@article{tagmouti_neurodevelopmental_2026,
	title = {Neurodevelopmental assessment at 24 months in infants with esophageal atresia: {A} prospective cohort study},
	volume = {82},
	copyright = {© 2026 The Author(s). Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.},
	issn = {1536-4801},
	shorttitle = {Neurodevelopmental assessment at 24 months in infants with esophageal atresia},
	url = {https://onlinelibrary.wiley.com/doi/abs/10.1002/jpn3.70406},
	doi = {10.1002/jpn3.70406},
	abstract = {Objective To evaluate neurodevelopment at 24 months in infants surgically treated for esophageal atresia (EA), using the ages and stages questionnaire (ASQ), and identify perinatal and early-life factors associated with developmental delay. Methods Infants with EA were prospectively enrolled in a structured multidisciplinary follow-up program. Total ASQ scores ≤185 were considered indicative of significant neurodevelopmental delay, while scores {\textgreater}220 reflected reassuring neurodevelopment. Five skill domains (communication, gross motor skills, fine motor skills, problem solving, and social skills) were also assessed. Clinical, surgical, and growth data were analyzed to identify associated risk factors. Results Of the 74 infants included, 13 patients (18\%) had an ASQ score ≤185. Lower ASQ scores were significantly associated with prematurity, prolonged hospitalization, multiple surgeries, poor weight gain at 1 and 2 years, and feeding disorders. After adjusting for gestational age and genetic anomalies, prolonged hospitalization remained associated with lower ASQ scores, and higher weight-for-age at 1 and 2 years with better ASQ scores. Communication and social skills were the most severely affected, with approximately 80\% of infants in the ASQ ≤ 185 group scoring below –2 standard deviations. Conclusion Although most children with EA demonstrated normal development at 2 years, a minority experienced significant delays, especially in communication and social skills. Early screening and targeted multidisciplinary follow-up are essential to support optimal outcomes in this high-risk population.},
	language = {en},
	number = {6},
	urldate = {2026-06-08},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Tagmouti, Julia and Midavaine, Manon and Sartorius, Victor and Vinit, Nicolas and Leroy-Terquem, Elise and Beaudoin, Sylvie and Kermorvant-Duchemin, Elsa and Rousseau, Véronique and Lapillonne, Alexandre},
	year = {2026},
	note = {\_eprint: https://onlinelibrary.wiley.com/doi/pdf/10.1002/jpn3.70406},
	keywords = {congenital gastrointestinal malformations, feeding disorders, neonatal surgery, neurodevelopment disorders, perinatal factors},
	pages = {1386--1393},
}

Objective To evaluate neurodevelopment at 24 months in infants surgically treated for esophageal atresia (EA), using the ages and stages questionnaire (ASQ), and identify perinatal and early-life factors associated with developmental delay. Methods Infants with EA were prospectively enrolled in a structured multidisciplinary follow-up program. Total ASQ scores ≤185 were considered indicative of significant neurodevelopmental delay, while scores \textgreater220 reflected reassuring neurodevelopment. Five skill domains (communication, gross motor skills, fine motor skills, problem solving, and social skills) were also assessed. Clinical, surgical, and growth data were analyzed to identify associated risk factors. Results Of the 74 infants included, 13 patients (18%) had an ASQ score ≤185. Lower ASQ scores were significantly associated with prematurity, prolonged hospitalization, multiple surgeries, poor weight gain at 1 and 2 years, and feeding disorders. After adjusting for gestational age and genetic anomalies, prolonged hospitalization remained associated with lower ASQ scores, and higher weight-for-age at 1 and 2 years with better ASQ scores. Communication and social skills were the most severely affected, with approximately 80% of infants in the ASQ ≤ 185 group scoring below –2 standard deviations. Conclusion Although most children with EA demonstrated normal development at 2 years, a minority experienced significant delays, especially in communication and social skills. Early screening and targeted multidisciplinary follow-up are essential to support optimal outcomes in this high-risk population.

@article{koivusalo_progression_2026,
	title = {Progression of esophageal atresia associated {Barrett}’s esophagus in adulthood – {Is} endoscopic surveillance worth it?},
	volume = {61},
	issn = {0022-3468},
	url = {https://www.sciencedirect.com/science/article/pii/S0022346826001429},
	doi = {10.1016/j.jpedsurg.2026.163059},
	abstract = {Objectives
Barrett's esophagus (BE) is a late sequela after esophageal atresia (EA) repair. In order to assess the need for an endoscopic BE surveillance program, we studied evolution of BE in 71 adults with C-type EA in two successive endoscopies.
Methods
Endoscopic Prague Classification of BE was used. Endoscopic BE was graded as True BE (with goblet cell metaplasia) or BE without goblet cell metaplasia.
Results
The median patient age was 36 (IQR 28–43) years at E1 (index endoscopy) and 50 (41–58) years at E2 (follow-up endoscopy) with median interval of 16 (12–17) years. Prevalence of endoscopic BE increased from 15 \% to 42 \% (p = 0.002) and of true BE from 1.4 \% to 15 \%, (p = 0.04). Dysplasia and cancer were not observed. De novo true BE (n = 10) developed from previous BE without goblet cell metaplasia (n = 4) or from normal epithelium (n = 6). In four (5.6 \%) patients true BE extended ≥ 3 cm. True BE was predicted by previous endoscopic BE (RR = 9.2; 95\%CI 2.0-4-1, p = 0.004) and esophagitis (RR = 5.8; 95\%CI 1.4–38, p = 0.02).
Conclusions
The prevalence of endoscopic and true BE increased by 3- and 10-fold, respectively, between median ages of 36 and 50 years. High-risk BE was rare and no dysplasia or cancer was found. Esophagitis and endoscopic BE predisposed to true BE. Endoscopic surveillance of patients with EA before the age 50 years seems unnecessary.},
	number = {7},
	urldate = {2026-03-27},
	journal = {Journal of Pediatric Surgery},
	author = {Koivusalo, Antti and Tenca, Andrea and Lohi, Jouko and Pakarinen, Mikko P.},
	month = jul,
	year = {2026},
	keywords = {Barrett’s esophagus, Endoscopy, Esophageal atresia, Gastroesophageal reflux},
	pages = {163059},
}

Objectives Barrett's esophagus (BE) is a late sequela after esophageal atresia (EA) repair. In order to assess the need for an endoscopic BE surveillance program, we studied evolution of BE in 71 adults with C-type EA in two successive endoscopies. Methods Endoscopic Prague Classification of BE was used. Endoscopic BE was graded as True BE (with goblet cell metaplasia) or BE without goblet cell metaplasia. Results The median patient age was 36 (IQR 28–43) years at E1 (index endoscopy) and 50 (41–58) years at E2 (follow-up endoscopy) with median interval of 16 (12–17) years. Prevalence of endoscopic BE increased from 15 % to 42 % (p = 0.002) and of true BE from 1.4 % to 15 %, (p = 0.04). Dysplasia and cancer were not observed. De novo true BE (n = 10) developed from previous BE without goblet cell metaplasia (n = 4) or from normal epithelium (n = 6). In four (5.6 %) patients true BE extended ≥ 3 cm. True BE was predicted by previous endoscopic BE (RR = 9.2; 95%CI 2.0-4-1, p = 0.004) and esophagitis (RR = 5.8; 95%CI 1.4–38, p = 0.02). Conclusions The prevalence of endoscopic and true BE increased by 3- and 10-fold, respectively, between median ages of 36 and 50 years. High-risk BE was rare and no dysplasia or cancer was found. Esophagitis and endoscopic BE predisposed to true BE. Endoscopic surveillance of patients with EA before the age 50 years seems unnecessary.

@article{durkin_functional_2026,
	title = {Functional integration of an autologous engineered esophagus in a large-animal model},
	copyright = {2026 The Author(s)},
	issn = {1546-1696},
	url = {https://www.nature.com/articles/s41587-026-03043-1},
	doi = {10.1038/s41587-026-03043-1},
	abstract = {Tissue engineering of the esophagus has been limited by stent dependance and poor muscle regeneration. Here we report an integrated strategy to engineer a 2.5-cm esophageal segment by microinjecting autologous pericyte-like myogenic precursors and fibroblasts in a decellularized porcine scaffold to repair circumferential defects in 10-kg minipigs (n = 8), modeling pediatric use. Bioreactor maturation induced a proangiogenic phenotype, with in vivo support from biodegradable intraluminal stents and a vascularizing pleural wrap. This coordinated approach yielded safe and effective esophageal conduits; oral feeding supported normal growth, morbidity resembled that of clinical esophageal replacement and was endoscopically manageable, and 63\% (5/8) survived to the 6-month endpoint. Comprehensive multimodal analyses demonstrated progressive recapitulation of native architecture, with increasing neuromuscular regeneration and vascularization, correlating with functional recovery, absence of symptomatic stricture and the presence of secondary peristalsis by 6 months. These results demonstrate that the combination of complementary regenerative, conditioning and surgical strategies enables a functionally integrated, contractile esophageal graft with ongoing structural maturation without immunosuppression.},
	language = {en},
	urldate = {2026-03-27},
	journal = {Nature Biotechnology},
	publisher = {Nature Publishing Group},
	author = {Durkin, Natalie and Hall, George T. and Lutman, Roberto and Scuglia, Marianna and Xenakis, Theodoros and Patera, Giulia and Di Biagio, Daniele and Yamada, Koji and Tullie, Lucinda and Scaglioni, Dominic and Shibuya, Soichi and Nikaki, Kornilia and Beesley, Max Arran and Saleh, Tarek and Garrido Flores, Matias and Borselle, Dominika and Karaluka, Valerija and Hutchinson, J. Ciaran and Khalaf, Sahira and Ogunbiyi, Olumide and Wu, Lei and Huang, Xia and Song, Wenhui and Loukogeorgakis, Stavros and Pellegata, Alessandro Filippo and Mantero, Sara and Cossu, Giulio and Li, Vivian S. W. and Borrelli, Osvaldo and Bonfanti, Paola and Castellano, Sergi and Gerli, Mattia Francesco Maria and McCann, Conor J. and Eaton, Simon and Pellegrini, Marco and De Coppi, Paolo},
	month = mar,
	year = {2026},
	keywords = {Regeneration, Tissue engineering},
	pages = {1--14},
}

Tissue engineering of the esophagus has been limited by stent dependance and poor muscle regeneration. Here we report an integrated strategy to engineer a 2.5-cm esophageal segment by microinjecting autologous pericyte-like myogenic precursors and fibroblasts in a decellularized porcine scaffold to repair circumferential defects in 10-kg minipigs (n = 8), modeling pediatric use. Bioreactor maturation induced a proangiogenic phenotype, with in vivo support from biodegradable intraluminal stents and a vascularizing pleural wrap. This coordinated approach yielded safe and effective esophageal conduits; oral feeding supported normal growth, morbidity resembled that of clinical esophageal replacement and was endoscopically manageable, and 63% (5/8) survived to the 6-month endpoint. Comprehensive multimodal analyses demonstrated progressive recapitulation of native architecture, with increasing neuromuscular regeneration and vascularization, correlating with functional recovery, absence of symptomatic stricture and the presence of secondary peristalsis by 6 months. These results demonstrate that the combination of complementary regenerative, conditioning and surgical strategies enables a functionally integrated, contractile esophageal graft with ongoing structural maturation without immunosuppression.

@article{galai_feeding_2026,
	title = {Feeding difficulties in children with esophageal atresia: {A} parent-reported multicenter study},
	issn = {1536-4801},
	shorttitle = {Feeding difficulties in children with esophageal atresia},
	doi = {10.1002/jpn3.70339},
	abstract = {OBJECTIVE: Feeding difficulties (FDs) are common among children with esophageal atresia (EA) and tracheoesophageal fistula (TEF), but knowledge about their prevalence and risk factors is limited. This multicenter study aimed to assess the prevalence, subtypes, and associated factors of FD in children with EA/TEF.
METHODS: Parents of children who underwent surgery for EA/TEF in four tertiary centers in Israel (2005-2022) completed a structured questionnaire. Pediatric feeding disorder (PFD) was diagnosed by means of the Montreal Children's Hospital Feeding Scale and classified by consensus criteria into four subtypes: feeding skills, nutritional, medical, and psychosocial dysfunctions.
RESULTS: Seventy-five children were included (median age: 40 months; 48 males), of whom 57 (76\%) were reported to have FD, primarily due to impaired feeding skills (42\%). Lower gestational age, low birth weight, and delayed oral feeding were significantly associated with PFD (37 vs. 39 weeks, p = 0.001, 2130 g versus 3084 g, p = 0.001 and 14 versus 10 days, p = 0.05, respectively). Only half of the children received timely and appropriate multidisciplinary follow-up care.
CONCLUSION: FDs are highly prevalent in children with EA/TEF, mostly due to impaired feeding skills. Several clinical and perinatal factors are associated with the development of these problems, calling for early and multidisciplinary intervention to improve outcomes.},
	language = {eng},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Galai, Tut and Cohen, Shlomi and Weiss, Batia and Shaoul, Ron and Silbermintz, Ari and Shemer, Kim and Gal, Dana L. and Moran-Lev, Hadar},
	month = jan,
	year = {2026},
	keywords = {feeding disorder, multidisciplinary intervention, pediatrics, tracheoesophageal fistula},
}

OBJECTIVE: Feeding difficulties (FDs) are common among children with esophageal atresia (EA) and tracheoesophageal fistula (TEF), but knowledge about their prevalence and risk factors is limited. This multicenter study aimed to assess the prevalence, subtypes, and associated factors of FD in children with EA/TEF. METHODS: Parents of children who underwent surgery for EA/TEF in four tertiary centers in Israel (2005-2022) completed a structured questionnaire. Pediatric feeding disorder (PFD) was diagnosed by means of the Montreal Children's Hospital Feeding Scale and classified by consensus criteria into four subtypes: feeding skills, nutritional, medical, and psychosocial dysfunctions. RESULTS: Seventy-five children were included (median age: 40 months; 48 males), of whom 57 (76%) were reported to have FD, primarily due to impaired feeding skills (42%). Lower gestational age, low birth weight, and delayed oral feeding were significantly associated with PFD (37 vs. 39 weeks, p = 0.001, 2130 g versus 3084 g, p = 0.001 and 14 versus 10 days, p = 0.05, respectively). Only half of the children received timely and appropriate multidisciplinary follow-up care. CONCLUSION: FDs are highly prevalent in children with EA/TEF, mostly due to impaired feeding skills. Several clinical and perinatal factors are associated with the development of these problems, calling for early and multidisciplinary intervention to improve outcomes.

@article{oby_stricture_2026,
	title = {Stricture classification of pediatric esophageal strictures ({SCOPES}): {A} novel tool to predict response to endoscopic therapy},
	issn = {1536-4801},
	shorttitle = {Stricture classification of pediatric esophageal strictures ({SCOPES})},
	url = {https://onlinelibrary.wiley.com/doi/10.1002/jpn3.70403},
	doi = {10.1002/jpn3.70403},
	abstract = {Objectives
Esophageal anastomotic stricture is a common complication following esophageal atresia (EA) repair. While multiple endoscopic therapies exist, predicting individual stricture response rem...},
	language = {en},
	urldate = {2026-03-24},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	publisher = {John Wiley \& Sons, Ltd},
	author = {Oby, Brandon T. and Staffa, Steven J. and Ngo, Peter D. and Chang, Denis and Manfredi, Michael A. and Yasuda, Jessica L.},
	month = mar,
	year = {2026},
}

Objectives Esophageal anastomotic stricture is a common complication following esophageal atresia (EA) repair. While multiple endoscopic therapies exist, predicting individual stricture response rem...

@article{domingues_brazilian_2025,
	title = {{BRAZILIAN} {GASTROENTEROLOGY} {FEDERATION} ({FBG}) {CLINICAL} {GUIDELINE}: {DIAGNOSIS} {AND} {TREATMENT} {OF} {EOSINOPHILIC} {ESOPHAGITIS} {IN} {ADULTS} {AND} {ADOLESCENTS}},
	volume = {62},
	issn = {1678-4219},
	shorttitle = {{BRAZILIAN} {GASTROENTEROLOGY} {FEDERATION} ({FBG}) {CLINICAL} {GUIDELINE}},
	doi = {10.1590/S0004-2803.24612025-079},
	abstract = {BACKGROUND: Eosinophilic esophagitis (EoE) is a chronic immune-mediated inflammatory disease of the esophagus that affects both children and adults.
OBJECTIVE: The purpose of this article is to establish guidance and updates for the diagnosis and treatment of EoE.
METHODS: The Brazilian Federation of Gastroenterology, FBG, gathered a group of gastroenterology experts in the field of esophagus and conducted a Delphi process to develop updated guideline for the management of patients with EoE in the light of recent evidence. Grading of the strength and quality of evidence of the recommendations was performed using GRADE criteria. The database searched included MEDLINE, EMBASE, SciELO, LILACS, ClinicalTrials.gov, and CINAHL, using their respective search mechanisms with studies retrieved up to January 2025. The resulting document is presented in the form of 15 questions and answers on diagnosis and treatment, with practical approaches and particularities of EoE, based on the current best evidence-based medicine, indicating the quality of data and specialists' opinion on the subject.
RESULTS: From the standpoint of diagnosis, the present clinical guideline focused on the most prevalent aspects of EoE as well aiming to promote the benefits of the early diagnosis in clinical practice. The treatment is herein focused on the major advances in pharmacologic approach and therapeutic options available, as well as in the consistent corpus of evidence on dietary therapy.
CONCLUSION: This FBG guideline is established to support clinical practice and suggest preferable approaches to a typical patient with EoE based on the currently available published literature. In this context, physicians must take into account patient´s medical comorbidities, adherence to treatment and preferences.},
	language = {eng},
	journal = {Arquivos De Gastroenterologia},
	author = {Domingues, Gerson and Moraes-Filho, Joaquim Prado Pinto de and Chinzon, Décio and Delgado, Áureo and {FBG advisors}},
	year = {2025},
	pmid = {41172433},
	pmcid = {PMC12560844},
	keywords = {Adolescent, Adult, Brazil, Delphi Technique, Eosinophilic Esophagitis, Gastroenterology, Humans},
	pages = {e25079},
}

BACKGROUND: Eosinophilic esophagitis (EoE) is a chronic immune-mediated inflammatory disease of the esophagus that affects both children and adults. OBJECTIVE: The purpose of this article is to establish guidance and updates for the diagnosis and treatment of EoE. METHODS: The Brazilian Federation of Gastroenterology, FBG, gathered a group of gastroenterology experts in the field of esophagus and conducted a Delphi process to develop updated guideline for the management of patients with EoE in the light of recent evidence. Grading of the strength and quality of evidence of the recommendations was performed using GRADE criteria. The database searched included MEDLINE, EMBASE, SciELO, LILACS, ClinicalTrials.gov, and CINAHL, using their respective search mechanisms with studies retrieved up to January 2025. The resulting document is presented in the form of 15 questions and answers on diagnosis and treatment, with practical approaches and particularities of EoE, based on the current best evidence-based medicine, indicating the quality of data and specialists' opinion on the subject. RESULTS: From the standpoint of diagnosis, the present clinical guideline focused on the most prevalent aspects of EoE as well aiming to promote the benefits of the early diagnosis in clinical practice. The treatment is herein focused on the major advances in pharmacologic approach and therapeutic options available, as well as in the consistent corpus of evidence on dietary therapy. CONCLUSION: This FBG guideline is established to support clinical practice and suggest preferable approaches to a typical patient with EoE based on the currently available published literature. In this context, physicians must take into account patient´s medical comorbidities, adherence to treatment and preferences.

@article{dellon_acg_2025,
	title = {{ACG} {Clinical} {Guideline}: {Diagnosis} and {Management} of {Eosinophilic} {Esophagitis}},
	volume = {120},
	issn = {1572-0241},
	shorttitle = {{ACG} {Clinical} {Guideline}},
	doi = {10.14309/ajg.0000000000003194},
	abstract = {Eosinophilic esophagitis (EoE) is a chronic immune-mediated disease of the esophagus. It is diagnosed in the setting of symptoms of esophageal dysfunction and an eosinophilic predominant infiltrate in the esophagus. The condition is rapidly increasing in incidence and prevalence and is commonly encountered in gastroenterology and allergy practices, emergency departments, and primary care settings. Over the past decade, there have been paradigm shifts in disease diagnosis and management, increases in knowledge about EoE risk factors, natural history, and pathogenesis, and development of validated outcome metrics. This updated American College of Gastroenterology Clinical Guideline uses Grading of Recommendations, Assessment, Development, and Evaluation methodology to make recommendations across domains of diagnosis, treatment, monitoring and assessment of response, and pediatric-specific considerations. Proton pump inhibitors, topical steroids, empiric diet elimination, a biologic, and esophageal dilation are all recommended treatments; feeding therapy is used adjunctively in children with food aversion or feeding dysfunction. Monitoring with clinical, endoscopic, and histologic assessments is recommended to assess for treatment response and follow patients over time with maintenance therapy. When evaluating and following patients with EoE, consideration should be given to assessing and controlling both the inflammatory and fibrostenotic aspects of disease.},
	language = {eng},
	number = {1},
	journal = {The American Journal of Gastroenterology},
	author = {Dellon, Evan S. and Muir, Amanda B. and Katzka, David A. and Shah, Shailja C. and Sauer, Bryan G. and Aceves, Seema S. and Furuta, Glenn T. and Gonsalves, Nirmala and Hirano, Ikuo},
	month = jan,
	year = {2025},
	pmid = {39745304},
	keywords = {Child, Dilatation, Eosinophilic Esophagitis, Esophagoscopy, Humans, Proton Pump Inhibitors},
	pages = {31--59},
}

Eosinophilic esophagitis (EoE) is a chronic immune-mediated disease of the esophagus. It is diagnosed in the setting of symptoms of esophageal dysfunction and an eosinophilic predominant infiltrate in the esophagus. The condition is rapidly increasing in incidence and prevalence and is commonly encountered in gastroenterology and allergy practices, emergency departments, and primary care settings. Over the past decade, there have been paradigm shifts in disease diagnosis and management, increases in knowledge about EoE risk factors, natural history, and pathogenesis, and development of validated outcome metrics. This updated American College of Gastroenterology Clinical Guideline uses Grading of Recommendations, Assessment, Development, and Evaluation methodology to make recommendations across domains of diagnosis, treatment, monitoring and assessment of response, and pediatric-specific considerations. Proton pump inhibitors, topical steroids, empiric diet elimination, a biologic, and esophageal dilation are all recommended treatments; feeding therapy is used adjunctively in children with food aversion or feeding dysfunction. Monitoring with clinical, endoscopic, and histologic assessments is recommended to assess for treatment response and follow patients over time with maintenance therapy. When evaluating and following patients with EoE, consideration should be given to assessing and controlling both the inflammatory and fibrostenotic aspects of disease.

@article{prasad_fdas_2025,
	title = {{FDA}'s {New} {Plausible} {Mechanism} {Pathway}},
	issn = {1533-4406},
	doi = {10.1056/NEJMsb2512695},
	language = {eng},
	journal = {The New England Journal of Medicine},
	author = {Prasad, Vinay and Makary, Martin A.},
	month = nov,
	year = {2025},
}

@article{dellenmark-blom_experiences_2025,
	title = {Experiences of symptom burden among young children born with esophageal atresia–tracheoesophageal fistula: a {US} focus group study},
	volume = {20},
	issn = {1750-1172},
	shorttitle = {Experiences of symptom burden among young children born with esophageal atresia–tracheoesophageal fistula},
	url = {https://doi.org/10.1186/s13023-025-03939-2},
	doi = {10.1186/s13023-025-03939-2},
	abstract = {Children born with esophageal atresia–tracheoesophageal fistula (EA–TEF) can suffer from aerodigestive morbidity that impairs their quality of life and can persist into adulthood. Ameliorating their symptom burden requires a thorough understanding of the symptom experiences that children have early in life. We aimed to explore parents’ experiences of their children’s aerodigestive symptom burden during the first years of life after being born with EA–TEF. This exploration also aimed to help determine whether a disease-specific measurement of symptom burden is needed.},
	language = {en},
	number = {1},
	urldate = {2025-09-02},
	journal = {Orphanet Journal of Rare Diseases},
	author = {Dellenmark-Blom, Michaela and Bennett, John and Micalizzi, Rosella and Cole, Lianne and Woods, Kaylee and Cardoni, Lauren and Frain, Leah and Mohamed, Abdimajid and Yasuda, Jessica and Ngo, Peter and Widenmann, Anke and Slater, Graham and Zendejas, Benjamin},
	month = aug,
	year = {2025},
	keywords = {Esophageal atresia, Patient-reported outcome, Rare disease, Symptom burden, Tracheobronchomalacia, Tracheomalacia},
	pages = {438},
}

Children born with esophageal atresia–tracheoesophageal fistula (EA–TEF) can suffer from aerodigestive morbidity that impairs their quality of life and can persist into adulthood. Ameliorating their symptom burden requires a thorough understanding of the symptom experiences that children have early in life. We aimed to explore parents’ experiences of their children’s aerodigestive symptom burden during the first years of life after being born with EA–TEF. This exploration also aimed to help determine whether a disease-specific measurement of symptom burden is needed.

@misc{gonzalez_veille_2025,
	title = {Veille bibliographique {CRACMO}},
	url = {https://www.fimatho.fr/cracmo/recherche/veille-bibliographique-cracmo},
	abstract = {La Filière des Maladies Rares Abdomino-Thoraciques. Amélioration de la prise en charge, Formation/Information, Développement de la recherche.},
	language = {fr-fr},
	urldate = {2025-07-28},
	author = {Gonzalez, Marine},
	month = jan,
	year = {2025},
}

La Filière des Maladies Rares Abdomino-Thoraciques. Amélioration de la prise en charge, Formation/Information, Développement de la recherche.

@article{leroy_long-term_2025,
	title = {Long-term outcome of oesophageal atresia in adolescence ({TransEAsome}): a national {French} cohort study protocol},
	volume = {15},
	issn = {2044-6055},
	shorttitle = {Long-term outcome of oesophageal atresia in adolescence ({TransEAsome})},
	url = {http://hdl.handle.net/20.500.12210/119304},
	doi = {10.1136/bmjopen-2024-086303},
	abstract = {INTRODUCTION: The TransEAsome project, funded by the Agence Nationale de la Recherche, aims to evaluate the long-term outcomes of patients with oesophageal atresia (OA) between 13 and 14 years old and establish multiomics profiles using data from the world's biggest OA registry.
METHODS AND ANALYSIS: TransEAsome is a national multicentre population-based cohort study recruiting participants from all qualified French centres for OA surgery at birth. The primary objective is to assess the prevalence of gastro-oesophageal reflux disease in adolescence among patients with OA, with several secondary objectives including the identification of risk factors and multiomic profiles from oesophageal biopsies and blood samples collected between 13 and 14 years old, compared with a control group. This comprehensive characterisation of phenotype and omic profiles aims to enhance the understanding of disease evolution in patients with OA and inform tailored care management strategies.
ETHICS AND DISSEMINATION: The study, coconstructed with input from patients, parents and research-expert adolescents, has obtained approval from the ethics research committee: Comité de protection des personnes Est II. Findings will be disseminated to various target audiences, including the scientific community, research participants, the patient community, the general public, regulatory authorities and policymakers. Data will be made available in a Findable, Accessible, Interoperable, Reusable format on the France Cohortes platform on study completion.
TRIAL REGISTRATION NUMBER: NCT05995171:Clinical trial.},
	language = {eng},
	number = {1},
	journal = {BMJ open},
	author = {Leroy, Mélanie and Aumar, Madeleine and Duhamel, Marie and Dauchet, Luc and Figeac, Martin and Gaillard, Segolene and Hankard, Regis and Labreuche, Julien and Marot, Guillemette and Reversat, Julie and Armand, Viviane and Salzet, Michel and Sfeir, Rony and Vandel, Jimmy and Gottrand, Frédéric},
	month = jan,
	year = {2025},
	keywords = {Adolescent, Adolescents, Cohort Studies, Esophageal Atresia, Female, France, Gastroesophageal Reflux, Humans, Male, Oesophageal disease, Paediatric gastroenterology, Patient Reported Outcome Measures, Prevalence, Quality of Life, Registries, Research Design, Risk Factors},
	pages = {e086303},
}

INTRODUCTION: The TransEAsome project, funded by the Agence Nationale de la Recherche, aims to evaluate the long-term outcomes of patients with oesophageal atresia (OA) between 13 and 14 years old and establish multiomics profiles using data from the world's biggest OA registry. METHODS AND ANALYSIS: TransEAsome is a national multicentre population-based cohort study recruiting participants from all qualified French centres for OA surgery at birth. The primary objective is to assess the prevalence of gastro-oesophageal reflux disease in adolescence among patients with OA, with several secondary objectives including the identification of risk factors and multiomic profiles from oesophageal biopsies and blood samples collected between 13 and 14 years old, compared with a control group. This comprehensive characterisation of phenotype and omic profiles aims to enhance the understanding of disease evolution in patients with OA and inform tailored care management strategies. ETHICS AND DISSEMINATION: The study, coconstructed with input from patients, parents and research-expert adolescents, has obtained approval from the ethics research committee: Comité de protection des personnes Est II. Findings will be disseminated to various target audiences, including the scientific community, research participants, the patient community, the general public, regulatory authorities and policymakers. Data will be made available in a Findable, Accessible, Interoperable, Reusable format on the France Cohortes platform on study completion. TRIAL REGISTRATION NUMBER: NCT05995171:Clinical trial.

@article{noauthor_pfmg2025integrating_2025,
	title = {{PFMG2025}–integrating genomic medicine into the national healthcare system in {France}},
	volume = {50},
	issn = {2666-7762},
	url = {https://www.thelancet.com/journals/lanepe/article/PIIS2666-7762(24)00352-1/fulltext},
	doi = {10.1016/j.lanepe.2024.101183},
	language = {English},
	urldate = {2025-03-20},
	journal = {The Lancet Regional Health – Europe},
	publisher = {Elsevier},
	author = {, PFMG2025 Contributors},
	month = mar,
	year = {2025},
	keywords = {Cancer predisposition, Cancers, French genomic medicine initiative, Genome sequencing, Genomic medicine, PFMG2025, Rare diseases},
}

@article{de_bortoli_1st_2024,
	title = {The 1st {EoETALY} {Consensus} on the {Diagnosis} and {Management} of {Eosinophilic} {Esophagitis}-{Current} {Treatment} and {Monitoring}},
	volume = {56},
	issn = {1878-3562},
	doi = {10.1016/j.dld.2024.02.020},
	abstract = {The present document constitutes Part 2 of the EoETALY Consensus Statements guideline on the diagnosis and management of eosinophilic esophagitis (EoE) developed by experts in the field of EoE across Italy (i.e., EoETALY Consensus Group). Part 1 was published as a different document, and included three chapters discussing 1) definition, epidemiology, and pathogenesis; 2) clinical presentation and natural history and 3) diagnosis of EoE. The present work provides guidelines on the management of EoE in two final chapters: 4) treatment and 5) monitoring and follow-up, and also includes considerations on knowledge gaps and a proposed research agenda for the coming years. The guideline was developed through a Delphi process, with grading of the strength and quality of the evidence of the recommendations performed according to accepted GRADE criteria.This document has received the endorsement of three Italian national societies including the Italian Society of Gastroenterology (SIGE), the Italian Society of Neurogastroenterology and Motility (SINGEM), and the Italian Society of Allergology, Asthma, and Clinical Immunology (SIAAIC). The guidelines also involved the contribution of members of ESEO Italia, the Italian Association of Families Against EoE.},
	language = {eng},
	number = {7},
	journal = {Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver},
	author = {de Bortoli, Nicola and Visaggi, Pierfrancesco and Penagini, Roberto and Annibale, Bruno and Baiano Svizzero, Federica and Barbara, Giovanni and Bartolo, Ottavia and Battaglia, Edda and Di Sabatino, Antonio and De Angelis, Paola and Docimo, Ludovico and Frazzoni, Marzio and Furnari, Manuele and Iori, Andrea and Iovino, Paola and Lenti, Marco Vincenzo and Marabotto, Elisa and Marasco, Giovanni and Mauro, Aurelio and Oliva, Salvatore and Pellegatta, Gaia and Pesce, Marcella and Privitera, Antonino Carlo and Puxeddu, Ilaria and Racca, Francesca and Ribolsi, Mentore and Ridolo, Erminia and Russo, Salvatore and Sarnelli, Giovanni and Tolone, Salvatore and Zentilin, Patrizia and Zingone, Fabiana and Barberio, Brigida and Ghisa, Matteo and Savarino, Edoardo Vincenzo},
	month = jul,
	year = {2024},
	pmid = {38521670},
	keywords = {Delphi Technique, EoE, EoETALY, Eosinophilic Esophagitis, Eosinophilic esophagitis, Guidelines, Humans, Italy, Proton Pump Inhibitors},
	pages = {1173--1184},
}

The present document constitutes Part 2 of the EoETALY Consensus Statements guideline on the diagnosis and management of eosinophilic esophagitis (EoE) developed by experts in the field of EoE across Italy (i.e., EoETALY Consensus Group). Part 1 was published as a different document, and included three chapters discussing 1) definition, epidemiology, and pathogenesis; 2) clinical presentation and natural history and 3) diagnosis of EoE. The present work provides guidelines on the management of EoE in two final chapters: 4) treatment and 5) monitoring and follow-up, and also includes considerations on knowledge gaps and a proposed research agenda for the coming years. The guideline was developed through a Delphi process, with grading of the strength and quality of the evidence of the recommendations performed according to accepted GRADE criteria.This document has received the endorsement of three Italian national societies including the Italian Society of Gastroenterology (SIGE), the Italian Society of Neurogastroenterology and Motility (SINGEM), and the Italian Society of Allergology, Asthma, and Clinical Immunology (SIAAIC). The guidelines also involved the contribution of members of ESEO Italia, the Italian Association of Families Against EoE.

@article{leroy_time_2024,
	title = {Time to consider oesophageal atresia as a life-long disease},
	volume = {110},
	issn = {1743-9159},
	url = {http://hdl.handle.net/20.500.12210/109819},
	doi = {10.1097/JS9.0000000000001167},
	language = {eng},
	number = {5},
	journal = {International Journal of Surgery (London, England)},
	author = {Leroy, Mélanie and Aumar, Madeleine and Seguy, David and Vandamme, Florent and Widenmann, Anke and Sfeir, Rony and Gottrand, Frédéric},
	month = may,
	year = {2024},
	keywords = {Esophageal Atresia, Humans, Infant, Newborn},
	pages = {2506--2507},
}

@article{corsello_over--scope_2024,
	title = {Over-the-scope clip for closure of persistent gastrocutaneous fistula after gastrostomy tube removal: a multicenter pediatric experience},
	volume = {38},
	issn = {1432-2218},
	shorttitle = {Over-the-scope clip for closure of persistent gastrocutaneous fistula after gastrostomy tube removal},
	doi = {10.1007/s00464-024-11166-2},
	abstract = {BACKGROUND: Percutaneous endoscopic gastrostomy is commonly used for enteral nutritional access, but gastrocutaneous fistulae (GCF) may persist after tube removal, posing clinical challenges. The use of endoscopic closure devices, including over-the-scope clips (OTSC), has shown promise in managing non-healing fistulae, although data in the pediatric population are limited.
METHODS: A retrospective multicenter study analyzed pediatric patients who underwent GCF closure following gastrostomy tube removal. Data from seven centers across multiple countries were collected, including patient demographics, procedural details, complications, and outcomes. Closure techniques were compared between OTSC and surgical closure.
RESULTS: Of 67 pediatric patients included, 21 underwent OTSC closure and 46 had surgical closure. Surgical closure demonstrated a higher success rate (100\%) compared to OTSC closure (61.9\%, P {\textless} 0.001). While procedural duration was shorter for OTSC closure (25 vs. 40 min, P = 0.002), complications, and scar quality were comparable between techniques. A subsequent sub-analysis did not reveal differences based on center experience.
CONCLUSION: OTSC closure is feasible and safe in pediatric patients, but surgical closure remains superior in achieving sustained GCF closure, although OTSC offers benefits, such as shorter procedural duration, potentially reducing the duration of general anesthesia exposure. Non-operative approaches, including OTSC, may be a valuable alternative to surgical closure.},
	language = {eng},
	number = {11},
	journal = {Surgical Endoscopy},
	author = {Corsello, Antonio and Antoine, Matthieu and Sharma, Shishu and Bertrand, Valérie and Oliva, Salvatore and Fava, Giorgio and Destro, Francesca and Huang, Andrew and Fong, Wei S. W. and Ichino, Martina and Thomson, Mike and Gottrand, Frederic},
	month = nov,
	year = {2024},
	keywords = {Adolescent, Child, Child, Preschool, Closure techniques, Cutaneous Fistula, Device Removal, Enteral Nutrition, Female, Gastric Fistula, Gastrocutaneous fistula, Gastrostomy, Humans, Infant, Male, OTSC, Ovesco, PEG removal, Pediatric surgery, Postoperative Complications, Retrospective Studies, Surgical Instruments, Treatment Outcome},
	pages = {6305--6311},
}

BACKGROUND: Percutaneous endoscopic gastrostomy is commonly used for enteral nutritional access, but gastrocutaneous fistulae (GCF) may persist after tube removal, posing clinical challenges. The use of endoscopic closure devices, including over-the-scope clips (OTSC), has shown promise in managing non-healing fistulae, although data in the pediatric population are limited. METHODS: A retrospective multicenter study analyzed pediatric patients who underwent GCF closure following gastrostomy tube removal. Data from seven centers across multiple countries were collected, including patient demographics, procedural details, complications, and outcomes. Closure techniques were compared between OTSC and surgical closure. RESULTS: Of 67 pediatric patients included, 21 underwent OTSC closure and 46 had surgical closure. Surgical closure demonstrated a higher success rate (100%) compared to OTSC closure (61.9%, P \textless 0.001). While procedural duration was shorter for OTSC closure (25 vs. 40 min, P = 0.002), complications, and scar quality were comparable between techniques. A subsequent sub-analysis did not reveal differences based on center experience. CONCLUSION: OTSC closure is feasible and safe in pediatric patients, but surgical closure remains superior in achieving sustained GCF closure, although OTSC offers benefits, such as shorter procedural duration, potentially reducing the duration of general anesthesia exposure. Non-operative approaches, including OTSC, may be a valuable alternative to surgical closure.

@article{dimitrov_proton_2024,
	title = {Proton pump inhibitors in esophageal atresia: {A} systematic review and meta-analysis},
	volume = {78},
	issn = {1536-4801},
	shorttitle = {Proton pump inhibitors in esophageal atresia},
	url = {http://hdl.handle.net/20.500.12210/105660},
	doi = {10.1002/jpn3.12115},
	abstract = {Gastroesophageal reflux disease (GERD) is frequent and prolonged in esophageal atresia (EA) pediatric patients requiring routine use of proton pump inhibitors (PPIs). However, there are still controversies on the prophylactic use of PPIs and the efficacy of PPIs on GERD and EA complications in this special condition. The aim of the study is to assess the prophylactic use of PPIs in pediatric patients with EA and its complications. We, therefore, performed a systematic review including all reports on the subject from 1980 to 2022. We conducted meta-analysis of the pooled proportion of PPI-and no PPI groups using random effect model, meta-regression, and estimate heterogeneity by heterogeneity index I2 . Thirty-eight reports on the topic met the criteria selection, representing a cumulative 6044 patients with EA. Prophylactic PPI prescription during the first year of life does not appear to prevent GERD persistence at follow-up and is not associated with a significantly reduced rate of antireflux surgical procedures (ARP). PPIs improve peptic esophagitis and induce remission of eosinophilic esophagitis at a rate of 50\%. Their effect on other GERD outcomes is uncertain. Evidence suggests that PPIs do not prevent anastomotic stricture, Barrett's esophagus, or respiratory complications. PPI use in EA can improve peptic and eosinophilic esophagitis but is ineffective on the other EA complications. Side effects of PPIs in EA are almost unknown.},
	language = {eng},
	number = {3},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Dimitrov, Georges and Aumar, Madeleine and Duhamel, Alain and Wanneveich, Mathilde and Gottrand, Frédéric},
	month = mar,
	year = {2024},
	keywords = {Barrett's esophagus, Child, Esophageal Atresia, Gastroesophageal Reflux, Humans, Proton Pump Inhibitors, anastomotic stricture, child, eosinophilic esophagitis, gastroesophageal reflux disease},
	pages = {457--470},
}

Gastroesophageal reflux disease (GERD) is frequent and prolonged in esophageal atresia (EA) pediatric patients requiring routine use of proton pump inhibitors (PPIs). However, there are still controversies on the prophylactic use of PPIs and the efficacy of PPIs on GERD and EA complications in this special condition. The aim of the study is to assess the prophylactic use of PPIs in pediatric patients with EA and its complications. We, therefore, performed a systematic review including all reports on the subject from 1980 to 2022. We conducted meta-analysis of the pooled proportion of PPI-and no PPI groups using random effect model, meta-regression, and estimate heterogeneity by heterogeneity index I2 . Thirty-eight reports on the topic met the criteria selection, representing a cumulative 6044 patients with EA. Prophylactic PPI prescription during the first year of life does not appear to prevent GERD persistence at follow-up and is not associated with a significantly reduced rate of antireflux surgical procedures (ARP). PPIs improve peptic esophagitis and induce remission of eosinophilic esophagitis at a rate of 50%. Their effect on other GERD outcomes is uncertain. Evidence suggests that PPIs do not prevent anastomotic stricture, Barrett's esophagus, or respiratory complications. PPI use in EA can improve peptic and eosinophilic esophagitis but is ineffective on the other EA complications. Side effects of PPIs in EA are almost unknown.

@article{krishnan_international_2023,
	title = {The {International} {Network} on {Oesophageal} {Atresia} ({INoEA}) consensus guidelines on the transition of patients with oesophageal atresia-tracheoesophageal fistula},
	volume = {20},
	issn = {1759-5053},
	url = {https://pubmed.ncbi.nlm.nih.gov/37286639/},
	doi = {10.1038/s41575-023-00789-w},
	abstract = {Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon.},
	language = {eng},
	number = {11},
	journal = {Nature Reviews. Gastroenterology \& Hepatology},
	author = {Krishnan, Usha and Dumont, Michael W. and Slater, Hayley and Gold, Benjamin D. and Seguy, David and Bouin, Mikael and Wijnen, Rene and Dall'Oglio, Luigi and Costantini, Mario and Koumbourlis, Anastassios C. and Kovesi, Thomas A. and Rutter, Michael J. and Soma, Marlene and Menzies, Jessica and Van Malleghem, Audrey and Rommel, Nathalie and Dellenmark-Blom, Michaela and Wallace, Vuokko and Culnane, Evelyn and Slater, Graham and Gottrand, Frederic and Faure, Christophe},
	month = nov,
	year = {2023},
	keywords = {Esophageal Atresia, Gastrointestinal Diseases, Humans, Quality of Life, Tracheoesophageal Fistula},
	pages = {735--755},
}

Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon.

@article{wyllie_prophylactic_2023,
	title = {Prophylactic {Acid}-suppression {Medication} to {Prevent} {Anastomotic} {Strictures} {After} {Oesophageal} {Atresia} {Surgery}: {A} {Systematic} {Review} and {Meta}-analysis},
	volume = {58},
	issn = {1531-5037},
	shorttitle = {Prophylactic {Acid}-suppression {Medication} to {Prevent} {Anastomotic} {Strictures} {After} {Oesophageal} {Atresia} {Surgery}},
	doi = {10.1016/j.jpedsurg.2023.05.024},
	abstract = {BACKGROUND: Anastomotic stricture is a common postoperative complication of oesophageal atresia ± tracheoesophageal fistula (OA/TOF) repair. Acid gastro-oesophageal reflux disease (GORD) is considered to be a factor in stricture formation and acid suppression medication is recommended post-operatively in consensus guidance. We aimed to investigate whether patients who were treated prophylactically with acid suppression medication had a reduced incidence of strictures compared to those who did not receive it.
METHODS: A systematic review of studies was performed, searching multiple databases without language or date restrictions. Multiple reviewers independently assessed study eligibility and literature quality. The primary outcome was anastomotic stricture formation, with secondary outcomes of GORD, anastomotic leak, and oesophagitis. Meta-analysis was performed using a random effects model, and the results were expressed as an odds ratio (OR) with 95\% confidence intervals (CI).
RESULTS: No randomised studies on the topic were identified. Twelve observational studies were included in the analysis with ten reporting the primary outcome. The quality assessment showed a high risk of bias in several papers, predominantly due to non-objective methods of assessment of oesophageal stricture and the non-prospective, non-randomised nature of the studies. Overall, 1395 patients were evaluated, of which 753 received acid suppression medication. Meta-analysis revealed a trend towards increased odds of anastomotic strictures in infants receiving prophylactic medication, but this was not statistically significant (OR 1.33; 95\% CI 0.92, 1.92). No significant differences were found in secondary outcomes.
CONCLUSIONS: This meta-analysis found no evidence of a statistically significant link between the prophylactic prescribing of acid suppression medication and the risk of developing anastomotic stricture after OA repair. The literature in this area is limited to observational studies and a randomised controlled trial is recommended to explore this question.
LEVEL OF EVIDENCE: Level III.},
	language = {eng},
	number = {10},
	journal = {Journal of Pediatric Surgery},
	author = {Wyllie, Thomas and Folaranmi, Eniola and Sekaran, Prabhu and Watkins, W. John and Chakraborty, Mallinath},
	month = oct,
	year = {2023},
	keywords = {Acid suppression, Anastomosis, Anastomosis, Surgical, Constriction, Pathologic, Esophageal Atresia, Esophageal Stenosis, Esophageal atresia, Gastroesophageal Reflux, H2-blockers, Humans, Infant, Observational Studies as Topic, PPI, Postoperative Complications, Randomized Controlled Trials as Topic, Stricture, Tracheo-esophageal fistula, Tracheoesophageal Fistula},
	pages = {1954--1962},
}

BACKGROUND: Anastomotic stricture is a common postoperative complication of oesophageal atresia ± tracheoesophageal fistula (OA/TOF) repair. Acid gastro-oesophageal reflux disease (GORD) is considered to be a factor in stricture formation and acid suppression medication is recommended post-operatively in consensus guidance. We aimed to investigate whether patients who were treated prophylactically with acid suppression medication had a reduced incidence of strictures compared to those who did not receive it. METHODS: A systematic review of studies was performed, searching multiple databases without language or date restrictions. Multiple reviewers independently assessed study eligibility and literature quality. The primary outcome was anastomotic stricture formation, with secondary outcomes of GORD, anastomotic leak, and oesophagitis. Meta-analysis was performed using a random effects model, and the results were expressed as an odds ratio (OR) with 95% confidence intervals (CI). RESULTS: No randomised studies on the topic were identified. Twelve observational studies were included in the analysis with ten reporting the primary outcome. The quality assessment showed a high risk of bias in several papers, predominantly due to non-objective methods of assessment of oesophageal stricture and the non-prospective, non-randomised nature of the studies. Overall, 1395 patients were evaluated, of which 753 received acid suppression medication. Meta-analysis revealed a trend towards increased odds of anastomotic strictures in infants receiving prophylactic medication, but this was not statistically significant (OR 1.33; 95% CI 0.92, 1.92). No significant differences were found in secondary outcomes. CONCLUSIONS: This meta-analysis found no evidence of a statistically significant link between the prophylactic prescribing of acid suppression medication and the risk of developing anastomotic stricture after OA repair. The literature in this area is limited to observational studies and a randomised controlled trial is recommended to explore this question. LEVEL OF EVIDENCE: Level III.

@article{dreyfus_respiratory_2023,
	title = {Respiratory physiology during {NAVA} ventilation in neonates born with a congenital diaphragmatic hernia: {The} "{NAVA}-diaph" pilot study},
	volume = {58},
	issn = {1099-0496},
	shorttitle = {Respiratory physiology during {NAVA} ventilation in neonates born with a congenital diaphragmatic hernia},
	doi = {10.1002/ppul.26357},
	abstract = {BACKGROUND: Neurally adjusted ventilatory assist (NAVA) is a ventilatory mode that delivers synchronized ventilation, proportional to the electrical activity of the diaphragm (EAdi). Although it has been proposed in infants with a congenital diaphragmatic hernia (CDH), the diaphragmatic defect and the surgical repair could alter the physiology of the diaphragm.
AIM: To evaluate, in a pilot study, the relationship between the respiratory drive (EAdi) and the respiratory effort in neonates with CDH during the postsurgical period under either NAVA ventilation or conventional ventilation (CV).
METHODS: This prospective physiological study included eight neonates admitted to a neonatal intensive care unit with a diagnosis of CDH. EAdi, esophageal, gastric, and transdiaphragmatic pressure, as well as clinical parameters, were recorded during NAVA and CV (synchronized intermittent mandatory pressure ventilation) in the postsurgical period.
RESULTS: EAdi was detectable and there was a correlation between the ΔEAdi (maximal - minimal values) and the transdiaphragmatic pressure (r = 0.26, 95\% confidence interval [CI] [0.222; 0.299]). There was no significant difference in terms of clinical or physiological parameters during NAVA compared to CV, including work of breathing.
CONCLUSION: Respiratory drive and effort were correlated in infants with CDH and therefore NAVA is a suitable proportional mode in this population. EAdi can also be used to monitor the diaphragm for individualized support.},
	language = {eng},
	number = {5},
	journal = {Pediatric Pulmonology},
	author = {Dreyfus, Lélia and Butin, Marine and Plaisant, Frank and Claris, Olivier and Baudin, Florent},
	month = may,
	year = {2023},
	keywords = {Diaphragm, Hernias, Diaphragmatic, Congenital, Humans, Infant, Infant, Newborn, Interactive Ventilatory Support, NAVA ventilation, Pilot Projects, Prospective Studies, Respiration, Artificial, Respiratory Rate, congenital diaphragmatic hernia, respiratory physiology, transpulmonary pressure, work of breathing},
	pages = {1542--1550},
}

BACKGROUND: Neurally adjusted ventilatory assist (NAVA) is a ventilatory mode that delivers synchronized ventilation, proportional to the electrical activity of the diaphragm (EAdi). Although it has been proposed in infants with a congenital diaphragmatic hernia (CDH), the diaphragmatic defect and the surgical repair could alter the physiology of the diaphragm. AIM: To evaluate, in a pilot study, the relationship between the respiratory drive (EAdi) and the respiratory effort in neonates with CDH during the postsurgical period under either NAVA ventilation or conventional ventilation (CV). METHODS: This prospective physiological study included eight neonates admitted to a neonatal intensive care unit with a diagnosis of CDH. EAdi, esophageal, gastric, and transdiaphragmatic pressure, as well as clinical parameters, were recorded during NAVA and CV (synchronized intermittent mandatory pressure ventilation) in the postsurgical period. RESULTS: EAdi was detectable and there was a correlation between the ΔEAdi (maximal - minimal values) and the transdiaphragmatic pressure (r = 0.26, 95% confidence interval [CI] [0.222; 0.299]). There was no significant difference in terms of clinical or physiological parameters during NAVA compared to CV, including work of breathing. CONCLUSION: Respiratory drive and effort were correlated in infants with CDH and therefore NAVA is a suitable proportional mode in this population. EAdi can also be used to monitor the diaphragm for individualized support.

@article{depoortere_nutritional_2022,
	title = {Nutritional status at age 1 year in patients born with esophageal atresia: {A} population-based, prospective cohort study},
	volume = {10},
	issn = {2296-2360},
	shorttitle = {Nutritional status at age 1 year in patients born with esophageal atresia},
	url = {http://hdl.handle.net/20.500.12210/79192},
	doi = {10.3389/fped.2022.969617},
	abstract = {OBJECTIVE: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure.
STUDY DESIGN: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P {\textless} 0.20 in univariate analyses were retained in a logistic regression model.
RESULTS: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61\%. Among these, 15.2\% were undernourished and 19\% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41\%), small for gestational age (17\%), or with associated abnormalities (55\%) were at higher risk of undernutrition and stunting at age 1 year (P {\textless} 0.05). Neither EA type nor surgical treatment was associated with growth failure.
CONCLUSION: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies.},
	language = {eng},
	journal = {Frontiers in Pediatrics},
	author = {Depoortere, Suzanne and Lapillonne, Alexandre and Sfeir, Rony and Bonnard, Arnaud and Gelas, Thomas and Panait, Nicoleta and Rabattu, Pierre-Yves and Guignot, Audrey and Lamireau, Thierry and Irtan, Sabine and Habonimana, Edouard and Breton, Anne and Fouquet, Virginie and Allal, Hossein and Elbaz, Frédéric and Talon, Isabelle and Ranke, Aline and Abely, Michel and Michel, Jean-Luc and Lirussi Borgnon, Joséphine and Buisson, Philippe and Schmitt, Françoise and Lardy, Hubert and Petit, Thierry and Chaussy, Yann and Borderon, Corinne and Levard, Guillaume and Cremillieux, Clara and Tolg, Cécilia and Breaud, Jean and Jaby, Olivier and Grossos, Céline and De Vries, Philine and Arnould, Myriam and Pelatan, Cécile and Geiss, Stephan and Laplace, Christophe and Kyheng, Maéva and Nicolas, Audrey and Aumar, Madeleine and Gottrand, Frédéric},
	year = {2022},
	keywords = {catch-up, growth, prematurity, small for gestational age, stunting, syndromic, undernutrition},
	pages = {969617},
}

OBJECTIVE: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure. STUDY DESIGN: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P \textless 0.20 in univariate analyses were retained in a logistic regression model. RESULTS: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year (P \textless 0.05). Neither EA type nor surgical treatment was associated with growth failure. CONCLUSION: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies.

@article{dellenmark-blom_postoperative_2022,
	title = {Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia: a nationwide {Swedish} study},
	volume = {17},
	issn = {1750-1172},
	shorttitle = {Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia},
	doi = {10.1186/s13023-022-02381-y},
	abstract = {BACKGROUND: In 10-15\% of children with esophageal atresia (EA) delayed reconstruction of esophageal atresia (DREA) is necessary due to long-gap EA and/or prematurity/low birth weight. They represent a patient subgroup with high risk of complications. We aimed to evaluate postoperative morbidity and health-related quality of life (HRQOL) in a Swedish national cohort of children with DREA.
METHODS: Postoperative morbidity, age-specific generic HRQOL (PedsQL™ 4.0) and condition-specific HRQOL (The EA-QOL questionnaires) in children with DREA were compared with children with EA who had primary anastomosis (PA). Factors associated with the DREA group's HRQOL scores were analyzed using Mann-Whitney U-test and Spearman's rho. Clinical data was extracted from the medical records. Significance level was p {\textless} 0.05.
RESULTS: Thirty-four out of 45 families of children with DREA were included and 30 returned the questionnaires(n = 8 children aged 2-7 years; n = 22 children aged 8-18 years). Compared to children with PA(42 children aged 2-7 years; 64 children aged 8-18 years), there were no significant differences in most early postoperative complications. At follow-up, symptom prevalence in children aged 2-7 with DREA ranged from 37.5\% (heartburn) to 75\% (cough). Further digestive and respiratory symptoms were present in ≥ 50\%. In children aged 8-18, it ranged from 14.3\% (vomiting) to 40.9\% (cough), with other digestive and airway symptoms present in 19.0-27.3\%. Except for chest tightness (2-7 years), there were no significant differences in symptom prevalence between children with DREA and PA, nor between their generic or condition-specific HRQOL scores (p {\textgreater} 0.05). More children with DREA underwent esophageal dilatations (both age groups), gastrostomy feeding (2-7 years), and antireflux treatment (8-18 years), p {\textless} 0.05. Days to hospital discharge after EA repair and a number of associated anomalies showed a strong negative correlation with HRQOL scores (2-7 years). Presence of cough, airway infection, swallowing difficulties and heartburn were associated with lower HRQOL scores (8-18 years), p {\textless} 0.05.
CONCLUSIONS: Although children with DREA need more treatments, they are not a risk group for postoperative morbidity and impaired HRQOL compared with children with PA. However, those with a long initial hospital stay, several associated anomalies and digestive or respiratory symptoms risk worse HRQOL. This is important information for clinical practice, families and patient stakeholders.},
	language = {eng},
	number = {1},
	journal = {Orphanet Journal of Rare Diseases},
	author = {Dellenmark-Blom, Michaela and Örnö Ax, Sofie and Öst, Elin and Svensson, Jan F. and Kassa, Ann-Marie and Jönsson, Linus and Abrahamsson, Kate and Gatzinsky, Vladimir and Stenström, Pernilla and Tollne, AnnaMaria and Omling, Erik and Engstrand Lilja, Helene},
	month = jun,
	year = {2022},
	keywords = {Child, Cough, Delayed reconstruction, Esophageal Atresia, Esophageal atresia, Health-related quality of life, Heartburn, Humans, Long-gap esophageal atresia, Long-term morbidity, Morbidity, Postoperative outcomes, Quality of Life, Sweden, Treatment Outcome},
	pages = {239},
}

BACKGROUND: In 10-15% of children with esophageal atresia (EA) delayed reconstruction of esophageal atresia (DREA) is necessary due to long-gap EA and/or prematurity/low birth weight. They represent a patient subgroup with high risk of complications. We aimed to evaluate postoperative morbidity and health-related quality of life (HRQOL) in a Swedish national cohort of children with DREA. METHODS: Postoperative morbidity, age-specific generic HRQOL (PedsQL™ 4.0) and condition-specific HRQOL (The EA-QOL questionnaires) in children with DREA were compared with children with EA who had primary anastomosis (PA). Factors associated with the DREA group's HRQOL scores were analyzed using Mann-Whitney U-test and Spearman's rho. Clinical data was extracted from the medical records. Significance level was p \textless 0.05. RESULTS: Thirty-four out of 45 families of children with DREA were included and 30 returned the questionnaires(n = 8 children aged 2-7 years; n = 22 children aged 8-18 years). Compared to children with PA(42 children aged 2-7 years; 64 children aged 8-18 years), there were no significant differences in most early postoperative complications. At follow-up, symptom prevalence in children aged 2-7 with DREA ranged from 37.5% (heartburn) to 75% (cough). Further digestive and respiratory symptoms were present in ≥ 50%. In children aged 8-18, it ranged from 14.3% (vomiting) to 40.9% (cough), with other digestive and airway symptoms present in 19.0-27.3%. Except for chest tightness (2-7 years), there were no significant differences in symptom prevalence between children with DREA and PA, nor between their generic or condition-specific HRQOL scores (p \textgreater 0.05). More children with DREA underwent esophageal dilatations (both age groups), gastrostomy feeding (2-7 years), and antireflux treatment (8-18 years), p \textless 0.05. Days to hospital discharge after EA repair and a number of associated anomalies showed a strong negative correlation with HRQOL scores (2-7 years). Presence of cough, airway infection, swallowing difficulties and heartburn were associated with lower HRQOL scores (8-18 years), p \textless 0.05. CONCLUSIONS: Although children with DREA need more treatments, they are not a risk group for postoperative morbidity and impaired HRQOL compared with children with PA. However, those with a long initial hospital stay, several associated anomalies and digestive or respiratory symptoms risk worse HRQOL. This is important information for clinical practice, families and patient stakeholders.

@article{dhar_british_2022,
	title = {British {Society} of {Gastroenterology} ({BSG}) and {British} {Society} of {Paediatric} {Gastroenterology}, {Hepatology} and {Nutrition} ({BSPGHAN}) joint consensus guidelines on the diagnosis and management of eosinophilic oesophagitis in children and adults},
	volume = {71},
	issn = {1468-3288},
	doi = {10.1136/gutjnl-2022-327326},
	abstract = {BACKGROUND: Eosinophilic oesophagitis (EoE) is an increasingly common cause of dysphagia in both children and adults, as well as one of the most prevalent oesophageal diseases with a significant impact on physical health and quality of life. We have provided a single comprehensive guideline for both paediatric and adult gastroenterologists on current best practice for the evaluation and management of EoE.
METHODS: The Oesophageal Section of the British Society of Gastroenterology was commissioned by the Clinical Standards Service Committee to develop these guidelines. The Guideline Development Group included adult and paediatric gastroenterologists, surgeons, dietitians, allergists, pathologists and patient representatives. The Population, Intervention, Comparator and Outcomes process was used to generate questions for a systematic review of the evidence. Published evidence was reviewed and updated to June 2021. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system was used to assess the evidence and make recommendations. Two rounds of voting were held to assess the level of agreement and the strength of recommendations, with 80\% consensus required for acceptance.
RESULTS: Fifty-seven statements on EoE presentation, diagnosis, investigation, management and complications were produced with further statements created on areas for future research.
CONCLUSIONS: These comprehensive adult and paediatric guidelines of the British Society of Gastroenterology and British Society of Paediatric Gastroenterology, Hepatology and Nutrition are based on evidence and expert consensus from a multidisciplinary group of healthcare professionals, including patient advocates and patient support groups, to help clinicians with the management patients with EoE and its complications.},
	language = {eng},
	number = {8},
	journal = {Gut},
	author = {Dhar, Anjan and Haboubi, Hasan N. and Attwood, Stephen E. and Auth, Marcus K. H. and Dunn, Jason M. and Sweis, Rami and Morris, Danielle and Epstein, Jenny and Novelli, Marco R. and Hunter, Hannah and Cordell, Amanda and Hall, Sharon and Hayat, Jamal O. and Kapur, Kapil and Moore, Andrew Robert and Read, Carol and Sami, Sarmed S. and Turner, Paul J. and Trudgill, Nigel J.},
	month = aug,
	year = {2022},
	keywords = {Adult, Child, Consensus, DIET, DYSPHAGIA, ENDOSCOPY, Eosinophilic Esophagitis, Gastroenterology, Humans, OESOPHAGEAL DISEASE, PAEDIATRIC GASTROENTEROLOGY, Quality of Life, Societies, Medical},
	pages = {1459--1487},
}

BACKGROUND: Eosinophilic oesophagitis (EoE) is an increasingly common cause of dysphagia in both children and adults, as well as one of the most prevalent oesophageal diseases with a significant impact on physical health and quality of life. We have provided a single comprehensive guideline for both paediatric and adult gastroenterologists on current best practice for the evaluation and management of EoE. METHODS: The Oesophageal Section of the British Society of Gastroenterology was commissioned by the Clinical Standards Service Committee to develop these guidelines. The Guideline Development Group included adult and paediatric gastroenterologists, surgeons, dietitians, allergists, pathologists and patient representatives. The Population, Intervention, Comparator and Outcomes process was used to generate questions for a systematic review of the evidence. Published evidence was reviewed and updated to June 2021. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system was used to assess the evidence and make recommendations. Two rounds of voting were held to assess the level of agreement and the strength of recommendations, with 80% consensus required for acceptance. RESULTS: Fifty-seven statements on EoE presentation, diagnosis, investigation, management and complications were produced with further statements created on areas for future research. CONCLUSIONS: These comprehensive adult and paediatric guidelines of the British Society of Gastroenterology and British Society of Paediatric Gastroenterology, Hepatology and Nutrition are based on evidence and expert consensus from a multidisciplinary group of healthcare professionals, including patient advocates and patient support groups, to help clinicians with the management patients with EoE and its complications.

@article{comella_radiation_2021,
	title = {Radiation burden in patients with esophageal atresia: a systematic review},
	volume = {37},
	issn = {1437-9813},
	shorttitle = {Radiation burden in patients with esophageal atresia},
	doi = {10.1007/s00383-021-04892-4},
	abstract = {Esophageal atresia (EA) is the most common congenital esophageal disorder. Radiological imaging facilitates diagnosis, surgical interventions, and follow-up. Despite this, standardized monitoring guidelines are lacking. We aimed to: (1) review the literature regarding radiation burden in children with EA; (2) establish the presence of guidelines for diagnosis and follow-up in children with EA. The systematic review was performed according to PRISMA protocol. Two investigators conducted independent searches (PubMed, Ovid, Cochrane Review) and data extraction. Analysis focused on pre- and post-operative imaging type and frequency to determine the radiation burden. Seven studies met the inclusion criteria (337 patients). All authors agreed upon the need to minimize radiation burden, recommending symptoms-guided management, use of dosimeters, and non-radiating imaging. One study identified a median 130-fold increase in cumulative lifetime cancer risk in children with EA compared with other babies in the special care unit. The most common investigations were X-ray and CT (pre-operatively), and X-ray and contrast swallow (post-operatively). Standardized guidelines focused upon the frequency and type of radiological imaging for children with EA are lacking. Children with EA are subjected to more radiation exposure than the general population. Implementation of non-radiating imaging (ultrasonography, manometry) is recommended.},
	language = {eng},
	number = {7},
	journal = {Pediatric Surgery International},
	author = {Comella, Assia and Tan Tanny, Sharman P. and Hutson, John M. and Omari, Taher I. and Teague, Warwick J. and Nataraja, Ramesh M. and King, Sebastian K.},
	month = jul,
	year = {2021},
	keywords = {Esophageal Atresia, Esophageal atresia, Esophagoplasty, Exposure, Humans, Infant, Manometry, Outcomes, Radiation, Radiation Exposure, Radiography, Systematic review},
	pages = {919--927},
}

Esophageal atresia (EA) is the most common congenital esophageal disorder. Radiological imaging facilitates diagnosis, surgical interventions, and follow-up. Despite this, standardized monitoring guidelines are lacking. We aimed to: (1) review the literature regarding radiation burden in children with EA; (2) establish the presence of guidelines for diagnosis and follow-up in children with EA. The systematic review was performed according to PRISMA protocol. Two investigators conducted independent searches (PubMed, Ovid, Cochrane Review) and data extraction. Analysis focused on pre- and post-operative imaging type and frequency to determine the radiation burden. Seven studies met the inclusion criteria (337 patients). All authors agreed upon the need to minimize radiation burden, recommending symptoms-guided management, use of dosimeters, and non-radiating imaging. One study identified a median 130-fold increase in cumulative lifetime cancer risk in children with EA compared with other babies in the special care unit. The most common investigations were X-ray and CT (pre-operatively), and X-ray and contrast swallow (post-operatively). Standardized guidelines focused upon the frequency and type of radiological imaging for children with EA are lacking. Children with EA are subjected to more radiation exposure than the general population. Implementation of non-radiating imaging (ultrasonography, manometry) is recommended.

@article{yang_risk_2021,
	title = {Risk {Factors} and {Reasons} for {Treatment} {Abandonment} for {Patients} {With} {Esophageal} {Atresia}: {A} {Study} {From} a {Tertiary} {Care} {Hospital} in {Beijing}, {China}},
	volume = {9},
	issn = {2296-2360},
	shorttitle = {Risk {Factors} and {Reasons} for {Treatment} {Abandonment} for {Patients} {With} {Esophageal} {Atresia}},
	doi = {10.3389/fped.2021.634573},
	abstract = {Background: This study aims to identify the risk factors and reasons for treatment abandonment for patients with esophageal atresia (EA) in a tertiary care hospital in China. Methods: A retrospective study was conducted on 360 patients with EA admitted to Beijing Children's Hospital between January 1, 2007 and June 1, 2020. Medical records for treatment abandonment and non-treatment abandonment patients were compared. Univariate and multivariate logistic regression analyses were conducted to identify potential risk factors for treatment abandonment. Results: After the diagnosis of EA, parents of 107 patients refused surgical repair and discharged against medical advice, and 253 patients underwent surgical repair. Among these 253 patients, parents of 59 patients abandoned treatment after surgery; 52 patients were discharged in an unstable condition, and parents of seven patients abandoned resuscitation leading to death in the hospital. By comparing clinical characteristics between treatment abandonment before surgery (n = 107) and non-treatment abandonment (n = 253) groups, we found that mother's parity {\textgreater}1, unplanned admission to intensive care unit before surgery, associated anomalies, and Gross type A/B were significant independent risk factors for treatment abandonment before surgery. Furthermore, birth weight {\textless}2,545 g, being discharged from neonatal center/intensive care unit and other departments, unplanned admission to intensive care unit after surgery, operative time {\textgreater}133 min, admission before 2016, pneumothorax, and anastomotic leakage were significant independent risk factors for treatment abandonment after surgery. The reasons for treatment abandonment included financial difficulties, multiple malformations with poor prognosis, belief of incurability and concerns about the prognosis of the diseases, postoperative complications, and extensive length of intensive care unit stay. Conclusions: Treatment abandonment of children with EA/TEF is still a common and serious problem in China. This study showed that EA/TEF patients in critical conditions, with associated anomalies, Gross type A/B, and who had occurrence of complications had high-risk for treatment abandonment.},
	language = {eng},
	journal = {Frontiers in Pediatrics},
	author = {Yang, Shen and Liao, Junmin and Li, Siqi and Hua, Kaiyun and Wang, Peize and Zhang, Yanan and Zhao, Yong and Gu, Yichao and Li, Shuangshuang and Huang, Jinshi},
	year = {2021},
	keywords = {children, esophageal atresia, reasons, risk factors, treatment abandonment},
	pages = {634573},
}

Background: This study aims to identify the risk factors and reasons for treatment abandonment for patients with esophageal atresia (EA) in a tertiary care hospital in China. Methods: A retrospective study was conducted on 360 patients with EA admitted to Beijing Children's Hospital between January 1, 2007 and June 1, 2020. Medical records for treatment abandonment and non-treatment abandonment patients were compared. Univariate and multivariate logistic regression analyses were conducted to identify potential risk factors for treatment abandonment. Results: After the diagnosis of EA, parents of 107 patients refused surgical repair and discharged against medical advice, and 253 patients underwent surgical repair. Among these 253 patients, parents of 59 patients abandoned treatment after surgery; 52 patients were discharged in an unstable condition, and parents of seven patients abandoned resuscitation leading to death in the hospital. By comparing clinical characteristics between treatment abandonment before surgery (n = 107) and non-treatment abandonment (n = 253) groups, we found that mother's parity \textgreater1, unplanned admission to intensive care unit before surgery, associated anomalies, and Gross type A/B were significant independent risk factors for treatment abandonment before surgery. Furthermore, birth weight \textless2,545 g, being discharged from neonatal center/intensive care unit and other departments, unplanned admission to intensive care unit after surgery, operative time \textgreater133 min, admission before 2016, pneumothorax, and anastomotic leakage were significant independent risk factors for treatment abandonment after surgery. The reasons for treatment abandonment included financial difficulties, multiple malformations with poor prognosis, belief of incurability and concerns about the prognosis of the diseases, postoperative complications, and extensive length of intensive care unit stay. Conclusions: Treatment abandonment of children with EA/TEF is still a common and serious problem in China. This study showed that EA/TEF patients in critical conditions, with associated anomalies, Gross type A/B, and who had occurrence of complications had high-risk for treatment abandonment.

@article{lejeune_esophageal_2021,
	title = {Esophageal {Atresia} and {Respiratory} {Morbidity}},
	volume = {148},
	issn = {1098-4275},
	url = {http://hdl.handle.net/20.500.12210/79191},
	doi = {10.1542/peds.2020-049778},
	abstract = {BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children.
METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value {\textless}.10 in univariate analyses were retained in logistic regression models.
RESULTS: Among 1460 patients born with EA, 97 (7\%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89\%, preterm birth was observed in 38\%, and associated malformations were observed in 52\%. Collectively, 61\% were readmitted after initial discharge in the first year, 31\% for a respiratory cause. Among these, respiratory infections occurred in 64\%, and 35\% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft.
CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for {\textgreater}50\% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies.},
	language = {eng},
	number = {3},
	journal = {Pediatrics},
	author = {Lejeune, Stéphanie and Sfeir, Rony and Rousseau, Véronique and Bonnard, Arnaud and Gelas, Thomas and Aumar, Madeleine and Panait, Nicoleta and Rabattu, Pierre-Yves and Irtan, Sabine and Fouquet, Virginie and Le Mandat, Aurélie and Cocci, Stephan De Napoli and Habonimana, Edouard and Lamireau, Thierry and Lemelle, Jean-Louis and Elbaz, Frédéric and Talon, Isabelle and Boudaoud, Nadia and Allal, Hossein and Buisson, Philippe and Petit, Thierry and Sapin, Emmanuel and Lardy, Hubert and Schmitt, Françoise and Levard, Guillaume and Scalabre, Aurélien and Michel, Jean-Luc and Jaby, Olivier and Pelatan, Cécile and De Vries, Philine and Borderon, Corinne and Fourcade, Laurent and Breaud, Jean and Arnould, Myriam and Tolg, Cécilia and Chaussy, Yann and Geiss, Stephan and Laplace, Christophe and Drumez, Elodie and El Mourad, Sawsan and Thumerelle, Caroline and Gottrand, Frédéric},
	month = sep,
	year = {2021},
	keywords = {Cohort Studies, Congenital Abnormalities, Enteral Nutrition, Esophageal Atresia, Female, Follow-Up Studies, France, Gastroesophageal Reflux, Humans, Infant, Infant, Small for Gestational Age, Male, Patient Readmission, Premature Birth, Registries, Respiration Disorders, Tracheoesophageal Fistula},
	pages = {e2020049778},
}

BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children. METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value \textless.10 in univariate analyses were retained in logistic regression models. RESULTS: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft. CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for \textgreater50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies.

@article{ravindra_exposure_2021,
	title = {Exposure to air pollutants and risk of congenital anomalies: {A} systematic review and metaanalysis},
	volume = {765},
	issn = {1879-1026},
	shorttitle = {Exposure to air pollutants and risk of congenital anomalies},
	doi = {10.1016/j.scitotenv.2020.142772},
	abstract = {BACKGROUND: Congenital malformations are considered as one of the significant causes of preterm as well as neonatal morbidity and mortality. Literature suggests the association of diverse congenital deformities with maternal exposure to air pollutants. However, the evidence is still inconclusive on the manifestation of these during pregnancy. Thus, systematic review was done on the available epidemiological studies studying the effect of air pollutants on congenital malformations. Furthermore, the meta-analysis was conducted for several combinations of air pollutants and congenital defects.
METHODS: Twenty six epidemiological studies were extracted from the databases and examined for association of risk of congenital defects with air pollutant concentrations. Metaanalysis was done if the risk estimates of the same anomaly and pollutant group were reported in at least three studies.
RESULTS: Each study reported a statistically significant increased risk of congenital malformation with some air pollutant, amid the several tested combinations. Our meta-analysis reported that nitrogen dioxide and PM2.5 were associated with the risk of pulmonary valve stenosis with OR = 1.74 and OR = 1.42 respectively. The risk of developing tetralogy of Fallot (TOF) was observed to be associated with PM2.5 with OR = 1.52. SO2 exposure was related to a high risk of the ventricular septal defect (VSD) with OR = 1.15 and orofacial defects (OR = 1.27).
CONCLUSION: It is evidenced that ambient air pollutants have some effect on congenital malformations. Standard case definitions, improved methods of exposure, and better control of confounders will improve future research in this area.},
	language = {eng},
	journal = {The Science of the Total Environment},
	author = {Ravindra, Khaiwal and Chanana, Neha and Mor, Suman},
	month = apr,
	year = {2021},
	keywords = {Air Pollutants, Air Pollution, Birth defects, Congenital Abnormalities, Congenital anomalies, Congenital malformations, Databases, Factual, Environmental Pollutants, Female, Humans, Infant, Newborn, Maternal Exposure, Meta-analysis, Nitrogen Dioxide, Particulate Matter, Pregnancy},
	pages = {142772},
}

BACKGROUND: Congenital malformations are considered as one of the significant causes of preterm as well as neonatal morbidity and mortality. Literature suggests the association of diverse congenital deformities with maternal exposure to air pollutants. However, the evidence is still inconclusive on the manifestation of these during pregnancy. Thus, systematic review was done on the available epidemiological studies studying the effect of air pollutants on congenital malformations. Furthermore, the meta-analysis was conducted for several combinations of air pollutants and congenital defects. METHODS: Twenty six epidemiological studies were extracted from the databases and examined for association of risk of congenital defects with air pollutant concentrations. Metaanalysis was done if the risk estimates of the same anomaly and pollutant group were reported in at least three studies. RESULTS: Each study reported a statistically significant increased risk of congenital malformation with some air pollutant, amid the several tested combinations. Our meta-analysis reported that nitrogen dioxide and PM2.5 were associated with the risk of pulmonary valve stenosis with OR = 1.74 and OR = 1.42 respectively. The risk of developing tetralogy of Fallot (TOF) was observed to be associated with PM2.5 with OR = 1.52. SO2 exposure was related to a high risk of the ventricular septal defect (VSD) with OR = 1.15 and orofacial defects (OR = 1.27). CONCLUSION: It is evidenced that ambient air pollutants have some effect on congenital malformations. Standard case definitions, improved methods of exposure, and better control of confounders will improve future research in this area.

@article{darmaun_quality_2021,
	title = {Quality of life was similar in children with congenital diaphragmatic hernia and oesophageal atresia and related to respiratory morbidity},
	volume = {110},
	issn = {1651-2227},
	url = {http://hdl.handle.net/20.500.12210/40704},
	doi = {10.1111/apa.15426},
	abstract = {AIM: To assess quality of life (QoL) in children with congenital diaphragmatic hernia (CDH) and to compare it with oesophageal atresia (OA).
METHODS: A cross-sectional study in CDH children (≥7 years) was conducted in Lille University Hospital, France, from January 2013 to April 2014. History, lung function (rest, exercise) and Pediatric Quality of Life Inventory questionnaires (PedsQoL 4.0) were collected. Data of OA children were previously published.
RESULTS: Fifty-four CDH patients (male: 53\%, median age: 11 years, IQR 9-14) were compared to 54 OA patients (male: 61\%, median age: 13 years, IQR: 11-15). CDH children had significantly more frequent history of pneumonia (30\% vs 13\%), exercise limitation (54\% vs 35\%) and chest deformity (39\% vs 11\%); 46\% had an obstructive pattern and 66\% an abnormal cardiopulmonary exercise test. The median PedsQoL total score in children was 81 (IQR 73-90) in CDH and 81 (IQR 72-91) in OA (P = .8). In CDH, duration of neonatal oxygen therapy, hospitalisation for respiratory disease, exercise limitation, inhaled corticosteroids treatment, chest deformity, abnormal cardiopulmonary exercise test and lower forced expiratory volume in one second were significantly associated with lower QoL scores.
CONCLUSION: PedsQoL scores remained satisfactory in CDH children with CDH, with no difference compared to OA. Patients with respiratory morbidity and lung function impairment, who displayed lower scores, should be identified in order to optimise their management in reference centres.},
	language = {eng},
	number = {2},
	journal = {Acta Paediatrica (Oslo, Norway: 1992)},
	author = {Darmaun, Laura and Lejeune, Stephanie and Drumez, Elodie and Mur, Sebastien and de Langle-Chevalier, Fanny and Nève, Véronique and Storme, Laurent and Michaud, Laurent and Gottrand, Frederic and Thumerelle, Caroline and Deschildre, Antoine},
	month = feb,
	year = {2021},
	keywords = {Adolescent, Child, Cross-Sectional Studies, Esophageal Atresia, France, Hernias, Diaphragmatic, Congenital, Humans, Infant, Newborn, Male, Morbidity, Quality of Life, congenital diaphragmatic hernia, lung function tests, oesophageal atresia, quality of life, respiratory morbidity},
	pages = {695--703},
}

AIM: To assess quality of life (QoL) in children with congenital diaphragmatic hernia (CDH) and to compare it with oesophageal atresia (OA). METHODS: A cross-sectional study in CDH children (≥7 years) was conducted in Lille University Hospital, France, from January 2013 to April 2014. History, lung function (rest, exercise) and Pediatric Quality of Life Inventory questionnaires (PedsQoL 4.0) were collected. Data of OA children were previously published. RESULTS: Fifty-four CDH patients (male: 53%, median age: 11 years, IQR 9-14) were compared to 54 OA patients (male: 61%, median age: 13 years, IQR: 11-15). CDH children had significantly more frequent history of pneumonia (30% vs 13%), exercise limitation (54% vs 35%) and chest deformity (39% vs 11%); 46% had an obstructive pattern and 66% an abnormal cardiopulmonary exercise test. The median PedsQoL total score in children was 81 (IQR 73-90) in CDH and 81 (IQR 72-91) in OA (P = .8). In CDH, duration of neonatal oxygen therapy, hospitalisation for respiratory disease, exercise limitation, inhaled corticosteroids treatment, chest deformity, abnormal cardiopulmonary exercise test and lower forced expiratory volume in one second were significantly associated with lower QoL scores. CONCLUSION: PedsQoL scores remained satisfactory in CDH children with CDH, with no difference compared to OA. Patients with respiratory morbidity and lung function impairment, who displayed lower scores, should be identified in order to optimise their management in reference centres.

@article{tavares_proton-pump_2021,
	title = {Proton-{Pump} {Inhibitors}: {Do} {Children} {Break} a {Leg} by {Using} {Them}?},
	volume = {73},
	issn = {1536-4801},
	shorttitle = {Proton-{Pump} {Inhibitors}},
	doi = {10.1097/MPG.0000000000003246},
	abstract = {The risk of bone fracture in children under proton-pump inhibitors (PPI) treatment has been the subject of recent publications and naturally raises concerns among prescribing doctors, patients and their parents.Currently, there is no consistency in those risk claims according to the available evidence and an update on it is beneficial to reduce anxiety on one hand, and prompt for well-planned studies addressing the issue on the other. Furthermore, common sense and well-founded prescriptions must be the general rule for this as for any other therapeutic drug.},
	language = {eng},
	number = {6},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Tavares, Marta and Amil-Dias, Jorge},
	month = dec,
	year = {2021},
	keywords = {Child, Humans, Leg, Proton Pump Inhibitors},
	pages = {665--669},
}

The risk of bone fracture in children under proton-pump inhibitors (PPI) treatment has been the subject of recent publications and naturally raises concerns among prescribing doctors, patients and their parents.Currently, there is no consistency in those risk claims according to the available evidence and an update on it is beneficial to reduce anxiety on one hand, and prompt for well-planned studies addressing the issue on the other. Furthermore, common sense and well-founded prescriptions must be the general rule for this as for any other therapeutic drug.

@article{mougey_stat6_2021,
	title = {{STAT6} {Variants} {Associate} {With} {Relapse} of {Eosinophilic} {Esophagitis} in {Patients} {Receiving} {Long}-term {Proton} {Pump} {Inhibitor} {Therapy}},
	volume = {19},
	issn = {1542-7714},
	doi = {10.1016/j.cgh.2020.08.020},
	abstract = {BACKGROUND \& AIMS: Based on histologic features, variants in STAT6 are associated with a poor initial response to proton pump inhibitor (PPI) therapy in pediatric patients with eosinophilic esophagitis (EoE). We investigated whether these genetic variants are associated with a poor long-term response in children with EoE who initially responded to PPI therapy.
METHODS: We performed a prospective longitudinal cohort study of children ages 2 to 16 years who met the diagnostic criteria for EoE (≥15 eosinophils/high-power field [eos/hpf]), responded to 8 weeks of treatment with 2 mg/kg/d PPI ({\textless}15 eos/hpf), and whose dose then was reduced to 1 mg/kg/d PPI (maintenance therapy) for 1 year, at which point biopsy specimens were collected by endoscopy. Genomic DNA was isolated from formalin-fixed paraffin-embedded biopsy tissue and was genotyped for variants of STAT6. Remission of inflammation was assessed at eos/hpf thresholds of {\textless}15 and ≤5.
RESULTS: Among 73 patients who received 1 mg/kg/d PPI maintenance therapy for 1 year, 13 patients (18\%) had 6 to 14 eos/hpf, 36 patients (49\%) had 5 or fewer eos/hpf, and 24 patients (33\%) relapsed to EoE (≥15 eos/hpf). Carriage of any of 3 STAT6 variants in linkage disequilibrium (r2 ≥0.8; rs324011, rs167769, or rs12368672) was associated with a 2.3- to 2.8-fold increase in the odds of EoE relapse, and with a 2.8- to 4.1-fold increase in the odds of having 6 to 14 eos/hpf. For rs324011, the odds ratio [95\% CI] for relapse was 2.77 [1.11, 6.92]; P = .029, and the odds ratio [95\% CI] for having 6 to 14 eos/hpf was 3.06 [1.27, 7.36]; P = .012.
CONCLUSIONS: Pediatric EoE patients who initially respond to PPI therapy and carry STAT6 variants rs324011, rs167769, or rs12368672 are at increased risk of relapse after 1 year of PPI maintenance therapy.},
	language = {eng},
	number = {10},
	journal = {Clinical Gastroenterology and Hepatology: The Official Clinical Practice Journal of the American Gastroenterological Association},
	author = {Mougey, Edward B. and Nguyen, Vivian and Gutiérrez-Junquera, Carolina and Fernández-Fernández, Sonia and Cilleruelo, Maria Luz and Rayo, Ana and Borrell, Belén and Román, Enriqueta and González-Lois, Carmen and Chao, Montserrat and Al-Atrash, Hadeel and Franciosi, James P.},
	month = oct,
	year = {2021},
	keywords = {Adolescent, Biomarker, Child, Child, Preschool, Eosinophilic Esophagitis, Esophagus, Humans, Immune Response, Longitudinal Studies, Prospective Studies, Proton Pump Inhibitors, Recurrence, Response to Treatment, STAT6 Transcription Factor},
	pages = {2046--2053.e2},
}

BACKGROUND & AIMS: Based on histologic features, variants in STAT6 are associated with a poor initial response to proton pump inhibitor (PPI) therapy in pediatric patients with eosinophilic esophagitis (EoE). We investigated whether these genetic variants are associated with a poor long-term response in children with EoE who initially responded to PPI therapy. METHODS: We performed a prospective longitudinal cohort study of children ages 2 to 16 years who met the diagnostic criteria for EoE (≥15 eosinophils/high-power field [eos/hpf]), responded to 8 weeks of treatment with 2 mg/kg/d PPI (\textless15 eos/hpf), and whose dose then was reduced to 1 mg/kg/d PPI (maintenance therapy) for 1 year, at which point biopsy specimens were collected by endoscopy. Genomic DNA was isolated from formalin-fixed paraffin-embedded biopsy tissue and was genotyped for variants of STAT6. Remission of inflammation was assessed at eos/hpf thresholds of \textless15 and ≤5. RESULTS: Among 73 patients who received 1 mg/kg/d PPI maintenance therapy for 1 year, 13 patients (18%) had 6 to 14 eos/hpf, 36 patients (49%) had 5 or fewer eos/hpf, and 24 patients (33%) relapsed to EoE (≥15 eos/hpf). Carriage of any of 3 STAT6 variants in linkage disequilibrium (r2 ≥0.8; rs324011, rs167769, or rs12368672) was associated with a 2.3- to 2.8-fold increase in the odds of EoE relapse, and with a 2.8- to 4.1-fold increase in the odds of having 6 to 14 eos/hpf. For rs324011, the odds ratio [95% CI] for relapse was 2.77 [1.11, 6.92]; P = .029, and the odds ratio [95% CI] for having 6 to 14 eos/hpf was 3.06 [1.27, 7.36]; P = .012. CONCLUSIONS: Pediatric EoE patients who initially respond to PPI therapy and carry STAT6 variants rs324011, rs167769, or rs12368672 are at increased risk of relapse after 1 year of PPI maintenance therapy.

@article{koumbourlis_care_2020,
	title = {Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula},
	volume = {55},
	copyright = {© 2020 Wiley Periodicals LLC},
	issn = {1099-0496},
	url = {https://onlinelibrary.wiley.com/doi/abs/10.1002/ppul.24982},
	doi = {10.1002/ppul.24982},
	abstract = {Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on “strong”, “moderate” or “weak” agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long-term follow-up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients’ age were also developed.},
	language = {en},
	number = {10},
	urldate = {2025-07-28},
	journal = {Pediatric Pulmonology},
	author = {Koumbourlis, Anastassios C. and Belessis, Yvonne and Cataletto, Mary and Cutrera, Renato and DeBoer, Emily and Kazachkov, Mikhail and Laberge, Sophie and Popler, Jonathan and Porcaro, Federica and Kovesi, Thomas},
	year = {2020},
	note = {\_eprint: https://onlinelibrary.wiley.com/doi/pdf/10.1002/ppul.24982},
	keywords = {aspiration, bronchiectasis, chronic bronchitis, gastroesophageal reflux, tracheoesophageal fistula, tracheomalacia},
	pages = {2713--2729},
}

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on “strong”, “moderate” or “weak” agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long-term follow-up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients’ age were also developed.

@article{lejeune_bronchopulmonary_2020,
	title = {Bronchopulmonary and vascular anomalies are frequent in children with oesophageal atresia},
	volume = {109},
	issn = {1651-2227},
	url = {http://hdl.handle.net/20.500.12210/79209},
	doi = {10.1111/apa.15086},
	abstract = {AIM: Oesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition.
METHOD: This was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist.
RESULTS: More than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation.
CONCLUSION: Intrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms.},
	language = {eng},
	number = {6},
	journal = {Acta Paediatrica (Oslo, Norway: 1992)},
	author = {Lejeune, Stephanie and Le Mee, Armelle and Petyt, Laurent and Hutt, Antoine and Sfeir, Rony and Michaud, Laurent and Fayoux, Pierre and Deschildre, Antoine and Gottrand, Frederic and Thumerelle, Caroline},
	month = jun,
	year = {2020},
	keywords = {Adolescent, Adult, Child, Child, Preschool, Cystic Adenomatoid Malformation of Lung, Congenital, Esophageal Atresia, Humans, Infant, Infant, Newborn, Lung, Retrospective Studies, Vena Cava, Superior, computed tomography, congenital malformations, diagnostic imaging, oesophageal atresia, thoracic malformations},
	pages = {1221--1228},
}

AIM: Oesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition. METHOD: This was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist. RESULTS: More than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation. CONCLUSION: Intrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms.

@article{donnet_esophageal_2020,
	title = {Esophageal perforation in eosinophilic esophagitis: five cases in children},
	volume = {8},
	issn = {2364-3722},
	shorttitle = {Esophageal perforation in eosinophilic esophagitis},
	url = {https://pubmed.ncbi.nlm.nih.gov/32617387/},
	doi = {10.1055/a-0914-2711},
	abstract = {Background and study aims  Eosinophilic esophagitis (EoE) is a chronic immune disease with increasing incidence. It is clinically defined by symptoms of esophageal dysfunction and histologically by eosinophilic polynuclear cell infiltration of the esophageal mucosa. Symptoms are not specific and include gastroesophageal reflux disease (GERD), dysphagia, vomiting or dietary blockages. Chronic inflammation of the mucosa may lead to narrowing of the esophageal lumen responsible for impactions. Extraction procedures can be complicated by dissection and perforation. Rare spontaneous ruptures of the esophagus known as Boerhaave syndrome are also possible. We report five cases of esophageal perforation in children with EoE, three with spontaneous rupture and two after an endoscopic procedure. The evolution was favorable under medical treatment.},
	language = {eng},
	number = {7},
	journal = {Endoscopy International Open},
	author = {Donnet, Camille and Destombe, Sylvie and Lachaux, Alain and Michaud, Laurent and Triolo, Valérie and Heissat, Sophie and Stephan, Jean-Louis and Patural, Hugues},
	month = jul,
	year = {2020},
	pages = {E830--E833},
}

Background and study aims  Eosinophilic esophagitis (EoE) is a chronic immune disease with increasing incidence. It is clinically defined by symptoms of esophageal dysfunction and histologically by eosinophilic polynuclear cell infiltration of the esophageal mucosa. Symptoms are not specific and include gastroesophageal reflux disease (GERD), dysphagia, vomiting or dietary blockages. Chronic inflammation of the mucosa may lead to narrowing of the esophageal lumen responsible for impactions. Extraction procedures can be complicated by dissection and perforation. Rare spontaneous ruptures of the esophagus known as Boerhaave syndrome are also possible. We report five cases of esophageal perforation in children with EoE, three with spontaneous rupture and two after an endoscopic procedure. The evolution was favorable under medical treatment.

@article{courbette_characterization_2020,
	title = {Characterization of {Esophageal} {Motility} in {Children} {With} {Operated} {Esophageal} {Atresia} {Using} {High}-resolution {Impedance} {Manometry} and {Pressure} {Flow} {Analysis}},
	volume = {71},
	issn = {1536-4801},
	doi = {10.1097/MPG.0000000000002806},
	abstract = {OBJECTIVES: Esophageal dysmotility is common in patients with esophageal atresia (EA). High-resolution impedance manometry and pressure flow analysis (PFA) allow characterization of biomechanical events that drive bolus flow. The aims were to assess esophageal motility in children with EA, using PFA, and to test whether there is a correlation between PFA parameters and symptoms or endoscopic/histologic findings.
METHODS: High-resolution impedance manometry was performed in 16 children with EA (median age 11 years), compared with 13 patient controls (median age 14 years; P = NS vs patients). Wet swallows were analyzed using PFA. Medical charts were reviewed for symptoms and pathology results of the attendant esophagoscopy. Patients with EA were arbitrarily subgrouped according to their motility pattern: group A with presence of distal contraction in ≥50\% of the swallows and group B with presence of distal contractions in {\textless}50\% of the swallows.
RESULTS: Esophageal peristaltic motor patterns were abnormal in all patients with EA. Bolus transport was impaired as shown by the higher impedance ratio in EA than in controls (0.47 vs 0.22; P {\textless} 0.001). Impedance ratio was also higher in group B (n = 8) versus group A (n = 8) (P {\textless} 0.001). Symptoms of dysphagia were not correlated with the PFA measures. Contractile segment impedance, a marker of mucosal integrity, was significantly lower in the EA group.
CONCLUSIONS: Bolus transport was severely altered in patients with EA but was not predictive of symptoms. The presence of residual distal contractions is associated with a more efficient bolus propulsion.},
	language = {eng},
	number = {3},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Courbette, Olivier and Omari, Taher and Aspirot, Ann and Faure, Christophe},
	month = sep,
	year = {2020},
	keywords = {Adolescent, Child, Deglutition, Electric Impedance, Esophageal Atresia, Esophageal Motility Disorders, Humans, Manometry},
	pages = {304--309},
}

OBJECTIVES: Esophageal dysmotility is common in patients with esophageal atresia (EA). High-resolution impedance manometry and pressure flow analysis (PFA) allow characterization of biomechanical events that drive bolus flow. The aims were to assess esophageal motility in children with EA, using PFA, and to test whether there is a correlation between PFA parameters and symptoms or endoscopic/histologic findings. METHODS: High-resolution impedance manometry was performed in 16 children with EA (median age 11 years), compared with 13 patient controls (median age 14 years; P = NS vs patients). Wet swallows were analyzed using PFA. Medical charts were reviewed for symptoms and pathology results of the attendant esophagoscopy. Patients with EA were arbitrarily subgrouped according to their motility pattern: group A with presence of distal contraction in ≥50% of the swallows and group B with presence of distal contractions in \textless50% of the swallows. RESULTS: Esophageal peristaltic motor patterns were abnormal in all patients with EA. Bolus transport was impaired as shown by the higher impedance ratio in EA than in controls (0.47 vs 0.22; P \textless 0.001). Impedance ratio was also higher in group B (n = 8) versus group A (n = 8) (P \textless 0.001). Symptoms of dysphagia were not correlated with the PFA measures. Contractile segment impedance, a marker of mucosal integrity, was significantly lower in the EA group. CONCLUSIONS: Bolus transport was severely altered in patients with EA but was not predictive of symptoms. The presence of residual distal contractions is associated with a more efficient bolus propulsion.

@article{glinianaia_long-term_2020,
	title = {Long-term survival of children born with congenital anomalies: {A} systematic review and meta-analysis of population-based studies},
	volume = {17},
	issn = {1549-1676},
	shorttitle = {Long-term survival of children born with congenital anomalies},
	doi = {10.1371/journal.pmed.1003356},
	abstract = {BACKGROUND: Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing. Although infant survival of children born with congenital anomalies has improved for many anomaly types in recent decades, there is less evidence on survival beyond infancy. We aimed to systematically review, summarise, and quantify the existing population-based data on long-term survival of individuals born with specific major congenital anomalies and examine the factors associated with survival.
METHODS AND FINDINGS: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, and citations of the included articles for studies published 1 January 1995 to 30 April 2020 were searched. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate. We included original population-based studies that reported long-term survival (beyond 1 year of life) of children born with a major congenital anomaly with the follow-up starting from birth that were published in the English language as peer-reviewed papers. Studies on congenital heart defects (CHDs) were excluded because of a recent systematic review of population-based studies of CHD survival. Meta-analysis was performed to pool survival estimates, accounting for trends over time. Of 10,888 identified articles, 55 (n = 367,801 live births) met the inclusion criteria and were summarised narratively, 41 studies (n = 54,676) investigating eight congenital anomaly types (spina bifida [n = 7,422], encephalocele [n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6,176], gastroschisis [n = 4,845], Down syndrome by presence of CHD [n = 22,317], and trisomy 18 [n = 2,174]) were included in the meta-analysis. These studies covered birth years from 1970 to 2015. Survival for children with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, and Down syndrome with an associated CHD has significantly improved over time, with the pooled odds ratios (ORs) of surviving per 10-year increase in birth year being OR = 1.34 (95\% confidence interval [95\% CI] 1.24-1.46), OR = 1.50 (95\% CI 1.38-1.62), OR = 1.62 (95\% CI 1.28-2.05), OR = 1.57 (95\% CI 1.37-1.81), OR = 1.24 (95\% CI 1.02-1.5), and OR = 1.99 (95\% CI 1.67-2.37), respectively (p {\textless} 0.001 for all, except for gastroschisis [p = 0.029]). There was no observed improvement for children with encephalocele (OR = 0.98, 95\% CI 0.95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96, 95\% CI 0.88-1.03, p = 0.26). The presence of additional structural anomalies, low birth weight, and earlier year of birth were the most commonly reported predictors of reduced survival for any congenital anomaly type. The main limitation of the meta-analysis was the small number of studies and the small size of the cohorts, which limited the predictive capabilities of the models resulting in wide confidence intervals.
CONCLUSIONS: This systematic review and meta-analysis summarises estimates of long-term survival associated with major congenital anomalies. We report a significant improvement in survival of children with specific congenital anomalies over the last few decades and predict survival estimates up to 20 years of age for those born in 2020. This information is important for the planning and delivery of specialised medical, social, and education services and for counselling affected families. This trial was registered on the PROSPERO database (CRD42017074675).},
	language = {eng},
	number = {9},
	journal = {PLoS medicine},
	author = {Glinianaia, Svetlana V. and Morris, Joan K. and Best, Kate E. and Santoro, Michele and Coi, Alessio and Armaroli, Annarita and Rankin, Judith},
	month = sep,
	year = {2020},
	keywords = {Adult, Age Factors, Child, Child Mortality, Child, Preschool, Congenital Abnormalities, Female, Humans, Infant, Live Birth, Parturition, Pregnancy, Registries, Young Adult},
	pages = {e1003356},
}

BACKGROUND: Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing. Although infant survival of children born with congenital anomalies has improved for many anomaly types in recent decades, there is less evidence on survival beyond infancy. We aimed to systematically review, summarise, and quantify the existing population-based data on long-term survival of individuals born with specific major congenital anomalies and examine the factors associated with survival. METHODS AND FINDINGS: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, and citations of the included articles for studies published 1 January 1995 to 30 April 2020 were searched. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate. We included original population-based studies that reported long-term survival (beyond 1 year of life) of children born with a major congenital anomaly with the follow-up starting from birth that were published in the English language as peer-reviewed papers. Studies on congenital heart defects (CHDs) were excluded because of a recent systematic review of population-based studies of CHD survival. Meta-analysis was performed to pool survival estimates, accounting for trends over time. Of 10,888 identified articles, 55 (n = 367,801 live births) met the inclusion criteria and were summarised narratively, 41 studies (n = 54,676) investigating eight congenital anomaly types (spina bifida [n = 7,422], encephalocele [n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6,176], gastroschisis [n = 4,845], Down syndrome by presence of CHD [n = 22,317], and trisomy 18 [n = 2,174]) were included in the meta-analysis. These studies covered birth years from 1970 to 2015. Survival for children with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, and Down syndrome with an associated CHD has significantly improved over time, with the pooled odds ratios (ORs) of surviving per 10-year increase in birth year being OR = 1.34 (95% confidence interval [95% CI] 1.24-1.46), OR = 1.50 (95% CI 1.38-1.62), OR = 1.62 (95% CI 1.28-2.05), OR = 1.57 (95% CI 1.37-1.81), OR = 1.24 (95% CI 1.02-1.5), and OR = 1.99 (95% CI 1.67-2.37), respectively (p \textless 0.001 for all, except for gastroschisis [p = 0.029]). There was no observed improvement for children with encephalocele (OR = 0.98, 95% CI 0.95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96, 95% CI 0.88-1.03, p = 0.26). The presence of additional structural anomalies, low birth weight, and earlier year of birth were the most commonly reported predictors of reduced survival for any congenital anomaly type. The main limitation of the meta-analysis was the small number of studies and the small size of the cohorts, which limited the predictive capabilities of the models resulting in wide confidence intervals. CONCLUSIONS: This systematic review and meta-analysis summarises estimates of long-term survival associated with major congenital anomalies. We report a significant improvement in survival of children with specific congenital anomalies over the last few decades and predict survival estimates up to 20 years of age for those born in 2020. This information is important for the planning and delivery of specialised medical, social, and education services and for counselling affected families. This trial was registered on the PROSPERO database (CRD42017074675).

@article{zeck_gastrointestinal_2019,
	title = {Gastrointestinal diseases among relatives of patients with esophageal atresia with or without tracheoesophageal fistula},
	volume = {8},
	issn = {2224-4344},
	doi = {10.21037/tp.2019.04.01},
	abstract = {BACKGROUND: Several studies have identified genetic factors that are associated with the formation of isolated and non-isolated esophageal atresia with or without tracheoesophageal fistula (EA/TEF) in human and mice. Some of these genetic factors like FOXF1/Foxf1 are associated with Barrett syndrome, esophageal carcinoma or tumors of the gastrointestinal tract. Here, we investigated the prevalence of common gastrointestinal diseases among EA/TEF patients and their first- and second-degree relatives (parents and grandparents).
METHODS: We send out a questionnaire to 280 EA/TEF families asking for the presence of Barrett syndrome, Achalasia and carcinoma of the esophagus, the stomach, the small and large intestine among first- and second-degree relatives.
RESULTS: In 32 of 124 families we found at least one affected family member with a possible association of colon carcinoma and the occurrence of EA/TEF within the same family.
CONCLUSIONS: Further studies are needed to evaluate a possible association.},
	language = {eng},
	number = {5},
	journal = {Translational Pediatrics},
	author = {Zeck, Florian and Reutter, Heiko},
	month = dec,
	year = {2019},
	keywords = {Esophageal atresia (EA), gastrointestinal disease, genetic risk factors},
	pages = {378--382},
}

BACKGROUND: Several studies have identified genetic factors that are associated with the formation of isolated and non-isolated esophageal atresia with or without tracheoesophageal fistula (EA/TEF) in human and mice. Some of these genetic factors like FOXF1/Foxf1 are associated with Barrett syndrome, esophageal carcinoma or tumors of the gastrointestinal tract. Here, we investigated the prevalence of common gastrointestinal diseases among EA/TEF patients and their first- and second-degree relatives (parents and grandparents). METHODS: We send out a questionnaire to 280 EA/TEF families asking for the presence of Barrett syndrome, Achalasia and carcinoma of the esophagus, the stomach, the small and large intestine among first- and second-degree relatives. RESULTS: In 32 of 124 families we found at least one affected family member with a possible association of colon carcinoma and the occurrence of EA/TEF within the same family. CONCLUSIONS: Further studies are needed to evaluate a possible association.

@article{van_lennep_oesophageal_2019,
	title = {Oesophageal atresia},
	volume = {5},
	issn = {2056-676X},
	url = {https://pubmed.ncbi.nlm.nih.gov/31000707/},
	doi = {10.1038/s41572-019-0077-0},
	abstract = {Oesophageal atresia (EA) is a congenital abnormality of the oesophagus that is caused by incomplete embryonic compartmentalization of the foregut. EA commonly occurs with a tracheo-oesophageal fistula (TEF). Associated birth defects or anomalies, such as VACTERL association, trisomy 18 or 21 and CHARGE syndrome, occur in the majority of patients born with EA. Although several studies have revealed signalling pathways and genes potentially involved in the development of EA, our understanding of the pathophysiology of EA lags behind the improvements in surgical and clinical care of patients born with this anomaly. EA is treated surgically to restore the oesophageal interruption and, if present, ligate and divide the TEF. Survival is now {\textasciitilde}90\% in those born with EA with severe associated anomalies and even higher in those born with EA alone. Despite these achievements, long-term gastrointestinal and respiratory complications and comorbidities in patients born with EA are common and lead to decreased quality of life. Oesophageal motility disorders are probably ubiquitous in patients after undergoing EA repair and often underlie these complications and comorbidities. The implementation of several new diagnostic and screening tools in clinical care, including high-resolution impedance manometry, pH-multichannel intraluminal impedance testing and disease-specific quality of life questionnaires now provide better insight into these problems and may contribute to better long-term outcomes in the future.},
	language = {eng},
	number = {1},
	journal = {Nature Reviews. Disease Primers},
	author = {van Lennep, Marinde and Singendonk, Maartje M. J. and Dall'Oglio, Luigi and Gottrand, Fréderic and Krishnan, Usha and Terheggen-Lagro, Suzanne W. J. and Omari, Taher I. and Benninga, Marc A. and van Wijk, Michiel P.},
	month = apr,
	year = {2019},
	keywords = {Comorbidity, Congenital Abnormalities, Esophageal Atresia, Esophagoscopy, Gastroesophageal Reflux, Humans, Magnetic Resonance Imaging, Mass Screening, Tracheoesophageal Fistula, Ultrasonography},
	pages = {26},
}

Oesophageal atresia (EA) is a congenital abnormality of the oesophagus that is caused by incomplete embryonic compartmentalization of the foregut. EA commonly occurs with a tracheo-oesophageal fistula (TEF). Associated birth defects or anomalies, such as VACTERL association, trisomy 18 or 21 and CHARGE syndrome, occur in the majority of patients born with EA. Although several studies have revealed signalling pathways and genes potentially involved in the development of EA, our understanding of the pathophysiology of EA lags behind the improvements in surgical and clinical care of patients born with this anomaly. EA is treated surgically to restore the oesophageal interruption and, if present, ligate and divide the TEF. Survival is now ~90% in those born with EA with severe associated anomalies and even higher in those born with EA alone. Despite these achievements, long-term gastrointestinal and respiratory complications and comorbidities in patients born with EA are common and lead to decreased quality of life. Oesophageal motility disorders are probably ubiquitous in patients after undergoing EA repair and often underlie these complications and comorbidities. The implementation of several new diagnostic and screening tools in clinical care, including high-resolution impedance manometry, pH-multichannel intraluminal impedance testing and disease-specific quality of life questionnaires now provide better insight into these problems and may contribute to better long-term outcomes in the future.

@article{goday_pediatric_2019,
	title = {Pediatric {Feeding} {Disorder}: {Consensus} {Definition} and {Conceptual} {Framework}},
	volume = {68},
	issn = {1536-4801},
	shorttitle = {Pediatric {Feeding} {Disorder}},
	doi = {10.1097/MPG.0000000000002188},
	abstract = {Pediatric feeding disorders (PFDs) lack a universally accepted definition. Feeding disorders require comprehensive assessment and treatment of 4 closely related, complementary domains (medical, psychosocial, and feeding skill-based systems and associated nutritional complications). Previous diagnostic paradigms have, however, typically defined feeding disorders using the lens of a single professional discipline and fail to characterize associated functional limitations that are critical to plan appropriate interventions and improve quality of life. Using the framework of the World Health Organization International Classification of Functioning, Disability, and Health, a unifying diagnostic term is proposed: "Pediatric Feeding Disorder" (PFD), defined as impaired oral intake that is not age-appropriate, and is associated with medical, nutritional, feeding skill, and/or psychosocial dysfunction. By incorporating associated functional limitations, the proposed diagnostic criteria for PFD should enable practitioners and researchers to better characterize the needs of heterogeneous patient populations, facilitate inclusion of all relevant disciplines in treatment planning, and promote the use of common, precise, terminology necessary to advance clinical practice, research, and health-care policy.},
	language = {eng},
	number = {1},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Goday, Praveen S. and Huh, Susanna Y. and Silverman, Alan and Lukens, Colleen T. and Dodrill, Pamela and Cohen, Sherri S. and Delaney, Amy L. and Feuling, Mary B. and Noel, Richard J. and Gisel, Erika and Kenzer, Amy and Kessler, Daniel B. and Kraus de Camargo, Olaf and Browne, Joy and Phalen, James A.},
	month = jan,
	year = {2019},
	keywords = {Child, Child Nutrition Sciences, Child Nutritional Physiological Phenomena, Consensus, Feeding and Eating Disorders, Gastroenterology, Humans, International Classification of Diseases, International Classification of Functioning, Disability and Health, Pediatrics, World Health Organization},
	pages = {124--129},
}

Pediatric feeding disorders (PFDs) lack a universally accepted definition. Feeding disorders require comprehensive assessment and treatment of 4 closely related, complementary domains (medical, psychosocial, and feeding skill-based systems and associated nutritional complications). Previous diagnostic paradigms have, however, typically defined feeding disorders using the lens of a single professional discipline and fail to characterize associated functional limitations that are critical to plan appropriate interventions and improve quality of life. Using the framework of the World Health Organization International Classification of Functioning, Disability, and Health, a unifying diagnostic term is proposed: "Pediatric Feeding Disorder" (PFD), defined as impaired oral intake that is not age-appropriate, and is associated with medical, nutritional, feeding skill, and/or psychosocial dysfunction. By incorporating associated functional limitations, the proposed diagnostic criteria for PFD should enable practitioners and researchers to better characterize the needs of heterogeneous patient populations, facilitate inclusion of all relevant disciplines in treatment planning, and promote the use of common, precise, terminology necessary to advance clinical practice, research, and health-care policy.

@article{ley_efficacy_2019,
	title = {Efficacy and {Safety} of the {Local} {Application} of {Mitomycin} {C} to {Recurrent} {Esophageal} {Strictures} in {Children}},
	volume = {69},
	issn = {1536-4801},
	url = {https://pubmed.ncbi.nlm.nih.gov/31436711/},
	doi = {10.1097/MPG.0000000000002445},
	abstract = {OBJECTIVES: Research on long-term use of mitomycin C (MC) for recurrent esophageal stenoses is limited. We assessed the long-term efficacy and safety of local application of MC for recurrent esophageal stenoses in children.
METHODS: This was a retrospective study of 39 patients (17 girls) with a median age of 19.5 months (range: 2.4-196.0) at the time of MC application. The etiologies of stenosis were esophageal atresia (n = 25), caustic ingestion (n = 9), congenital esophageal stenosis (n = 3), and other causes (n = 2). Stenosis was single in 35 (90\%) patients and multiple in 4 (10\%). Before MC, patients underwent multiple repeated dilations (median: 3 dilations per child [range: 2-26]) over a median period of 7 months (range: 2.6-49.3). Treatment success was defined a priori as a reduction in the number of dilations over the same period from before to after the application of MC.
RESULTS: For 26 (67\%) patients, the application of MC was considered a success: 102 versus 17 dilatations (P {\textless} 0.0001). Sixteen (41\%) patients never required additional dilation during the follow-up after MC application (median: 3.1 years [range: 0.6-8.5]). No complication related to MC was observed. Biopsies at the site of MC application were performed at maximal follow-up in 16 patients and revealed no dysplasia. Three factors were associated with success of MC: single stenosis, short stenosis, and esophageal atresia type III.
CONCLUSIONS: This study is the largest series reported showing that topical application of MC is an efficient and safe treatment for recurrent esophageal stenosis in children.},
	language = {eng},
	number = {5},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Ley, Delphine and Bridenne, Marie and Gottrand, Frédéric and Lemale, Julie and Hauser, Bruno and Lachaux, Alain and Rebouissoux, Laurent and Viala, Jérôme and Fayoux, Pierre and Michaud, Laurent},
	month = nov,
	year = {2019},
	keywords = {Administration, Mucosal, Adolescent, Antibiotics, Antineoplastic, Child, Child, Preschool, Esophageal Stenosis, Esophagoscopy, Female, Humans, Infant, Male, Mitomycin, Postoperative Complications, Retrospective Studies},
	pages = {528--532},
}

OBJECTIVES: Research on long-term use of mitomycin C (MC) for recurrent esophageal stenoses is limited. We assessed the long-term efficacy and safety of local application of MC for recurrent esophageal stenoses in children. METHODS: This was a retrospective study of 39 patients (17 girls) with a median age of 19.5 months (range: 2.4-196.0) at the time of MC application. The etiologies of stenosis were esophageal atresia (n = 25), caustic ingestion (n = 9), congenital esophageal stenosis (n = 3), and other causes (n = 2). Stenosis was single in 35 (90%) patients and multiple in 4 (10%). Before MC, patients underwent multiple repeated dilations (median: 3 dilations per child [range: 2-26]) over a median period of 7 months (range: 2.6-49.3). Treatment success was defined a priori as a reduction in the number of dilations over the same period from before to after the application of MC. RESULTS: For 26 (67%) patients, the application of MC was considered a success: 102 versus 17 dilatations (P \textless 0.0001). Sixteen (41%) patients never required additional dilation during the follow-up after MC application (median: 3.1 years [range: 0.6-8.5]). No complication related to MC was observed. Biopsies at the site of MC application were performed at maximal follow-up in 16 patients and revealed no dysplasia. Three factors were associated with success of MC: single stenosis, short stenosis, and esophageal atresia type III. CONCLUSIONS: This study is the largest series reported showing that topical application of MC is an efficient and safe treatment for recurrent esophageal stenosis in children.

@article{wittekindt_epidemiology_2019,
	title = {Epidemiology and {Outcome} of {Major} {Congenital} {Malformations} in a {Large} {German} {County}},
	volume = {29},
	issn = {1439-359X},
	doi = {10.1055/s-0038-1642630},
	abstract = {INTRODUCTION: Congenital malformations are associated with substantial neonatal morbidity and mortality. Furthermore, only sparse data are available on the modalities of care provided to and the associated clinical outcomes in affected neonates. In this study, we focused on five malformations that require surgery during the neonatal period: duodenal stenosis and atresia (DA), gastroschisis (GA), omphalocele (OM), congenital diaphragmatic herniation (CDH), and esophageal atresia (EA).
MATERIALS AND METHODS: We reviewed the Hessian neonatal registry (2010-2015) to identify records including the ICD-10 (International Classification of Diseases, Tenth Edition) codes for the aforementioned diagnoses and identified 283 patients who were affected by at least one of these conditions. Multiple regression analyses were performed to further identify risk factors for mortality and extended length of hospital stay.
RESULTS: The incidence rates per 10,000 live births and inhospital mortality rates were as follows: DA: 1.79 and 3.6\%; GA: 1.79 and 1.8\%; OM: 1.60 and 24\%; CDH: 1.32 and 27.5\%; and EA: 2.67 and 11.1\%, respectively. Thirty-three percent of the patients had not been born in a perinatal center in which corrective surgeries were performed. The following risk factors were significantly associated with early mortality: trisomy 13 and 18, congenital heart defects, prematurity, and high-risk malformations (OM and CDH). The predictors of length of stay were as follows: gestational age, number of additional malformations, and treatment in the center with the highest patient volume.
CONCLUSION: Epidemiology and outcome of major congenital malformations in Hesse, Germany, are comparable to previously published data. In addition, our data revealed a volume-outcome association with regard to the length of hospital stay.},
	language = {eng},
	number = {3},
	journal = {European Journal of Pediatric Surgery: Official Journal of Austrian Association of Pediatric Surgery ... [et Al] = Zeitschrift Fur Kinderchirurgie},
	author = {Wittekindt, Boris and Schloesser, Rolf and Doberschuetz, Nora and Salzmann-Manrique, Emilia and Grossmann, Jasmin and Misselwitz, Bjoern and Rolle, Udo},
	month = jun,
	year = {2019},
	keywords = {Congenital Abnormalities, Female, Germany, Humans, Infant, Newborn, Length of Stay, Logistic Models, Male, Registries, Risk Factors, Survival Analysis, Treatment Outcome},
	pages = {282--289},
}

INTRODUCTION: Congenital malformations are associated with substantial neonatal morbidity and mortality. Furthermore, only sparse data are available on the modalities of care provided to and the associated clinical outcomes in affected neonates. In this study, we focused on five malformations that require surgery during the neonatal period: duodenal stenosis and atresia (DA), gastroschisis (GA), omphalocele (OM), congenital diaphragmatic herniation (CDH), and esophageal atresia (EA). MATERIALS AND METHODS: We reviewed the Hessian neonatal registry (2010-2015) to identify records including the ICD-10 (International Classification of Diseases, Tenth Edition) codes for the aforementioned diagnoses and identified 283 patients who were affected by at least one of these conditions. Multiple regression analyses were performed to further identify risk factors for mortality and extended length of hospital stay. RESULTS: The incidence rates per 10,000 live births and inhospital mortality rates were as follows: DA: 1.79 and 3.6%; GA: 1.79 and 1.8%; OM: 1.60 and 24%; CDH: 1.32 and 27.5%; and EA: 2.67 and 11.1%, respectively. Thirty-three percent of the patients had not been born in a perinatal center in which corrective surgeries were performed. The following risk factors were significantly associated with early mortality: trisomy 13 and 18, congenital heart defects, prematurity, and high-risk malformations (OM and CDH). The predictors of length of stay were as follows: gestational age, number of additional malformations, and treatment in the center with the highest patient volume. CONCLUSION: Epidemiology and outcome of major congenital malformations in Hesse, Germany, are comparable to previously published data. In addition, our data revealed a volume-outcome association with regard to the length of hospital stay.

@article{navarro_systematic_2019,
	title = {Systematic review with meta-analysis: the growing incidence and prevalence of eosinophilic oesophagitis in children and adults in population-based studies},
	volume = {49},
	issn = {1365-2036},
	shorttitle = {Systematic review with meta-analysis},
	doi = {10.1111/apt.15231},
	abstract = {BACKGROUND: The frequency of eosinophilic oesophagitis (EoE) occurrence is escalating. Current diagnostic criteria recently proposed for the disease, determine that previous estimates of incidence and prevalence are outdated.
AIM: To gauge the current incidence and prevalence of EoE by performing a systematic review of population-based studies.
METHODS: Three electronic databases were searched from their inception dates to September 2018. A total of 2386 documents were screened; 29 studies reported on the prevalence and incidence of EoE in the general population.
RESULTS: The pooled prevalence of EoE was 34.4 cases per 100 000 inhabitants (95\% CI, 23.1-47.5), and was higher for adults (42.2; 95\% CI, 31.1-55) than for children (34; 95\% CI, 22.3-49.2). The pooled EoE incidence rates were 6.6/100 000 person-years (95\% CI, 3-11.7) in children and 7.7/100 000 (95\% CI, 1.8-17.8) in adults. No differences were found between North American and European studies using varied sources of data (insurance and administrative databases compared to hospital-bases case series). Subgroup analysis according to risk of bias did not change results significantly. A steady rise in EoE incidence and prevalence rates was observed over time, comparing studies conducted under subsequent definitions for EoE. No significant publication bias was found.
CONCLUSIONS: In a systematic review and meta-analysis, we found a sharp increase, higher than previous estimates, in the incidence and prevalence of EoE in population based studies. Results from studies carried out in developed countries show broad consistency and provide evidence of increasing pooled prevalence and incidence of EoE rates over time.},
	language = {eng},
	number = {9},
	journal = {Alimentary Pharmacology \& Therapeutics},
	author = {Navarro, Pilar and Arias, Ángel and Arias-González, Laura and Laserna-Mendieta, Emilio J. and Ruiz-Ponce, Miriam and Lucendo, Alfredo J.},
	month = may,
	year = {2019},
	keywords = {Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Databases, Factual, Developed Countries, Eosinophilic Esophagitis, Female, Humans, Incidence, Male, Middle Aged, Prevalence, Young Adult},
	pages = {1116--1125},
}

BACKGROUND: The frequency of eosinophilic oesophagitis (EoE) occurrence is escalating. Current diagnostic criteria recently proposed for the disease, determine that previous estimates of incidence and prevalence are outdated. AIM: To gauge the current incidence and prevalence of EoE by performing a systematic review of population-based studies. METHODS: Three electronic databases were searched from their inception dates to September 2018. A total of 2386 documents were screened; 29 studies reported on the prevalence and incidence of EoE in the general population. RESULTS: The pooled prevalence of EoE was 34.4 cases per 100 000 inhabitants (95% CI, 23.1-47.5), and was higher for adults (42.2; 95% CI, 31.1-55) than for children (34; 95% CI, 22.3-49.2). The pooled EoE incidence rates were 6.6/100 000 person-years (95% CI, 3-11.7) in children and 7.7/100 000 (95% CI, 1.8-17.8) in adults. No differences were found between North American and European studies using varied sources of data (insurance and administrative databases compared to hospital-bases case series). Subgroup analysis according to risk of bias did not change results significantly. A steady rise in EoE incidence and prevalence rates was observed over time, comparing studies conducted under subsequent definitions for EoE. No significant publication bias was found. CONCLUSIONS: In a systematic review and meta-analysis, we found a sharp increase, higher than previous estimates, in the incidence and prevalence of EoE in population based studies. Results from studies carried out in developed countries show broad consistency and provide evidence of increasing pooled prevalence and incidence of EoE rates over time.

@article{morris_trends_2018,
	title = {Trends in congenital anomalies in {Europe} from 1980 to 2012},
	volume = {13},
	issn = {1932-6203},
	doi = {10.1371/journal.pone.0194986},
	abstract = {BACKGROUND: Surveillance of congenital anomalies is important to identify potential teratogens.
METHODS: This study analysed the prevalence of 61 congenital anomaly subgroups (excluding chromosomal) in 25 population-based EUROCAT registries (1980-2012). Live births, fetal deaths and terminations of pregnancy for fetal anomaly were analysed with multilevel random-effects Poisson regression models.
RESULTS: Seventeen anomaly subgroups had statistically significant trends from 2003-2012; 12 increasing and 5 decreasing.
CONCLUSIONS: The annual increasing prevalence of severe congenital heart defects, single ventricle, atrioventricular septal defects and tetralogy of Fallot of 1.4\% (95\% CI: 0.7\% to 2.0\%), 4.6\% (1.0\% to 8.2\%), 3.4\% (1.3\% to 5.5\%) and 4.1\% (2.4\% to 5.7\%) respectively may reflect increases in maternal obesity and diabetes (known risk factors). The increased prevalence of cystic adenomatous malformation of the lung [6.5\% (3.5\% to 9.4\%)] and decreased prevalence of limb reduction defects [-2.8\% (-4.2\% to -1.5\%)] are unexplained. For renal dysplasia and maternal infections, increasing trends may be explained by increased screening, and deceases in patent ductus arteriosus at term and increases in craniosynostosis, by improved follow up period after birth and improved diagnosis. For oesophageal atresia, duodenal atresia/stenosis and ano-rectal atresia/stenosis recent changes in prevalence appeared incidental when compared with larger long term fluctuations. For microcephaly and congenital hydronephrosis trends could not be interpreted due to discrepancies in diagnostic criteria. The trends for club foot and syndactyly disappeared once registries with disparate results were excluded. No decrease in neural tube defects was detected, despite efforts at prevention through folic acid supplementation.},
	language = {eng},
	number = {4},
	journal = {PloS One},
	author = {Morris, Joan K. and Springett, Anna L. and Greenlees, Ruth and Loane, Maria and Addor, Marie-Claude and Arriola, Larraitz and Barisic, Ingeborg and Bergman, Jorieke E. H. and Csaky-Szunyogh, Melinda and Dias, Carlos and Draper, Elizabeth S. and Garne, Ester and Gatt, Miriam and Khoshnood, Babak and Klungsoyr, Kari and Lynch, Catherine and McDonnell, Robert and Nelen, Vera and Neville, Amanda J. and O'Mahony, Mary and Pierini, Anna and Queisser-Luft, Annette and Randrianaivo, Hanitra and Rankin, Judith and Rissmann, Anke and Kurinczuk, Jennifer and Tucker, David and Verellen-Dumoulin, Christine and Wellesley, Diana and Dolk, Helen},
	year = {2018},
	keywords = {Congenital Abnormalities, Europe, Female, History, 20th Century, History, 21st Century, Humans, Male, Population Surveillance, Pregnancy, Prevalence, Registries},
	pages = {e0194986},
}

BACKGROUND: Surveillance of congenital anomalies is important to identify potential teratogens. METHODS: This study analysed the prevalence of 61 congenital anomaly subgroups (excluding chromosomal) in 25 population-based EUROCAT registries (1980-2012). Live births, fetal deaths and terminations of pregnancy for fetal anomaly were analysed with multilevel random-effects Poisson regression models. RESULTS: Seventeen anomaly subgroups had statistically significant trends from 2003-2012; 12 increasing and 5 decreasing. CONCLUSIONS: The annual increasing prevalence of severe congenital heart defects, single ventricle, atrioventricular septal defects and tetralogy of Fallot of 1.4% (95% CI: 0.7% to 2.0%), 4.6% (1.0% to 8.2%), 3.4% (1.3% to 5.5%) and 4.1% (2.4% to 5.7%) respectively may reflect increases in maternal obesity and diabetes (known risk factors). The increased prevalence of cystic adenomatous malformation of the lung [6.5% (3.5% to 9.4%)] and decreased prevalence of limb reduction defects [-2.8% (-4.2% to -1.5%)] are unexplained. For renal dysplasia and maternal infections, increasing trends may be explained by increased screening, and deceases in patent ductus arteriosus at term and increases in craniosynostosis, by improved follow up period after birth and improved diagnosis. For oesophageal atresia, duodenal atresia/stenosis and ano-rectal atresia/stenosis recent changes in prevalence appeared incidental when compared with larger long term fluctuations. For microcephaly and congenital hydronephrosis trends could not be interpreted due to discrepancies in diagnostic criteria. The trends for club foot and syndactyly disappeared once registries with disparate results were excluded. No decrease in neural tube defects was detected, despite efforts at prevention through folic acid supplementation.

@article{bastard_thoracic_2018,
	title = {Thoracic skeletal anomalies following surgical treatment of esophageal atresia. {Lessons} from a national cohort},
	volume = {53},
	issn = {1531-5037},
	url = {https://pubmed.ncbi.nlm.nih.gov/28778692/},
	doi = {10.1016/j.jpedsurg.2017.07.013},
	abstract = {INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity.
MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies.
RESULTS: Among 322 inclusions from 32 centers, 110 (34.2\%) X-rays were normal and 25 (7.7\%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1\%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1\%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2\%; p=0.04).
CONCLUSION: About 60 \% of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood.
LEVELS OF EVIDENCE: Level III retrospective comparative treatment study.},
	language = {eng},
	number = {4},
	journal = {Journal of Pediatric Surgery},
	author = {Bastard, François and Bonnard, Arnaud and Rousseau, Véronique and Gelas, Thomas and Michaud, Laurent and Irtan, Sabine and Piolat, Christian and Ranke-Chrétien, Aline and Becmeur, François and Dariel, Anne and Lamireau, Thierry and Petit, Thierry and Fouquet, Virginie and Le Mandat, Aurélie and Lefebvre, Francis and Allal, Hossein and Borgnon, Josephine and Boubnova, Julia and Habonimana, Edouard and Panait, Nicoleta and Buisson, Philippe and Margaryan, Marc and Michel, Jean-Luc and Gaudin, Jean and Lardy, Hubert and Auber, Frédéric and Borderon, Corinne and De Vries, Philine and Jaby, Olivier and Fourcade, Laurent and Lecompte, Jean François and Tolg, Cécilia and Delorme, Benoit and Schmitt, Françoise and Podevin, Guillaume},
	month = apr,
	year = {2018},
	keywords = {Child, Digestive System Surgical Procedures, Esophageal Atresia, Esophageal atresia, Female, Humans, Male, Musculoskeletal Abnormalities, Radiography, Radiography, Thoracic, Retrospective Studies, Thoracic Diseases, Thoracic wall, Thoracoscopy, Thoracotomy, Treatment Outcome},
	pages = {605--609},
}

INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. RESULTS: Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). CONCLUSION: About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. LEVELS OF EVIDENCE: Level III retrospective comparative treatment study.

@article{alsebayel_congenital_2018,
	title = {Congenital {Esophageal} {Atresia} and {Microtia} in a {Newborn} {Secondary} to {Mycophenolate} {Mofetil} {Exposure} {During} {Pregnancy}: {A} {Case} {Report} and {Review} of the {Literature}},
	volume = {19},
	issn = {1941-5923},
	shorttitle = {Congenital {Esophageal} {Atresia} and {Microtia} in a {Newborn} {Secondary} to {Mycophenolate} {Mofetil} {Exposure} {During} {Pregnancy}},
	doi = {10.12659/AJCR.908433},
	abstract = {BACKGROUND Mycophenolate mofetil (MMF) is one of the most commonly prescribed drugs to prevent organ transplant rejection in combination with calcineurin inhibitors and steroids. It has a different toxicity profile than tacrolimus and cyclosporine.  Gastrointestinal tract disturbances are the most common adverse effects. The use of MMF in pregnant women, however, holds great risk of miscarriage and fetal development defects such as external ear malformation, ocular anomalies, cleft lip and palate, and abnormality of distal limbs, heart, esophagus, and kidneys. Based on post-marketing studies, its pregnancy category was reclassified as category D by the US FDA in 2007. CASE REPORT A 20-year-old woman received a deceased-donor liver transplant for end-stage liver disease secondary to autoimmune hepatitis. She had 3 miscarriages while on MMF. In her fourth pregnancy she was exposed to MMF in the first trimester, which was stopped by week 20 of the pregnancy. Obstetric ultrasound suggested a cephalic presentation fetus with abdominal circumference. Her pregnancy resulted in an infant with tracheoesophageal fistula, esophageal atresia, and a bilateral ear canal atresia (microtia) with normal sensorineural conduction. There were no other congenital abnormalities. Thoracoscopic ligation of fistula and thoracotomy with esophageal repair were performed and a bone-anchored hearing aid for conductive hearing loss was implanted. Here, we report a case of congenital esophageal atresia and microtia secondary to mycophenolate mofetil. CONCLUSIONS MMF should be avoided during pregnancy. Transplanted female patients of reproductive age should receive appropriate counseling.},
	language = {eng},
	journal = {The American Journal of Case Reports},
	author = {Alsebayel, Musaed Mohammed and Abaalkhail, Faisal Abdulrahman and Alsebayel, Faisal Mohammed and Alissa, Dema A. and Al-Jedai, Ahmed Hamdan and Elsiesy, Hussien},
	month = may,
	year = {2018},
	keywords = {Congenital Microtia, Enzyme Inhibitors, Esophageal Atresia, Female, Humans, Infant, Newborn, Liver Transplantation, Maternal-Fetal Exchange, Mycophenolic Acid, Pregnancy, Transplant Recipients},
	pages = {523--526},
}

BACKGROUND Mycophenolate mofetil (MMF) is one of the most commonly prescribed drugs to prevent organ transplant rejection in combination with calcineurin inhibitors and steroids. It has a different toxicity profile than tacrolimus and cyclosporine.  Gastrointestinal tract disturbances are the most common adverse effects. The use of MMF in pregnant women, however, holds great risk of miscarriage and fetal development defects such as external ear malformation, ocular anomalies, cleft lip and palate, and abnormality of distal limbs, heart, esophagus, and kidneys. Based on post-marketing studies, its pregnancy category was reclassified as category D by the US FDA in 2007. CASE REPORT A 20-year-old woman received a deceased-donor liver transplant for end-stage liver disease secondary to autoimmune hepatitis. She had 3 miscarriages while on MMF. In her fourth pregnancy she was exposed to MMF in the first trimester, which was stopped by week 20 of the pregnancy. Obstetric ultrasound suggested a cephalic presentation fetus with abdominal circumference. Her pregnancy resulted in an infant with tracheoesophageal fistula, esophageal atresia, and a bilateral ear canal atresia (microtia) with normal sensorineural conduction. There were no other congenital abnormalities. Thoracoscopic ligation of fistula and thoracotomy with esophageal repair were performed and a bone-anchored hearing aid for conductive hearing loss was implanted. Here, we report a case of congenital esophageal atresia and microtia secondary to mycophenolate mofetil. CONCLUSIONS MMF should be avoided during pregnancy. Transplanted female patients of reproductive age should receive appropriate counseling.

@article{manfredi_endoscopic_2018,
	title = {Endoscopic {Electrocautery} {Incisional} {Therapy} as a {Treatment} for {Refractory} {Benign} {Pediatric} {Esophageal} {Strictures}},
	volume = {67},
	issn = {1536-4801},
	doi = {10.1097/MPG.0000000000002008},
	abstract = {BACKGROUND AND AIM: Refractory esophageal strictures are rare conditions in pediatrics, and are often due to anastomotic, congenital, or caustic strictures. Traditional treatment options include serial dilation and surgical stricture resection; endoscopic intralesional steroid injections, mitomycin C, and externally removable stents combined with dilation have had variable success rates. Although not as widely used, endoscopic electrocautery incisional therapy (EIT) has been reported as an alternative treatment for refractory strictures in a small number of adult series. The aim of the study was to evaluate the safety and efficacy of EIT in a pediatric population with refractory esophageal strictures.
METHODS: A retrospective chart review was conducted on all patients who underwent EIT for esophageal strictures (May 2011-September 2017) at our tertiary-care referral center. A total of 57 patients underwent EIT. Procedural success was defined as no stricture resection, appropriate diameter for age, and fewer than 7 dilations within 24 months of first EIT session. This corresponded to the 90th percentile of the observed number of dilations in the data. All patients included in the study had at least 2-year follow-up.
RESULTS: A total of 133 EIT sessions on 58 distinct anastomotic strictures were performed on 57 patients (24 girls). The youngest patient to have EIT was 3 months old and 4.8 kg. There were 36 strictures that met the criteria for refractory stricture and 22 non-refractory (NR) strictures. The median number of dilations before EIT therapy was 8 (interquartile range [IQR]: 6-10) in the refractory group and 3 (IQR: 0-3) in the NR group. In the refractory group, 61\% of the patients met the criteria for treatment success. The median number of dilations within 2 years of EIT in the refractory group was 2 (IQR: 0-4). In the NR group, 100\% of the patients met criteria for success. The median number of dilations within 2 years of EIT in the NR was 1 (IQR: 0-2). The overall adverse event rate was 5.3\% (7/133), with 3 major (2.3\%) and 4 minor events (3\%).
CONCLUSIONS: EIT shows promise as an adjunct treatment option for pediatric refractory esophageal strictures and may be considered before surgical resection even in severe cases. The complication rate, albeit low, is significant, and EIT should only be considered by experienced endoscopists in close consultation with surgery. Further prospective longitudinal studies are needed to validate this treatment.},
	language = {eng},
	number = {4},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Manfredi, Michael A. and Clark, Susannah J. and Medford, Shawn and Staffa, Steven J. and Ngo, Peter D. and Hamilton, Thomas E. and Smithers, C. Jason and Jennings, Russell W.},
	month = oct,
	year = {2018},
	keywords = {Child, Child, Preschool, Electrocoagulation, Esophageal Stenosis, Esophagoscopy, Female, Humans, Infant, Male, Postoperative Complications, Retrospective Studies, Treatment Outcome},
	pages = {464--468},
}

BACKGROUND AND AIM: Refractory esophageal strictures are rare conditions in pediatrics, and are often due to anastomotic, congenital, or caustic strictures. Traditional treatment options include serial dilation and surgical stricture resection; endoscopic intralesional steroid injections, mitomycin C, and externally removable stents combined with dilation have had variable success rates. Although not as widely used, endoscopic electrocautery incisional therapy (EIT) has been reported as an alternative treatment for refractory strictures in a small number of adult series. The aim of the study was to evaluate the safety and efficacy of EIT in a pediatric population with refractory esophageal strictures. METHODS: A retrospective chart review was conducted on all patients who underwent EIT for esophageal strictures (May 2011-September 2017) at our tertiary-care referral center. A total of 57 patients underwent EIT. Procedural success was defined as no stricture resection, appropriate diameter for age, and fewer than 7 dilations within 24 months of first EIT session. This corresponded to the 90th percentile of the observed number of dilations in the data. All patients included in the study had at least 2-year follow-up. RESULTS: A total of 133 EIT sessions on 58 distinct anastomotic strictures were performed on 57 patients (24 girls). The youngest patient to have EIT was 3 months old and 4.8 kg. There were 36 strictures that met the criteria for refractory stricture and 22 non-refractory (NR) strictures. The median number of dilations before EIT therapy was 8 (interquartile range [IQR]: 6-10) in the refractory group and 3 (IQR: 0-3) in the NR group. In the refractory group, 61% of the patients met the criteria for treatment success. The median number of dilations within 2 years of EIT in the refractory group was 2 (IQR: 0-4). In the NR group, 100% of the patients met criteria for success. The median number of dilations within 2 years of EIT in the NR was 1 (IQR: 0-2). The overall adverse event rate was 5.3% (7/133), with 3 major (2.3%) and 4 minor events (3%). CONCLUSIONS: EIT shows promise as an adjunct treatment option for pediatric refractory esophageal strictures and may be considered before surgical resection even in severe cases. The complication rate, albeit low, is significant, and EIT should only be considered by experienced endoscopists in close consultation with surgery. Further prospective longitudinal studies are needed to validate this treatment.

@article{pellegrino_impact_2018,
	title = {Impact of {Esophageal} {Atresia} on the {Success} of {Fundoplication} for {Gastroesophageal} {Reflux}},
	volume = {198},
	issn = {1097-6833},
	doi = {10.1016/j.jpeds.2018.02.059},
	abstract = {OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation.
STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed. Redo fundoplication was used as a surrogate for surgical failure.
RESULTS: A total of 767 patients (patients with EA 85, those who did not have EA 682) underwent fundoplication during the study period. Median age (months) at primary fundoplication was lower in patients with EA (7.2 vs those who did not have EA 23.3; P {\textless} .001). Redo fundoplication rates between groups were not significantly different (EA 11/85 vs 53/682; P = .14). Median time (months) between primary and redo fundoplication was greater in patients with EA (36.2 vs 11.7; P = .03).
CONCLUSIONS: Contrary to popular belief, the incidence of redo fundoplication was not significantly increased in patients with a history of EA. However, patients with EA underwent fundoplication at younger ages, which may be related to early life-threatening events in these patients. These results inform perioperative counseling, and highlight the importance of sustained surgical follow-up in patients with EA.},
	language = {eng},
	journal = {The Journal of Pediatrics},
	author = {Pellegrino, Samantha A. and King, Sebastian K. and McLeod, Elizabeth and Hawley, Alisa and Brooks, Jo-Anne and Hutson, John M. and Teague, Warwick J.},
	month = jul,
	year = {2018},
	keywords = {Child, Preschool, Esophageal Atresia, Female, Follow-Up Studies, Fundoplication, Gastroesophageal Reflux, Humans, Infant, Laparoscopy, Male, Reoperation, Retrospective Studies, Treatment Outcome, esophageal atresia, fundoplication, gastroesophageal reflux, postoperative complication},
	pages = {60--66},
}

OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation. STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed. Redo fundoplication was used as a surrogate for surgical failure. RESULTS: A total of 767 patients (patients with EA 85, those who did not have EA 682) underwent fundoplication during the study period. Median age (months) at primary fundoplication was lower in patients with EA (7.2 vs those who did not have EA 23.3; P \textless .001). Redo fundoplication rates between groups were not significantly different (EA 11/85 vs 53/682; P = .14). Median time (months) between primary and redo fundoplication was greater in patients with EA (36.2 vs 11.7; P = .03). CONCLUSIONS: Contrary to popular belief, the incidence of redo fundoplication was not significantly increased in patients with a history of EA. However, patients with EA underwent fundoplication at younger ages, which may be related to early life-threatening events in these patients. These results inform perioperative counseling, and highlight the importance of sustained surgical follow-up in patients with EA.

@article{fayoux_laryngotracheal_2018,
	title = {Laryngotracheal anomalies associated with esophageal atresia: importance of early diagnosis},
	volume = {275},
	issn = {1434-4726},
	shorttitle = {Laryngotracheal anomalies associated with esophageal atresia},
	url = {https://pubmed.ncbi.nlm.nih.gov/29299746/},
	doi = {10.1007/s00405-017-4856-5},
	abstract = {OBJECTIVE: Esophageal atresia (EA) is the most common congenital esophageal malformation. Airway pathology, in particular, tracheomalacia and laryngotracheal anomalies is a major cause of morbidity and mortalilty in patients with EA. The aim of this study was to report the incidence and type of laryngotracheal anomalies seen in a large series of patients with EA, and to evaluate their impact on the management of children with EA.
STUDY DESIGN: Retrospective study.
MATERIALS AND METHODS: Retrospective cohort including all patients referred to the EA National Reference Center from January 2002 to December 2014. Airway assessment was based on endoscopy performed before, during and/or after esophageal surgery.
RESULTS: One-hundred and fifty-eight patients were included in the study. Endoscopy revealed tracheomalacia in 141 cases (89.2\%) and other laryngotracheal anomalies in 43 patients (27.2\%). Ninety-six patients (60.7\%) presented with persistent respiratory symptoms, including acute life-threatening events in 21 cases, leading to death in 6 cases. A correlation was observed between degree of tracheal collapse and presence of acute life-threatening events. Laryngotracheal surgery was required in 35 cases (22\%).
CONCLUSION: Laryngotracheal anomalies are frequently associated with EA and represent an important etiology of morbidity and mortality that can be prevented by early and systematic diagnosis and aggressive management. An early systematic endoscopic evaluation is recommended to coordinate the airway management with the EA surgery.},
	language = {eng},
	number = {2},
	journal = {European archives of oto-rhino-laryngology: official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS): affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery},
	author = {Fayoux, Pierre and Morisse, Martin and Sfeir, Rony and Michaud, Laurent and Daniel, Sam},
	month = feb,
	year = {2018},
	keywords = {Abnormalities, Multiple, Early Diagnosis, Endoscopy, Esophageal Atresia, Esophageal atresia, Esophagoscopy, Female, Humans, Infant, Infant, Newborn, Laryngeal cleft, Laryngotracheal anomalies, Larynx, Male, Retrospective Studies, Surgery, Trachea, Tracheomalacia},
	pages = {477--481},
}

OBJECTIVE: Esophageal atresia (EA) is the most common congenital esophageal malformation. Airway pathology, in particular, tracheomalacia and laryngotracheal anomalies is a major cause of morbidity and mortalilty in patients with EA. The aim of this study was to report the incidence and type of laryngotracheal anomalies seen in a large series of patients with EA, and to evaluate their impact on the management of children with EA. STUDY DESIGN: Retrospective study. MATERIALS AND METHODS: Retrospective cohort including all patients referred to the EA National Reference Center from January 2002 to December 2014. Airway assessment was based on endoscopy performed before, during and/or after esophageal surgery. RESULTS: One-hundred and fifty-eight patients were included in the study. Endoscopy revealed tracheomalacia in 141 cases (89.2%) and other laryngotracheal anomalies in 43 patients (27.2%). Ninety-six patients (60.7%) presented with persistent respiratory symptoms, including acute life-threatening events in 21 cases, leading to death in 6 cases. A correlation was observed between degree of tracheal collapse and presence of acute life-threatening events. Laryngotracheal surgery was required in 35 cases (22%). CONCLUSION: Laryngotracheal anomalies are frequently associated with EA and represent an important etiology of morbidity and mortality that can be prevented by early and systematic diagnosis and aggressive management. An early systematic endoscopic evaluation is recommended to coordinate the airway management with the EA surgery.

@article{yousef_radiation_2018,
	title = {Radiation {Exposure} and {Attributable} {Cancer} {Risk} in {Patients} {With} {Esophageal} {Atresia}},
	volume = {66},
	issn = {1536-4801},
	doi = {10.1097/MPG.0000000000001701},
	abstract = {OBJECTIVES: Cases of esophageal carcinoma have been documented in survivors of esophageal atresia (EA). Children with EA undergo considerable amounts of diagnostic imaging and consequent radiation exposure potentially increasing their lifetime cancer mortality risk. This study evaluates the radiological procedures performed on patients with EA and estimates their cumulative radiation exposure and attributable lifetime cancer mortality risk.
METHODS: Medical records of patients with EA managed at a tertiary care center were reviewed for demographics, EA subtype, and number and type of radiological investigations. Existing normative data were used to estimate the cumulative radiation exposure and lifetime cancer risk per patient.
RESULTS: The present study included 53 patients with a mean follow-up of 5.7 years. The overall median and maximum estimated effective radiation dose in the neonatal period was 5521.4 μSv/patient and 66638.6 μSv/patient, respectively. This correlates to a median and maximum estimated cumulative lifetime cancer mortality risk of 1:1530 and 1:130, respectively. Hence, radiation exposure in the neonatal period increased the cumulative cancer mortality risk a median of 130-fold and a maximum of 1575-fold in EA survivors.
CONCLUSIONS: Children with EA are exposed to significant amounts of radiation and an increased estimated cumulative cancer mortality risk. Efforts should be made to eliminate superfluous imaging.},
	language = {eng},
	number = {2},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Yousef, Yasmine and Baird, Robert},
	month = feb,
	year = {2018},
	keywords = {Esophageal Atresia, Esophageal Neoplasms, Female, Humans, Infant, Newborn, Male, Radiation Dosage, Radiation Exposure, Retrospective Studies, Risk Assessment, Risk Factors},
	pages = {234--238},
}

OBJECTIVES: Cases of esophageal carcinoma have been documented in survivors of esophageal atresia (EA). Children with EA undergo considerable amounts of diagnostic imaging and consequent radiation exposure potentially increasing their lifetime cancer mortality risk. This study evaluates the radiological procedures performed on patients with EA and estimates their cumulative radiation exposure and attributable lifetime cancer mortality risk. METHODS: Medical records of patients with EA managed at a tertiary care center were reviewed for demographics, EA subtype, and number and type of radiological investigations. Existing normative data were used to estimate the cumulative radiation exposure and lifetime cancer risk per patient. RESULTS: The present study included 53 patients with a mean follow-up of 5.7 years. The overall median and maximum estimated effective radiation dose in the neonatal period was 5521.4 μSv/patient and 66638.6 μSv/patient, respectively. This correlates to a median and maximum estimated cumulative lifetime cancer mortality risk of 1:1530 and 1:130, respectively. Hence, radiation exposure in the neonatal period increased the cumulative cancer mortality risk a median of 130-fold and a maximum of 1575-fold in EA survivors. CONCLUSIONS: Children with EA are exposed to significant amounts of radiation and an increased estimated cumulative cancer mortality risk. Efforts should be made to eliminate superfluous imaging.

@article{gottrand_can_2018,
	title = {Can {Patients} {With} {Neonatal} {Digestive} {Diseases} {Be} {Protected} {From} {Unnecessary} {Radiation}?},
	volume = {66},
	issn = {1536-4801},
	url = {https://pubmed.ncbi.nlm.nih.gov/28937542/},
	doi = {10.1097/MPG.0000000000001751},
	language = {eng},
	number = {2},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Gottrand, Frédéric and Avni, Freddy},
	month = feb,
	year = {2018},
	keywords = {Digestion, Digestive System Diseases, Esophageal Atresia, Humans, Infant, Newborn, Neoplasms, Radiation Exposure},
	pages = {187},
}

@article{vergouwe_longitudinal_2017,
	title = {Longitudinal evaluation of growth in oesophageal atresia patients up to 12 years},
	volume = {102},
	issn = {1468-2052},
	doi = {10.1136/archdischild-2016-311598},
	abstract = {OBJECTIVE: Previous studies reported diminished growth after oesophageal atresia (OA) repair. We evaluated long-term follow-up data on growth.
METHODS: Longitudinal cohort study up to 12 years. Patients with OA, born 1999-2013, who participated in a longitudinal follow-up programme were included. Children with genetic syndromes associated with growth disorders were excluded. SD scores (SDS) for height-for-age (HFA), weight-for-height (WFH) and distance-to-target-height were calculated for routine visits (0.5/1/2/5/8/12 years). Linear mixed models were used to estimate SDS until 12 years of age and to evaluate explanatory factors for growth.
RESULTS: We included 126/155 children (32\% prematurely born, 20\% small for gestational age), 32 reached the age of 12 years. Fundoplication surgery was performed in 24\%. SDS-HFA was below normal up to 8 years but improved over these years (mean (SE) -0.48 (0.09), -0.31 (0.09) and -0.20 (0.13) at 0.5, 8 and 12 years). Scores improved after correction for target height (mean (SE) -0.29 (0.10), -0.17 (0.09) and -0.10 (0.14) at 0.5, 8 and 12 years). SDS-WFH was below normal from age 1-5 years (mean (SE) -0.53 (0.09), -0.24 (0.09) and 0.03 (0.14) at 1, 5 and 12 years). Low birth weight and fundoplication surgery were negatively associated with growth.
CONCLUSIONS: The growth of patients with OA was below the reference norm during the first years of life, but normalised at 12 years. Large longitudinal cohort studies should evaluate if normal growth persists into adolescence. Early nutritional assessment with timely dietary intervention should be considered especially in those with low birth weight or following fundoplication surgery.},
	language = {eng},
	number = {5},
	journal = {Archives of Disease in Childhood. Fetal and Neonatal Edition},
	author = {Vergouwe, Floor W. T. and Spoel, Marjolein and van Beelen, Nicole W. G. and Gischler, Saskia J. and Wijnen, René M. H. and van Rosmalen, Joost and IJsselstijn, Hanneke},
	month = sep,
	year = {2017},
	keywords = {Body Height, Body Weight, Child, Child Development, Cohort Studies, Esophageal Atresia, Female, Fundoplication, Growth, Humans, Infant, Low Birth Weight, Infant, Newborn, Linear Models, Longitudinal Studies, Male, Malnutrition, Netherlands, Oesophageal atresia, Outcome, Reference Values, Risk factors},
	pages = {F417--F422},
}

OBJECTIVE: Previous studies reported diminished growth after oesophageal atresia (OA) repair. We evaluated long-term follow-up data on growth. METHODS: Longitudinal cohort study up to 12 years. Patients with OA, born 1999-2013, who participated in a longitudinal follow-up programme were included. Children with genetic syndromes associated with growth disorders were excluded. SD scores (SDS) for height-for-age (HFA), weight-for-height (WFH) and distance-to-target-height were calculated for routine visits (0.5/1/2/5/8/12 years). Linear mixed models were used to estimate SDS until 12 years of age and to evaluate explanatory factors for growth. RESULTS: We included 126/155 children (32% prematurely born, 20% small for gestational age), 32 reached the age of 12 years. Fundoplication surgery was performed in 24%. SDS-HFA was below normal up to 8 years but improved over these years (mean (SE) -0.48 (0.09), -0.31 (0.09) and -0.20 (0.13) at 0.5, 8 and 12 years). Scores improved after correction for target height (mean (SE) -0.29 (0.10), -0.17 (0.09) and -0.10 (0.14) at 0.5, 8 and 12 years). SDS-WFH was below normal from age 1-5 years (mean (SE) -0.53 (0.09), -0.24 (0.09) and 0.03 (0.14) at 1, 5 and 12 years). Low birth weight and fundoplication surgery were negatively associated with growth. CONCLUSIONS: The growth of patients with OA was below the reference norm during the first years of life, but normalised at 12 years. Large longitudinal cohort studies should evaluate if normal growth persists into adolescence. Early nutritional assessment with timely dietary intervention should be considered especially in those with low birth weight or following fundoplication surgery.

@article{van_der_zee_position_2017,
	title = {Position {Paper} of {INoEA} {Working} {Group} on {Long}-{Gap} {Esophageal} {Atresia}: {For} {Better} {Care}},
	volume = {5},
	issn = {2296-2360},
	shorttitle = {Position {Paper} of {INoEA} {Working} {Group} on {Long}-{Gap} {Esophageal} {Atresia}},
	url = {https://pubmed.ncbi.nlm.nih.gov/28409148/},
	doi = {10.3389/fped.2017.00063},
	abstract = {INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF). LGEA is considerably more complex than EA with distal TEFs and should be referred to a center of expertise. The first choice is to preserve the native esophagus and pursue primary repair, delayed primary anastomosis, or traction/growth techniques to achieve anastomosis. A cervical esophagostomy should be avoided if possible. Only if primary anastomosis is not possible, replacement techniques should be used. Jejunal interposition is proposed as the best option among the major EA centers. In light of the infrequent occurrence of LGEA and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients.},
	language = {eng},
	journal = {Frontiers in Pediatrics},
	author = {van der Zee, David C. and Bagolan, Pietro and Faure, Christophe and Gottrand, Frederic and Jennings, Russell and Laberge, Jean-Martin and Martinez Ferro, Marcela Hernan and Parmentier, Benoît and Sfeir, Rony and Teague, Warwick},
	year = {2017},
	keywords = {centers of expertise, definition, diagnosis, long-gap esophageal atresia, management},
	pages = {63},
}

INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF). LGEA is considerably more complex than EA with distal TEFs and should be referred to a center of expertise. The first choice is to preserve the native esophagus and pursue primary repair, delayed primary anastomosis, or traction/growth techniques to achieve anastomosis. A cervical esophagostomy should be avoided if possible. Only if primary anastomosis is not possible, replacement techniques should be used. Jejunal interposition is proposed as the best option among the major EA centers. In light of the infrequent occurrence of LGEA and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients.

@article{toussaint-duyster_determinants_2017,
	title = {Determinants of exercise capacity in school-aged esophageal atresia patients},
	volume = {52},
	issn = {1099-0496},
	doi = {10.1002/ppul.23687},
	abstract = {BACKGROUND AND AIMS: Data on long-term outcome of exercise capacity in school-aged children with esophageal atresia (EA) are scarce. We evaluated maximal exercise capacity and its relation to lung function. Moreover, we studied other possible determinants of exercise capacity and lung function.
METHODS: Exercise capacity of 63 children with EA born 1999-2007 was evaluated at the age of 8 years with the Bruce-protocol. Dynamic and static lung volumes, bronchodilator response and diffusion capacity were measured. Furthermore, perinatal characteristics, hospital admissions for lower respiratory tract infections (RTIs), RTIs treated with antibiotics in the past year, symptoms of gastroesophageal reflux, weight-for-height, and sports participation were evaluated as other potential determinants.
RESULTS: Exercise capacity was significantly below normal: mean (SD) SDS -0.91 (0.97); P {\textless} 0.001. All spirometric parameters were significantly below normal with significant reversibility of airflow obstruction in 13.5\% of patients. Static lung volumes were significantly decreased (mean (SD) SDS TLChe -1.06 (1.29); P {\textless} 0.001). Diffusion capacity corrected for alveolar volume was normal (mean (SD) SDS KCO -0.12 (1.04)). Exercise capacity was positively associated with total lung capacity and negatively with SDS weight-for-height. Spirometric parameters were negatively associated with congenital cardiac malformation, duration of ventilation, and persistent respiratory morbidity.
CONCLUSION: Eight-year-old children with EA had reduced exercise capacity which was only associated with the reduction in TLChe and higher SDS weight-for-height. We speculate that diminished physical activity with recurrent respiratory tract infections may also play a role in reduced exercise capacity. This should be subject to further research to optimize appropriate intervention.},
	language = {eng},
	number = {9},
	journal = {Pediatric Pulmonology},
	author = {Toussaint-Duyster, Leontien C. C. and van der Cammen-van Zijp, Monique H. M. and Spoel, Marjolein and Lam, Mhanfei and Wijnen, Rene M. H. and de Jongste, Johan C. and Tibboel, Dick and van Rosmalen, Joost and IJsselstijn, Hanneke},
	month = sep,
	year = {2017},
	keywords = {Anti-Bacterial Agents, Bronchodilator Agents, Child, Esophageal Atresia, Exercise, Exercise Test, Female, Gastroesophageal Reflux, Humans, Lung, Male, Respiratory Tract Infections, Spirometry, Total Lung Capacity, congenital malformations, esophageal atresia, exercise capacity, pulmonary function testing, respiratory tract infections, school-aged children},
	pages = {1198--1205},
}

BACKGROUND AND AIMS: Data on long-term outcome of exercise capacity in school-aged children with esophageal atresia (EA) are scarce. We evaluated maximal exercise capacity and its relation to lung function. Moreover, we studied other possible determinants of exercise capacity and lung function. METHODS: Exercise capacity of 63 children with EA born 1999-2007 was evaluated at the age of 8 years with the Bruce-protocol. Dynamic and static lung volumes, bronchodilator response and diffusion capacity were measured. Furthermore, perinatal characteristics, hospital admissions for lower respiratory tract infections (RTIs), RTIs treated with antibiotics in the past year, symptoms of gastroesophageal reflux, weight-for-height, and sports participation were evaluated as other potential determinants. RESULTS: Exercise capacity was significantly below normal: mean (SD) SDS -0.91 (0.97); P \textless 0.001. All spirometric parameters were significantly below normal with significant reversibility of airflow obstruction in 13.5% of patients. Static lung volumes were significantly decreased (mean (SD) SDS TLChe -1.06 (1.29); P \textless 0.001). Diffusion capacity corrected for alveolar volume was normal (mean (SD) SDS KCO -0.12 (1.04)). Exercise capacity was positively associated with total lung capacity and negatively with SDS weight-for-height. Spirometric parameters were negatively associated with congenital cardiac malformation, duration of ventilation, and persistent respiratory morbidity. CONCLUSION: Eight-year-old children with EA had reduced exercise capacity which was only associated with the reduction in TLChe and higher SDS weight-for-height. We speculate that diminished physical activity with recurrent respiratory tract infections may also play a role in reduced exercise capacity. This should be subject to further research to optimize appropriate intervention.

@article{weusten_endoscopic_2017,
	title = {Endoscopic management of {Barrett}'s esophagus: {European} {Society} of {Gastrointestinal} {Endoscopy} ({ESGE}) {Position} {Statement}},
	volume = {49},
	issn = {1438-8812},
	shorttitle = {Endoscopic management of {Barrett}'s esophagus},
	doi = {10.1055/s-0042-122140},
	abstract = {Current practices for the management of Barrett's esophagus (BE) vary across Europe, as several national European guidelines exist. This Position Statement from the European Society of Gastrointestinal Endoscopy (ESGE) is an attempt to homogenize recommendations and, hence, patient management according to the best scientific evidence and other considerations (e.g. health policy). A Working Group developed consensus statements, using the existing national guidelines as a starting point and considering new evidence in the literature. The Position Statement wishes to contribute to a more cost-effective approach to the care of patients with BE by reducing the number of surveillance endoscopies for patients with a low risk of malignant progression and centralizing care in expert centers for those with high progression rates.Main statements MS1 The diagnosis of BE is made if the distal esophagus is lined with columnar epithelium with a minimum length of 1 cm (tongues or circular) containing specialized intestinal metaplasia at histopathological examination. MS2 The ESGE recommends varying surveillance intervals for different BE lengths. For patients with an irregular Z-line/columnar-lined esophagus of {\textless} 1 cm, no routine biopsies or endoscopic surveillance is advised. For BE ≥ 1 cm and {\textless} 3 cm, BE surveillance should be repeated every 5 years. For BE ≥ 3 cm and {\textless} 10 cm, the interval for endoscopic surveillance should be 3 years. Patients with BE with a maximum extent ≥ 10 cm should be referred to a BE expert center for surveillance endoscopies. Patients with limited life expectancy and advanced age should be discharged from endoscopic surveillance. MS3 The diagnosis of any degree of dysplasia (including "indefinite for dysplasia") in BE requires confirmation by an expert gastrointestinal pathologist. MS4 Patients with visible lesions in BE diagnosed as dysplasia or early cancer should be referred to a BE expert center. All visible abnormalities, regardless of the degree of dysplasia, should be removed by means of endoscopic resection techniques in order to obtain optimal histopathological staging MS5 All patients with a BE ≥ 10 cm, a confirmed diagnosis of low grade dysplasia, high grade dysplasia (HGD), or early cancer should be referred to a BE expert center for surveillance and/or treatment. BE expert centers should meet the following criteria: annual case load of ≥10 new patients undergoing endoscopic treatment for HGD or early carcinoma per BE expert endoscopist; endoscopic and histological care provided by endoscopists and pathologists who have followed additional training; at least 30 supervised endoscopic resection and 30 endoscopic ablation procedures to acquire competence in technical skills, management pathways, and complications; multidisciplinary meetings with gastroenterologists, surgeons, oncologists, and pathologists to discuss patients with Barrett's neoplasia; access to experienced esophageal surgery; and all BE patients registered prospectively in a database.},
	language = {eng},
	number = {2},
	journal = {Endoscopy},
	author = {Weusten, Bas and Bisschops, Raf and Coron, Emanuel and Dinis-Ribeiro, Mário and Dumonceau, Jean-Marc and Esteban, José-Miguel and Hassan, Cesare and Pech, Oliver and Repici, Alessandro and Bergman, Jacques and di Pietro, Massimiliano},
	month = feb,
	year = {2017},
	keywords = {Barrett Esophagus, Consensus, Endoscopy, Gastrointestinal, Europe, Humans, Patient Care Management, Practice Guidelines as Topic, Societies, Medical},
	pages = {191--198},
}

Current practices for the management of Barrett's esophagus (BE) vary across Europe, as several national European guidelines exist. This Position Statement from the European Society of Gastrointestinal Endoscopy (ESGE) is an attempt to homogenize recommendations and, hence, patient management according to the best scientific evidence and other considerations (e.g. health policy). A Working Group developed consensus statements, using the existing national guidelines as a starting point and considering new evidence in the literature. The Position Statement wishes to contribute to a more cost-effective approach to the care of patients with BE by reducing the number of surveillance endoscopies for patients with a low risk of malignant progression and centralizing care in expert centers for those with high progression rates.Main statements MS1 The diagnosis of BE is made if the distal esophagus is lined with columnar epithelium with a minimum length of 1 cm (tongues or circular) containing specialized intestinal metaplasia at histopathological examination. MS2 The ESGE recommends varying surveillance intervals for different BE lengths. For patients with an irregular Z-line/columnar-lined esophagus of \textless 1 cm, no routine biopsies or endoscopic surveillance is advised. For BE ≥ 1 cm and \textless 3 cm, BE surveillance should be repeated every 5 years. For BE ≥ 3 cm and \textless 10 cm, the interval for endoscopic surveillance should be 3 years. Patients with BE with a maximum extent ≥ 10 cm should be referred to a BE expert center for surveillance endoscopies. Patients with limited life expectancy and advanced age should be discharged from endoscopic surveillance. MS3 The diagnosis of any degree of dysplasia (including "indefinite for dysplasia") in BE requires confirmation by an expert gastrointestinal pathologist. MS4 Patients with visible lesions in BE diagnosed as dysplasia or early cancer should be referred to a BE expert center. All visible abnormalities, regardless of the degree of dysplasia, should be removed by means of endoscopic resection techniques in order to obtain optimal histopathological staging MS5 All patients with a BE ≥ 10 cm, a confirmed diagnosis of low grade dysplasia, high grade dysplasia (HGD), or early cancer should be referred to a BE expert center for surveillance and/or treatment. BE expert centers should meet the following criteria: annual case load of ≥10 new patients undergoing endoscopic treatment for HGD or early carcinoma per BE expert endoscopist; endoscopic and histological care provided by endoscopists and pathologists who have followed additional training; at least 30 supervised endoscopic resection and 30 endoscopic ablation procedures to acquire competence in technical skills, management pathways, and complications; multidisciplinary meetings with gastroenterologists, surgeons, oncologists, and pathologists to discuss patients with Barrett's neoplasia; access to experienced esophageal surgery; and all BE patients registered prospectively in a database.

@article{gottrand_motility_2016,
	title = {Motility, digestive and nutritional problems in {Esophageal} {Atresia}},
	volume = {19},
	issn = {1526-0550},
	url = {https://pubmed.ncbi.nlm.nih.gov/26752295/},
	doi = {10.1016/j.prrv.2015.11.005},
	abstract = {Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a rare congenital malformation. Digestive and nutritional problems remain frequent in children with EA both in early infancy and at long-term follow-up. These patients are at major risk of presenting with gastroesophageal reflux and its complications, such as anastomotic strictures. Esophageal dysmotility is constant, and can have important consequences on feeding and nutritional status. Patients with EA need a systematic follow-up with a multidisciplinary team.},
	language = {eng},
	journal = {Paediatric Respiratory Reviews},
	author = {Gottrand, Madeleine and Michaud, Laurent and Sfeir, Rony and Gottrand, Frédéric},
	month = jun,
	year = {2016},
	keywords = {Anastomotic stricture, Children, Constriction, Pathologic, Deglutition Disorders, Diagnostic Imaging, Dysmotility, Dysphagia, Esophageal Atresia, Esophageal Motility Disorders, Esophageal pH Monitoring, Esophagitis, Esophagoscopy, Gastro-Esophageal Reflux, Gastroesophageal Reflux, Growth Disorders, Growth Retardation, Humans, Infant, Infant, Newborn, Nutrition, Tracheoesophageal Fistula},
	pages = {28--33},
}

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a rare congenital malformation. Digestive and nutritional problems remain frequent in children with EA both in early infancy and at long-term follow-up. These patients are at major risk of presenting with gastroesophageal reflux and its complications, such as anastomotic strictures. Esophageal dysmotility is constant, and can have important consequences on feeding and nutritional status. Patients with EA need a systematic follow-up with a multidisciplinary team.

@article{smits_pediatric_2016,
	title = {Pediatric {Achalasia} in the {Netherlands}: {Incidence}, {Clinical} {Course}, and {Quality} of {Life}},
	volume = {169},
	issn = {1097-6833},
	shorttitle = {Pediatric {Achalasia} in the {Netherlands}},
	doi = {10.1016/j.jpeds.2015.10.057},
	abstract = {OBJECTIVE: To assess incidence and clinical course of Dutch patients with achalasia diagnosed before 18 years of age as well as their current symptoms and quality of life (QoL).
STUDY DESIGN: Retrospective medical chart review and a cross-sectional study assessing current clinical status using the Eckardt score and reflux disease questionnaire. General QoL was measured using Kidscreen-52 for patients {\textless}18 years of age or to 36-Item Short Form Health Survey for patients ≥18 years of age.
RESULTS: Between 1990 and 2013, 87 children (mean age 11.4 ± 3.4 years, 60\% male) diagnosed with achalasia in the Netherlands were included. Mean incidence was 0.1/100,000/y (range 0.03-0.21). Initial treatment was pneumodilation (PD) in 68 (79\%) patients and Heller myotomy (HM) in 18 (21\%) patients. Retreatment was required more often after initial PD compared with initial HM (88\% vs 22\%; P {\textless} .0001). More complications of initial treatment occurred after HM compared with PD (55.6\% vs 1.5\%; P {\textless} .0001). Three esophageal perforations were seen after HM (16.7\%), 1 after PD (1.5\%). Sixty-three of 87 (72\%) patients were prospectively contacted. Median Eckardt score was 3 (IQR 2-5), with 32 patients (44.5\%) having positive scores suggesting active disease. Reflux disease questionnaire scores were higher after initial HM vs PD (1.71 [0.96-2.90] vs 0.58 [0-1.56]; P = .005). The 36-Item Short Form Health Survey (n = 52) was lower compared with healthy population norms for 7/8 domains. Kidscreen-52 (n = 20) was similar to population norms.
CONCLUSIONS: Pediatric achalasia is rare and relapse rates are high after initial treatment, especially after pneumodilation, but with more complications after HM. Symptoms often persist into adulthood, without any clinical follow-up. QoL in adulthood was decreased.},
	language = {eng},
	journal = {The Journal of Pediatrics},
	author = {Smits, Marije and van Lennep, Marinde and Vrijlandt, Remy and Benninga, Marc and Oors, Jac and Houwen, Roderick and Kokke, Freddy and van der Zee, David and Escher, Johanne and van den Neucker, Anita and de Meij, Tim and Bodewes, Frank and Schweizer, Joachim and Damen, Gerard and Busch, Olivier and van Wijk, Michiel},
	month = feb,
	year = {2016},
	keywords = {Adolescent, Child, Cross-Sectional Studies, Esophageal Achalasia, Female, Humans, Incidence, Male, Netherlands, Quality of Life, Retrospective Studies},
	pages = {110--115.e3},
}

OBJECTIVE: To assess incidence and clinical course of Dutch patients with achalasia diagnosed before 18 years of age as well as their current symptoms and quality of life (QoL). STUDY DESIGN: Retrospective medical chart review and a cross-sectional study assessing current clinical status using the Eckardt score and reflux disease questionnaire. General QoL was measured using Kidscreen-52 for patients \textless18 years of age or to 36-Item Short Form Health Survey for patients ≥18 years of age. RESULTS: Between 1990 and 2013, 87 children (mean age 11.4 ± 3.4 years, 60% male) diagnosed with achalasia in the Netherlands were included. Mean incidence was 0.1/100,000/y (range 0.03-0.21). Initial treatment was pneumodilation (PD) in 68 (79%) patients and Heller myotomy (HM) in 18 (21%) patients. Retreatment was required more often after initial PD compared with initial HM (88% vs 22%; P \textless .0001). More complications of initial treatment occurred after HM compared with PD (55.6% vs 1.5%; P \textless .0001). Three esophageal perforations were seen after HM (16.7%), 1 after PD (1.5%). Sixty-three of 87 (72%) patients were prospectively contacted. Median Eckardt score was 3 (IQR 2-5), with 32 patients (44.5%) having positive scores suggesting active disease. Reflux disease questionnaire scores were higher after initial HM vs PD (1.71 [0.96-2.90] vs 0.58 [0-1.56]; P = .005). The 36-Item Short Form Health Survey (n = 52) was lower compared with healthy population norms for 7/8 domains. Kidscreen-52 (n = 20) was similar to population norms. CONCLUSIONS: Pediatric achalasia is rare and relapse rates are high after initial treatment, especially after pneumodilation, but with more complications after HM. Symptoms often persist into adulthood, without any clinical follow-up. QoL in adulthood was decreased.

@article{schneider_prevalence_2016,
	title = {Prevalence of {Barrett} {Esophagus} in {Adolescents} and {Young} {Adults} {With} {Esophageal} {Atresia}},
	volume = {264},
	issn = {1528-1140},
	url = {https://pubmed.ncbi.nlm.nih.gov/26720426/},
	doi = {10.1097/SLA.0000000000001540},
	abstract = {OBJECTIVE: To study the prevalence of Barrett esophagus (BE) (gastric and/or intestinal metaplasia) in adolescents treated for esophageal atresia (EA).
SUMMARY OF BACKGROUND DATA: EA patients are at high risk of BE.
METHODS: This multicenter prospective study included EA patients aged 15 to 19 years. All eligible patients were proposed an upper endoscopy with multistaged esophageal biopsies under general anesthesia. Histological suspicion of metaplasia was confirmed centrally.
RESULTS: One hundred twenty patients [mean age, 16.5 years (±1.4)] were included; 70\% had been treated for gastroesophageal reflux disease (GERD) during infancy. At evaluation, 8\% were undernourished, 41\% had received antireflux surgery, and 41\% presented with GERD symptoms, although only 28\% were receiving medical treatment. Esophagitis was found at endoscopy in 34\% and confirmed at histology in 67\%. BE was suspected after endoscopy in 37\% and was confirmed by histology for 43\% of patients (50 gastric and 1 intestinal metaplasia). No endoscopic or histological anomalies were found at the anastomosis site. BE was not significantly related to clinical symptoms. In multivariate analysis, BE was associated with EA without fistula (P = 0.03), previous multiple antireflux surgery (P = 0.04), esophageal dilation (P = 0.04), suspicion of BE at endoscopy (P {\textless} 0.001), and histological esophagitis (P = 0.02).
CONCLUSIONS: Patients with EA are at high risk of persistent GERD and BE. The development of BE is related to GERD history. Long-term systematic follow-up of the esophageal mucosa including multistaged biopsies is required, even in asymptomatic patients. (NCT02495051).},
	language = {eng},
	number = {6},
	journal = {Annals of Surgery},
	author = {Schneider, Anne and Gottrand, Frédéric and Bellaiche, Marc and Becmeur, François and Lachaux, Alain and Bridoux-Henno, Laure and Michel, Jean-Luc and Faure, Christophe and Philippe, Paul and Vandenplas, Yvan and Dupont, Claire and Breton, Anne and Gaudin, Jean and Lamireau, Thierry and Muyshont, Laurence and Podevin, Guillaume and Viola, Sheila and Bertrand, Valérie and Caldari, Dominique and Colinet, Stéphanie and Wanty, Catherine and Sauleau, Erik and Leteurtre, Emmanuelle and Michaud, Laurent},
	month = dec,
	year = {2016},
	keywords = {Adolescent, Barrett Esophagus, Biopsy, Esophageal Atresia, Esophagitis, Esophagoscopy, Female, France, Gastroesophageal Reflux, Humans, Male, Prevalence, Prospective Studies, Young Adult},
	pages = {1004--1008},
}

OBJECTIVE: To study the prevalence of Barrett esophagus (BE) (gastric and/or intestinal metaplasia) in adolescents treated for esophageal atresia (EA). SUMMARY OF BACKGROUND DATA: EA patients are at high risk of BE. METHODS: This multicenter prospective study included EA patients aged 15 to 19 years. All eligible patients were proposed an upper endoscopy with multistaged esophageal biopsies under general anesthesia. Histological suspicion of metaplasia was confirmed centrally. RESULTS: One hundred twenty patients [mean age, 16.5 years (±1.4)] were included; 70% had been treated for gastroesophageal reflux disease (GERD) during infancy. At evaluation, 8% were undernourished, 41% had received antireflux surgery, and 41% presented with GERD symptoms, although only 28% were receiving medical treatment. Esophagitis was found at endoscopy in 34% and confirmed at histology in 67%. BE was suspected after endoscopy in 37% and was confirmed by histology for 43% of patients (50 gastric and 1 intestinal metaplasia). No endoscopic or histological anomalies were found at the anastomosis site. BE was not significantly related to clinical symptoms. In multivariate analysis, BE was associated with EA without fistula (P = 0.03), previous multiple antireflux surgery (P = 0.04), esophageal dilation (P = 0.04), suspicion of BE at endoscopy (P \textless 0.001), and histological esophagitis (P = 0.02). CONCLUSIONS: Patients with EA are at high risk of persistent GERD and BE. The development of BE is related to GERD history. Long-term systematic follow-up of the esophageal mucosa including multistaged biopsies is required, even in asymptomatic patients. (NCT02495051).

@article{hochart_contribution_2015,
	title = {The contribution of fetal {MR} imaging to the assessment of oesophageal atresia},
	volume = {25},
	issn = {1432-1084},
	url = {https://pubmed.ncbi.nlm.nih.gov/25304819/},
	doi = {10.1007/s00330-014-3444-y},
	abstract = {OBJECTIVE: Present challenges are to improve the diagnosis rate of oesophageal atresia (OA) and evaluate as completely as possible a fetus affected by OA, specifically the type of OA and the length of the gap. Our aim was to evaluate the accuracy of fetal MR imaging (fMRI) for diagnosis of OA.
METHODS: We reviewed fMRI performed because of sonographic suspicion of an OA. The signs reviewed included stomach size, "pouch sign", bowing of the trachea and visualization of the lower oesophageal lumen. The fetuses were assigned by consensus as having or not having EA, as well as having a tracheaoesophageal fistula (TOF). All findings were correlated with postnatal data. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated.
RESULTS: Se, Sp, PPV and NPV of the technique were respectively 91\%, 100\%, 100\% and 88\%. The presence of the pouch sign yielded corresponding values of 82\%, 100\%, 100\% and 78\%. Mid-tracheal bowing was correlated positively with EA. The type of atresia was correctly evaluated in 90\% of patients.
CONCLUSION: fMRI is useful for the diagnosis of EA through the visualization of the oesophageal pouch or through associated signs such as tracheal bowing. Visualization of the lower oesophageal lumen seems to be a good sign of TEF.
KEY POINTS: • Challenges are to improve the prenatal diagnosis of EA and associated malformations. • fMRI is able to diagnose EA through demonstration of the pouch sign. • Tracheal bowing is a promising indirect sign of EA. • Tracheoesophageal fistula can also be suspected thanks to fMRI. • Obstetrical US, fMRI and fetal CT are complementary for assessing associated malformations.},
	language = {eng},
	number = {2},
	journal = {European Radiology},
	author = {Hochart, V. and Verpillat, P. and Langlois, C. and Garabedian, C. and Bigot, J. and Debarge, V. Houfflin and Sfeir, R. and Avni, F. E.},
	month = feb,
	year = {2015},
	keywords = {Adult, Esophageal Atresia, Female, Fetal Diseases, Gestational Age, Humans, Magnetic Resonance Imaging, Pregnancy, Prenatal Diagnosis, Retrospective Studies, Sensitivity and Specificity, Stomach, Tracheoesophageal Fistula},
	pages = {306--314},
}

OBJECTIVE: Present challenges are to improve the diagnosis rate of oesophageal atresia (OA) and evaluate as completely as possible a fetus affected by OA, specifically the type of OA and the length of the gap. Our aim was to evaluate the accuracy of fetal MR imaging (fMRI) for diagnosis of OA. METHODS: We reviewed fMRI performed because of sonographic suspicion of an OA. The signs reviewed included stomach size, "pouch sign", bowing of the trachea and visualization of the lower oesophageal lumen. The fetuses were assigned by consensus as having or not having EA, as well as having a tracheaoesophageal fistula (TOF). All findings were correlated with postnatal data. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated. RESULTS: Se, Sp, PPV and NPV of the technique were respectively 91%, 100%, 100% and 88%. The presence of the pouch sign yielded corresponding values of 82%, 100%, 100% and 78%. Mid-tracheal bowing was correlated positively with EA. The type of atresia was correctly evaluated in 90% of patients. CONCLUSION: fMRI is useful for the diagnosis of EA through the visualization of the oesophageal pouch or through associated signs such as tracheal bowing. Visualization of the lower oesophageal lumen seems to be a good sign of TEF. KEY POINTS: • Challenges are to improve the prenatal diagnosis of EA and associated malformations. • fMRI is able to diagnose EA through demonstration of the pouch sign. • Tracheal bowing is a promising indirect sign of EA. • Tracheoesophageal fistula can also be suspected thanks to fMRI. • Obstetrical US, fMRI and fetal CT are complementary for assessing associated malformations.

@article{mccann_congenital_2015,
	title = {Congenital esophageal stenosis associated with esophageal atresia},
	volume = {28},
	issn = {1442-2050},
	url = {https://pubmed.ncbi.nlm.nih.gov/24446921/},
	doi = {10.1111/dote.12176},
	abstract = {Congenital esophageal stenosis (CES) is a rare clinical condition but is frequently associated with esophageal atresia (EA). The aim of this study is to report the diagnosis, management, and outcome of CES associated with EA. Medical charts of CES-EA patients from Lille University Hospital, Sainte-Justine Hospital, and Montreal Children's Hospital were retrospectively reviewed. Seventeen patients (13 boys) were included. The incidence of CES in patients with EA was 3.6\%. Fifteen patients had a type C EA, one had a type A EA, and one had an isolated tracheoesophageal fistula. Seven patients had associated additional malformations. The mean age at diagnosis was 11.6 months. All but two patients had non-specific symptoms such as regurgitations or dysphagia. One CES was diagnosed at the time of surgical repair of EA. In 12 patients, CES was suspected based on abnormal barium swallow. In the remaining four, the diagnostic was confirmed by esophagoscopy. Eleven patients were treated by dilation only (1-3 dilations/patient). Six patients underwent surgery (resection and anastomosis) because of failure of attempted dilations (1-7 dilations/patient). Esophageal perforation was encountered in three patients (18\%). Three patients had histologically proven tracheobronchial remnants. CES associated with EA is frequent. A high index of suspicion for CES must remain in the presence of EA. Dilatation may be effective to treat some of them, but perforation is frequent. Surgery may be required, especially in CES secondary to ectopic tracheobronchial remnants.},
	language = {eng},
	number = {3},
	journal = {Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus},
	author = {McCann, F. and Michaud, L. and Aspirot, A. and Levesque, D. and Gottrand, F. and Faure, C.},
	month = apr,
	year = {2015},
	keywords = {Abnormalities, Multiple, Anastomosis, Surgical, Child, Preschool, Deglutition Disorders, Dilatation, Esophageal Atresia, Esophageal Perforation, Esophageal Stenosis, Esophagoscopy, Female, Gastroesophageal Reflux, Humans, Infant, Infant, Newborn, Male, Retrospective Studies, Tracheoesophageal Fistula, children, congenital stenosis, esophageal atresia},
	pages = {211--215},
}

Congenital esophageal stenosis (CES) is a rare clinical condition but is frequently associated with esophageal atresia (EA). The aim of this study is to report the diagnosis, management, and outcome of CES associated with EA. Medical charts of CES-EA patients from Lille University Hospital, Sainte-Justine Hospital, and Montreal Children's Hospital were retrospectively reviewed. Seventeen patients (13 boys) were included. The incidence of CES in patients with EA was 3.6%. Fifteen patients had a type C EA, one had a type A EA, and one had an isolated tracheoesophageal fistula. Seven patients had associated additional malformations. The mean age at diagnosis was 11.6 months. All but two patients had non-specific symptoms such as regurgitations or dysphagia. One CES was diagnosed at the time of surgical repair of EA. In 12 patients, CES was suspected based on abnormal barium swallow. In the remaining four, the diagnostic was confirmed by esophagoscopy. Eleven patients were treated by dilation only (1-3 dilations/patient). Six patients underwent surgery (resection and anastomosis) because of failure of attempted dilations (1-7 dilations/patient). Esophageal perforation was encountered in three patients (18%). Three patients had histologically proven tracheobronchial remnants. CES associated with EA is frequent. A high index of suspicion for CES must remain in the presence of EA. Dilatation may be effective to treat some of them, but perforation is frequent. Surgery may be required, especially in CES secondary to ectopic tracheobronchial remnants.

@article{heuschkel_espghan_2015,
	title = {{ESPGHAN} position paper on management of percutaneous endoscopic gastrostomy in children and adolescents},
	volume = {60},
	issn = {1536-4801},
	url = {https://pubmed.ncbi.nlm.nih.gov/25023584/},
	doi = {10.1097/MPG.0000000000000501},
	abstract = {OBJECTIVES: This European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) position statement provides a comprehensive guide for health care providers to manage percutaneous endoscopic gastrostomy tubes in a safe, effective, and appropriate way.
METHODS: Relevant literature from searches of PubMed, CINAHL, and recent guidelines was reviewed. In the absence of evidence, recommendations reflect the expert opinion of the authors. Final consensus was obtained by multiple e-mail exchange and during 3 face-to-face meetings of the gastroenterology committee of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
RESULTS: Endoscopically placed gastrostomy devices are essential in the management of children with feeding and nutritional problems. The article focuses on practical issues such as indications and contraindications.
CONCLUSIONS: The decision to place an endoscopic gastrostomy has to be made by an appropriate multidisciplinary team, which then provides active follow-up and care for the child and the device.},
	language = {eng},
	number = {1},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Heuschkel, R. B. and Gottrand, F. and Devarajan, K. and Poole, H. and Callan, J. and Dias, J. A. and Karkelis, S. and Papadopoulou, A. and Husby, S. and Ruemmele, F. and Schäppi, M. G. and Wilschanski, M. and Lionetti, P. and Orel, R. and Tovar, J. and Thapar, N. and Vandenplas, Y. and {European Society for Pediatric Gastroenterology, Hepatology, and Nutrition}},
	month = jan,
	year = {2015},
	keywords = {Adolescent, Adolescent Nutritional Physiological Phenomena, Child, Child Nutritional Physiological Phenomena, Enteral Nutrition, Europe, Evidence-Based Medicine, Gastrostomy, Humans, Infant, Infant Nutritional Physiological Phenomena, Interdisciplinary Communication, Postoperative Complications, Societies, Scientific},
	pages = {131--141},
}

OBJECTIVES: This European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) position statement provides a comprehensive guide for health care providers to manage percutaneous endoscopic gastrostomy tubes in a safe, effective, and appropriate way. METHODS: Relevant literature from searches of PubMed, CINAHL, and recent guidelines was reviewed. In the absence of evidence, recommendations reflect the expert opinion of the authors. Final consensus was obtained by multiple e-mail exchange and during 3 face-to-face meetings of the gastroenterology committee of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. RESULTS: Endoscopically placed gastrostomy devices are essential in the management of children with feeding and nutritional problems. The article focuses on practical issues such as indications and contraindications. CONCLUSIONS: The decision to place an endoscopic gastrostomy has to be made by an appropriate multidisciplinary team, which then provides active follow-up and care for the child and the device.

@article{jacob_safety_2015,
	title = {Safety of the {One}-{Step} {Percutaneous} {Endoscopic} {Gastrostomy} {Button} in {Children}},
	volume = {166},
	issn = {1097-6833},
	url = {https://pubmed.ncbi.nlm.nih.gov/25868429/},
	doi = {10.1016/j.jpeds.2015.03.008},
	abstract = {Pediatric experience using the single-stage percutaneous endoscopic gastrostomy button has been reported anecdotally. This 3-year prospective monocentric study, including 183 children, demonstrates that this technique is safe, with a low rate of infection, and compares favorably with the pull technique of percutaneous endoscopic gastrostomy while necessitating only one general anesthesia.},
	language = {eng},
	number = {6},
	journal = {The Journal of Pediatrics},
	author = {Jacob, Anne and Delesalle, Dorothée and Coopman, Stéphanie and Bridenne, Marie and Guimber, Dominique and Turck, Dominique and Gottrand, Frédéric and Michaud, Laurent},
	month = jun,
	year = {2015},
	keywords = {Adolescent, Child, Child, Preschool, Costs and Cost Analysis, Feasibility Studies, Female, Gastroscopy, Gastrostomy, Humans, Infant, Male, Postoperative Complications, Prospective Studies},
	pages = {1526--1528},
}

Pediatric experience using the single-stage percutaneous endoscopic gastrostomy button has been reported anecdotally. This 3-year prospective monocentric study, including 183 children, demonstrates that this technique is safe, with a low rate of infection, and compares favorably with the pull technique of percutaneous endoscopic gastrostomy while necessitating only one general anesthesia.

@article{poghosyan_circumferential_2015,
	title = {Circumferential esophageal replacement using a tube-shaped tissue-engineered substitute: {An} experimental study in minipigs},
	volume = {158},
	issn = {1532-7361},
	shorttitle = {Circumferential esophageal replacement using a tube-shaped tissue-engineered substitute},
	url = {http://hdl.handle.net/20.500.12210/4703},
	doi = {10.1016/j.surg.2015.01.020},
	abstract = {BACKGROUND: Esophageal replacement by the colon or the stomach for malignant and nonmalignant esophageal diseases exposes to significant morbidity and mortality. In this setting, tissue engineering seems to be a seductive alternative.
METHODS: In a porcine model, we performed a 5-cm-long circumferential replacement of the cervical esophagus by a tubulized acellular matrix (small intestinal submucosa) cellularized with autologous skeletal myoblasts and covered by a human amniotic membrane seeded with autologous oral epithelial cells. The substitute was grown for 2 weeks in the great omentum before esophageal replacement. Eighteen minipigs (divided into 3 groups: group A [substitute with esophageal endoprothesis; n = 6], group B [substitute alone; n = 6], and group C [endoprothesis alone; n = 6]) were included. The esophageal endoprothesis was removed at 6 months. Animals were killed sequentially over a 12 month-period. Clinical, endoscopic, radiologic and histologic outcomes were analyzed.
RESULTS: All animals except 1 of in groups B and C died during the first 2 months owing to refractory esophageal stenosis or endoprothesis extrusion. Nutritional autonomy without endoprothesis was observed in all animals of group A with a follow-up of {\textgreater}6 months (n = 3). A phenotype similar to that of native esophagus, consisting of a mature epithelium, submucosal glands, and a circular muscular layer, was observed after 9 months.
CONCLUSION: In this model, the circumferential replacement of the cervical esophagus by a tube-shaped tissue-engineered substitute under the temporary cover of an esophageal endoprothesis allowed nutritional autonomy and tissue remodeling toward an esophageal phenotype.},
	language = {eng},
	number = {1},
	journal = {Surgery},
	author = {Poghosyan, Tigran and Sfeir, Rony and Michaud, Laurent and Bruneval, Patrick and Domet, Thomas and Vanneaux, Valerie and Luong-Nguyen, Minh and Gaujoux, Sebastien and Gottrand, Frederic and Larghero, Jerome and Cattan, Pierre},
	month = jul,
	year = {2015},
	keywords = {Animals, Artificial Organs, Esophagus, Intestine, Small, Male, Models, Animal, Swine, Swine, Miniature, Tissue Engineering, Tissue Scaffolds},
	pages = {266--277},
}

BACKGROUND: Esophageal replacement by the colon or the stomach for malignant and nonmalignant esophageal diseases exposes to significant morbidity and mortality. In this setting, tissue engineering seems to be a seductive alternative. METHODS: In a porcine model, we performed a 5-cm-long circumferential replacement of the cervical esophagus by a tubulized acellular matrix (small intestinal submucosa) cellularized with autologous skeletal myoblasts and covered by a human amniotic membrane seeded with autologous oral epithelial cells. The substitute was grown for 2 weeks in the great omentum before esophageal replacement. Eighteen minipigs (divided into 3 groups: group A [substitute with esophageal endoprothesis; n = 6], group B [substitute alone; n = 6], and group C [endoprothesis alone; n = 6]) were included. The esophageal endoprothesis was removed at 6 months. Animals were killed sequentially over a 12 month-period. Clinical, endoscopic, radiologic and histologic outcomes were analyzed. RESULTS: All animals except 1 of in groups B and C died during the first 2 months owing to refractory esophageal stenosis or endoprothesis extrusion. Nutritional autonomy without endoprothesis was observed in all animals of group A with a follow-up of \textgreater6 months (n = 3). A phenotype similar to that of native esophagus, consisting of a mature epithelium, submucosal glands, and a circular muscular layer, was observed after 9 months. CONCLUSION: In this model, the circumferential replacement of the cervical esophagus by a tube-shaped tissue-engineered substitute under the temporary cover of an esophageal endoprothesis allowed nutritional autonomy and tissue remodeling toward an esophageal phenotype.

@article{garabedian_does_2015,
	title = {Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia?},
	volume = {212},
	issn = {1097-6868},
	url = {https://pubmed.ncbi.nlm.nih.gov/25265404/},
	doi = {10.1016/j.ajog.2014.09.030},
	abstract = {OBJECTIVE: Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA.
STUDY DESIGN: We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year.
RESULTS: Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3\%; 82.2\% of EA type I were diagnosed prenatally compared with 17.9\% of EA type III (P {\textless} .001). Transfer after birth was lower in case of prenatal diagnosis (25.6\% vs 82.5\%; P {\textless} .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P {\textless} .001). The composite variables were higher in prenatal diagnosis subset (44\% vs 27.6\%; P = .003) and in EA type I than in type III (58.1\% vs 28.3\%; P {\textless} .001).
CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers.},
	language = {eng},
	number = {3},
	journal = {American Journal of Obstetrics and Gynecology},
	author = {Garabedian, Charles and Sfeir, Rony and Langlois, Carole and Bonnard, Arnaud and Khen-Dunlop, Naziha and Gelas, Thomas and Michaud, Laurent and Auber, Fréderic and Gottrand, Fréderic and Houfflin-Debarge, Véronique and {French Network on Esophageal Atresia}},
	month = mar,
	year = {2015},
	keywords = {Combined Modality Therapy, Esophageal Atresia, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Pregnancy, Prenatal Diagnosis, Registries, Survival Analysis, Treatment Outcome, associated anomaly, esophageal atresia, outcome, postnatal diagnosis, prenatal diagnosis},
	pages = {340.e1--7},
}

OBJECTIVE: Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA. STUDY DESIGN: We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year. RESULTS: Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P \textless .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P \textless .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P \textless .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P \textless .001). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers.

@article{garabedian_esophageal_2014,
	title = {[{Esophageal} atresia: prevalence, prenatal diagnosis and prognosis]},
	volume = {43},
	issn = {1773-0430},
	shorttitle = {[{Esophageal} atresia},
	doi = {10.1016/j.jgyn.2013.11.014},
	abstract = {Esophageal atresia (EA) is a rare congenital malformation (1 in 2,500 to 3,500 births). Prenatal diagnosis (PN) is particularly interesting allowing search for associated malformations related to worse prognosis forms (reference ultrasound, MRI and amniocentesis) and planning the birth in an adapted medico-surgical center. Diagnosis of EA is usually suspected because of indirect and non-specific signs: association of polyhydramnios and absent or small stomach bubble. The visualization in ultrasound or MRI of cervical or thoracic fluid image corresponding to the expansion of the bottom of upper esophageal ("pouch sign") increases the specificity of diagnosis. However, prenatal diagnosis remains difficult and less than 50 \% of EA are diagnosed prenatally. Biochemical analysis could improve these results. If EA is confirmed at birth, surgical management consists in a primary end-to-end anastomosis in first days of life, or in two-steps surgery if the defect is too large. Although current prognosis of EA is good, frequency of surgical complications and esophageal lesions secondary to gastroesophageal reflux justify a systematic and multidisciplinary extended follow-up.},
	language = {fre},
	number = {6},
	journal = {Journal De Gynecologie, Obstetrique Et Biologie De La Reproduction},
	author = {Garabedian, C. and Vaast, P. and Bigot, J. and Sfeir, R. and Michaud, L. and Gottrand, F. and Verpillat, P. and Coulon, C. and Subtil, D. and Houfflin Debarge, V.},
	month = jun,
	year = {2014},
	keywords = {Analyse biochimique amniotique, Atrésie de l’œsophage, Biochemical amniotic fluid, Diagnostic anténatal, Esophageal Atresia, Esophageal atresia, Female, Fetal Diseases, Fetal ultrasound, Humans, IRM, MRI, Pregnancy, Prenatal Diagnosis, Prenatal diagnosis, Prognosis, Échographie fœtale},
	pages = {424--430},
}

Esophageal atresia (EA) is a rare congenital malformation (1 in 2,500 to 3,500 births). Prenatal diagnosis (PN) is particularly interesting allowing search for associated malformations related to worse prognosis forms (reference ultrasound, MRI and amniocentesis) and planning the birth in an adapted medico-surgical center. Diagnosis of EA is usually suspected because of indirect and non-specific signs: association of polyhydramnios and absent or small stomach bubble. The visualization in ultrasound or MRI of cervical or thoracic fluid image corresponding to the expansion of the bottom of upper esophageal ("pouch sign") increases the specificity of diagnosis. However, prenatal diagnosis remains difficult and less than 50 % of EA are diagnosed prenatally. Biochemical analysis could improve these results. If EA is confirmed at birth, surgical management consists in a primary end-to-end anastomosis in first days of life, or in two-steps surgery if the defect is too large. Although current prognosis of EA is good, frequency of surgical complications and esophageal lesions secondary to gastroesophageal reflux justify a systematic and multidisciplinary extended follow-up.

@article{garabedian_does_2014,
	title = {Does a combination of ultrasound, {MRI}, and biochemical amniotic fluid analysis improve prenatal diagnosis of esophageal atresia?},
	volume = {34},
	issn = {1097-0223},
	url = {https://pubmed.ncbi.nlm.nih.gov/24706336/},
	doi = {10.1002/pd.4376},
	abstract = {OBJECTIVE: Prenatal diagnosis of esophageal atresia (EA) remains a challenge. Our objective was to evaluate the combination of sonography, magnetic resonance imaging (MRI), and amniotic fluid biochemical markers in prenatal diagnosis of EA.
STUDY DESIGN: A retrospective study of all cases with prenatal suspicion of EA from January 2008 to May 2013 in our regional reference center was carried out. Patients were included if all the three tests were performed. For each test, sensitivity (Se), specificity (Sp), positive predictive value (PPV), and negative predictive value (NPV) were evaluated. Each test was compared using Fisher's exact test.
RESULTS: Fifteen patients were referred at a median gestational age of 28(+5)  weeks (24-36) for suspicion of EA on the basis of small or non-visualized fetal stomach bubble and/or polyhydramnios. Se, Sp, PPV, and NPV for sonographic pouch sign/MRI/biochemical amniotic fluid were respectively 40/100/100/45.5\%, 80/100/100/71.4\%, and 90/60/81.8/75\%. MRI was the best predictive test (p = 0.007).
CONCLUSION: In case of ultrasound prenatal suspicion of EA (with or without visualization of the pouch sign), an MRI at 30-32 weeks using fast imaging employing steady-state acquisition should be proposed. Biochemical amniotic fluid may be helpful and should be evaluated in a larger study.},
	language = {eng},
	number = {9},
	journal = {Prenatal Diagnosis},
	author = {Garabedian, C. and Verpillat, P. and Czerkiewicz, I. and Langlois, C. and Muller, F. and Avni, F. and Bigot, J. and Sfeir, R. and Vaast, P. and Coulon, C. and Subtil, D. and Houfflin-Debarge, V.},
	month = sep,
	year = {2014},
	keywords = {Amniocentesis, Amniotic Fluid, Biomarkers, Esophageal Atresia, Female, Humans, Magnetic Resonance Imaging, Predictive Value of Tests, Pregnancy, Pregnancy Trimester, Second, Pregnancy Trimester, Third, Retrospective Studies, Sensitivity and Specificity, Ultrasonography, Prenatal},
	pages = {839--842},
}

OBJECTIVE: Prenatal diagnosis of esophageal atresia (EA) remains a challenge. Our objective was to evaluate the combination of sonography, magnetic resonance imaging (MRI), and amniotic fluid biochemical markers in prenatal diagnosis of EA. STUDY DESIGN: A retrospective study of all cases with prenatal suspicion of EA from January 2008 to May 2013 in our regional reference center was carried out. Patients were included if all the three tests were performed. For each test, sensitivity (Se), specificity (Sp), positive predictive value (PPV), and negative predictive value (NPV) were evaluated. Each test was compared using Fisher's exact test. RESULTS: Fifteen patients were referred at a median gestational age of 28(+5)  weeks (24-36) for suspicion of EA on the basis of small or non-visualized fetal stomach bubble and/or polyhydramnios. Se, Sp, PPV, and NPV for sonographic pouch sign/MRI/biochemical amniotic fluid were respectively 40/100/100/45.5%, 80/100/100/71.4%, and 90/60/81.8/75%. MRI was the best predictive test (p = 0.007). CONCLUSION: In case of ultrasound prenatal suspicion of EA (with or without visualization of the pouch sign), an MRI at 30-32 weeks using fast imaging employing steady-state acquisition should be proposed. Biochemical amniotic fluid may be helpful and should be evaluated in a larger study.

@article{schneider_results_2014,
	title = {Results from the {French} {National} {Esophageal} {Atresia} register: one-year outcome},
	volume = {9},
	issn = {1750-1172},
	shorttitle = {Results from the {French} {National} {Esophageal} {Atresia} register},
	url = {https://pubmed.ncbi.nlm.nih.gov/25496976/},
	doi = {10.1186/s13023-014-0206-5},
	abstract = {BACKGROUND: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA).
METHODS: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database.
RESULTS: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98\%) patients, of whom 4\% were lost to follow-up and 5\% died. Medical complications occurred in 34\% of the patients: anastomotic leaks (8\%), recurrent tracheoesophageal fistula (4\%), and anastomotic stenosis (22\%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59\% of patients (2.5 hospitalizations/patient) for digestive (52\%) or respiratory (48\%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1\% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37\% presented with respiratory symptoms and 15\% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007).
CONCLUSIONS: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.},
	language = {eng},
	journal = {Orphanet Journal of Rare Diseases},
	author = {Schneider, Anne and Blanc, Sébastien and Bonnard, Arnaud and Khen-Dunlop, Naziha and Auber, Frédéric and Breton, Anne and Podevin, Guillaume and Sfeir, Rony and Fouquet, Virginie and Jacquier, Catherine and Lemelle, Jean-Louis and Lavrand, Frédéric and Becmeur, François and Petit, Thierry and Poli-Merol, Marie-Laurence and Elbaz, Frédéric and Merrot, Thierry and Michel, Jean-Luc and Hossein, Allal and Lopez, Manuel and Habonimana, Edouard and Pelatan, Cécile and De Lagausie, Pascal and Buisson, Philippe and de Vries, Philine and Gaudin, Jean and Lardy, Hubert and Borderon, Corine and Borgnon, Joséphine and Jaby, Olivier and Weil, Dominique and Aubert, Didier and Geiss, Stephan and Breaud, Jean and Echaieb, Anis and Languepin, Jane and Laplace, Christophe and Pouzac, Myriam and Lefebvre, François and Gottrand, Frédéric and Michaud, Laurent},
	month = dec,
	year = {2014},
	keywords = {Esophageal Atresia, Female, Follow-Up Studies, France, Hospitalization, Humans, Infant, Infant, Newborn, Male, Population Surveillance, Registries, Time Factors, Treatment Outcome},
	pages = {206},
}

BACKGROUND: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA). METHODS: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database. RESULTS: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007). CONCLUSIONS: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

@article{aite_short-term_2014,
	title = {Short-term neurodevelopmental outcome of babies operated on for low-risk esophageal atresia: a pilot study},
	volume = {27},
	issn = {1442-2050},
	shorttitle = {Short-term neurodevelopmental outcome of babies operated on for low-risk esophageal atresia},
	doi = {10.1111/dote.12114},
	abstract = {Data on the neurodevelopmental outcome of esophageal atresia (EA) survivors are scarce, controversial, and based on small samples. This is an observational prospective longitudinal study on a selected cohort of low-risk EA survivors. We considered a low-risk EA survivor a patient with the following characteristics: gestational age {\textgreater}32 weeks, no long gap, no genetic or chromosomic anomaly associated with neurodevelopmental delay, and no further major surgical congenital anomalies. Infants were evaluated with scales derived from the Bayley Scales of Infant and Toddler Development - 3rd Edition at 6 and 12 months, with a score of 100 considered normal for each scale. Analysis of variance was used to assess differences of cognitive and motor development. Linear regression was used to assess the impact of the following clinical and sociodemographic variables: gender, birthweight, gestational age, length of hospital stay, number of surgeries and number of esophageal dilatations during first hospitalization, days of mechanical ventilation, weight at follow up, number of surgeries and esophageal dilatations at follow up, parental age, educational level, and socioeconomic status. Thirty children form the object of the study. The mean (standard deviation [SD]) cognitive scale's score was 93.7 (7.5) and 98.2 (9.6) at 6 and 12 months, respectively (P {\textless} 0.05). The mean (SD) motor scale's score was 97.6 (9.3) and 98.0 (12.1) at 6 and 12 months, respectively (P = n.s.). Children with a body weight {\textless}5° percentile at 12 months showed a mean (SD) cognitive score significantly lower when compared with those with a body weight {\textgreater}5° percentile: 88.8 (6.3) and 100.5 (8.9), respectively. At 12 months, children with unemployed mothers had a mean (SD) motor score significantly lower when compared with those in the other socioeconomic classes: 87.7 (9.8) and 100.6 (12.4), respectively. In conclusion, parents of babies operated on for low-risk EA can be reassured about neurodevelopmental outcome at least up to 1 year of age. When offering a multidisciplinary follow-up program, underweight patients should deserve particular attention to promote their quality of life and support their global development.},
	language = {eng},
	number = {4},
	journal = {Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus},
	author = {Aite, L. and Bevilacqua, F. and Zaccara, A. and Ravà, L. and Valfrè, L. and Conforti, A. and Braguglia, A. and Bagolan, P.},
	year = {2014},
	keywords = {Child Development, Cognition, Cohort Studies, Esophageal Atresia, Female, Humans, Infant, Linear Models, Longitudinal Studies, Male, Motor Skills, Pilot Projects, Prospective Studies, Risk Factors, Socioeconomic Factors, Thinness, esophageal atresia, follow up, neurodevelopmental outcome},
	pages = {330--334},
}

Data on the neurodevelopmental outcome of esophageal atresia (EA) survivors are scarce, controversial, and based on small samples. This is an observational prospective longitudinal study on a selected cohort of low-risk EA survivors. We considered a low-risk EA survivor a patient with the following characteristics: gestational age \textgreater32 weeks, no long gap, no genetic or chromosomic anomaly associated with neurodevelopmental delay, and no further major surgical congenital anomalies. Infants were evaluated with scales derived from the Bayley Scales of Infant and Toddler Development - 3rd Edition at 6 and 12 months, with a score of 100 considered normal for each scale. Analysis of variance was used to assess differences of cognitive and motor development. Linear regression was used to assess the impact of the following clinical and sociodemographic variables: gender, birthweight, gestational age, length of hospital stay, number of surgeries and number of esophageal dilatations during first hospitalization, days of mechanical ventilation, weight at follow up, number of surgeries and esophageal dilatations at follow up, parental age, educational level, and socioeconomic status. Thirty children form the object of the study. The mean (standard deviation [SD]) cognitive scale's score was 93.7 (7.5) and 98.2 (9.6) at 6 and 12 months, respectively (P \textless 0.05). The mean (SD) motor scale's score was 97.6 (9.3) and 98.0 (12.1) at 6 and 12 months, respectively (P = n.s.). Children with a body weight \textless5° percentile at 12 months showed a mean (SD) cognitive score significantly lower when compared with those with a body weight \textgreater5° percentile: 88.8 (6.3) and 100.5 (8.9), respectively. At 12 months, children with unemployed mothers had a mean (SD) motor score significantly lower when compared with those in the other socioeconomic classes: 87.7 (9.8) and 100.6 (12.4), respectively. In conclusion, parents of babies operated on for low-risk EA can be reassured about neurodevelopmental outcome at least up to 1 year of age. When offering a multidisciplinary follow-up program, underweight patients should deserve particular attention to promote their quality of life and support their global development.

@article{padula_traffic-related_2013,
	title = {Traffic-related air pollution and selected birth defects in the {San} {Joaquin} {Valley} of {California}},
	volume = {97},
	issn = {1542-0760},
	doi = {10.1002/bdra.23175},
	abstract = {BACKGROUND: Birth defects are a leading cause of infant morbidity and mortality. Studies suggest associations between environmental contaminants and some structural anomalies, although evidence is limited and several anomalies have not been investigated previously.
METHODS: We used data from the California Center of the National Birth Defects Prevention Study and the Children's Health and Air Pollution Study to estimate the odds of 26 congenital birth defect phenotypes with respect to quartiles of seven ambient air pollutant and traffic exposures in California during the first 2 months of pregnancy, 1997 to 2006 (874 cases and 849 controls). We calculated odds ratios (adjusted for maternal race/ethnicity, education, and vitamin use; aOR) for 11 phenotypes that had at least 40 cases.
RESULTS: Few odds ratios had confidence intervals that did not include 1.0. Odds of esophageal atresia were increased for the highest versus lowest of traffic density (aOR = 2.8, 95\% confidence interval [CI], 1.1-7.4) and PM10 exposure (aOR 4.9; 95\% CI, 1.4-17.2). PM₁₀ was associated with a decreased risk of hydrocephaly (aOR= 0.3; 95\% CI, 0.1-0.9) and CO with decreased risk of anotia/microtia (aOR = 0.4; 95\% CI, 0.2-0.8) and transverse limb deficiency (aOR = 0.4; 95\% CI, 0.2-0.9), again reflecting highest versus lowest quartile comparisons.
CONCLUSION: Most analyses showed no substantive association between air pollution and the selected birth defects with few exceptions of mixed results.},
	language = {eng},
	number = {11},
	journal = {Birth Defects Research. Part A, Clinical and Molecular Teratology},
	author = {Padula, Amy M. and Tager, Ira B. and Carmichael, Suzan L. and Hammond, S. Katharine and Yang, Wei and Lurmann, Frederick W. and Shaw, Gary M.},
	month = nov,
	year = {2013},
	keywords = {Adult, Air Pollutants, Air Pollution, California, Congenital Abnormalities, Congenital Microtia, Ear, Female, Humans, Hydrocephalus, Maternal Exposure, Pregnancy, Retrospective Studies, air pollution, birth outcomes, congenital anomalies, traffic},
	pages = {730--735},
}

BACKGROUND: Birth defects are a leading cause of infant morbidity and mortality. Studies suggest associations between environmental contaminants and some structural anomalies, although evidence is limited and several anomalies have not been investigated previously. METHODS: We used data from the California Center of the National Birth Defects Prevention Study and the Children's Health and Air Pollution Study to estimate the odds of 26 congenital birth defect phenotypes with respect to quartiles of seven ambient air pollutant and traffic exposures in California during the first 2 months of pregnancy, 1997 to 2006 (874 cases and 849 controls). We calculated odds ratios (adjusted for maternal race/ethnicity, education, and vitamin use; aOR) for 11 phenotypes that had at least 40 cases. RESULTS: Few odds ratios had confidence intervals that did not include 1.0. Odds of esophageal atresia were increased for the highest versus lowest of traffic density (aOR = 2.8, 95% confidence interval [CI], 1.1-7.4) and PM10 exposure (aOR 4.9; 95% CI, 1.4-17.2). PM₁₀ was associated with a decreased risk of hydrocephaly (aOR= 0.3; 95% CI, 0.1-0.9) and CO with decreased risk of anotia/microtia (aOR = 0.4; 95% CI, 0.2-0.8) and transverse limb deficiency (aOR = 0.4; 95% CI, 0.2-0.9), again reflecting highest versus lowest quartile comparisons. CONCLUSION: Most analyses showed no substantive association between air pollution and the selected birth defects with few exceptions of mixed results.

@article{gottrand_barrett_2013,
	title = {Barrett esophagus and esophagojejunal anastomotic stenosis as complications of esophagogastric disconnection in children with esophageal atresia},
	volume = {57},
	issn = {1536-4801},
	url = {https://pubmed.ncbi.nlm.nih.gov/23535759/},
	doi = {10.1097/MPG.0b013e3182929ec2},
	abstract = {Total esophagogastric disconnection (TED) is an alternative surgical procedure in resistant gastroesophageal reflux disease. We report 2 severe, not yet described long-term complications of TED occurring in 4 children with a history of esophageal atresia. Three children presented with stenosis of the esophagojejunal anastomosis 5 months to 9 years after TED, requiring repeated dilations associated with mitomycin C application in one of them. Barrett esophagus was observed in 3 children 8 to 9 years after TED. Careful long-term clinical and endoscopic follow-up of children who underwent TED is required.},
	language = {eng},
	number = {1},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Gottrand, Madeleine and Michaud, Laurent and Guimber, Dominique and Coopman, Stéphanie and Sfeir, Rony and Bonnevalle, Michel and Leteurtre, Emmanuelle and Gottrand, Frédéric},
	month = jul,
	year = {2013},
	keywords = {Barrett Esophagus, Child, Child, Preschool, Esophageal Atresia, Esophageal Stenosis, Esophagogastric Junction, France, Humans, Male, Postoperative Complications, Severity of Illness Index},
	pages = {93--95},
}

Total esophagogastric disconnection (TED) is an alternative surgical procedure in resistant gastroesophageal reflux disease. We report 2 severe, not yet described long-term complications of TED occurring in 4 children with a history of esophageal atresia. Three children presented with stenosis of the esophagojejunal anastomosis 5 months to 9 years after TED, requiring repeated dilations associated with mitomycin C application in one of them. Barrett esophagus was observed in 3 children 8 to 9 years after TED. Careful long-term clinical and endoscopic follow-up of children who underwent TED is required.

@article{michaud_characteristics_2013,
	title = {Characteristics and management of congenital esophageal stenosis: findings from a multicenter study},
	volume = {8},
	issn = {1750-1172},
	shorttitle = {Characteristics and management of congenital esophageal stenosis},
	url = {https://pubmed.ncbi.nlm.nih.gov/24289834/},
	doi = {10.1186/1750-1172-8-186},
	abstract = {BACKGROUND: Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.
METHODS: Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.
RESULTS: Over 18 years, 61 patients (30 boys) had CES, and 29 (47\%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p {\textless} 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50\% presented with dysphasia, 40\% with vomiting, 50\% with food impaction, and 42\% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56\%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4\%). At follow-up, dysphagia remained in 36\% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27).
CONCLUSIONS: CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.},
	language = {eng},
	journal = {Orphanet Journal of Rare Diseases},
	author = {Michaud, Laurent and Coutenier, Frédéric and Podevin, Guillaume and Bonnard, Arnaud and Becmeur, François and Khen-Dunlop, Naziha and Auber, Frédéric and Maurel, Aude and Gelas, Thomas and Dassonville, Martine and Borderon, Corinne and Dabadie, Alain and Weil, Dominique and Piolat, Christian and Breton, Anne and Djeddi, Djamal and Morali, Alain and Bastiani, Florence and Lamireau, Thierry and Gottrand, Frédéric},
	month = dec,
	year = {2013},
	keywords = {Adolescent, Child, Child, Preschool, Esophageal Atresia, Esophageal Stenosis, Female, Humans, Infant, Infant, Newborn, Male, Retrospective Studies},
	pages = {186},
}

BACKGROUND: Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES. METHODS: Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome. RESULTS: Over 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p \textless 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27). CONCLUSIONS: CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

@article{bednarczyk_chromosome_2013,
	title = {Chromosome aberrations and gene mutations in patients with esophageal atresia},
	volume = {57},
	issn = {1536-4801},
	doi = {10.1097/MPG.0b013e3182a373dc},
	abstract = {Esophageal atresia (EA) is one of the most frequent congenital malformations of the gastrointestinal tract. Many genetic alterations in patients with EA have been described in the literature. It is thought that the etiology of EA is heterogeneous. This review of the literature provides detailed information about chromosomal aberrations, gene mutations, and clinical features of neonates with EA, and serves as an excellent source to compare newly diagnosed patients with those described in the literature.},
	language = {eng},
	number = {6},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Bednarczyk, Damian and Sasiadek, Maria M. and Smigiel, Robert},
	month = dec,
	year = {2013},
	keywords = {Chromosome Aberrations, Esophageal Atresia, Esophagus, Humans, Mutation, Tracheoesophageal Fistula},
	pages = {688--693},
}

Esophageal atresia (EA) is one of the most frequent congenital malformations of the gastrointestinal tract. Many genetic alterations in patients with EA have been described in the literature. It is thought that the etiology of EA is heterogeneous. This review of the literature provides detailed information about chromosomal aberrations, gene mutations, and clinical features of neonates with EA, and serves as an excellent source to compare newly diagnosed patients with those described in the literature.

@article{sfeir_epidemiology_2013,
	title = {Epidemiology of esophageal atresia},
	volume = {26},
	issn = {1442-2050},
	url = {https://pubmed.ncbi.nlm.nih.gov/23679022/},
	doi = {10.1111/dote.12051},
	abstract = {Esophageal atresia (EA) is a rare congenital malformation consisting of a lack of continuity between the upper and lower esophageal pouches, frequently associated with tracheoesophageal fistula. The prevalence of such rare abnormalities is established by global birth surveillance programs over the world. EUROCAT is a European program covering 1.7 million births since its creation. The prevalence of EA in Europe seems stable over decades. The National Birth Defects Prevention Network in the USA also shows a stable prevalence with a wide range between states or regions. In France, with the implementation of the national rare diseases plan, a reference center for congenital abnormalities of the esophagus was created in 2006 and a national registry for EA began patient inclusion in 2008. This has resulted in the establishment of the national live birth prevalence for EA, prenatal diagnosis rates, and clinical characteristics of EA patients, early survival, and early morbidity. Prevalence rates seem stable all over the world since many decades. Continuous surveillance of congenital abnormalities and specific registries are useful for epidemiologic data but also for public health authorities for helping families of rare diseases patients.},
	language = {eng},
	number = {4},
	journal = {Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus},
	author = {Sfeir, R. and Michaud, L. and Salleron, J. and Gottrand, F.},
	year = {2013},
	keywords = {Esophageal Atresia, Europe, Female, Global Health, Humans, Population Surveillance, Pregnancy, Prenatal Diagnosis, Prevalence, Registries, United States},
	pages = {354--355},
}

Esophageal atresia (EA) is a rare congenital malformation consisting of a lack of continuity between the upper and lower esophageal pouches, frequently associated with tracheoesophageal fistula. The prevalence of such rare abnormalities is established by global birth surveillance programs over the world. EUROCAT is a European program covering 1.7 million births since its creation. The prevalence of EA in Europe seems stable over decades. The National Birth Defects Prevention Network in the USA also shows a stable prevalence with a wide range between states or regions. In France, with the implementation of the national rare diseases plan, a reference center for congenital abnormalities of the esophagus was created in 2006 and a national registry for EA began patient inclusion in 2008. This has resulted in the establishment of the national live birth prevalence for EA, prenatal diagnosis rates, and clinical characteristics of EA patients, early survival, and early morbidity. Prevalence rates seem stable all over the world since many decades. Continuous surveillance of congenital abnormalities and specific registries are useful for epidemiologic data but also for public health authorities for helping families of rare diseases patients.

@article{rintala_long-term_2013,
	title = {Long-term outcome of esophageal anastomosis},
	volume = {23},
	issn = {1439-359X},
	doi = {10.1055/s-0033-1347912},
	abstract = {After repair of esophageal atresia (EA) in a newborn, esophageal dysmotility presenting as dysphagia and symptomatic gastroesophageal reflux are common. Significant esophageal morbidity associated with EA extends into adulthood. In adulthood approximately one-fifth of the patients have developed epithelial metaplastic changes, one-third of these have intestinal metaplasia (Barrett esophagus). Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, eight cases of esophageal cancer have been reported in young adults treated for EA. Incidence of esophageal cancer after EA repair is very much likely to increase in the future. Life-long endoscopic follow-up is warranted in patients with EA.},
	language = {eng},
	number = {3},
	journal = {European Journal of Pediatric Surgery: Official Journal of Austrian Association of Pediatric Surgery ... [et Al] = Zeitschrift Fur Kinderchirurgie},
	author = {Rintala, R. J. and Pakarinen, M. P.},
	month = jun,
	year = {2013},
	keywords = {Anastomosis, Surgical, Esophageal Atresia, Esophagus, Follow-Up Studies, Humans, Postoperative Complications, Time Factors, Treatment Outcome},
	pages = {219--225},
}

After repair of esophageal atresia (EA) in a newborn, esophageal dysmotility presenting as dysphagia and symptomatic gastroesophageal reflux are common. Significant esophageal morbidity associated with EA extends into adulthood. In adulthood approximately one-fifth of the patients have developed epithelial metaplastic changes, one-third of these have intestinal metaplasia (Barrett esophagus). Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, eight cases of esophageal cancer have been reported in young adults treated for EA. Incidence of esophageal cancer after EA repair is very much likely to increase in the future. Life-long endoscopic follow-up is warranted in patients with EA.

@article{bagolan_long-gap_2013,
	title = {Long-gap esophageal atresia: traction-growth and anastomosis - before and beyond},
	volume = {26},
	issn = {1442-2050},
	shorttitle = {Long-gap esophageal atresia},
	doi = {10.1111/dote.12050},
	abstract = {Long-gap esophageal atresia (LGEA) is still a major surgical challenge. Options for esophageal reconstruction include the use of native esophagus or esophageal replacement with stomach, colon, or small intestine. Nonetheless, there is a consensus among most pediatric surgeons that the preservation of the native esophagus is associated with better postoperative outcomes. Thus, every effort should be made to conserve the native esophagus. The present study is aimed at critically reporting our experience focused on a standardized protocol based on the preoperative assessment of the gap in all cases and reviewing the present literature because no consensus is available regarding many aspects of LGEA (from definition to treatment). All newborn infants treated since 1995 for esophageal atresia (EA), regardless of type, were included in the present study. Identification of LGEA patients (gap ≥3 vertebral bodies) was performed based on preoperative esophageal gap measurement. The selected patients were grouped based on EA type (A/B vs. C/D) and whether they were referred from an outside institution or not. Postoperative outcome was compared. Statistical analysis was performed with the Fisher's exact test and Mann-Whitney test as appropriate, with P {\textless} 0.05 considered statistically significant. Two hundred and nineteen patients have been consecutively treated between 1995 and 2012 with the following EA subtypes: type: A 25 (11.4\%); B 6 (2.7\%); C 182 (83.1\%); D 3 (1.4\%); E 3 (1.4\%). Fifty-seven patients (26\%) were classified as LGEA: type A-B, 31 (54.4\%); type C-D, 26 (45.6\%). Twenty seven (47\%) of these patients were referred after at least one failed attempt at esophageal correction: type A-B, 15 (55\%); type C-D, 12 (45\%). Only one patient ultimately required esophageal substitution, with an overall survival rate of 94\%. A standardized perioperative protocol enhances the possibility of preserving the native esophagus in cases of LGEA. Gap measurement can be accurately defined before surgery in all patients with EA. Esophageal anastomosis (either immediate or delayed repair) is almost always feasible; esophageal substitution should only be considered after a rigorous attempt at achieving end-to-end esophageal anastomosis.},
	language = {eng},
	number = {4},
	journal = {Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus},
	author = {Bagolan, P. and Valfrè, L. and Morini, F. and Conforti, A.},
	year = {2013},
	keywords = {Anastomosis, Surgical, Clinical Protocols, Esophageal Atresia, Esophagoplasty, Esophagus, Humans, Infant, Newborn, Postoperative Complications, Preoperative Care, Tissue Expansion, Tracheoesophageal Fistula, Treatment Outcome},
	pages = {372--379},
}

Long-gap esophageal atresia (LGEA) is still a major surgical challenge. Options for esophageal reconstruction include the use of native esophagus or esophageal replacement with stomach, colon, or small intestine. Nonetheless, there is a consensus among most pediatric surgeons that the preservation of the native esophagus is associated with better postoperative outcomes. Thus, every effort should be made to conserve the native esophagus. The present study is aimed at critically reporting our experience focused on a standardized protocol based on the preoperative assessment of the gap in all cases and reviewing the present literature because no consensus is available regarding many aspects of LGEA (from definition to treatment). All newborn infants treated since 1995 for esophageal atresia (EA), regardless of type, were included in the present study. Identification of LGEA patients (gap ≥3 vertebral bodies) was performed based on preoperative esophageal gap measurement. The selected patients were grouped based on EA type (A/B vs. C/D) and whether they were referred from an outside institution or not. Postoperative outcome was compared. Statistical analysis was performed with the Fisher's exact test and Mann-Whitney test as appropriate, with P \textless 0.05 considered statistically significant. Two hundred and nineteen patients have been consecutively treated between 1995 and 2012 with the following EA subtypes: type: A 25 (11.4%); B 6 (2.7%); C 182 (83.1%); D 3 (1.4%); E 3 (1.4%). Fifty-seven patients (26%) were classified as LGEA: type A-B, 31 (54.4%); type C-D, 26 (45.6%). Twenty seven (47%) of these patients were referred after at least one failed attempt at esophageal correction: type A-B, 15 (55%); type C-D, 12 (45%). Only one patient ultimately required esophageal substitution, with an overall survival rate of 94%. A standardized perioperative protocol enhances the possibility of preserving the native esophagus in cases of LGEA. Gap measurement can be accurately defined before surgery in all patients with EA. Esophageal anastomosis (either immediate or delayed repair) is almost always feasible; esophageal substitution should only be considered after a rigorous attempt at achieving end-to-end esophageal anastomosis.

@article{sfeir_esophageal_2013,
	title = {Esophageal atresia: data from a national cohort},
	volume = {48},
	issn = {1531-5037},
	shorttitle = {Esophageal atresia},
	url = {https://pubmed.ncbi.nlm.nih.gov/23932604/},
	doi = {10.1016/j.jpedsurg.2013.03.075},
	abstract = {PURPOSE: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan.
METHODS: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report.
RESULTS: Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95\%, and no correlation with caseload was noted.
CONCLUSIONS: Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.},
	language = {eng},
	number = {8},
	journal = {Journal of Pediatric Surgery},
	author = {Sfeir, Rony and Bonnard, Arnaud and Khen-Dunlop, Naziha and Auber, Frederic and Gelas, Thomas and Michaud, Laurent and Podevin, Guillaume and Breton, Anne and Fouquet, Virginie and Piolat, Christian and Lemelle, Jean Louis and Petit, Thierry and Lavrand, Frederic and Becmeur, Francis and Polimerol, Marie Laurence and Michel, Jean Luc and Elbaz, Frederic and Habonimana, Eric and Allal, Hassan and Lopez, Emmanuel and Lardy, Hubert and Morineau, Marianne and Pelatan, Cécile and Merrot, Thierry and Delagausie, Pascal and de Vries, Philline and Levard, Guillaume and Buisson, Phillippe and Sapin, Emmanuel and Jaby, Olivier and Borderon, Corinne and Weil, Dominique and Gueiss, Stephane and Aubert, Didier and Echaieb, Anais and Fourcade, Laurent and Breaud, Jean and Laplace, Christophe and Pouzac, Myriam and Duhamel, Alain and Gottrand, Frederic},
	month = aug,
	year = {2013},
	keywords = {Abnormalities, Multiple, Adolescent, Adult, Birth Weight, Cohort Studies, Cohort study, Combined Modality Therapy, Epidemiology, Esophageal Atresia, Esophageal atresia, Female, France, Gestational Age, Humans, Incidence, Infant, Newborn, Infant, Premature, Infant, Premature, Diseases, Male, Maternal Age, Middle Aged, Neonatal surgery, Polyhydramnios, Population Surveillance, Population-based registry, Pregnancy, Prenatal Diagnosis, Prenatal diagnosis, Prevalence, Prospective Studies, Rare disease, Registries, Surveys and Questionnaires, Survival Rate, Workload, Young Adult},
	pages = {1664--1669},
}

PURPOSE: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan. METHODS: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report. RESULTS: Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted. CONCLUSIONS: Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.

@article{schneider_esophageal_2013,
	title = {Esophageal atresia: metaplasia, {Barrett}},
	volume = {26},
	issn = {1442-2050},
	shorttitle = {Esophageal atresia},
	url = {https://pubmed.ncbi.nlm.nih.gov/23679037/},
	doi = {10.1111/dote.12057},
	abstract = {Barrett's esophagus is characterized by the replacement of squamous epithelium by columnar epithelium that is intestinal metaplasia-positive or -negative in the distal esophagus. Gastroesophageal reflux disease, which is frequent and prolonged in esophageal atresia, probably plays a major role in the development of Barrett's esophagus through repeated mucosal damage. Long-term acid exposure contributes to carcinogenesis in Barrett's esophagus of intestinal type, but its effect on gastric metaplasia is less well defined. Recent studies have suggest that metaplasia arises in about 15\% of patients with esophageal atresia, with a lag time to developing metaplasia from initial surgical correction of about 10 years. Preliminary data from an ongoing multicenter study including 88 patients with esophageal atresia aged 15-19 years showed gastric metaplasia in 42\% of patients (29 fundic and 7 cardial metaplasia), while one patient presented intestinal metaplasia. Esophageal mucosal abnormalities can be observed in esophageal atresia patients at endoscopy despite the absence of symptoms. Whether prolonged, aggressive, acid suppression is beneficial in these situations remains to be determined. Barrett's metaplasia can be removed by endoscopic mucosal resection or destroyed with endoscopic ablative techniques, such as photodynamic therapy, radiofrequency ablation, and cryotherapy. The risk of developing esophageal carcinoma is still a controversial issue as only a few clinical cases have been reported in young adults with esophageal atresia. As late complications of esophageal atresia, particularly esophagitis and Barrett's esophagus, are increasingly being recognized, long-time systematic follow up of the esophageal mucosa including multistage biopsies is therefore required even in asymptomatic patients.},
	language = {eng},
	number = {4},
	journal = {Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus},
	author = {Schneider, A. and Michaud, L. and Gottrand, F.},
	year = {2013},
	keywords = {Barrett Esophagus, Esophageal Atresia, Esophagoscopy, Humans},
	pages = {425--427},
}

Barrett's esophagus is characterized by the replacement of squamous epithelium by columnar epithelium that is intestinal metaplasia-positive or -negative in the distal esophagus. Gastroesophageal reflux disease, which is frequent and prolonged in esophageal atresia, probably plays a major role in the development of Barrett's esophagus through repeated mucosal damage. Long-term acid exposure contributes to carcinogenesis in Barrett's esophagus of intestinal type, but its effect on gastric metaplasia is less well defined. Recent studies have suggest that metaplasia arises in about 15% of patients with esophageal atresia, with a lag time to developing metaplasia from initial surgical correction of about 10 years. Preliminary data from an ongoing multicenter study including 88 patients with esophageal atresia aged 15-19 years showed gastric metaplasia in 42% of patients (29 fundic and 7 cardial metaplasia), while one patient presented intestinal metaplasia. Esophageal mucosal abnormalities can be observed in esophageal atresia patients at endoscopy despite the absence of symptoms. Whether prolonged, aggressive, acid suppression is beneficial in these situations remains to be determined. Barrett's metaplasia can be removed by endoscopic mucosal resection or destroyed with endoscopic ablative techniques, such as photodynamic therapy, radiofrequency ablation, and cryotherapy. The risk of developing esophageal carcinoma is still a controversial issue as only a few clinical cases have been reported in young adults with esophageal atresia. As late complications of esophageal atresia, particularly esophagitis and Barrett's esophagus, are increasingly being recognized, long-time systematic follow up of the esophageal mucosa including multistage biopsies is therefore required even in asymptomatic patients.

@article{yamada_esophagitis_2013,
	title = {Esophagitis with eosinophil infiltration associated with congenital esophageal atresia and stenosis},
	volume = {161 Suppl 2},
	issn = {1423-0097},
	doi = {10.1159/000350400},
	abstract = {BACKGROUND: The esophagus is physiologically devoid of eosinophils, so their presence would suggest some underlying pathology. The prevalence of eosinophilic esophagitis (EoE) has steadily increased in Western countries. Previous studies have described EoE in association with congenital esophageal atresia (CEA), which is the most common congenital anomaly of the esophagus. However, the association remains unclear.
METHODS: We performed a retrospective histological analysis examining for eosinophil infiltration in the esophagus of patients with CEA following surgical repair or congenital esophageal stenosis (CES) who underwent esophageal biopsy or surgical resection in our hospital between 2005 and 2012.
RESULTS: There were 6 patients with CEA following surgical repair or CES who had eosinophil-dominant infiltration in the esophagus. All had associated allergic disorders, including food allergies in 4. Moreover, all except for one fulfilled the histological criteria of EoE. Impairment of eosinophil infiltration and symptomatic improvement were observed in those treated with a proton pump inhibitor (PPI), either alone or in combination with steroids after esophageal dilatation.
CONCLUSIONS: These findings suggest that CEA repair or CES in conjunction with allergic conditions and coexisting gastroesophageal reflux disease (GERD) may induce greater esophageal eosinophilic inflammation. In addition, esophageal dilatation followed by PPI treatment, alone or with steroids, may be a therapeutic strategy that can provide symptomatic relief by reducing eosinophilic inflammation in esophageal strictures or GERD associated with CEA or CES.},
	language = {eng},
	journal = {International Archives of Allergy and Immunology},
	author = {Yamada, Yoshiyuki and Nishi, Akira and Kato, Masahiko and Toki, Fumiaki and Yamamoto, Hideki and Suzuki, Norio and Hirato, Junko and Hayashi, Yasuhide},
	year = {2013},
	keywords = {Adolescent, Child, Child, Preschool, Eosinophilic Esophagitis, Esophageal Atresia, Esophageal Stenosis, Esophagoscopy, Female, Humans, Infant, Male, Retrospective Studies},
	pages = {159--163},
}

BACKGROUND: The esophagus is physiologically devoid of eosinophils, so their presence would suggest some underlying pathology. The prevalence of eosinophilic esophagitis (EoE) has steadily increased in Western countries. Previous studies have described EoE in association with congenital esophageal atresia (CEA), which is the most common congenital anomaly of the esophagus. However, the association remains unclear. METHODS: We performed a retrospective histological analysis examining for eosinophil infiltration in the esophagus of patients with CEA following surgical repair or congenital esophageal stenosis (CES) who underwent esophageal biopsy or surgical resection in our hospital between 2005 and 2012. RESULTS: There were 6 patients with CEA following surgical repair or CES who had eosinophil-dominant infiltration in the esophagus. All had associated allergic disorders, including food allergies in 4. Moreover, all except for one fulfilled the histological criteria of EoE. Impairment of eosinophil infiltration and symptomatic improvement were observed in those treated with a proton pump inhibitor (PPI), either alone or in combination with steroids after esophageal dilatation. CONCLUSIONS: These findings suggest that CEA repair or CES in conjunction with allergic conditions and coexisting gastroesophageal reflux disease (GERD) may induce greater esophageal eosinophilic inflammation. In addition, esophageal dilatation followed by PPI treatment, alone or with steroids, may be a therapeutic strategy that can provide symptomatic relief by reducing eosinophilic inflammation in esophageal strictures or GERD associated with CEA or CES.

@article{lepeytre_medium-term_2013,
	title = {[{Medium}-term outcome, follow-up, and quality of life in children treated for type {III} esophageal atresia]},
	volume = {20},
	issn = {1769-664X},
	doi = {10.1016/j.arcped.2013.06.035},
	abstract = {The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65\%), asthma before the age of 3 years (66\%), hospitalization for a respiratory event (45\%), fundoplication (20\%), and esophageal dilatation (45\%). We noted current chronic cough (16\%), asthma (30\%), dysphagia (39\%), and symptomatic gastroesophageal reflux (9\%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good.},
	language = {fre},
	number = {10},
	journal = {Archives De Pediatrie: Organe Officiel De La Societe Francaise De Pediatrie},
	author = {Lepeytre, C. and De Lagausie, P. and Merrot, T. and Baumstarck, K. and Oudyi, M. and Dubus, J.-C.},
	month = oct,
	year = {2013},
	keywords = {Body Mass Index, Child, Child, Preschool, Deglutition Disorders, Esophageal Atresia, Female, Follow-Up Studies, Gastrostomy, Humans, Male, Patient Outcome Assessment, Quality of Life, Respiratory Tract Diseases, Surveys and Questionnaires},
	pages = {1096--1104},
}

The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good.

@article{walker_early_2013,
	title = {Early developmental outcome following surgery for oesophageal atresia},
	volume = {49},
	issn = {1440-1754},
	doi = {10.1111/jpc.12206},
	abstract = {AIM: To compare the developmental outcome of infants with oesophageal atresia with or without trachea-oesophageal fistula (OA/TOF) who underwent surgery in early infancy with healthy control infants in New South Wales, Australia.
METHODS: Infants diagnosed with OA/TOF requiring surgical intervention were enrolled prospectively between 1 August 2006 and the 31 December 2008. Healthy control infants were enrolled in the same time period. The children underwent a developmental assessment at 1 year of age (corrected) using the Bayley Scales of Infant and Toddler Development (Version III).
RESULTS: Of 34 infants with OA/TOF that were enrolled, 31 had developmental assessments. The majority (75\%) were term infants (≥37 weeks gestation) with a mean birth weight of 2717 g. Fourteen infants (44\%) had an associated birth defect and one infant with multiple associated anomalies subsequently died. Developmental assessments were also performed on 62 control infants matched for gestational age. Infants with OA/TOF had a mean score significantly lower on the expressive language subscale (P {\textless} 0.05) compared with the control infants.
CONCLUSIONS: This study found a lower than expected developmental score for infants following surgery for OA/TOF in the expressive language subscale compared with the healthy control infants. These findings support concerns over the potential impact of OA/TOF and its effects on development. Further studies, including continuing developmental review to determine whether these differences persist and their functional importance, should be performed.},
	language = {eng},
	number = {6},
	journal = {Journal of Paediatrics and Child Health},
	author = {Walker, Karen and Halliday, Robert and Badawi, Nadia and Stewart, Jan and Holland, Andrew Ja},
	month = jun,
	year = {2013},
	keywords = {Case-Control Studies, Child Development, Child Language, Developmental Disabilities, Esophageal Atresia, Humans, Infant, Prospective Studies, Tracheoesophageal Fistula, Treatment Outcome},
	pages = {467--470},
}

AIM: To compare the developmental outcome of infants with oesophageal atresia with or without trachea-oesophageal fistula (OA/TOF) who underwent surgery in early infancy with healthy control infants in New South Wales, Australia. METHODS: Infants diagnosed with OA/TOF requiring surgical intervention were enrolled prospectively between 1 August 2006 and the 31 December 2008. Healthy control infants were enrolled in the same time period. The children underwent a developmental assessment at 1 year of age (corrected) using the Bayley Scales of Infant and Toddler Development (Version III). RESULTS: Of 34 infants with OA/TOF that were enrolled, 31 had developmental assessments. The majority (75%) were term infants (≥37 weeks gestation) with a mean birth weight of 2717 g. Fourteen infants (44%) had an associated birth defect and one infant with multiple associated anomalies subsequently died. Developmental assessments were also performed on 62 control infants matched for gestational age. Infants with OA/TOF had a mean score significantly lower on the expressive language subscale (P \textless 0.05) compared with the control infants. CONCLUSIONS: This study found a lower than expected developmental score for infants following surgery for OA/TOF in the expressive language subscale compared with the healthy control infants. These findings support concerns over the potential impact of OA/TOF and its effects on development. Further studies, including continuing developmental review to determine whether these differences persist and their functional importance, should be performed.

@article{familiari_peroral_2013,
	title = {Peroral endoscopic myotomy for the treatment of achalasia in children},
	volume = {57},
	issn = {1536-4801},
	doi = {10.1097/MPG.0b013e3182a803f7},
	abstract = {Peroral endoscopic myotomy (POEM) is a new endoscopic technique for the treatment of esophageal achalasia, with excellent results in adults. Three children with classic-type achalasia (mean age 9.6 years) underwent POEM in a single center. Mean basal lower esophageal sphincter pressure and Eckard score were 34.6 mmHg and 5.3, respectively. Mean length of myotomy was 10 cm. On average, POEM was completed in 60.6 minutes. No patients experienced postoperative complications or gastroesophageal reflux disease. In the 2 patients who completed a 12-month follow-up, complete symptoms relief was documented (Eckard score 0), as well as a significant drop of the basal lower esophageal sphincter pressure.},
	language = {eng},
	number = {6},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Familiari, Pietro and Marchese, Michele and Gigante, Giovanni and Boskoski, Ivo and Tringali, Andrea and Perri, Vincenzo and Costamagna, Guido},
	month = dec,
	year = {2013},
	keywords = {Child, Esophageal Achalasia, Esophageal Sphincter, Lower, Esophagoscopy, Female, Follow-Up Studies, Gastroesophageal Reflux, Humans, Incidence, Muscles, Postoperative Complications, Pressure, Treatment Outcome},
	pages = {794--797},
}

Peroral endoscopic myotomy (POEM) is a new endoscopic technique for the treatment of esophageal achalasia, with excellent results in adults. Three children with classic-type achalasia (mean age 9.6 years) underwent POEM in a single center. Mean basal lower esophageal sphincter pressure and Eckard score were 34.6 mmHg and 5.3, respectively. Mean length of myotomy was 10 cm. On average, POEM was completed in 60.6 minutes. No patients experienced postoperative complications or gastroesophageal reflux disease. In the 2 patients who completed a 12-month follow-up, complete symptoms relief was documented (Eckard score 0), as well as a significant drop of the basal lower esophageal sphincter pressure.

@article{catalano_simple_2012,
	title = {A simple technique of oblique anastomosis can prevent stricture formation in primary repair of esophageal atresia},
	volume = {47},
	issn = {1531-5037},
	doi = {10.1016/j.jpedsurg.2012.04.021},
	abstract = {BACKGROUND: Anastomotic stricture is an important problem after esophageal atresia (EA) repair. This study evaluates a technique of oblique esophageal anastomosis without use of a flap in order to prevent stricture formation.
METHODS: Medical records of 16 patients (14 with EA type III and 2 with EA type IV Ladd-Gross classification) who underwent primary repair of EA at birth without anastomotic tension were reviewed, evaluating long-term follow-up results. All patients were studied with esophageal contrast study, pH-multichannel intraluminal impedance, and endoscopy. The incidence of complications and their management were analysed.
RESULTS: Contrast esophagogram and esophagoscopy always showed regular patency of the suture line.
CONCLUSIONS: Our technique of oblique anastomosis is simple, safe, and effective in preventing stricture formation even in the long-term follow-up.},
	language = {eng},
	number = {9},
	journal = {Journal of Pediatric Surgery},
	author = {Catalano, Pieralba and Di Pace, Maria Rita and Caruso, Anna Maria and Salerno, Sergio and Cimador, Marcello and De Grazia, Enrico},
	month = sep,
	year = {2012},
	keywords = {Anastomosis, Surgical, Esophageal Atresia, Esophageal Stenosis, Esophagoplasty, Female, Follow-Up Studies, Humans, Incidence, Infant, Newborn, Male, Postoperative Complications, Retrospective Studies, Treatment Outcome},
	pages = {1767--1771},
}

BACKGROUND: Anastomotic stricture is an important problem after esophageal atresia (EA) repair. This study evaluates a technique of oblique esophageal anastomosis without use of a flap in order to prevent stricture formation. METHODS: Medical records of 16 patients (14 with EA type III and 2 with EA type IV Ladd-Gross classification) who underwent primary repair of EA at birth without anastomotic tension were reviewed, evaluating long-term follow-up results. All patients were studied with esophageal contrast study, pH-multichannel intraluminal impedance, and endoscopy. The incidence of complications and their management were analysed. RESULTS: Contrast esophagogram and esophagoscopy always showed regular patency of the suture line. CONCLUSIONS: Our technique of oblique anastomosis is simple, safe, and effective in preventing stricture formation even in the long-term follow-up.

@article{pinheiro_current_2012,
	title = {Current knowledge on esophageal atresia},
	volume = {18},
	issn = {2219-2840},
	doi = {10.3748/wjg.v18.i28.3662},
	abstract = {Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.},
	language = {eng},
	number = {28},
	journal = {World Journal of Gastroenterology},
	author = {Pinheiro, Paulo Fernando Martins and Simões e Silva, Ana Cristina and Pereira, Regina Maria},
	month = jul,
	year = {2012},
	keywords = {Adult, Anastomosis, Surgical, Child, Child, Preschool, Esophageal Atresia, Esophageal Stenosis, Esophageal atresia, Esophageal stenosis, Esophagus, Gastro-esophageal reflux, Gastroenterology, Gastroesophageal Reflux, Humans, Infant, Infant, Newborn, Long-gap, Prognosis, Risk Factors, Surgical Procedures, Operative, Time Factors, Tracheoesophageal Fistula, Tracheoesophageal fistula, Treatment Outcome},
	pages = {3662--3672},
}

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.

@article{schneider_postoperative_2012,
	title = {Postoperative lower esophageal dilation in children following the performance of {Nissen} fundoplication},
	volume = {22},
	issn = {1439-359X},
	url = {https://pubmed.ncbi.nlm.nih.gov/22773348/},
	doi = {10.1055/s-0032-1315807},
	abstract = {OBJECTIVES: The purpose of this study is to study the frequency and factors associated with lower esophageal dilation (LED) after Nissen fundoplication.
METHODS: This retrospective monocentric study included 288 patients who had undergone Nissen fundoplication from 1998 to 2009. The frequency of children requiring LED was assessed. The clinical characteristics of the patients at the time of fundoplication, their symptoms, and outcomes were recorded. The population with LED (group 1) was compared with the population without LED (group 2) to identify factors associated with postfundoplication LED using multivariate analysis.
RESULTS: LED was required by 70 patients (24\%) because of postoperative dysphagia, and 45/70 were dilated within the first 6 months. The mean age at dilation was 72 months (standard deviation [SD] 65), with an average post-Nissen delay of 9 months (SD 13). Surgical revision was required by 11 patients because of LED failure (n = 10) or postdilation perforation (n = 1). Patients who required post-Nissen dilation were significantly more frequently fed orally than those in group 2 and had more postoperative complications (dumping syndrome, surgical revision).
CONCLUSIONS: A significant frequency of postfundoplication LED was observed in this pediatric population. Dilation was associated in children with preoperative feeding or postoperative complications (dumping syndrome, surgical revision).},
	language = {eng},
	number = {5},
	journal = {European Journal of Pediatric Surgery: Official Journal of Austrian Association of Pediatric Surgery ... [et Al] = Zeitschrift Fur Kinderchirurgie},
	author = {Schneider, Anne and Gottrand, Frédéric and Sfeir, Rony and Duhamel, Alain and Bonnevalle, Michel and Guimber, Dominique and Michaud, Laurent},
	month = oct,
	year = {2012},
	keywords = {Adolescent, Adult, Child, Child, Preschool, Deglutition Disorders, Dilatation, Dumping Syndrome, Enteral Nutrition, Female, France, Fundoplication, Humans, Infant, Male, Multivariate Analysis, Proportional Hazards Models, Reoperation, Retrospective Studies, Risk Factors, Treatment Outcome},
	pages = {399--403},
}

OBJECTIVES: The purpose of this study is to study the frequency and factors associated with lower esophageal dilation (LED) after Nissen fundoplication. METHODS: This retrospective monocentric study included 288 patients who had undergone Nissen fundoplication from 1998 to 2009. The frequency of children requiring LED was assessed. The clinical characteristics of the patients at the time of fundoplication, their symptoms, and outcomes were recorded. The population with LED (group 1) was compared with the population without LED (group 2) to identify factors associated with postfundoplication LED using multivariate analysis. RESULTS: LED was required by 70 patients (24%) because of postoperative dysphagia, and 45/70 were dilated within the first 6 months. The mean age at dilation was 72 months (standard deviation [SD] 65), with an average post-Nissen delay of 9 months (SD 13). Surgical revision was required by 11 patients because of LED failure (n = 10) or postdilation perforation (n = 1). Patients who required post-Nissen dilation were significantly more frequently fed orally than those in group 2 and had more postoperative complications (dumping syndrome, surgical revision). CONCLUSIONS: A significant frequency of postfundoplication LED was observed in this pediatric population. Dilation was associated in children with preoperative feeding or postoperative complications (dumping syndrome, surgical revision).

@article{kunisaki_surgical_2012,
	title = {Surgical advances in the fetus and neonate: esophageal atresia},
	volume = {39},
	issn = {1557-9840},
	shorttitle = {Surgical advances in the fetus and neonate},
	doi = {10.1016/j.clp.2012.04.007},
	abstract = {This article focuses on selected topics in the diagnosis and management of patients with esophageal atresia (EA) with or without tracheoesophageal fistula. The current status of prenatal diagnosis and recent advances in surgical techniques, including thoracoscopic repair for short-gap EA and tension-induced esophageal growth for long-gap EA, are reviewed. Although no consensus exists among pediatric surgeons regarding the role of these procedures in the treatment of EA, one can reasonably expect that, as they evolve, their application will become more widespread in this challenging patient population.},
	language = {eng},
	number = {2},
	journal = {Clinics in Perinatology},
	author = {Kunisaki, Shaun M. and Foker, John E.},
	month = jun,
	year = {2012},
	keywords = {Child, Digestive System Surgical Procedures, Esophageal Atresia, Female, Fetus, Humans, Infant, Newborn, Male, Thoracoscopy, Tracheoesophageal Fistula, Ultrasonography, Prenatal},
	pages = {349--361},
}

This article focuses on selected topics in the diagnosis and management of patients with esophageal atresia (EA) with or without tracheoesophageal fistula. The current status of prenatal diagnosis and recent advances in surgical techniques, including thoracoscopic repair for short-gap EA and tension-induced esophageal growth for long-gap EA, are reviewed. Although no consensus exists among pediatric surgeons regarding the role of these procedures in the treatment of EA, one can reasonably expect that, as they evolve, their application will become more widespread in this challenging patient population.

@article{elshafei_colonic_2012,
	title = {Colonic conduit for esophageal replacement: long-term endoscopic and histopathologic changes in colonic mucosa},
	volume = {47},
	issn = {1531-5037},
	shorttitle = {Colonic conduit for esophageal replacement},
	doi = {10.1016/j.jpedsurg.2012.04.002},
	abstract = {BACKGROUND: Long-term follow-up has substantiated the colon as a durable and highly acceptable esophageal substitute. Exposure of colonic conduit to gastric acid may lead to histopathologic changes in the form of chronic inflammation.
MATERIALS/METHODS: Thirty children with esophageal replacement were studied from 2 to 12 years (mean, 5.20 years) postoperatively. All cases underwent upper gastrointestinal tract endoscopy to evaluate the gross appearance of colonic conduit mucosa, and punch biopsies were taken from upper and lower junctions of the conduit. All biopsies were submitted to histopathologic examination.
RESULTS: Endoscopic findings were comparable with normal regarding the gross appearance of colonic mucosa in both upper and lower junctions (25 cases/83.3\%). Some abnormalities were seen including cervical anastomosis stricture (2 cases/6.7\%), redundancy (3 cases/10\%), mucosal ulcer in the lower residual esophagus (1 case/3.3\%), and hyperemia (3 cases/10\%). Pathologic changes were minimal regarding the change in position of the colon to a thoracic organ during follow-up. Most of the cases were normal (22 cases/73.3\%). Seven cases (23.4\%) showed mild chronic nonspecific inflammation of the colonic mucosa, whereas only 1 case (3.3\%) showed mildly active inflammation of colonic mucosa.
CONCLUSION: The use of the colon for esophageal replacement showed that no significant pathologic changes affecting its function as a conduit because its mucosa showed no significant change in response to gastric acid reflux in long-term follow-up and can be further protected by an antireflux procedure.},
	language = {eng},
	number = {9},
	journal = {Journal of Pediatric Surgery},
	author = {Elshafei, Hossam and Elshafei, Ehab and ElDebeiky, Mohamed and Hegazy, Nedal and Zaki, Ahmed and Abdel Hay, Sameh},
	month = sep,
	year = {2012},
	keywords = {Adolescent, Anastomosis, Surgical, Biopsy, Child, Child, Preschool, Colon, Esophageal Atresia, Esophagoscopy, Esophagus, Female, Follow-Up Studies, Gastroesophageal Reflux, Humans, Infant, Infant, Newborn, Intestinal Mucosa, Male, Postoperative Complications, Stomach, Treatment Outcome},
	pages = {1658--1661},
}

BACKGROUND: Long-term follow-up has substantiated the colon as a durable and highly acceptable esophageal substitute. Exposure of colonic conduit to gastric acid may lead to histopathologic changes in the form of chronic inflammation. MATERIALS/METHODS: Thirty children with esophageal replacement were studied from 2 to 12 years (mean, 5.20 years) postoperatively. All cases underwent upper gastrointestinal tract endoscopy to evaluate the gross appearance of colonic conduit mucosa, and punch biopsies were taken from upper and lower junctions of the conduit. All biopsies were submitted to histopathologic examination. RESULTS: Endoscopic findings were comparable with normal regarding the gross appearance of colonic mucosa in both upper and lower junctions (25 cases/83.3%). Some abnormalities were seen including cervical anastomosis stricture (2 cases/6.7%), redundancy (3 cases/10%), mucosal ulcer in the lower residual esophagus (1 case/3.3%), and hyperemia (3 cases/10%). Pathologic changes were minimal regarding the change in position of the colon to a thoracic organ during follow-up. Most of the cases were normal (22 cases/73.3%). Seven cases (23.4%) showed mild chronic nonspecific inflammation of the colonic mucosa, whereas only 1 case (3.3%) showed mildly active inflammation of colonic mucosa. CONCLUSION: The use of the colon for esophageal replacement showed that no significant pathologic changes affecting its function as a conduit because its mucosa showed no significant change in response to gastric acid reflux in long-term follow-up and can be further protected by an antireflux procedure.

@article{schulz_nine_2012,
	title = {Nine new twin pairs with esophageal atresia: a review of the literature and performance of a twin study of the disorder},
	volume = {94},
	issn = {1542-0760},
	shorttitle = {Nine new twin pairs with esophageal atresia},
	doi = {10.1002/bdra.22879},
	abstract = {BACKGROUND: Isolated esophageal atresia (EA) is a rare congenital malformation whose etiology remains largely unknown. Nine twin pairs with EA were identified from our clinical service, prompting the performance of a systematic review of the literature and the first reported twin study of isolated EA.
METHODS: A total of 330 twin pairs with EA were identified from the literature. The zygosity, concordance, and malformation (isolated vs. nonisolated) status of all 339 twin pairs were evaluated. A total of 72 twin pairs (4 of 9 / 68 of 330) fulfilled the criteria for inclusion in a classic twin study of isolated EA.
RESULTS: The pairwise concordance rates were 50\% (95\% confidence interval [CI], 34-66\%) for monozygous (MZ) twin pairs and 26\% (95\% CI, 15-42\%) for dizygous (DZ) twin pairs (p = 0.033). The probandwise concordance rates were 67\% (95\% CI, 53-78\%) for MZ twin pairs and 42\% (95\% CI, 29-56\%) for DZ twin pairs (p = 0.011). The MZ/DZ ratios were 1.9 for pairwise analysis and 1.6 for probandwise analysis. The familial risk ratios for MZ and DZ twin pairs were 1700 and 900, respectively.
CONCLUSION: The observation of higher concordance rates for MZ compared to DZ twin pairs indicates that genetic factors contribute to isolated EA.},
	language = {eng},
	number = {3},
	journal = {Birth Defects Research. Part A, Clinical and Molecular Teratology},
	author = {Schulz, Anna Carina and Bartels, Enrika and Stressig, Rüdiger and Ritgen, Jochen and Schmiedeke, Eberhard and Mattheisen, Manuel and Draaken, Markus and Ludwig, Michael and Bagci, Soyhan and Müller, Andreas and Gembruch, Ulrich and Geipel, Annegret and Berg, Christoph and Heydweiller, Andreas and Bachour, Haitham and Schumacher, Johannes and Bartmann, Peter and Nöthen, Markus M. and Reutter, Heiko},
	month = mar,
	year = {2012},
	keywords = {Diseases in Twins, Esophageal Atresia, Female, Genetic Predisposition to Disease, Humans, Male, Twins, Dizygotic, Twins, Monozygotic},
	pages = {182--186},
}

BACKGROUND: Isolated esophageal atresia (EA) is a rare congenital malformation whose etiology remains largely unknown. Nine twin pairs with EA were identified from our clinical service, prompting the performance of a systematic review of the literature and the first reported twin study of isolated EA. METHODS: A total of 330 twin pairs with EA were identified from the literature. The zygosity, concordance, and malformation (isolated vs. nonisolated) status of all 339 twin pairs were evaluated. A total of 72 twin pairs (4 of 9 / 68 of 330) fulfilled the criteria for inclusion in a classic twin study of isolated EA. RESULTS: The pairwise concordance rates were 50% (95% confidence interval [CI], 34-66%) for monozygous (MZ) twin pairs and 26% (95% CI, 15-42%) for dizygous (DZ) twin pairs (p = 0.033). The probandwise concordance rates were 67% (95% CI, 53-78%) for MZ twin pairs and 42% (95% CI, 29-56%) for DZ twin pairs (p = 0.011). The MZ/DZ ratios were 1.9 for pairwise analysis and 1.6 for probandwise analysis. The familial risk ratios for MZ and DZ twin pairs were 1700 and 900, respectively. CONCLUSION: The observation of higher concordance rates for MZ compared to DZ twin pairs indicates that genetic factors contribute to isolated EA.

@article{maselli_peroral_2012,
	title = {Peroral endoscopic myotomy ({POEM}) in a 3-year-old girl with severe growth retardation, achalasia, and {Down} syndrome},
	volume = {44 Suppl 2 UCTN},
	issn = {1438-8812},
	doi = {10.1055/s-0032-1309924},
	language = {eng},
	journal = {Endoscopy},
	author = {Maselli, R. and Inoue, H. and Misawa, M. and Ikeda, H. and Hosoya, T. and Onimaru, M. and Yoshida, A. and Eleftheriadis, N. and Suzuki, K. and Kudo, S.},
	year = {2012},
	keywords = {Child, Preschool, Down Syndrome, Esophageal Achalasia, Esophageal Sphincter, Lower, Female, Growth Disorders, Humans, Natural Orifice Endoscopic Surgery},
	pages = {E285--287},
}

@article{gordon_eftud2_2012,
	title = {{EFTUD2} haploinsufficiency leads to syndromic oesophageal atresia},
	volume = {49},
	issn = {1468-6244},
	url = {https://pubmed.ncbi.nlm.nih.gov/23188108/},
	doi = {10.1136/jmedgenet-2012-101173},
	abstract = {BACKGROUND: Oesophageal atresia (OA) and mandibulofacial dysostosis (MFD) are two congenital malformations for which the molecular bases of syndromic forms are being identified at a rapid rate. In particular, the EFTUD2 gene encoding a protein of the spliceosome complex has been found mutated in patients with MFD and microcephaly (MIM610536). Until now, no syndrome featuring both MFD and OA has been clearly delineated.
RESULTS: We report on 10 cases presenting with MFD, eight of whom had OA, either due to de novo 17q21.31 deletions encompassing EFTUD2 and neighbouring genes or de novo heterozygous EFTUD2 loss-of-function mutations. No EFTUD2 deletions or mutations were found in a series of patients with isolated OA or isolated oculoauriculovertebral spectrum (OAVS).
CONCLUSIONS: These data exclude a contiguous gene syndrome for the association of MFD and OA, broaden the spectrum of clinical features ascribed to EFTUD2 haploinsufficiency, define a novel syndromic OA entity, and emphasise the necessity of mRNA maturation through the spliceosome complex for global growth and within specific regions of the embryo during development. Importantly, the majority of patients reported here with EFTUD2 lesions were previously diagnosed with Feingold or CHARGE syndromes or presented with OAVS plus OA, highlighting the variability of expression and the wide range of differential diagnoses.},
	language = {eng},
	number = {12},
	journal = {Journal of Medical Genetics},
	author = {Gordon, Christopher T. and Petit, Florence and Oufadem, Myriam and Decaestecker, Charles and Jourdain, Anne-Sophie and Andrieux, Joris and Malan, Valérie and Alessandri, Jean-Luc and Baujat, Geneviève and Baumann, Clarisse and Boute-Benejean, Odile and Caumes, Roseline and Delobel, Bruno and Dieterich, Klaus and Gaillard, Dominique and Gonzales, Marie and Lacombe, Didier and Escande, Fabienne and Manouvrier-Hanu, Sylvie and Marlin, Sandrine and Mathieu-Dramard, Michèle and Mehta, Sarju G. and Simonic, Ingrid and Munnich, Arnold and Vekemans, Michel and Porchet, Nicole and de Pontual, Loïc and Sarnacki, Sabine and Attie-Bitach, Tania and Lyonnet, Stanislas and Holder-Espinasse, Muriel and Amiel, Jeanne},
	month = dec,
	year = {2012},
	keywords = {Adolescent, Child, Child, Preschool, Chromosome Deletion, Chromosomes, Human, Pair 17, Comparative Genomic Hybridization, Esophageal Atresia, Facies, Female, Haploinsufficiency, Humans, Infant, Male, Peptide Elongation Factors, Phenotype, Ribonucleoprotein, U5 Small Nuclear, Syndrome},
	pages = {737--746},
}

BACKGROUND: Oesophageal atresia (OA) and mandibulofacial dysostosis (MFD) are two congenital malformations for which the molecular bases of syndromic forms are being identified at a rapid rate. In particular, the EFTUD2 gene encoding a protein of the spliceosome complex has been found mutated in patients with MFD and microcephaly (MIM610536). Until now, no syndrome featuring both MFD and OA has been clearly delineated. RESULTS: We report on 10 cases presenting with MFD, eight of whom had OA, either due to de novo 17q21.31 deletions encompassing EFTUD2 and neighbouring genes or de novo heterozygous EFTUD2 loss-of-function mutations. No EFTUD2 deletions or mutations were found in a series of patients with isolated OA or isolated oculoauriculovertebral spectrum (OAVS). CONCLUSIONS: These data exclude a contiguous gene syndrome for the association of MFD and OA, broaden the spectrum of clinical features ascribed to EFTUD2 haploinsufficiency, define a novel syndromic OA entity, and emphasise the necessity of mRNA maturation through the spliceosome complex for global growth and within specific regions of the embryo during development. Importantly, the majority of patients reported here with EFTUD2 lesions were previously diagnosed with Feingold or CHARGE syndromes or presented with OAVS plus OA, highlighting the variability of expression and the wide range of differential diagnoses.

@article{gorter_eosinophilic_2012,
	title = {Eosinophilic esophagitis after esophageal atresia: is there an association? {Case} presentation and literature review},
	volume = {47},
	issn = {1531-5037},
	shorttitle = {Eosinophilic esophagitis after esophageal atresia},
	doi = {10.1016/j.jpedsurg.2012.01.079},
	abstract = {Eosinophilic esophagitis (EoE) is a relatively new condition resulting in dysphagia or symptoms resembling gastroesophageal reflux disease, symptoms that also are common in patients with a history of esophageal atresia. We present 2 patients with persistent dysphagia after repair of esophageal atresia that was caused by EoE. Although the exact etiology and pathogenesis of EoE remain unclear, it is now generally accepted that it is the result of a T-helper cell 2-type immune response with a crucial role for the eosinophil-specific chemotaxis factor eotaxin 3 and eosinophils. Because there are genetic similarities between esophageal atresia and EoE, we speculate that patients with esophageal atresia are at increased risk for developing EoE.},
	language = {eng},
	number = {6},
	journal = {Journal of Pediatric Surgery},
	author = {Gorter, Ramon R. and Heij, Hugo A. and van der Voorn, J. Patrick and Kneepkens, C. M. Frank},
	month = jun,
	year = {2012},
	keywords = {Abnormalities, Multiple, Anal Canal, Anti-Ulcer Agents, Barrett Esophagus, Chemokine CCL26, Chemokines, CC, Child, Combined Modality Therapy, Deglutition Disorders, Disease Susceptibility, Eosinophilia, Esophageal Atresia, Esophagitis, Esophagus, Female, Follow-Up Studies, Food Hypersensitivity, Forkhead Transcription Factors, Fundoplication, Gastroesophageal Reflux, Gene Deletion, Heart Defects, Congenital, Hernia, Hiatal, Humans, Infant, Newborn, Kidney, Limb Deformities, Congenital, Male, Postoperative Complications, Spine, Th2 Cells, Trachea, Tracheoesophageal Fistula},
	pages = {e9--13},
}

Eosinophilic esophagitis (EoE) is a relatively new condition resulting in dysphagia or symptoms resembling gastroesophageal reflux disease, symptoms that also are common in patients with a history of esophageal atresia. We present 2 patients with persistent dysphagia after repair of esophageal atresia that was caused by EoE. Although the exact etiology and pathogenesis of EoE remain unclear, it is now generally accepted that it is the result of a T-helper cell 2-type immune response with a crucial role for the eosinophil-specific chemotaxis factor eotaxin 3 and eosinophils. Because there are genetic similarities between esophageal atresia and EoE, we speculate that patients with esophageal atresia are at increased risk for developing EoE.

@article{burjonrappa_what_2011,
	title = {What is the incidence of {Barrett}'s and gastric metaplasia in esophageal atresia/tracheoesophageal fistula ({EA}/{TEF}) patients?},
	volume = {21},
	issn = {1439-359X},
	doi = {10.1055/s-0030-1262861},
	abstract = {OBJECTIVE: Aim of the study was to document the incidence of gastric and intestinal metaplasia (Barrett's esophagus) in a closely followed group with EA/TEF, determine the efficacy of pH probe studies in diagnosing reflux and, moreover, to determine the lag period between the development of gastroesophageal reflux disease (GERD) and metaplasia.
MATERIALS AND METHODS: Patients who underwent EA/TEF correction were followed up over an 18-year period (1990-2009) with regular pH probe and endoscopy investigations. Biopsies were performed at 3 year intervals to evaluate for metaplastic changes in the esophageal epithelium. The interval between esophageal atresia correction and the development of erosive esophagitis, gastric metaplasia and Barrett's esophagus was determined. The development of complicated GERD was statistically correlated to age at detection.
RESULTS: 51 patients (28 male) underwent EA/TEF correction during the study period. The ages of the children followed up ranged from 7 months to 19 years. There were 5 Type A, 44 Type C and 2 Type D fistulas. 37 patients (5 Type A, 31 Type C, 1 Type D) had documented reflux and 14 did not. 17 (46\%) of the patients with reflux underwent an anti-reflux operation. 4 of the 17 underwent 2 or more anti-reflux procedures. 21 patients had pH probe studies positive for reflux, 3 had negative pH probe studies after an anti-reflux operation. 18 of the 37 patients with reflux (about 50\%) needed esophageal dilatation for either anastamotic or non-anastamotic strictures. Only 4/14 (28.5\%) patients without reflux underwent dilatations and only in their first year of life (p=NS). 15 patients had mucosal abnormalities in endoscopic biopsies. 8 patients had gastric metaplasia and 1 patient had Barrett's esophagus. The mean age in the group with metaplasia (gastric and intestinal) was 13 years, which was significantly different from the mean age of the group (5.2 years) without mucosal changes (p{\textless}0.001). 2 patients with normal pH probe studies had metaplasia on biopsies.
CONCLUSION: Metaplasia arises in about 15\% of patients with EA/TEF. The lag time to developing metaplasia from the time of initial surgical correction is about 10 years. Endoscopy and biopsies are the best way of detecting mucosal changes.},
	language = {eng},
	number = {1},
	journal = {European Journal of Pediatric Surgery: Official Journal of Austrian Association of Pediatric Surgery ... [et Al] = Zeitschrift Fur Kinderchirurgie},
	author = {Burjonrappa, S. C. and Youssef, S. and St-Vil, D.},
	month = jan,
	year = {2011},
	keywords = {Adolescent, Barrett Esophagus, Child, Child, Preschool, Esophageal Atresia, Esophagus, Female, Gastroesophageal Reflux, Humans, Incidence, Infant, Male, Metaplasia, Tracheoesophageal Fistula, Young Adult},
	pages = {25--29},
}

OBJECTIVE: Aim of the study was to document the incidence of gastric and intestinal metaplasia (Barrett's esophagus) in a closely followed group with EA/TEF, determine the efficacy of pH probe studies in diagnosing reflux and, moreover, to determine the lag period between the development of gastroesophageal reflux disease (GERD) and metaplasia. MATERIALS AND METHODS: Patients who underwent EA/TEF correction were followed up over an 18-year period (1990-2009) with regular pH probe and endoscopy investigations. Biopsies were performed at 3 year intervals to evaluate for metaplastic changes in the esophageal epithelium. The interval between esophageal atresia correction and the development of erosive esophagitis, gastric metaplasia and Barrett's esophagus was determined. The development of complicated GERD was statistically correlated to age at detection. RESULTS: 51 patients (28 male) underwent EA/TEF correction during the study period. The ages of the children followed up ranged from 7 months to 19 years. There were 5 Type A, 44 Type C and 2 Type D fistulas. 37 patients (5 Type A, 31 Type C, 1 Type D) had documented reflux and 14 did not. 17 (46%) of the patients with reflux underwent an anti-reflux operation. 4 of the 17 underwent 2 or more anti-reflux procedures. 21 patients had pH probe studies positive for reflux, 3 had negative pH probe studies after an anti-reflux operation. 18 of the 37 patients with reflux (about 50%) needed esophageal dilatation for either anastamotic or non-anastamotic strictures. Only 4/14 (28.5%) patients without reflux underwent dilatations and only in their first year of life (p=NS). 15 patients had mucosal abnormalities in endoscopic biopsies. 8 patients had gastric metaplasia and 1 patient had Barrett's esophagus. The mean age in the group with metaplasia (gastric and intestinal) was 13 years, which was significantly different from the mean age of the group (5.2 years) without mucosal changes (p\textless0.001). 2 patients with normal pH probe studies had metaplasia on biopsies. CONCLUSION: Metaplasia arises in about 15% of patients with EA/TEF. The lag time to developing metaplasia from the time of initial surgical correction is about 10 years. Endoscopy and biopsies are the best way of detecting mucosal changes.

@article{chang_endoscopic_2011,
	title = {Endoscopic balloon dilatation for esophageal strictures in children younger than 6 years: experience in a medical center},
	volume = {52},
	issn = {1875-9572},
	shorttitle = {Endoscopic balloon dilatation for esophageal strictures in children younger than 6 years},
	doi = {10.1016/j.pedneo.2011.05.005},
	abstract = {Esophageal strictures in children may be caused by congenital anomaly, caustic agent or foreign body ingestion, complication of reflux esophagitis, and after esophageal surgery. Accidental ingestion of alkaline fluid is the most common cause of corrosive esophagitis in children in Taiwan. In this article, we studied 10 pediatric patients who had esophageal strictures and required endoscopic balloon dilatation (EBD) therapy under general anesthesia from January 2003 to June 2009. The median age of the studied children who received their first EBD treatment was 36.2 months (13.4-60.9 months), with a dilator size of 8.0mm (5-12 mm). The interval between injury and initial EBD was 3.0 months (1.3-60.8 months). The treatment duration averaged 16.7 months (3.0-69.3 months), with 13.5 (4-31) instances of EBD therapy per patient. The greater the length of stricture, the more number of times EBD was needed. In these cases, no severe complication was found after the procedure. The result indicated that EBD under general anesthesia was a safe and effective method to resolve the symptom of dysphagia and diet condition. Because of the limited number of study cases, long-term studies are required to further confirm the clinical effect of EBD under general anesthesia.},
	language = {eng},
	number = {4},
	journal = {Pediatrics and Neonatology},
	author = {Chang, Chih-Feng and Kuo, Shih-Pin and Lin, Hung-Chih and Chuang, Chun-Chun and Tsai, Tien-Kai and Wu, Shu-Fen and Chen, An-Chyi and Chen, Walter and Peng, Ching-Tien},
	month = aug,
	year = {2011},
	keywords = {Catheterization, Child, Preschool, Esophageal Stenosis, Esophagoscopy, Female, Humans, Infant, Male, Retrospective Studies},
	pages = {196--202},
}

Esophageal strictures in children may be caused by congenital anomaly, caustic agent or foreign body ingestion, complication of reflux esophagitis, and after esophageal surgery. Accidental ingestion of alkaline fluid is the most common cause of corrosive esophagitis in children in Taiwan. In this article, we studied 10 pediatric patients who had esophageal strictures and required endoscopic balloon dilatation (EBD) therapy under general anesthesia from January 2003 to June 2009. The median age of the studied children who received their first EBD treatment was 36.2 months (13.4-60.9 months), with a dilator size of 8.0mm (5-12 mm). The interval between injury and initial EBD was 3.0 months (1.3-60.8 months). The treatment duration averaged 16.7 months (3.0-69.3 months), with 13.5 (4-31) instances of EBD therapy per patient. The greater the length of stricture, the more number of times EBD was needed. In these cases, no severe complication was found after the procedure. The result indicated that EBD under general anesthesia was a safe and effective method to resolve the symptom of dysphagia and diet condition. Because of the limited number of study cases, long-term studies are required to further confirm the clinical effect of EBD under general anesthesia.

@article{di_pace_evaluation_2011,
	title = {Evaluation of esophageal motility and reflux in children treated for esophageal atresia with the use of combined multichannel intraluminal impedance and {pH} monitoring},
	volume = {46},
	issn = {1531-5037},
	doi = {10.1016/j.jpedsurg.2010.08.012},
	abstract = {BACKGROUND: Gastroesophageal reflux (GER) and dysmotility are frequent in patients treated for esophageal atresia (EA). This aim of this study is to evaluate GER and dysmotility in young EA patients using pH-multichannel intraluminal impedance (pH-MII).
METHODS: Fifteen patients with a mean age of 7.5 years (group 1) have been studied and compared with 15 children without congenital malformation, submitted to pH-MII for suspected GER (group 2). These latter patients serve as a control group of healthy subjects. The following impedance reflux and motility parameters have been studied on 10 standardized swallows: number of reflux episodes, mean acid clearing time, median bolus clearing time, bolus presence time, total bolus transit time, segmental transit time, and total propagation velocity.
RESULTS: In the group of EA patients, mean acid clearing time and median bolus clearing time were pathological. In the control group, all reflux parameters were normal. Patients with EA had significantly longer median bolus presence time at each measuring site, median total bolus transit time, and median segmental transit time and slower total propagation velocity (P {\textless} .001).
CONCLUSIONS: pH-multichannel intraluminal impedance evaluates both GER and motility patterns. Our report studies impedance parameters of esophageal motility in healthy children and in EA patients using only pH-MII.},
	language = {eng},
	number = {3},
	journal = {Journal of Pediatric Surgery},
	author = {Di Pace, Maria Rita and Caruso, Anna Maria and Catalano, Pieralba and Casuccio, Alessandra and Cimador, Marcello and De Grazia, Enrico},
	month = mar,
	year = {2011},
	keywords = {Child, Child, Preschool, Deglutition, Electric Impedance, Esophageal Atresia, Esophageal Motility Disorders, Esophageal pH Monitoring, Esophagitis, Esophagoscopy, Esophagus, Female, Gastroesophageal Reflux, Humans, Male, Monitoring, Ambulatory, Peristalsis, Postoperative Complications, Rheology, Tracheoesophageal Fistula},
	pages = {443--451},
}

BACKGROUND: Gastroesophageal reflux (GER) and dysmotility are frequent in patients treated for esophageal atresia (EA). This aim of this study is to evaluate GER and dysmotility in young EA patients using pH-multichannel intraluminal impedance (pH-MII). METHODS: Fifteen patients with a mean age of 7.5 years (group 1) have been studied and compared with 15 children without congenital malformation, submitted to pH-MII for suspected GER (group 2). These latter patients serve as a control group of healthy subjects. The following impedance reflux and motility parameters have been studied on 10 standardized swallows: number of reflux episodes, mean acid clearing time, median bolus clearing time, bolus presence time, total bolus transit time, segmental transit time, and total propagation velocity. RESULTS: In the group of EA patients, mean acid clearing time and median bolus clearing time were pathological. In the control group, all reflux parameters were normal. Patients with EA had significantly longer median bolus presence time at each measuring site, median total bolus transit time, and median segmental transit time and slower total propagation velocity (P \textless .001). CONCLUSIONS: pH-multichannel intraluminal impedance evaluates both GER and motility patterns. Our report studies impedance parameters of esophageal motility in healthy children and in EA patients using only pH-MII.

@article{marlais_health-related_2011,
	title = {Health-related quality of life in children with achalasia},
	volume = {47},
	issn = {1440-1754},
	doi = {10.1111/j.1440-1754.2010.01884.x},
	abstract = {AIMS: To assess self-reported QoL in children with achalasia aged 5-18 and compare this with both disease and healthy control children in a prospective study.
METHODS: All children diagnosed with achalasia at one hospital were asked to participate in this study by completing the self-report module of the validated PedsQL™ generic QoL assessment. All children attending a tertiary paediatric gastroenterology clinic from February 2009 to May 2009 with chronic constipation or inflammatory bowel disease were asked to participate in this study as disease controls. The PedsQL™ considers physical, emotional, social and school domains and is scored from 0-100. Healthy children were also recruited from the same site. Groups were compared using Analysis of Variance with Tukey's post-hoc test.
RESULTS: One hundred and sixty one children completed the assessment (90 (56\%) male, mean age 11.3 yrs ± 3.4 years) including 17 children with achalasia, 44 with chronic constipation, 59 with inflammatory bowel disease and 41 healthy children. QoL was significantly lower in the achalasia group compared to both children with IBD (73 vs. 82, p = 0.035) and healthy children (73 vs. 84, p = 0.005), and was comparable to that of children with chronic constipation (73 vs. 74, p = 0.99).
CONCLUSION: Children with achalasia report a significantly lower QoL compared to children with inflammatory bowel disease and healthy children.},
	language = {eng},
	number = {1-2},
	journal = {Journal of Paediatrics and Child Health},
	author = {Marlais, Matko and Fishman, Julia R. and Fell, John Me and Rawat, David J. and Haddad, Munther J.},
	month = jan,
	year = {2011},
	keywords = {Adolescent, Case-Control Studies, Child, Constipation, Esophageal Achalasia, Female, Humans, Inflammatory Bowel Diseases, London, Male, Prospective Studies, Quality of Life, Sickness Impact Profile},
	pages = {18--21},
}

AIMS: To assess self-reported QoL in children with achalasia aged 5-18 and compare this with both disease and healthy control children in a prospective study. METHODS: All children diagnosed with achalasia at one hospital were asked to participate in this study by completing the self-report module of the validated PedsQL™ generic QoL assessment. All children attending a tertiary paediatric gastroenterology clinic from February 2009 to May 2009 with chronic constipation or inflammatory bowel disease were asked to participate in this study as disease controls. The PedsQL™ considers physical, emotional, social and school domains and is scored from 0-100. Healthy children were also recruited from the same site. Groups were compared using Analysis of Variance with Tukey's post-hoc test. RESULTS: One hundred and sixty one children completed the assessment (90 (56%) male, mean age 11.3 yrs ± 3.4 years) including 17 children with achalasia, 44 with chronic constipation, 59 with inflammatory bowel disease and 41 healthy children. QoL was significantly lower in the achalasia group compared to both children with IBD (73 vs. 82, p = 0.035) and healthy children (73 vs. 84, p = 0.005), and was comparable to that of children with chronic constipation (73 vs. 74, p = 0.99). CONCLUSION: Children with achalasia report a significantly lower QoL compared to children with inflammatory bowel disease and healthy children.

@article{schneider_pseudostratified_2011,
	title = {Pseudostratified ciliated metaplasia of the distal esophagus diagnosed at adolescence},
	volume = {43 Suppl 2 UCTN},
	issn = {1438-8812},
	url = {https://pubmed.ncbi.nlm.nih.gov/21557159/},
	doi = {10.1055/s-0030-1256301},
	language = {eng},
	journal = {Endoscopy},
	author = {Schneider, A. and Maunoury, V. and Leteurtre, E. and Turck, D. and Gottrand, F. and Michaud, L.},
	year = {2011},
	keywords = {Adolescent, Barrett Esophagus, Esophagus, Humans, Male, Metaplasia, Proton Pump Inhibitors},
	pages = {E184},
}

@article{djeddi_another_2011,
	title = {Another case of idiopathic megaesophagus in a girl with growth hormone deficiency},
	volume = {35},
	issn = {2210-741X},
	url = {https://pubmed.ncbi.nlm.nih.gov/21778130/},
	doi = {10.1016/j.clinre.2011.05.013},
	abstract = {BACKGROUND: Achalasia or idiopathic megaesophagus is rare in children, and an association with growth hormone (GH) deficiency has rarely been described.
CASE REPORT: This report is of a girl treated with recombinant GH (rGH) for isolated GH deficiency who simultaneously presented with idiopathic megaesophagus.
CONCLUSION: This case report highlights the fact that practitioners need to be aware of this possible morbid association.},
	language = {eng},
	number = {11},
	journal = {Clinics and Research in Hepatology and Gastroenterology},
	author = {Djeddi, D. and Vanrenterghem, A. and Léké, A. and Boudailliez, B. and Gottrand, F.},
	month = nov,
	year = {2011},
	keywords = {Child, Esophageal Achalasia, Female, Human Growth Hormone, Humans},
	pages = {768--770},
}

BACKGROUND: Achalasia or idiopathic megaesophagus is rare in children, and an association with growth hormone (GH) deficiency has rarely been described. CASE REPORT: This report is of a girl treated with recombinant GH (rGH) for isolated GH deficiency who simultaneously presented with idiopathic megaesophagus. CONCLUSION: This case report highlights the fact that practitioners need to be aware of this possible morbid association.

@article{di_palmo_rare_2011,
	title = {A rare association of inlet patch with laryngospasm: a report of two children and literature review},
	volume = {46},
	issn = {1099-0496},
	shorttitle = {A rare association of inlet patch with laryngospasm},
	doi = {10.1002/ppul.21457},
	abstract = {The inlet patch (IP) is an area of heterotopic gastric mucosa in the cervical esophagus commonly seen during upper endoscopy. Although generally asymptomatic IP has been associated with esophageal and supraesophageal symptoms and, though rare, in adult with malignant transformation. We describe two cases of recurrent episodes of laryngospasm associated with IP. In both cases there was a good response to prolonged acid suppression therapy.},
	language = {eng},
	number = {9},
	journal = {Pediatric Pulmonology},
	author = {di Palmo, E. and Cazzato, S. and Tursini, S. and Salfi, N. C. M. and Mazzotta, A. and Di Silverio Carulli, C. and Lima, M. and Bergamaschi, R. and Bernardi, F. and Cicognani, A.},
	month = sep,
	year = {2011},
	keywords = {Alginates, Anti-Ulcer Agents, Child, Child, Preschool, Esophageal Diseases, Female, Gastric Mucosa, Glucuronic Acid, Hexuronic Acids, Humans, Laryngismus, Omeprazole, Ranitidine, Severity of Illness Index, Treatment Outcome},
	pages = {934--938},
}

The inlet patch (IP) is an area of heterotopic gastric mucosa in the cervical esophagus commonly seen during upper endoscopy. Although generally asymptomatic IP has been associated with esophageal and supraesophageal symptoms and, though rare, in adult with malignant transformation. We describe two cases of recurrent episodes of laryngospasm associated with IP. In both cases there was a good response to prolonged acid suppression therapy.

@article{catalano_gastroesophageal_2011,
	title = {Gastroesophageal reflux in young children treated for esophageal atresia: evaluation with {pH}-multichannel intraluminal impedance},
	volume = {52},
	issn = {1536-4801},
	shorttitle = {Gastroesophageal reflux in young children treated for esophageal atresia},
	doi = {10.1097/MPG.0b013e318202a3e5},
	abstract = {OBJECTIVES: Gastroesophageal reflux (GER) and dismotility occur frequently after repair of esophageal atresia (EA). GER-associated complications can manifest either early or later; then precocious diagnosis and treatment are essential. The aim of the study was to evaluate characteristics of GER and esophageal clearance in children treated for EA with distal tracheoesophageal fistula, using pH-multichannel intraluminal impedance (pH-MII).
PATIENTS AND METHODS: Twenty-two children (ages 3-40 months) treated for EA at birth, and 20 normal children of similar age with suspected GER disease were included in the study. Impedance parameters were analyzed according to age and symptoms.
RESULTS: Reflux events were mainly nonacidic. About bolus exposure index, mean acid clearing time (MACT), and mean bolus clearing time (MBCT), a significant difference was found between 2 groups: the median MACT and MBCT were longer, with values of 281 and 39 seconds, respectively, in the EA group and 110 and 15 seconds in the control group (P {\textless} 0.0005).
CONCLUSIONS: Our data show that in young patients the majority of refluxes are not acid. This implies that the incidence of GER may be underestimated if pH-metry is used. The pH-MII is an ideal test in children because it studies both GER with its characteristics and motility pattern. The quality of reflux does not seem to influence the onset of symptoms that are related to an impaired esophageal clearance. Hence, this technique could be useful to study patients treated for EA, avoiding the onset of complications.},
	language = {eng},
	number = {6},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {Catalano, Pieralba and Di Pace, Maria R. and Caruso, Anna M. and Casuccio, Alessandra and De Grazia, Enrico},
	month = jun,
	year = {2011},
	keywords = {Case-Control Studies, Child, Preschool, Esophageal Atresia, Esophageal pH Monitoring, Esophagus, Gastric Acid, Gastroesophageal Reflux, Gastrointestinal Transit, Humans, Hydrogen-Ion Concentration, Infant, Postoperative Complications, Tracheoesophageal Fistula},
	pages = {686--690},
}

OBJECTIVES: Gastroesophageal reflux (GER) and dismotility occur frequently after repair of esophageal atresia (EA). GER-associated complications can manifest either early or later; then precocious diagnosis and treatment are essential. The aim of the study was to evaluate characteristics of GER and esophageal clearance in children treated for EA with distal tracheoesophageal fistula, using pH-multichannel intraluminal impedance (pH-MII). PATIENTS AND METHODS: Twenty-two children (ages 3-40 months) treated for EA at birth, and 20 normal children of similar age with suspected GER disease were included in the study. Impedance parameters were analyzed according to age and symptoms. RESULTS: Reflux events were mainly nonacidic. About bolus exposure index, mean acid clearing time (MACT), and mean bolus clearing time (MBCT), a significant difference was found between 2 groups: the median MACT and MBCT were longer, with values of 281 and 39 seconds, respectively, in the EA group and 110 and 15 seconds in the control group (P \textless 0.0005). CONCLUSIONS: Our data show that in young patients the majority of refluxes are not acid. This implies that the incidence of GER may be underestimated if pH-metry is used. The pH-MII is an ideal test in children because it studies both GER with its characteristics and motility pattern. The quality of reflux does not seem to influence the onset of symptoms that are related to an impaired esophageal clearance. Hence, this technique could be useful to study patients treated for EA, avoiding the onset of complications.

@article{gupta_long-term_2011,
	title = {Long-term follow-up of patients with esophageal replacement by reversed gastric tube},
	volume = {21},
	issn = {1439-359X},
	doi = {10.1055/s-0030-1267240},
	abstract = {PURPOSE: Various surgical procedures are used for esophageal substitution in children. The purpose of this study was to evaluate the long-term outcome in children who underwent esophageal replacement by reversed gastric tube and to assess some aspects of the physiological function and behavior of the reversed gastric tube.
METHODS: This study was a retrospective and prospective review of 16 patients who had undergone reversed gastric tube esophagoplasty between March 1990 and March 2009. Indications for esophageal replacement included esophageal atresia with or without fistula (10) and caustic injury (6). There were 10 boys and 6 girls. Age at the time of this study varied from 1.7-20 years. The interval between operation and follow-up varied; it was more than 3 months in all cases, with an average of 8 months. Follow-up studies included clinical evaluation, anthropometry, barium study (to look for anastomotic leak, stricture, passage of contrast, ulcer, diverticulum, peristalsis and emptying), neoesophagus motility patterns by manometry, 24-h pH monitoring and radionuclide studies (to investigate esophageal transit and gastric emptying times and gastroesophageal reflux). Statistical analysis was carried out using STATA 9.0 and continuous variables were compared between the tests using the Wilcoxon rank sum test.
RESULTS: Of the 16 children, 12 were between the 3 (rd) and 97 (th) percentiles for weight and 10 were between the 3 (rd) and 97 (th) percentiles for height for their respective age group. 14 were eating and swallowing normally. 2 patients had slight swallowing difficulties with lumpy foods but did not require dilatations. None of our patients had respiratory problems. The children with corrosive stricture were almost normal in growth and development compared to esophageal atresia patients. Barium swallow showed normal swallowing in all patients and did not show gastroesophageal reflux. Mass contractions were seen in two-thirds of the patients on manometry. 24-h pH monitoring showed that the intraluminal pH remained consistently low, even long after operative treatment. Scintigraphy and barium swallow showed almost similar results except that scintigraphy was much more sensitive in detecting reflux.
CONCLUSION: Almost all children remained asymptomatic and led a normal life. The function of the neoesophagus was good and growth and nutrition in the majority of patients was satisfactory.},
	language = {eng},
	number = {2},
	journal = {European Journal of Pediatric Surgery: Official Journal of Austrian Association of Pediatric Surgery ... [et Al] = Zeitschrift Fur Kinderchirurgie},
	author = {Gupta, L. and Bhatnagar, V. and Gupta, A. K. and Kumar, R.},
	month = mar,
	year = {2011},
	keywords = {Burns, Chemical, Child, Preschool, Deglutition, Esophageal Atresia, Esophagoplasty, Esophagus, Female, Follow-Up Studies, Humans, Infant, Male, Prospective Studies, Recovery of Function, Retrospective Studies, Stomach, Time Factors, Treatment Outcome},
	pages = {88--93},
}

PURPOSE: Various surgical procedures are used for esophageal substitution in children. The purpose of this study was to evaluate the long-term outcome in children who underwent esophageal replacement by reversed gastric tube and to assess some aspects of the physiological function and behavior of the reversed gastric tube. METHODS: This study was a retrospective and prospective review of 16 patients who had undergone reversed gastric tube esophagoplasty between March 1990 and March 2009. Indications for esophageal replacement included esophageal atresia with or without fistula (10) and caustic injury (6). There were 10 boys and 6 girls. Age at the time of this study varied from 1.7-20 years. The interval between operation and follow-up varied; it was more than 3 months in all cases, with an average of 8 months. Follow-up studies included clinical evaluation, anthropometry, barium study (to look for anastomotic leak, stricture, passage of contrast, ulcer, diverticulum, peristalsis and emptying), neoesophagus motility patterns by manometry, 24-h pH monitoring and radionuclide studies (to investigate esophageal transit and gastric emptying times and gastroesophageal reflux). Statistical analysis was carried out using STATA 9.0 and continuous variables were compared between the tests using the Wilcoxon rank sum test. RESULTS: Of the 16 children, 12 were between the 3 (rd) and 97 (th) percentiles for weight and 10 were between the 3 (rd) and 97 (th) percentiles for height for their respective age group. 14 were eating and swallowing normally. 2 patients had slight swallowing difficulties with lumpy foods but did not require dilatations. None of our patients had respiratory problems. The children with corrosive stricture were almost normal in growth and development compared to esophageal atresia patients. Barium swallow showed normal swallowing in all patients and did not show gastroesophageal reflux. Mass contractions were seen in two-thirds of the patients on manometry. 24-h pH monitoring showed that the intraluminal pH remained consistently low, even long after operative treatment. Scintigraphy and barium swallow showed almost similar results except that scintigraphy was much more sensitive in detecting reflux. CONCLUSION: Almost all children remained asymptomatic and led a normal life. The function of the neoesophagus was good and growth and nutrition in the majority of patients was satisfactory.

@article{raam_long-term_2011,
	title = {Long-term outcomes of adults with features of {VACTERL} association},
	volume = {54},
	issn = {1878-0849},
	doi = {10.1016/j.ejmg.2010.09.007},
	abstract = {VACTERL association involves the presence of specific congenital, multi-organ malformations that tend to co-occur. Clinical and research efforts typically center on pediatric patients, and there is a scarcity of information in the literature regarding VACTERL-related issues and outcomes in adulthood. We describe here 11 adults with features of VACTERL association ascertained through our research study on the condition. In our cohort of adult patients, approximately 25\% of medically significant malformations that are component features of VACTERL association, including 40\% of vertebral, 50\% of cardiac, and 50\% of renal anomalies, were not identified during childhood. Additionally, medical sequelae of many of the primary malformations identified in infancy or early childhood persist or are first reported in adulthood. These sequelae can involve challenging medical and surgical management in adulthood. As most adults with VACTERL association are not specifically followed for VACTERL-related issues, a more uniform diagnostic work-up and a low threshold for investigation of medical sequelae of the primary disorder may enhance the quality of clinical management in these patients.},
	language = {eng},
	number = {1},
	journal = {European Journal of Medical Genetics},
	author = {Raam, Manu S. and Pineda-Alvarez, Daniel E. and Hadley, Donald W. and Solomon, Benjamin D.},
	year = {2011},
	keywords = {Abnormalities, Multiple, Adult, Anal Canal, Cohort Studies, Esophagus, Female, Heart Defects, Congenital, Humans, Kidney, Limb Deformities, Congenital, Male, Middle Aged, Spine, Time Factors, Trachea},
	pages = {34--41},
}

VACTERL association involves the presence of specific congenital, multi-organ malformations that tend to co-occur. Clinical and research efforts typically center on pediatric patients, and there is a scarcity of information in the literature regarding VACTERL-related issues and outcomes in adulthood. We describe here 11 adults with features of VACTERL association ascertained through our research study on the condition. In our cohort of adult patients, approximately 25% of medically significant malformations that are component features of VACTERL association, including 40% of vertebral, 50% of cardiac, and 50% of renal anomalies, were not identified during childhood. Additionally, medical sequelae of many of the primary malformations identified in infancy or early childhood persist or are first reported in adulthood. These sequelae can involve challenging medical and surgical management in adulthood. As most adults with VACTERL association are not specifically followed for VACTERL-related issues, a more uniform diagnostic work-up and a low threshold for investigation of medical sequelae of the primary disorder may enhance the quality of clinical management in these patients.

@article{szavay_perioperative_2011,
	title = {Perioperative outcome of patients with esophageal atresia and tracheo-esophageal fistula undergoing open versus thoracoscopic surgery},
	volume = {21},
	issn = {1557-9034},
	doi = {10.1089/lap.2010.0349},
	abstract = {INTRODUCTION: Thoracoscopic approach for repair of esophageal atresia (EA) and tracheo-esophageal fistula (TEF) has become a standard procedure in many pediatric surgical centers. Thoracoscopic surgery in a newborn is demanding from both the surgeon and the patient. The potential benefits for the newborn are still discussed by neonatologists, pediatric intensive care physicians, and also parents. The aim of our investigation was to clearly define perioperative outcome and complication rates in children undergoing thoracoscopic versus open surgery for EA and TEF repair.
PATIENTS AND METHODS: We reviewed the records of 68 newborns undergoing surgery for EA and TEF between March 2002 and February 2010. Patient data of open versus thoracoscopic approach were compared regarding operating time, intraoperative as well as postoperative pCO(2)max values, postoperative ventilation time, and complications. Specific patient data are reported with the median and range. Data analysis was done with the JMP(®) 7.0.2 statistical software (SAS Institute, Cary, NC).
RESULTS: For the 68 patients, the mean gestational age was 35 weeks (28-41), the median birth weight was 2720 g (1500-3510 g) in the thoracoscopic group and 2090 g (780-3340 g) in the open group. There were 36 girls and 32 boys. Thirty-two children had associated anomalies. Twenty-five children were undergoing a thoracoscopic procedure. In 8 cases, the operation was converted to open thoracotomy. Another 32 children received a thoracotomy. In 11 newborns, a cervical esophagostomy was performed because of long-gap EA and these patients were excluded from the study. Operating time was 141 minutes (77-201 minutes) in the thoracoscopic group and 106 minutes (48-264 minutes) in the thoracotomy group, with significant difference (P=.014). Values of pCO(2)max during operation were 62 mm Hg (34-101 mm Hg) in the thoracoscopic group and 48 mm Hg (28-89 mm Hg) in the open group, with significant difference (P=.014). Postoperative ventilation time was 3 days (1-51 days) in all groups, with no significant difference (P=.79). Early complications were noticed in 9 children undergoing thoracoscopy and in 8 patients of the thoracotomy group, again with no significant difference (P{\textgreater}.05).
CONCLUSION: Thoracoscopic repair of EA with TEF is justified because of a comparable perioperative outcome to open surgery, competitive operating times, decreased trauma to the thoracic cavity, and improved cosmesis despite skeptical considerations. Complication rates are not higher than in children operated on through a thoracotomy. However, a learning curve has to be taken into account and large experience in minimal invasive surgery is mandatory for this procedure. Larger series have to be expected for a more objective evaluation of perioperative as well as long-term outcomes. To our opinion, the thoracoscopic approach appears to be favorable and could be a future standard.},
	language = {eng},
	number = {5},
	journal = {Journal of Laparoendoscopic \& Advanced Surgical Techniques. Part A},
	author = {Szavay, Philipp O. and Zundel, Sabine and Blumenstock, Gunnar and Kirschner, Hans Joachim and Luithle, Tobias and Girisch, Monika and Luenig, Holger and Fuchs, Joerg},
	month = jun,
	year = {2011},
	keywords = {Digestive System Surgical Procedures, Esophageal Atresia, Female, Humans, Infant, Newborn, Male, Retrospective Studies, Thoracoscopy, Tracheoesophageal Fistula, Treatment Outcome},
	pages = {439--443},
}

INTRODUCTION: Thoracoscopic approach for repair of esophageal atresia (EA) and tracheo-esophageal fistula (TEF) has become a standard procedure in many pediatric surgical centers. Thoracoscopic surgery in a newborn is demanding from both the surgeon and the patient. The potential benefits for the newborn are still discussed by neonatologists, pediatric intensive care physicians, and also parents. The aim of our investigation was to clearly define perioperative outcome and complication rates in children undergoing thoracoscopic versus open surgery for EA and TEF repair. PATIENTS AND METHODS: We reviewed the records of 68 newborns undergoing surgery for EA and TEF between March 2002 and February 2010. Patient data of open versus thoracoscopic approach were compared regarding operating time, intraoperative as well as postoperative pCO(2)max values, postoperative ventilation time, and complications. Specific patient data are reported with the median and range. Data analysis was done with the JMP(®) 7.0.2 statistical software (SAS Institute, Cary, NC). RESULTS: For the 68 patients, the mean gestational age was 35 weeks (28-41), the median birth weight was 2720 g (1500-3510 g) in the thoracoscopic group and 2090 g (780-3340 g) in the open group. There were 36 girls and 32 boys. Thirty-two children had associated anomalies. Twenty-five children were undergoing a thoracoscopic procedure. In 8 cases, the operation was converted to open thoracotomy. Another 32 children received a thoracotomy. In 11 newborns, a cervical esophagostomy was performed because of long-gap EA and these patients were excluded from the study. Operating time was 141 minutes (77-201 minutes) in the thoracoscopic group and 106 minutes (48-264 minutes) in the thoracotomy group, with significant difference (P=.014). Values of pCO(2)max during operation were 62 mm Hg (34-101 mm Hg) in the thoracoscopic group and 48 mm Hg (28-89 mm Hg) in the open group, with significant difference (P=.014). Postoperative ventilation time was 3 days (1-51 days) in all groups, with no significant difference (P=.79). Early complications were noticed in 9 children undergoing thoracoscopy and in 8 patients of the thoracotomy group, again with no significant difference (P\textgreater.05). CONCLUSION: Thoracoscopic repair of EA with TEF is justified because of a comparable perioperative outcome to open surgery, competitive operating times, decreased trauma to the thoracic cavity, and improved cosmesis despite skeptical considerations. Complication rates are not higher than in children operated on through a thoracotomy. However, a learning curve has to be taken into account and large experience in minimal invasive surgery is mandatory for this procedure. Larger series have to be expected for a more objective evaluation of perioperative as well as long-term outcomes. To our opinion, the thoracoscopic approach appears to be favorable and could be a future standard.

@article{burford_repair_2011,
	title = {Repair of esophageal atresia with tracheoesophageal fistula via thoracotomy: a contemporary series},
	volume = {202},
	issn = {1879-1883},
	shorttitle = {Repair of esophageal atresia with tracheoesophageal fistula via thoracotomy},
	doi = {10.1016/j.amjsurg.2010.09.035},
	abstract = {BACKGROUND: A recent series detailing thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) reported lower complication rates compared with historic controls. This study provides a contemporary cohort of patients repaired via thoracotomy for comparison with the recent large multi-institutional thoracoscopic series.
METHODS: Records of patients with EA/TEF between 1993 and 2008 were reviewed. Attention was focused on demographics and complications including anastomotic leak, recurrent fistulae, stricture formation, and need for fundoplication.
RESULTS: Seventy-two patients underwent repair of EA/TEF via thoracotomy. Complication rates in the current series compared with the thoracoscopic series were anastomotic leak, 2.7\% versus 7.6\%; recurrent fistulae, 2.7\% versus 1.9\%; stricture, 5.5\% versus 3.8\%; and need for fundoplication, 12\% versus 24\%. Differences in complication rates did not reach statistical significance. Two children in this cohort developed mild scoliosis attributed to congenital vertebral anomalies, neither of whom required intervention.
CONCLUSIONS: Thoracoscopic repair of EA/TEF yielded complication rates similar to this contemporary series; however, trends toward increased anastomotic leaks and greater need for fundoplication were noted. No musculoskeletal sequelae were directly attributable to thoracotomy.},
	language = {eng},
	number = {2},
	journal = {American Journal of Surgery},
	author = {Burford, Jeffrey M. and Dassinger, Melvin S. and Copeland, Daniel R. and Keller, Jennifer E. and Smith, Samuel D.},
	month = aug,
	year = {2011},
	keywords = {Abnormalities, Multiple, Esophageal Atresia, Female, Humans, Infant, Infant, Newborn, Male, Retrospective Studies, Thoracoscopy, Thoracotomy, Tracheoesophageal Fistula, Treatment Outcome},
	pages = {203--206},
}

BACKGROUND: A recent series detailing thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) reported lower complication rates compared with historic controls. This study provides a contemporary cohort of patients repaired via thoracotomy for comparison with the recent large multi-institutional thoracoscopic series. METHODS: Records of patients with EA/TEF between 1993 and 2008 were reviewed. Attention was focused on demographics and complications including anastomotic leak, recurrent fistulae, stricture formation, and need for fundoplication. RESULTS: Seventy-two patients underwent repair of EA/TEF via thoracotomy. Complication rates in the current series compared with the thoracoscopic series were anastomotic leak, 2.7% versus 7.6%; recurrent fistulae, 2.7% versus 1.9%; stricture, 5.5% versus 3.8%; and need for fundoplication, 12% versus 24%. Differences in complication rates did not reach statistical significance. Two children in this cohort developed mild scoliosis attributed to congenital vertebral anomalies, neither of whom required intervention. CONCLUSIONS: Thoracoscopic repair of EA/TEF yielded complication rates similar to this contemporary series; however, trends toward increased anastomotic leaks and greater need for fundoplication were noted. No musculoskeletal sequelae were directly attributable to thoracotomy.

@article{de_cunto_safety_2011,
	title = {Safety and efficacy of high-dose acarbose treatment for dumping syndrome},
	volume = {53},
	issn = {1536-4801},
	doi = {10.1097/MPG.0b013e31820ae6d1},
	abstract = {Dumping syndrome (DS) is a complication of Nissen fundoplication. Dietary strategies can ameliorate symptoms, but this approach is not always foolproof. Limited evidence reports the efficacy of acarbose for children who are unresponsive to feeding manipulations. We report 8 patients with DS aged between 7 and 24 months. In 4 of 8 nutritional strategies failed, and acarbose treatment was started. The initial dose was 25 mg for meals, and increased until postprandial glucose was stable. In 3 of 4 children the final dose was higher than previously reported, without adverse effects. Acarbose is useful to treat DS in cases of failure of dietary strategies.},
	language = {eng},
	number = {1},
	journal = {Journal of Pediatric Gastroenterology and Nutrition},
	author = {De Cunto, Angela and Barbi, Egidio and Minen, Federico and Ventura, Alessandro},
	month = jul,
	year = {2011},
	keywords = {Acarbose, Child, Preschool, Dumping Syndrome, Female, Humans, Hyperglycemia, Hypoglycemic Agents, Infant, Male, Postprandial Period, Treatment Outcome},
	pages = {113--114},
}

Dumping syndrome (DS) is a complication of Nissen fundoplication. Dietary strategies can ameliorate symptoms, but this approach is not always foolproof. Limited evidence reports the efficacy of acarbose for children who are unresponsive to feeding manipulations. We report 8 patients with DS aged between 7 and 24 months. In 4 of 8 nutritional strategies failed, and acarbose treatment was started. The initial dose was 25 mg for meals, and increased until postprandial glucose was stable. In 3 of 4 children the final dose was higher than previously reported, without adverse effects. Acarbose is useful to treat DS in cases of failure of dietary strategies.

@article{li_13-year_2010,
	title = {13-year follow-up of a prospective comparison of the long-term clinical efficacy of temporary self-expanding metallic stents and pneumatic dilatation for the treatment of achalasia in 120 patients},
	volume = {195},
	issn = {1546-3141},
	doi = {10.2214/AJR.10.4407},
	abstract = {OBJECTIVE: The purpose of this article is to compare the efficacy of self-expanding metallic stents and pneumatic dilation for the long-term clinical treatment of achalasia.
SUBJECTS AND METHODS: Patients diagnosed with achalasia (n = 120) were allocated for treatment with pneumatic dilation (n = 30; group A) or a temporary self-expanding metallic stent with a diameter of 20 mm (n = 30; group B), 25 mm (n = 30; group C), or 30 mm (n = 30; group D). Data on clinical symptoms, complications, and long-term clinical outcomes were collected, and follow-up was performed at 6 months and at 1, 3-5, 5-8, 8-10, and more than 10 years after surgery.
RESULTS: Pneumatic dilation and stent placement were technically successful in all patients. The follow-up at more than 10 years revealed that the clinical remission rate in group D (83.3\%) was higher than that in groups A (0\%), B (0\%), and C (28.6\%), and the overall cumulative clinical failure rate in group D (13\%) was lower than that in groups A (76.7\%), B (53.3\%), and C (26.7\%). Patients in group D exhibited reduced dysphagia scores and lower esophageal sphincter pressures and had normal levels of barium height and width during the follow-up periods, whereas these markers increased with time in the other groups. The duration of primary patency in group D was also longer than that in groups A, B, and C.
CONCLUSION: A temporary self-expanding metallic stent with a diameter of 30 mm has superior clinical efficacy for the treatment of achalasia compared with pneumatic dilation or self-expanding metallic stents with diameters of 20 or 25 mm.},
	language = {eng},
	number = {6},
	journal = {AJR. American journal of roentgenology},
	author = {Li, Yong-Dong and Tang, Guang-Yu and Cheng, Ying-Sheng and Chen, Ni-Wei and Chen, Wei-Xiong and Zhao, Jun-Gong},
	month = dec,
	year = {2010},
	keywords = {Adolescent, Adult, Aged, Aged, 80 and over, Chi-Square Distribution, Child, Contrast Media, Device Removal, Dilatation, Esophageal Achalasia, Female, Follow-Up Studies, Gastroscopy, Humans, Iohexol, Male, Metals, Middle Aged, Pilot Projects, Prospective Studies, Radiography, Interventional, Statistics, Nonparametric, Stents, Treatment Outcome},
	pages = {1429--1437},
}

OBJECTIVE: The purpose of this article is to compare the efficacy of self-expanding metallic stents and pneumatic dilation for the long-term clinical treatment of achalasia. SUBJECTS AND METHODS: Patients diagnosed with achalasia (n = 120) were allocated for treatment with pneumatic dilation (n = 30; group A) or a temporary self-expanding metallic stent with a diameter of 20 mm (n = 30; group B), 25 mm (n = 30; group C), or 30 mm (n = 30; group D). Data on clinical symptoms, complications, and long-term clinical outcomes were collected, and follow-up was performed at 6 months and at 1, 3-5, 5-8, 8-10, and more than 10 years after surgery. RESULTS: Pneumatic dilation and stent placement were technically successful in all patients. The follow-up at more than 10 years revealed that the clinical remission rate in group D (83.3%) was higher than that in groups A (0%), B (0%), and C (28.6%), and the overall cumulative clinical failure rate in group D (13%) was lower than that in groups A (76.7%), B (53.3%), and C (26.7%). Patients in group D exhibited reduced dysphagia scores and lower esophageal sphincter pressures and had normal levels of barium height and width during the follow-up periods, whereas these markers increased with time in the other groups. The duration of primary patency in group D was also longer than that in groups A, B, and C. CONCLUSION: A temporary self-expanding metallic stent with a diameter of 30 mm has superior clinical efficacy for the treatment of achalasia compared with pneumatic dilation or self-expanding metallic stents with diameters of 20 or 25 mm.

@article{burgos_colonic_2010,
	title = {Colonic interposition for esophageal replacement in children remains a good choice: 33-year median follow-up of 65 patients},
	volume = {45},
	issn = {1531-5037},
	shorttitle = {Colonic interposition for esophageal replacement in children remains a good choice},
	doi = {10.1016/j.jpedsurg.2009.10.065},
	abstract = {INTRODUCTION: Gastric pull-up has become the predominant technique for esophageal replacement because of allegedly deficient results of colon grafts. This retrospective study examines the long-term results in a large series of colonic interpositions.
PATIENTS AND METHOD: One hundred six children (median, 2.9 years; range, 0.32-15 years) had their esophagus replaced between 1965 and 2008, of which 96 had colon grafts. Those survivors who were 18 years and older were contacted and, if willing, interviewed, examined, and had their functional ability rated using the Karnofsky index.
RESULTS: Ninety-six children had undergone a colon graft, and of these, 9 (9.3\%) died. There were 65 long-term survivors whose indications for surgery included caustic injury (n = 32), failed tracheoesophageal fistula repair (n = 16), pure esophageal atresia (n = 14), and others (n = 3). The graft was either retrosternal (n = 49) or mediastinal (n = 16 patients). Twelve patients were unavailable for long-term assessment. After a median follow-up of 33.3 (11-41) years, 23 (43\%) of 53 individuals experienced mild symptoms of reflux; scoliosis, 12 (22\%) of 53, and/or other complications, 15 (27\%) of 53 required further surgery. Thirty-two patients (60\%) with Karnofsky indexes of 80\% or higher felt healthy, 19 (36\%) had mild life-style limitations (index, 40\%-80\%), and 2 had indexes less than 40\%. Most patients live normal lives.
CONCLUSIONS: Colon conduits restored gastrointestinal continuity with limited mortality and considerable morbidity but good functional outcome and health perception in the long-term. Our study suggests that colon grafts are no worse than gastric pull-ups in the long-term.},
	language = {eng},
	number = {2},
	journal = {Journal of Pediatric Surgery},
	author = {Burgos, Laura and Barrena, Saturnino and Andrés, Ane M. and Martínez, Leopoldo and Hernández, Francisco and Olivares, Pedro and Lassaletta, Luis and Tovar, Juan A.},
	month = feb,
	year = {2010},
	keywords = {Adult, Anastomosis, Surgical, Child, Child, Preschool, Colon, Digestive System Surgical Procedures, Disease-Free Survival, Esophageal Atresia, Esophagoplasty, Esophagus, Female, Humans, Infant, Karnofsky Performance Status, Longitudinal Studies, Male, Quality of Life, Retrospective Studies, Trachea, Tracheoesophageal Fistula, Treatment Outcome, Young Adult},
	pages = {341--345},
}

INTRODUCTION: Gastric pull-up has become the predominant technique for esophageal replacement because of allegedly deficient results of colon grafts. This retrospective study examines the long-term results in a large series of colonic interpositions. PATIENTS AND METHOD: One hundred six children (median, 2.9 years; range, 0.32-15 years) had their esophagus replaced between 1965 and 2008, of which 96 had colon grafts. Those survivors who were 18 years and older were contacted and, if willing, interviewed, examined, and had their functional ability rated using the Karnofsky index. RESULTS: Ninety-six children had undergone a colon graft, and of these, 9 (9.3%) died. There were 65 long-term survivors whose indications for surgery included caustic injury (n = 32), failed tracheoesophageal fistula repair (n = 16), pure esophageal atresia (n = 14), and others (n = 3). The graft was either retrosternal (n = 49) or mediastinal (n = 16 patients). Twelve patients were unavailable for long-term assessment. After a median follow-up of 33.3 (11-41) years, 23 (43%) of 53 individuals experienced mild symptoms of reflux; scoliosis, 12 (22%) of 53, and/or other complications, 15 (27%) of 53 required further surgery. Thirty-two patients (60%) with Karnofsky indexes of 80% or higher felt healthy, 19 (36%) had mild life-style limitations (index, 40%-80%), and 2 had indexes less than 40%. Most patients live normal lives. CONCLUSIONS: Colon conduits restored gastrointestinal continuity with limited mortality and considerable morbidity but good functional outcome and health perception in the long-term. Our study suggests that colon grafts are no worse than gastric pull-ups in the long-term.

@article{yoo_congenital_2010,
	title = {Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features},
	volume = {40},
	issn = {1432-1998},
	shorttitle = {Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula},
	doi = {10.1007/s00247-010-1603-0},
	abstract = {BACKGROUND: Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF.
OBJECTIVE: To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula.
MATERIALS AND METHODS: We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children.
RESULTS: CES was diagnosed in 22 of 187 EA/TEF children (12\%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45\%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45\%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87\%) or mid-esophagus (n = 3, 13\%). The degree of stenosis was severe (n = 6, 26\%), moderate (n = 10, 43\%), or mild (n = 7, 30\%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months.
CONCLUSION: Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed.},
	language = {eng},
	number = {8},
	journal = {Pediatric Radiology},
	author = {Yoo, Hye Jin and Kim, Woo Sun and Cheon, Jung-Eun and Yoo, So-Young and Park, Kwi-Won and Jung, Sung-Eun and Shin, Su-Mi and Kim, In-One and Yeon, Kyung Mo},
	month = aug,
	year = {2010},
	keywords = {Child, Digestive System Abnormalities, Esophageal Atresia, Esophageal Stenosis, Female, Humans, Longitudinal Studies, Male, Radiography, Retrospective Studies, Treatment Outcome},
	pages = {1353--1359},
}

BACKGROUND: Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF. OBJECTIVE: To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula. MATERIALS AND METHODS: We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children. RESULTS: CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months. CONCLUSION: Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed.

@article{sistonen_esophageal_2010,
	title = {Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula: a population-based long-term follow-up},
	volume = {251},
	issn = {1528-1140},
	shorttitle = {Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula},
	doi = {10.1097/SLA.0b013e3181c9b613},
	abstract = {OBJECTIVE: We assessed esophageal morbidity and relationships between surgical complications, symptoms, endoscopic findings, immunohistochemistry, and esophageal motility in adults with repaired esophageal atresia (EA).
SUMMARY OF BACKGROUND DATA: There exist no previous population-based long-term follow-up studies on EA.
METHODS: Participants were interviewed, and they underwent esophageal endoscopy and manometry. Matched control subjects (n = 287) served as controls.
RESULTS: A total of 101 (42\%) individuals representative of the entire study population participated at a mean age of 36 years (range, 21-57). Symptomatic gastroesophageal reflux had occurred in 34\% and dysphagia in 85\% of the patients and in 8\% and 2\% of the controls (P {\textless} 0.001 for both). Endoscopic findings included hiatal hernia (28\%), Barrett's esophagus (11\%), esophagitis (8\%), and anastomotic stricture (8\%). Immunohistochemistry revealed esophagitis in 25\%, and CDX2-positive columnar epithelial metaplasia in 21\%, with additional goblet cells and MUC2 positivity in 6\%. Gastroesophageal reflux and dysphagia were equally common in individuals with normal histology, esophagitis, or epithelial metaplasia. Manometry demonstrated nonpropagating peristalsis in 80\% of the patients, and low distal wave amplitudes of the esophagus in all the changes being significantly worse in those with epithelial metaplasia (P {\textless} or = 0.022 metaplasia vs. esophagitis/normal). Anastomotic complications (odds ratio [OR]: 8.6-24, 95\% confidence interval [CI]: 1.7-260, P = 0.011-0.008), age (OR: 20, 95\% CI: 1.3-310, P = 0.034), low distal esophageal body pressure (OR: 2.6, 95\% CI: 0.7-10, P = 0.002), and defective esophageal peristalsis (OR: 2.2, 95\% CI: 0.4-11, P = 0.014) predicted development of epithelial metaplasia.
CONCLUSIONS: Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict development of epithelial metaplasia after repair of EA.},
	language = {eng},
	number = {6},
	journal = {Annals of Surgery},
	author = {Sistonen, Saara J. and Koivusalo, Antti and Nieminen, Urpo and Lindahl, Harry and Lohi, Jouko and Kero, Mia and Kärkkäinen, Päivi A. and Färkkilä, Martti A. and Sarna, Seppo and Rintala, Risto J. and Pakarinen, Mikko P.},
	month = jun,
	year = {2010},
	keywords = {Adult, Barrett Esophagus, Endoscopy, Gastrointestinal, Esophageal Atresia, Esophageal Diseases, Esophagitis, Esophagus, Female, Follow-Up Studies, Gastroesophageal Reflux, Humans, Male, Manometry, Middle Aged, Tracheoesophageal Fistula},
	pages = {1167--1173},
}

OBJECTIVE: We assessed esophageal morbidity and relationships between surgical complications, symptoms, endoscopic findings, immunohistochemistry, and esophageal motility in adults with repaired esophageal atresia (EA). SUMMARY OF BACKGROUND DATA: There exist no previous population-based long-term follow-up studies on EA. METHODS: Participants were interviewed, and they underwent esophageal endoscopy and manometry. Matched control subjects (n = 287) served as controls. RESULTS: A total of 101 (42%) individuals representative of the entire study population participated at a mean age of 36 years (range, 21-57). Symptomatic gastroesophageal reflux had occurred in 34% and dysphagia in 85% of the patients and in 8% and 2% of the controls (P \textless 0.001 for both). Endoscopic findings included hiatal hernia (28%), Barrett's esophagus (11%), esophagitis (8%), and anastomotic stricture (8%). Immunohistochemistry revealed esophagitis in 25%, and CDX2-positive columnar epithelial metaplasia in 21%, with additional goblet cells and MUC2 positivity in 6%. Gastroesophageal reflux and dysphagia were equally common in individuals with normal histology, esophagitis, or epithelial metaplasia. Manometry demonstrated nonpropagating peristalsis in 80% of the patients, and low distal wave amplitudes of the esophagus in all the changes being significantly worse in those with epithelial metaplasia (P \textless or = 0.022 metaplasia vs. esophagitis/normal). Anastomotic complications (odds ratio [OR]: 8.6-24, 95% confidence interval [CI]: 1.7-260, P = 0.011-0.008), age (OR: 20, 95% CI: 1.3-310, P = 0.034), low distal esophageal body pressure (OR: 2.6, 95% CI: 0.7-10, P = 0.002), and defective esophageal peristalsis (OR: 2.2, 95% CI: 0.4-11, P = 0.014) predicted development of epithelial metaplasia. CONCLUSIONS: Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict development of epithelial metaplasia after repair of EA.

@article{peetsold_health-related_2010,
	title = {Health-related quality of life and its determinants in children and adolescents born with oesophageal atresia},
	volume = {99},
	issn = {1651-2227},
	doi = {10.1111/j.1651-2227.2009.01579.x},
	abstract = {AIM: Following surgical correction in the neonatal period, patients born with oesophageal atresia have significant co-morbidity, particularly in childhood. This study evaluates health-related quality of life and its determinants such as concomitant anomalies and the presence of respiratory and/or gastro-intestinal symptoms 6-18 years after repair of oesophageal atresia.
METHODS: Parents of 24 patients with oesophageal atresia completed the child health questionnaire for parents and 37 patients completed the child form. Gastro-intestinal symptoms were assessed by a validated standardized reflux questionnaire. Results were compared with a healthy reference population.
RESULTS: Parents as well as patients themselves scored significantly lower on the domain general health perception. According to parents, general health perception was negatively affected by age at follow-up and concomitant anomalies. Patients reported that reflux symptoms reduced general health perception.
CONCLUSION: In this first study describing health-related quality of life in children and adolescents born with oesophageal atresia, we demonstrated that general health remains impaired because of a high incidence of concomitant anomalies and gastrointestinal symptoms in patients with oesophageal atresia when compared with the healthy reference population.},
	language = {eng},
	number = {3},
	journal = {Acta Paediatrica (Oslo, Norway: 1992)},
	author = {Peetsold, M. G. and Heij, H. A. and Deurloo, J. A. and Gemke, R. J. B. J.},
	month = mar,
	year = {2010},
	keywords = {Adolescent, Case-Control Studies, Child, Comorbidity, Esophageal Atresia, Female, Follow-Up Studies, Gastroesophageal Reflux, Health Status, Health Surveys, Humans, Male, Outcome Assessment, Health Care, Parents, Quality of Life, Regression Analysis, Surveys and Questionnaires},
	pages = {411--417},
}

AIM: Following surgical correction in the neonatal period, patients born with oesophageal atresia have significant co-morbidity, particularly in childhood. This study evaluates health-related quality of life and its determinants such as concomitant anomalies and the presence of respiratory and/or gastro-intestinal symptoms 6-18 years after repair of oesophageal atresia. METHODS: Parents of 24 patients with oesophageal atresia completed the child health questionnaire for parents and 37 patients completed the child form. Gastro-intestinal symptoms were assessed by a validated standardized reflux questionnaire. Results were compared with a healthy reference population. RESULTS: Parents as well as patients themselves scored significantly lower on the domain general health perception. According to parents, general health perception was negatively affected by age at follow-up and concomitant anomalies. Patients reported that reflux symptoms reduced general health perception. CONCLUSION: In this first study describing health-related quality of life in children and adolescents born with oesophageal atresia, we demonstrated that general health remains impaired because of a high incidence of concomitant anomalies and gastrointestinal symptoms in patients with oesophageal atresia when compared with the healthy reference population.

@article{tannuri_laparoscopic_2010,
	title = {Laparoscopic extended cardiomyotomy in children: an effective procedure for the treatment of esophageal achalasia},
	volume = {45},
	issn = {1531-5037},
	shorttitle = {Laparoscopic extended cardiomyotomy in children},
	doi = {10.1016/j.jpedsurg.2009.08.023},
	abstract = {PURPOSE: Achalasia of the esophagus is characterized by aperistalsis and incomplete relaxation of the lower esophageal sphincter in response to swallowing. The objective of the present study is to present the experience of a modified Heller myotomy via a laparoscopic approach for the treatment of children who had this condition.
METHODS: A retrospective review of medical records of all patients who underwent this procedure from 2000 to 2009 was performed. The procedure consisted of an extended esophagomyotomy beginning on the lower part of the lower esophageal sphincter and continuing 5 to 6 cm above on the lower third of the esophagus, and then extended 3 to 4 cm below to the stomach, associated with an anterior 180-degree hemi-fundoplication according to Dor's technique.
RESULTS: Fifteen patients were included in the study. There were 8 female and 7 male patients. Mean operating time was 190 minutes with no intraoperative complications and 1 conversion to open surgery because of difficulty in dissecting an inflamed distal esophagus. In a mean follow-up period of 32.3 months, 2 patients had recurrence of mild dysphagia that disappeared spontaneously, and 1 required a single botulinum toxin injection with complete resolution of symptoms.
CONCLUSION: We conclude that the laparoscopic extended Heller myotomy with Dor fundoplication is a safe and effective method for the treatment for achalasia in the pediatric population even in advanced cases.},
	language = {eng},
	number = {7},
	journal = {Journal of Pediatric Surgery},
	author = {Tannuri, Ana Cristina Aoun and Tannuri, Uenis and Velhote, Manoel Carlos Prieto and Romão, Rodrigo Luiz Pinto},
	month = jul,
	year = {2010},
	keywords = {Adolescent, Cardia, Child, Esophageal Achalasia, Female, Fundoplication, Humans, Laparoscopy, Male, Minimally Invasive Surgical Procedures, Retrospective Studies},
	pages = {1463--1466},
}

PURPOSE: Achalasia of the esophagus is characterized by aperistalsis and incomplete relaxation of the lower esophageal sphincter in response to swallowing. The objective of the present study is to present the experience of a modified Heller myotomy via a laparoscopic approach for the treatment of children who had this condition. METHODS: A retrospective review of medical records of all patients who underwent this procedure from 2000 to 2009 was performed. The procedure consisted of an extended esophagomyotomy beginning on the lower part of the lower esophageal sphincter and continuing 5 to 6 cm above on the lower third of the esophagus, and then extended 3 to 4 cm below to the stomach, associated with an anterior 180-degree hemi-fundoplication according to Dor's technique. RESULTS: Fifteen patients were included in the study. There were 8 female and 7 male patients. Mean operating time was 190 minutes with no intraoperative complications and 1 conversion to open surgery because of difficulty in dissecting an inflamed distal esophagus. In a mean follow-up period of 32.3 months, 2 patients had recurrence of mild dysphagia that disappeared spontaneously, and 1 required a single botulinum toxin injection with complete resolution of symptoms. CONCLUSION: We conclude that the laparoscopic extended Heller myotomy with Dor fundoplication is a safe and effective method for the treatment for achalasia in the pediatric population even in advanced cases.

@article{leeuwenburgh_long-term_2010,
	title = {Long-term esophageal cancer risk in patients with primary achalasia: a prospective study},
	volume = {105},
	issn = {1572-0241},
	shorttitle = {Long-term esophageal cancer risk in patients with primary achalasia},
	doi = {10.1038/ajg.2010.263},
	abstract = {OBJECTIVES: Achalasia patients are considered at increased risk for esophageal cancer, but the reported relative risks vary. Identification of this risk is relevant for patient management. We performed a prospective evaluation of the esophageal cancer risk in a large cohort of achalasia patients with long-term follow-up.
METHODS: Between 1975 and 2006, all patients diagnosed with primary achalasia in our hospital were treated and followed by the same protocol. After graded pneumatic dilatation, all patients were offered a fixed surveillance protocol including gastrointestinal endoscopy with esophageal biopsy sampling.
RESULTS: We surveyed a cohort of 448 achalasia patients (218 men, mean age 51 years at diagnosis, range 4-92 years) for a mean follow-up of 9.6 years (range 0.1-32). Overall, 15 (3.3\%) patients (10 men) developed esophageal cancer (annual incidence 0.34 (95\% confidence interval 0.20-0.56)). The mean age at cancer diagnosis was 71 years (range 36-90) after a mean of 11 years (range 2-23) following initial presentation, and a mean of 24 years (range 10-43) after symptom onset. The relative hazard rate of esophageal cancer was 28 (confidence interval 17-46) compared with an age- and sex-identical population in the same timeframe. Five patients received a potential curative treatment.
CONCLUSIONS: Although the gastro-esophageal cancer risk in patients with longstanding achalasia is much higher than in the general population, the absolute risk is rather low. Despite structured endoscopical surveillance, most neoplastic lesions remain undetected until an advanced stage. Efforts should be made to identify high-risk groups and develop adequate surveillance strategies.},
	language = {eng},
	number = {10},
	journal = {The American Journal of Gastroenterology},
	author = {Leeuwenburgh, I. and Scholten, P. and Alderliesten, J. and Tilanus, H. W. and Looman, C. W. N. and Steijerberg, E. W. and Kuipers, E. J.},
	month = oct,
	year = {2010},
	keywords = {Adolescent, Adult, Aged, Aged, 80 and over, Catheterization, Child, Child, Preschool, Cohort Studies, Esophageal Achalasia, Esophageal Neoplasms, Female, Follow-Up Studies, Humans, Incidence, Male, Middle Aged, Prospective Studies, Risk, Risk Assessment},
	pages = {2144--2149},
}

OBJECTIVES: Achalasia patients are considered at increased risk for esophageal cancer, but the reported relative risks vary. Identification of this risk is relevant for patient management. We performed a prospective evaluation of the esophageal cancer risk in a large cohort of achalasia patients with long-term follow-up. METHODS: Between 1975 and 2006, all patients diagnosed with primary achalasia in our hospital were treated and followed by the same protocol. After graded pneumatic dilatation, all patients were offered a fixed surveillance protocol including gastrointestinal endoscopy with esophageal biopsy sampling. RESULTS: We surveyed a cohort of 448 achalasia patients (218 men, mean age 51 years at diagnosis, range 4-92 years) for a mean follow-up of 9.6 years (range 0.1-32). Overall, 15 (3.3%) patients (10 men) developed esophageal cancer (annual incidence 0.34 (95% confidence interval 0.20-0.56)). The mean age at cancer diagnosis was 71 years (range 36-90) after a mean of 11 years (range 2-23) following initial presentation, and a mean of 24 years (range 10-43) after symptom onset. The relative hazard rate of esophageal cancer was 28 (confidence interval 17-46) compared with an age- and sex-identical population in the same timeframe. Five patients received a potential curative treatment. CONCLUSIONS: Although the gastro-esophageal cancer risk in patients with longstanding achalasia is much higher than in the general population, the absolute risk is rather low. Despite structured endoscopical surveillance, most neoplastic lesions remain undetected until an advanced stage. Efforts should be made to identify high-risk groups and develop adequate surveillance strategies.

@article{esquibies_pulmonary_2010,
	title = {Pulmonary squamous cell carcinoma associated with repaired congenital tracheoesophageal fistula and esophageal atresia},
	volume = {45},
	issn = {1099-0496},
	doi = {10.1002/ppul.21148},
	abstract = {We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood.},
	language = {eng},
	number = {2},
	journal = {Pediatric Pulmonology},
	author = {Esquibies, Americo E. and Zambrano, Eduardo and Ziai, James and Kesebir, Deniz and Touloukian, Robert J. and Egan, Marie E. and Reyes-Múgica, Miguel and Bazzy-Asaad, Alia},
	month = feb,
	year = {2010},
	keywords = {Abnormalities, Multiple, Carcinoma, Squamous Cell, Esophageal Atresia, Fatal Outcome, Humans, Lung Neoplasms, Male, Postoperative Complications, Risk Factors, Tracheoesophageal Fistula, Treatment Outcome, Young Adult},
	pages = {202--204},
}

We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood.

@article{michaud_dumping_2010,
	title = {Dumping syndrome after esophageal atresia repair without antireflux surgery},
	volume = {45},
	issn = {1531-5037},
	url = {https://pubmed.ncbi.nlm.nih.gov/20385264/},
	doi = {10.1016/j.jpedsurg.2010.01.016},
	abstract = {In childhood, the surgical treatment of gastroesophageal reflux is the main cause of dumping syndrome. We report the cases of 2 children with esophageal atresia who presented with dumping syndrome without any precipitating known factors, such as gastroesophageal reflux surgery or associated microgastria. Our data suggest (1) that dumping syndrome can occur after primary anastomosis of esophageal atresia without antireflux surgery and (2) that dumping syndrome should be considered in every child treated surgically for esophageal atresia presenting with digestive symptoms, malaise, failure to thrive, or refusal to eat.},
	language = {eng},
	number = {4},
	journal = {Journal of Pediatric Surgery},
	author = {Michaud, Laurent and Sfeir, Rony and Couttenier, Frédéric and Turck, Dominique and Gottrand, Frédéric},
	month = apr,
	year = {2010},
	keywords = {Anastomosis, Surgical, Dumping Syndrome, Esophageal Atresia, Humans, Infant, Male},
	pages = {E13--15},
}

In childhood, the surgical treatment of gastroesophageal reflux is the main cause of dumping syndrome. We report the cases of 2 children with esophageal atresia who presented with dumping syndrome without any precipitating known factors, such as gastroesophageal reflux surgery or associated microgastria. Our data suggest (1) that dumping syndrome can occur after primary anastomosis of esophageal atresia without antireflux surgery and (2) that dumping syndrome should be considered in every child treated surgically for esophageal atresia presenting with digestive symptoms, malaise, failure to thrive, or refusal to eat.

@article{park_heterotopic_2010,
	title = {Heterotopic pancreas of the esophagus and stomach associated with pure esophageal atresia},
	volume = {45},
	issn = {1531-5037},
	doi = {10.1016/j.jpedsurg.2009.12.018},
	abstract = {Esophageal atresia with or without tracheoeosphageal fistula is a frequent congenital malformation that may be associated with other congenital anomalies. The combination of heterotopic pancreas of the esophagus and pure esophageal atresia, however, is rare. We treated a patient with esophageal atresia without tracheoesophageal fistula (type A) associated with heterotopic pancreas of the esophagus and stomach who developed a gastric perforation.},
	language = {eng},
	number = {3},
	journal = {Journal of Pediatric Surgery},
	author = {Park, Jinyoung},
	month = mar,
	year = {2010},
	keywords = {Abnormalities, Multiple, Choristoma, Digestive System Surgical Procedures, Esophageal Atresia, Esophageal Diseases, Esophagoscopy, Female, Follow-Up Studies, Humans, Immunohistochemistry, Infant, Newborn, Laparotomy, Pancreas, Risk Assessment, Stomach Diseases, Treatment Outcome},
	pages = {E25--27},
}

Esophageal atresia with or without tracheoeosphageal fistula is a frequent congenital malformation that may be associated with other congenital anomalies. The combination of heterotopic pancreas of the esophagus and pure esophageal atresia, however, is rare. We treated a patient with esophageal atresia without tracheoesophageal fistula (type A) associated with heterotopic pancreas of the esophagus and stomach who developed a gastric perforation.

@article{wessels_polyalanine_2010,
	title = {Polyalanine expansion in the {ZIC3} gene leading to {X}-linked heterotaxy with {VACTERL} association: a new polyalanine disorder?},
	volume = {47},
	issn = {1468-6244},
	shorttitle = {Polyalanine expansion in the {ZIC3} gene leading to {X}-linked heterotaxy with {VACTERL} association},
	doi = {10.1136/jmg.2008.060913},
	abstract = {BACKGROUND: The VACTERL association is a non-random association of congenital defects with an unknown aetiology in the majority of patients.
METHODS: A male newborn is reported with features of the VACTERL association, including anal atresia, laryngeal and oesophageal atresia with tracheo-oesophageal fistula, dextroposition of the heart with persistent left superior vena cava, and unilateral multicystic kidney. As the clinical picture of this patient overlaps with that of X-linked heterotaxy caused by ZIC3 mutations, the ZIC3 coding region was sequenced.
RESULTS: In a patient with the VACTERL association a 6-nucleotide insertion was found in the GCC repeat of the ZIC3 gene, which is predicted to expand the amino-terminal polyalanine repeat from 10 to 12 polyalanines. The polyalanine expansion is a novel ZIC3 mutation which was not found in 336 chromosomes from 192 ethnically matched controls. The mutation was also not present in the mother, suggesting it occurred de novo in the patient and is therefore a pathogenetic mutation.
CONCLUSION: It is hypothesized that this novel and de novo polyalanine expansion in ZIC3 contributes to the VACTERL association in this patient. A newborn male is described with features of the VACTERL association, including anal atresia, laryngeal and oesophageal atresia with tracheo-oesophageal fistula, dextroposition of the heart with persistent left superior vena cava, and unilateral multicystic kidney. As the clinical picture of the VACTERL association overlaps with X-linked heterotaxy caused by ZIC3 mutations, the ZIC3 coding region was sequenced, and a 6-nucleotide insertion was found that is predicted to expand the amino-terminal polyalanine repeat from 10 to 12 polyalanines. This novel mutation was not present in the mother, nor in 336 chromosomes from 192 ethnically matched controls. It is hypothesised that this novel and de novo polyalanine expansion in the ZIC3 gene contributes to the VACTERL association in this patient.},
	language = {eng},
	number = {5},
	journal = {Journal of Medical Genetics},
	author = {Wessels, Marja W. and Kuchinka, Brian and Heydanus, Rogier and Smit, Bert J. and Dooijes, Dennis and de Krijger, Ronald R. and Lequin, Maarten H. and de Jong, Elisabeth M. and Husen, Margreet and Willems, Patrick J. and Casey, Brett},
	month = may,
	year = {2010},
	keywords = {Abnormalities, Multiple, Anus, Imperforate, Female, Genes, X-Linked, Genetic Diseases, X-Linked, Heart Defects, Congenital, Homeodomain Proteins, Humans, Infant, Newborn, Male, Peptides, Pregnancy, Transcription Factors, Trinucleotide Repeat Expansion, Zinc Fingers},
	pages = {351--355},
}

BACKGROUND: The VACTERL association is a non-random association of congenital defects with an unknown aetiology in the majority of patients. METHODS: A male newborn is reported with features of the VACTERL association, including anal atresia, laryngeal and oesophageal atresia with tracheo-oesophageal fistula, dextroposition of the heart with persistent left superior vena cava, and unilateral multicystic kidney. As the clinical picture of this patient overlaps with that of X-linked heterotaxy caused by ZIC3 mutations, the ZIC3 coding region was sequenced. RESULTS: In a patient with the VACTERL association a 6-nucleotide insertion was found in the GCC repeat of the ZIC3 gene, which is predicted to expand the amino-terminal polyalanine repeat from 10 to 12 polyalanines. The polyalanine expansion is a novel ZIC3 mutation which was not found in 336 chromosomes from 192 ethnically matched controls. The mutation was also not present in the mother, suggesting it occurred de novo in the patient and is therefore a pathogenetic mutation. CONCLUSION: It is hypothesized that this novel and de novo polyalanine expansion in ZIC3 contributes to the VACTERL association in this patient. A newborn male is described with features of the VACTERL association, including anal atresia, laryngeal and oesophageal atresia with tracheo-oesophageal fistula, dextroposition of the heart with persistent left superior vena cava, and unilateral multicystic kidney. As the clinical picture of the VACTERL association overlaps with X-linked heterotaxy caused by ZIC3 mutations, the ZIC3 coding region was sequenced, and a 6-nucleotide insertion was found that is predicted to expand the amino-terminal polyalanine repeat from 10 to 12 polyalanines. This novel mutation was not present in the mother, nor in 336 chromosomes from 192 ethnically matched controls. It is hypothesised that this novel and de novo polyalanine expansion in the ZIC3 gene contributes to the VACTERL association in this patient.

@article{faugli_mental_2009,
	title = {Mental health and psychosocial functioning in adolescents with esophageal atresia},
	volume = {44},
	issn = {1531-5037},
	doi = {10.1016/j.jpedsurg.2008.09.027},
	abstract = {PURPOSE: We examined mental health and psychosocial functioning in adolescents with esophageal atresia (EA) and searched for predictors of impaired outcome.
METHODS: The study group comprised 21 adolescents with EA and 1 or both parents. A comparison group comprised 36 adolescents from the general population. Mental health, self-esteem, psychosocial functioning, and parental/family functioning were assessed by standardized questionnaires and semistructured interviews. Physical health was assessed by growth and clinical symptoms.
RESULTS: Mental health and psychosocial functioning did not differ from the comparison group. Seven of 21 EA adolescents had special education. Dilatations of esophagus, birth weight, well-being, and maternal psychological distress were prognostic factors predicting mental health. Height, birth weight, well-being, dissociative symptoms, and family strain were prognostic factors predicting psychosocial functioning.
CONCLUSION: Adolescents with EA adjusted well, and mental health and psychosocial functioning did not differ from controls. Dilatations of esophagus and birth weight were significant predictors of mental health and psychosocial functioning.},
	language = {eng},
	number = {4},
	journal = {Journal of Pediatric Surgery},
	author = {Faugli, Anne and Bjørnland, Kristin and Emblem, Ragnhild and Nøvik, Torunn S. and Diseth, Trond H.},
	month = apr,
	year = {2009},
	keywords = {Adaptation, Physiological, Adaptation, Psychological, Adolescent, Esophageal Atresia, Female, Humans, Interpersonal Relations, Male, Mental Health, Norway, Parent-Child Relations, Psychology, Quality of Life, Reference Values, Retrospective Studies, Risk Assessment, Self Concept, Sex Factors, Sickness Impact Profile, Statistics, Nonparametric, Surveys and Questionnaires},
	pages = {729--737},
}

PURPOSE: We examined mental health and psychosocial functioning in adolescents with esophageal atresia (EA) and searched for predictors of impaired outcome. METHODS: The study group comprised 21 adolescents with EA and 1 or both parents. A comparison group comprised 36 adolescents from the general population. Mental health, self-esteem, psychosocial functioning, and parental/family functioning were assessed by standardized questionnaires and semistructured interviews. Physical health was assessed by growth and clinical symptoms. RESULTS: Mental health and psychosocial functioning did not differ from the comparison group. Seven of 21 EA adolescents had special education. Dilatations of esophagus, birth weight, well-being, and maternal psychological distress were prognostic factors predicting mental health. Height, birth weight, well-being, dissociative symptoms, and family strain were prognostic factors predicting psychosocial functioning. CONCLUSION: Adolescents with EA adjusted well, and mental health and psychosocial functioning did not differ from controls. Dilatations of esophagus and birth weight were significant predictors of mental health and psychosocial functioning.

@article{macher_retrograde_2009,
	title = {Retrograde dilatation via gastrostomy of a proximal esophagoileal anastomotic stricture in an infant with esophageal atresia},
	volume = {41 Suppl 2},
	issn = {1438-8812},
	url = {https://pubmed.ncbi.nlm.nih.gov/19668010/},
	doi = {10.1055/s-2008-1077649},
	language = {eng},
	journal = {Endoscopy},
	author = {Macher, E. and Michaud, L. and Guimber, D. and Bonnevalle, M. and Turck, D. and Gottrand, F.},
	year = {2009},
	keywords = {Anastomosis, Surgical, Catheterization, Constriction, Pathologic, Esophageal Atresia, Esophageal Stenosis, Esophagus, Follow-Up Studies, Gastrostomy, Humans, Ileum, Infant, Male, Risk Assessment, Treatment Outcome},
	pages = {E10},
}

@article{rintala_outcome_2009,
	title = {Outcome of esophageal atresia beyond childhood},
	volume = {18},
	issn = {1055-8586},
	doi = {10.1053/j.sempedsurg.2008.10.010},
	abstract = {Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.},
	language = {eng},
	number = {1},
	journal = {Seminars in Pediatric Surgery},
	author = {Rintala, R. J. and Sistonen, S. and Pakarinen, M. P.},
	month = feb,
	year = {2009},
	keywords = {Adult, Age Factors, Anastomosis, Surgical, Esophageal Atresia, Esophagus, Humans, Survival Analysis, Thoracotomy, Time Factors, Treatment Outcome},
	pages = {50--56},
}

Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.

@misc{noauthor_topical_nodate,
	title = {Topical mitomycin {C} application is effective in management of localized caustic esophageal stricture: a double-blinded, randomized, placebo-controlled trial - {PubMed}},
	url = {https://pubmed.ncbi.nlm.nih.gov/23895984/},
	urldate = {2025-01-09},
}

@misc{noauthor_terminal_nodate,
	title = {Terminal 4q deletion and 8q duplication in a patient with {CHARGE}-like features - {PubMed}},
	url = {https://pubmed.ncbi.nlm.nih.gov/21094707/},
	urldate = {2025-01-09},
}

@misc{noauthor_postoperative_nodate,
	title = {Postoperative appearances of esophageal atresia repair: retrospective study of 210 patients with review of literature - what the radiologist should know - {PubMed}},
	url = {https://pubmed.ncbi.nlm.nih.gov/23117198/},
	urldate = {2025-01-09},
}

@misc{noauthor_peut-diagnostiquer_nodate,
	title = {Peut-on diagnostiquer l’atrésie de l’œsophage en prénatal ? {\textbar} {Revue} de médecine périnatale},
	url = {https://link.springer.com/article/10.1007/s12611-012-0189-1},
	urldate = {2025-01-09},
}

@misc{noauthor_outcomes_nodate,
	title = {Outcomes of treatment of childhood achalasia - {PubMed}},
	url = {https://pubmed.ncbi.nlm.nih.gov/20620315/},
	urldate = {2025-01-09},
}

@misc{noauthor_long-term_nodate,
	title = {Long-term outcome of children with oesophageal atresia type {III} - {PubMed}},
	url = {https://pubmed.ncbi.nlm.nih.gov/22753768/},
	urldate = {2025-01-09},
}

@misc{noauthor_endoscopic_nodate,
	title = {Endoscopic incision plus esophageal stenting for refractory esophageal stricture in children - {PubMed}},
	url = {https://pubmed.ncbi.nlm.nih.gov/24676814/},
	urldate = {2025-01-09},
}

@misc{noauthor_contemporary_nodate,
	title = {Contemporary management and outcomes for infants born with oesophageal atresia - {PubMed}},
	url = {https://pubmed.ncbi.nlm.nih.gov/23334932/},
	urldate = {2025-01-09},
}