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.
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Pedersen, R. N., Calzolari, E., Husby, S. & Garne, E., EUROCAT Working group. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch. Dis. Child. 97, 227–232 (2012).
Nassar, N. et al. Prevalence of esophageal atresia among 18 international birth defects surveillance programs. Birth Defects Res. A Clin. Mol. Teratol. 94, 893–899 (2012). This study, together with that of Pedersen et al. (2012), reports the prevalence of EA with or without TEF and its associated anomalies as calculated from national and international databases for congenital anomalies and reports the number of spontaneous intrauterine deaths and induced abortions.
Bogs, T. et al. Esophageal atresia with or without tracheoesophageal fistula (EA/TEF): association of different EA/TEF subtypes with specific co-occurring congenital anomalies and implications for diagnostic workup. Eur. J. Pediatr. Surg. 28, 176–182 (2017). This study is the first to specifically look at different EA subtypes and co-occurring congenital anomalies.
Spitz, L. Oesophageal atresia. Orphanet J. Rare Dis. 2, 24 (2007).
Vogt, E. C. Congenital esophageal atresia. Am. J. Roentgenol. 22, 463–465 (1929).
Gross, R. E. & Ladd, W. E. The Surgery of Infancy and Childhood: Its Principles and Techniques (Philadelphia and London: W. B. Saunders Co., 1953).
Faure, C. & Righini Grunder, F. Dysmotility in esophageal atresia: pathophysiology, characterization, and treatment. Front. Pediatr. 5, 130 (2017).
Mousa, H. et al. How to care for patients with EA-TEF: the known and the unknown. Curr. Gastroenterol. Rep. 19, 65 (2017).
Pultrum, B. B., Bijleveld, C. M., de Langen, Z. J. & Plukker, J. T. M. Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia. J. Pediatr. Surg. 40, e1–e4 (2005).
Alfaro, L., Bermas, H., Fenoglio, M., Parker, R. & Janik, J. S. Are patients who have had a tracheoesophageal fistula repair during infancy at risk for esophageal adenocarcinoma during adulthood? J. Pediatr. Surg. 40, 719–720 (2005).
Vergouwe, F. W. et al. Four cancer cases after esophageal atresia repair: time to start screening the upper gastrointestinal tract. World J. Gastroenterol. 24, 1056–1062 (2018).
Dellenmark-Blom, M. et al. The Esophageal-Atresia-Quality-of-life questionnaires: feasibility, validity and reliability in Sweden and Germany. J. Pediatr. Gastroenterol. Nutr. 67, 469–477 (2018). This article presents the first disease-specific QOL questionnaire for patients born with EA.
Donoso, F., Kassa, A. M., Gustafson, E., Meurling, S. & Lilja, H. E. Outcome and management in infants with esophageal atresia - a single centre observational study. J. Pediatr. Surg. 51, 1421–1425 (2016).
Lilja, H. E. & Wester, T. Outcome in neonates with esophageal atresia treated over the last 20 years. Pediatr. Surg. Int. 24, 531–536 (2008).
Cassina, M. et al. Prevalence, characteristics, and survival of children with esophageal atresia: a 32-year population-based study including 1,417,724 consecutive newborns. Birth Defects Res. A Clin. Mol. Teratol. 106, 542–548 (2016).
Sulkowski, J. P. et al. Morbidity and mortality in patients with esophageal atresia. Surgery 156, 483–491 (2014).
Powell, L., Frawley, J., Crameri, J., Teague, W. J. & Frawley, G. P. Oesophageal atresia: are “long gap” patients at greater anesthetic risk? Pediatr. Anaesth. 28, 249–256 (2018).
Stoll, C., Alembik, Y., Dott, B. & Roth, M. P. Associated anomalies in cases with esophageal atresia. Am. J. Med. Genet. A 173, 2139–2157 (2017).
Solomon, B. D. VACTERL/VATER association. Orphanet J. Rare Dis. 6, 56 (2011).
Wyse, R. K. H., Al-Mahdawi, S., Burn, J. & Blake, K. Congenital heart disease in CHARGE association. Pediatr. Cardiol. 14, 75–81 (1993).
Sanlaville, D. & Verloes, A. CHARGE syndrome: an update. Eur. J. Hum. Genet. 15, 389 (2007).
Krishnan, U. et al. ESPGHAN-NASPGHAN guidelines for the evaluation and treatment of gastrointestinal and nutritional complications in children with esophageal atresia-tracheoesophageal fistula. J. Pediatr. Gastroenterol. Nutr. 63, 550–570 (2016). This paper provides the current international guidelines for the management of EA.
Gibreel, W. et al. Swallowing dysfunction and quality of life in adults with surgically corrected esophageal atresia/tracheoesophageal fistula as infants: forty years of follow-up. Ann. Surg. 266, 305–310 (2017).
Lemoine, C. et al. Characterization of esophageal motility following esophageal atresia repair using high-resolution esophageal manometry. J. Pediatr. Gastroenterol. Nutr. 56, 609–614 (2013). This study of HRM in patients born with EA observes patterns ranging from normal motility to absent peristalsis. Manometries are compared with symptomatology; GER symptoms were most common in patients with aperistalsis.
Menzies, J., Hughes, J., Leach, S., Belessis, Y. & Krishnan, U. Prevalence of malnutrition and feeding difficulties in children with esophageal atresia. J. Pediatr. Gastroenterol. Nutr. 64, e100–e105 (2017).
Legrand, C. et al. Long-term outcome of children with oesophageal atresia type III. Arch. Dis. Child. 97, 808–811 (2012).
Chetcuti, P., Myers, N. A., Phelan, P. D. & Beasley, S. W. Adults who survived repair of congenital oesophageal atresia and tracheo-oesophageal fistula. BMJ 297, 344–346 (1988).
Chetcuti, P. & Phelan, P. D. Respiratory morbidity after repair of oesophageal atresia and tracheo-oesophageal fistula. Arch. Dis. Child. 68, 167–170 (1993).
Spoel, M. et al. Respiratory morbidity and growth after open thoracotomy or thoracoscopic repair of esophageal atresia. J. Pediatr. Surg. 47, 1975–1983 (2012).
Catalano, P., Di Pace, M. R., Caruso, A. M., Casuccio, A. & De Grazia, E. Gastroesophageal reflux in young children treated for esophageal atresia: evaluation with pH-multichannel intraluminal impedance. J. Pediatr. Gastroenterol. Nutr. 52, 686–690 (2011).
Castilloux, J., Soglio, D. B.-D. & Faure, C. Endoscopic assessment of children with esophageal atresia: lack of relationship of esophagitis and esophageal metaplasia to symptomatology. Can. J. Gastroenterol. 24, 312–316 (2010).
Koivusalo, A., Pakarinen, M. P. & Rintala, R. J. The cumulative incidence of significant gastrooesophageal reflux in patients with oesophageal atresia with a distal fistula—a systematic clinical, pH-metric, and endoscopic follow-up study. J. Pediatr. Surg. 42, 370–374 (2007).
Krug, E. et al. Gastroesophageal reflux and Barrett’s esophagus in adults born with esophageal atresia. Am. J. Gastroenterol. 94, 2825 (1999).
Taylor, A. C. F. et al. Gastroesophageal reflux and related pathology in adults who were born with esophageal atresia: a long-term follow-up study. Clin. Gastroenterol. Hepatol. 5, 702–706 (2007).
Burjonrappa, S. C., Youssef, S. & St-Vil, D. What is the incidence of Barrett’s and gastric metaplasia in esophageal atresia/tracheoesophageal fistula (EA/TEF) patients? Eur. J. Pediatr. Surg. 21, 25–29 (2011).
Lindahl, H. & Rintala, R. Long-term complications in cases of isolated esophageal atresia treated with esophageal anastomosis. J. Pediatr. Surg. 30, 1222–1223 (1995).
Dhaliwal, J. et al. Eosinophilic esophagitis in children with esophageal atresia. Dis. Esophagus 27, 340–347 (2014).
Gupta, S. K., Hassall, E., Chiu, Y.-L., Amer, F. & Heyman, M. B. Presenting symptoms of nonerosive and erosive esophagitis in pediatric patients. Dig. Dis. Sci. 51, 858–863 (2006).
Schneider, A. et al. Prevalence of Barrett esophagus in adolescents and young adults with esophageal atresia. Ann. Surg. 264, 1004–1008 (2016).
Vergouwe, F. W. T. et al. High prevalence of Barrett’s esophagus and esophageal squamous cell carcinoma after repair of esophageal atresia. Clin. Gastroenterol. Hepatol. 16, 513–521 (2018). This study in patients born with EA reveals an extremely high prevalence of oesophagitis, Barrett oesophagus and oesophageal squamous cell cancer.
Sistonen, S. J. et al. Esophageal morbidity and function in adults with repaired esophageal atresia with tracheoesophageal fistula: a population-based long-term follow-up. Ann. Surg. 251, 1167–1173 (2010).
Hsieh, H. et al. Intestinal metaplasia of the esophagus in children with esophageal atresia. J. Pediatr. Gastroenterol. Nutr. 65, e1–e4 (2017).
Liacouras, C. A. et al. Eosinophilic esophagitis: updated consensus recommendations for children and adults. J. Allergy Clin. Immunol. 128, 3–20 (2011).
Arias, Á., Pérez-Martínez, I., Tenías, J. M. & Lucendo, A. J. Systematic review with meta-analysis: the incidence and prevalence of eosinophilic oesophagitis in children and adults in population-based studies. Aliment. Pharmacol. Ther. 43, 3–15 (2016).
Vergouwe, F. W. T. et al. Risk factors for refractory anastomotic strictures after oesophageal atresia repair: a multicentre study. Arch. Dis. Child. 104, 152–157 (2019).
Morini, F., Conforti, A. & Bagolan, P. Perioperative complications of esophageal atresia. Eur. J. Pediatr. Surg. 28, 133–140 (2018).
Chittmittrapap, S., Spitz, L., Kiely, E. M. & Brereton, R. J. Anastomotic stricture following repair of esophageal atresia. J. Pediatr. Surg. 25, 508–511 (1990).
Spitz, L. Esophageal atresia. Lessons I have learned in a 40-year experience. J. Pediatr. Surg. 41, 1635–1640 (2006).
Davidson, A. J. et al. Neurodevelopmental outcome at 2 years of age after general anaesthesia and awake-regional anaesthesia in infancy (GAS): an international multicentre, randomised controlled trial. Lancet 387, 239–250 (2016).
Sun, L. S. et al. Association between a single general anesthesia exposure before age 36 months and neurocognitive outcomes in later childhood. JAMA 315, 2312–2320 (2016).
Kawahara, H., Imura, K., Yagi, M. & Kubota, A. Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia. Surgery 129, 29–38 (2001).
Vasudevan, S. A., Kerendi, F., Lee, H. & Ricketts, R. R. Management of congenital esophageal stenosis. J. Pediatr. Surg. 37, 1024–1026 (2002).
McCann, F. et al. Congenital esophageal stenosis associated with esophageal atresia. Dis. Esophagus 28, 211–215 (2015).
Ioannides, A. S. & Copp, A. J. Embryology of oesophageal atresia. Semin. Pediatr. Surg. 18, 2–11 (2009).
De Santa Barbara, P., Van Den Brink, G. R. & Roberts, D. J. Molecular etiology of gut malformations and diseases. Am. J. Med. Genet. 115, 221–230 (2002).
Fausett, S. R. & Klingensmith, J. Compartmentalization of the foregut tube: developmental origins of the trachea and esophagus. Wiley Interdiscip. Rev. Dev. Biol. 1, 184–202 (2012).
Qi, B. Q. & Beasley, S. W. Stages of normal tracheo-bronchial development in rat embryos: resolution of a controversy. Dev. Growth Differ. 42, 145–153 (2000).
Sasaki, T., Kusafuka, T. & Okada, A. Analysis of the development of normal foregut and tracheoesophageal fistula in an adriamycin rat model using three-dimensional image reconstruction. Surg. Today 31, 133–139 (2001).
Fagman, H., Andersson, L. & Nilsson, M. The developing mouse thyroid: embryonic vessel contacts and parenchymal growth pattern during specification, budding, migration, and lobulation. Dev. Dyn. 235, 444–455 (2006).
Ioannides, A. S. et al. Foregut separation and tracheo-oesophageal malformations: the role of tracheal outgrowth, dorso-ventral patterning and programmed cell death. Dev. Biol. 337, 351–362 (2010). This study in mice embryos assesses foregut compartmentalization and the signalling pathways involved.
Li, Y., Litingtung, Y., Ten Dijke, P. & Chiang, C. Aberrant Bmp signaling and notochord delamination in the pathogenesis of esophageal atresia. Dev. Dyn. 236, 746–754 (2007).
Que, J., Choi, M., Ziel, J. W., Klingensmith, J. & Hogan, B. L. M. Morphogenesis of the trachea and esophagus: current players and new roles for noggin and Bmps. Differentiation 74, 422–437 (2006).
Que, J. et al. Multiple dose-dependent roles for Sox2 in the patterning and differentiation of anterior foregut endoderm. Development 134, 2521–2531 (2007).
Litingtung, Y., Lei, L., Westphal, H. & Chiang, C. Sonic hedgehog is essential to foregut development. Nat. Genet. 20, 58 (1998).
Shaw-Smith, C. Genetic factors in esophageal atresia, tracheo-esophageal fistula and the VACTERL association: roles for FOXF1 and the 16q24.1 FOX transcription factor gene cluster, and review of the literature. Eur. J. Med. Genet. 53, 6–13 (2010).
Mendelsohn, C. et al. Function of the retinoic acid receptors (RARs) during development (II). Multiple abnormalities at various stages of organogenesis in RAR double mutants. Development 120, 2749–2771 (1994).
Qi, B., Diez-Pardo, J. A., Navarro, C. & Tovar, J. A. Narrowing the embryologic window of the adriamycin-induced fetal rat model of esophageal atresia and tracheoesophageal fistula. Pediatr. Surg. Int. 11, 444–447 (1996).
Orford, J., Manglick, P., Cass, D. T. & Tam, P. P. L. Mechanisms for the development of esophageal atresia. J. Pediatr. Surg. 36, 985–994 (2001).
Qi, B. Q. & Beasley, S. W. Relationship of the notochord to foregut development in the fetal rat model of esophageal atresia. J. Pediatr. Surg. 34, 1593–1598 (1999).
Nakazato, Y., R. Wells, T. & H. Landing, B. Abnormal tracheal innervation in patients with esophageal atresia and tracheoesophageal fistula: Study of the intrinsic tracheal nerve plexuses by a microdissection technique. 21, 838–844 (1986).
Boleken, M. et al. Reduced neuronal innervation in the distal end of the proximal esophageal atretic segment in cases of esophageal atresia with distal tracheoesophageal fistula. World J. Surg. 31, 1512–1517 (2007).
Midrio, P. et al. Reduction of interstitial cells of cajal in esophageal atresia. J. Pediatr. Gastroenterol. Nutr. 51, 610–617 (2010).
Qi, B. Q. et al. The vagus and recurrent laryngeal nerves in the rodent experimental model of esophageal atresia. J. Pediatr. Surg. 32, 1580–1586 (1997).
Cheng, W., Bishop, A. E., Spitz, L. & Polak, J. M. Abnormal enteric nerve morphology in atretic esophagus of fetal rats with adriamycin-induced esophageal atresia. Pediatr. Surg. Int. 15, 8–10 (1999).
Zuccarello, B. et al. Intramural ganglion structures in esophageal atresia: a morphologic and immunohistochemical study. Int. J. Pediatr. 2009, 695837–695837 (2009).
Lemoine, C., Aspirot, A., Morris, M. & Faure, C. Esophageal dysmotility is present before surgery in isolated tracheoesophageal fistula. J. Pediatr. Gastroenterol. Nutr. 60, 642–644 (2015).
Shono, T. et al. Motility function of the esophagus before primary anastomosis in esophageal atresia. J. Pediatr. Surg. 28, 673–676 (1993).
Davies, M. R. Q. Anatomy of the extrinsic motor nerve supply to mobilized segments of the oesophagus disrupted by dissection during repair of oesophageal atresia with distal fistula. Br. J. Surg. 83, 1268–1270 (1996).
Kahrilas, P. J. et al. Esophageal peristaltic dysfunction in peptic esophagitis. Gastroenterology 91, 897–904 (1986).
Cucchiara, S. et al. Esophageal motor abnormalities in children with gastroesophageal reflux and peptic esophagitis. J. Pediatr. 108, 907–910 (1986).
Deprez, P. & Fiasse, R. Healing of severe esophagitis improves esophageal peristaltic dysfunction. Dig. Dis. Sci. 44, 125–133 (1999).
Duvoisin, G. & Krishnan, U. Gastric function in children with oesophageal atresia and tracheoesophageal fistula. Front. Pediatr. 5, 76 (2017).
van Beek, A. P., Emous, M., Laville, M. & Tack, J. Dumping syndrome after esophageal, gastric or bariatric surgery: pathophysiology, diagnosis, and management. Obes. Rev. 18, 68–85 (2017).
Mason, S. J., Harris, G. & Blissett, J. Tube feeding in infancy: implications for the development of normal eating and drinking skills. Dysphagia 20, 46–61 (2005).
Rommel, N., De Meyer, A.-M., Feenstra, L. & Veereman-Wauters, G. The complexity of feeding problems in 700 infants and young children presenting to a tertiary care institution. J. Pediatr. Gastroenterol. Nutr. 37, 75–84 (2003).
Puntis, J. W., Ritson, D. G., Holden, C. E. & Buick, R. G. Growth and feeding problems after repair of oesophageal atresia. Arch. Dis. Child. 65, 84–88 (1990).
van Wijk, M., Knüppe, F., Omari, T., de Jong, J. & Benninga, M. Evaluation of gastroesophageal function and mechanisms underlying gastroesophageal reflux in infants and adults born with esophageal atresia. J. Pediatr. Surg. 48, 2496–2505 (2013). This study is the first and only study to evaluate mechanisms underlying GER and gastroesophageal function in patients born with EA.
Tong, S., Mallitt, K.-A. & Krishnan, U. Evaluation of gastroesophageal reflux by combined multichannel intraluminal impedance and pH monitoring and esophageal motility patterns in children with esophageal atresia. Eur. J. Pediatr. Surg. 26, 322–331 (2016).
Mitchell, D. J., McClure, B. G. & Tubman, T. R. J. Simultaneous monitoring of gastric and oesophageal pH reveals limitations of conventional oesophageal pH monitoring in milk fed infants. Arch. Dis. Child. 84, 273–276 (2001).
Montgomery, M., Escobar-Billing, R., Hellström, P., Karlsson, K. & Frenckner, B. Impaired gastric emptying in children with repaired esophageal atresia: a controlled study. J. Pediatr. Surg. 33, 476–480 (1998).
Rintala, R. J. Fundoplication in patients with esophageal atresia: patient selection, indications, and outcomes. Front. Pediatr. 5, 109 (2017).
Koivusalo, A. I., Rintala, R. J. & Pakarinen, M. P. Outcomes of fundoplication in oesophageal atresia associated gastrooesophageal reflux disease. J. Pediatr. Surg. 53, 230–233 (2018).
Kwiatek, M. A. et al. Esophagogastric junction distensibility after fundoplication assessed with a novel functional luminal imaging probe. J. Gastrointest. Surg. 14, 268–276 (2010).
Bufler, P., Ehringhaus, C. & Koletzko, S. Dumping syndrome: a common problem following Nissen fundoplication in young children. Pediatr. Surg. Int. 17, 351–355 (2001).
Rosen, R. et al. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J. Pediatr. Gastroenterol. Nutr. 66, 516–554 (2018).
Wanaguru, D. et al. Is fundoplication required after the Foker procedure for long gap esophageal atresia? J. Pediatr. Surg. 52, 1117–1120 (2017).
Straumann, A., Bauer, M., Fischer, B., Blaser, K. & Simon, H.-U. Idiopathic eosinophilic esophagitis is associated with a TH2-type allergic inflammatory response. J. Allergy Clin. Immunol. 108, 954–961 (2001).
Virchow, J. C. Eosinophilic esophagitis: asthma of the esophagus? Dig. Dis. 32, 54–60 (2014).
Blanchard, C. et al. Eotaxin-3 and a uniquely conserved gene-expression profile in eosinophilic esophagitis. J. Clin. Invest. 116, 536–547 (2006).
Gorter, R. R., Heij, H. A., van der Voorn, J. P. & Kneepkens, C. M. F. Eosinophilic esophagitis after esophageal atresia: is there an association? Case presentation and literature review. J. Pediatr. Surg. 47, e9–e13 (2012).
Costa, R. H., Kalinichenko, V. V. & Lim, L. Transcription factors in mouse lung development and function. Am. J. Physiol. Lung Cell. Mol. Physiol. 280, L823–L838 (2001).
Spechler, S. J., Genta, R. M. & Souza, R. F. Thoughts on the complex relationship between gastroesophageal reflux disease and eosinophilic esophagitis. Am. J. Gastroenterol. 102, 1301 (2007).
Yamada, Y. et al. Esophagitis with eosinophil infiltration associated with congenital esophageal atresia and stenosis. Int. Arch. Allergy Immunol. 161 (Suppl. 2), 159–163 (2013).
Pali-Schöll, I. & Jensen-Jarolim, E. Anti-acid medication as a risk factor for food allergy. Allergy 66, 469–477 (2011).
Krishnan, U., Lijuan, C., Andrew, G. J., Rothenberg, M. E. & Wen, T. Analysis of eosinophilic esophagitis in children with repaired congenital esophageal atresia. J. Allergy Clin. Immunol. https://doi.org/10.1016/j.jaci.2018.08.040 (2018). This transcriptome analysis shows that baseline epithelial barrier and type 2 associated genes are dysregulated in patients with EoE without EA, in patients born with EA and without EoE and in patients born with EA and without EoE, potentially explaining the higher prevalence in EA.
Shah, R., Varjavandi, V. & Krishnan, U. Predictive factors for complications in children with esophageal atresia and tracheoesophageal fistula. Dis. Esophagus 28, 216–223 (2015).
Serhal, L. et al. Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations. J. Pediatr. Surg. 45, 1459–1462 (2010).
Stenström, P., Anderberg, M., Börjesson, A. & Arnbjornsson, E. Prolonged use of proton pump inhibitors as stricture prophylaxis in infants with reconstructed esophageal atresia. Eur. J. Pediatr. Surg. 27, 192–195 (2017).
Miyake, H. et al. Are prophylactic anti-reflux medications effective after esophageal atresia repair? Systematic review and meta-analysis. Pediatr. Surg. Int. 34, 491–497 (2018).
Okata, Y. et al. Evaluation of the intraoperative risk factors for esophageal anastomotic complications after primary repair of esophageal atresia with tracheoesophageal fistula. Pediatr. Surg. Int. 32, 869–873 (2016).
Yeung, C. K., Spitz, L., Brereton, R. J., Kiely, E. M. & Leake, J. Congenital esophageal stenosis due to tracheobronchial remnants: a rare but important association with esophageal atresia. J. Pediatr. Surg. 27, 852–855 (1992).
El-Gohary, Y., Gittes, G. K. & Tovar, J. A. Congenital anomalies of the esophagus. Semin. Pediatr. Surg. 19, 186–193 (2010).
Spilde, T. L. et al. Fibroblast growth factor signaling in the developing tracheoesophageal fistula. J. Pediatr. Surg. 38, 474–477 (2003).
Calonge, W. M. et al. Expression of homeotic genes Hoxa3, Hoxb3, Hoxd3 and Hoxc4 is decreased in the lungs but not in the hearts of adriamycin-exposed mice. Pediatr. Surg. Int. 23, 419–424 (2007).
Liu, X. et al. Impaired VEGF signaling in lungs with hypoplastic esophageal atresia and effects on branching morphogenesis. Cell. Physiol. Biochem. 39, 385–394 (2016).
Xiaomei, L., Aras-Lopez, R., Martinez, L. & Tovar, J. A. Lung hypoplasia in rats with esophageal atresia and tracheo–esophageal fistula. Pediatr. Res. 71, 235 (2012).
Xia, H., Otten, C., Migliazza, L., Diez-Pardo, J. A. & Tovar, J. A. Tracheobronchial malformations in experimental esophageal atresia. J. Pediatr. Surg. 34, 536–539 (1999).
DeBoer, E. M. et al. Multidisciplinary care of children with repaired esophageal atresia and tracheoesophageal fistula. Pediatr. Pulmonol. 51, 576–581 (2016).
Kompare, M. & Weinberger, M. Protracted bacterial bronchitis in young children: association with airway malacia. J. Pediatr. 160, 88–92 (2012).
Fung, S. W. et al. Vocal cord dysfunction following esophageal atresia and tracheoesophageal fistula (EA/TEF) repair. J. Pediatr. Surg. https://doi.org/10.1016/j.jpedsurg.2018.08.041 (2018).
Hseu, A., Recko, T., Jennings, R. & Nuss, R. Upper airway anomalies in congenital tracheoesophageal fistula and esophageal atresia patients. Ann. Otol. Rhinol. Laryngol. 124, 808–813 (2015).
Ghandour, K. E., Spitz, L., Brereton, R. J. & Kiely, E. M. Recurrent tracheo-oesophageal fistula: experience with 24 patients. J. Paediatr. Child Health 26, 89–91 (1990).
Chetcuti, P., Phelan, P. D. & Greenwood, R. Lung function abnormalities in repaired oesophageal atresia and tracheo-oesophageal fistula. Thorax 47, 1030–1034 (1992).
Houben, C. H. & Curry, J. I. Current status of prenatal diagnosis, operative management and outcome of esophageal atresia/tracheo-esophageal fistula. Prenat. Diagn. 28, 667–675 (2008).
Sfeir, R. et al. Esophageal atresia: data from a national cohort. J. Pediatr. Surg. 48, 1664–1669 (2013).
Garabedian, C. et al. Does a combination of ultrasound, MRI, and biochemical amniotic fluid analysis improve prenatal diagnosis of esophageal atresia? Prenat. Diagn. 34, 839–842 (2014).
Spaggiari, E. et al. Performance of prenatal diagnosis in esophageal atresia. Prenat Diagn. 35, 888–893 (2015).
Garabedian, C. et al. Management and outcome of neonates with a prenatal diagnosis of esophageal atresia type A: a population-based study. Prenat. Diagn. 38, 517–522 (2018).
Pretorius, D. H., Gosink, B. B., Clautice-Engle, T., Leopold, G. R. & Minnick, C. M. Sonographic evaluation of the fetal stomach: significance of nonvisualization. Am. J. Roentgenol. 151, 987–989 (1988).
Eyheremendy, E. & Pfister, M. Antenatal real-time diagnosis of esophageal atresias. J. Clin. Ultrasound 11, 395–397 (1983).
Kalache, K. D., Wauer, R., Mau, H., Chaoui, R. & Bollmann, R. Prognostic significance of the pouch sign in fetuses with prenatally diagnosed esophageal atresia. Am. J. Obstet. Gynecol. 182, 978–981 (2000).
Solt, I., Rotmensch, S. & Bronshtein, M. The esophageal ‘pouch sign’: a benign transient finding. Prenat. Diagn. 30, 845–848 (2010).
Brantberg, A., Blaas, H.-G. K., Haugen, S. E. & Eik-Nes, S. H. Esophageal obstruction—prenatal detection rate and outcome. Ultrasound Obstet. Gynecol. 30, 180–187 (2007).
Pardy, C., D‘Antonio, F., Khalil, A. & Giuliani, S. Prenatal detection of esophageal atresia: a systematic review and meta-analysis. Acta Obstet. Gynecol. Scand. https://doi.org/10.1111/aogs.13536 (2019).
Parolini, F., Bulotta, A. L., Battaglia, S. & Alberti, D. Preoperative management of children with esophageal atresia: current perspectives. Pediatric Health Med. Ther. 8, 1–7 (2017).
Hochart, V. et al. The contribution of fetal MR imaging to the assessment of oesophageal atresia. Eur. Radiol. 25, 306–314 (2015).
Tracy, S. et al. The distended fetal hypopharynx: a sensitive and novel sign for the prenatal diagnosis of esophageal atresia. J. Pediatr. Surg. 53, 1137–1141 (2018).
Potier, M., Dallaire, L. & Melançon, S. B. Prenatal detection of intestinal obstruction by disaccharidase assay in amniotic fluid. Lancet 310, 982–983 (1977).
Solomon, B. D. et al. An approach to the identification of anomalies and etiologies in neonates with identified or suspected VACTERL (vertebral defects, anal atresia, tracheo-esophageal fistula with esophageal atresia, cardiac anomalies, renal anomalies, and limb anomalies) association. J. Pediatr. 164, 451–457 (2014).
Kahrilas, P. J., Shaheen, N. J. & Vaezi, M. F. American Gastroenterological Association medical position statement on the management of gastroesophageal reflux disease. Gastroenterology 135, 1383–1391 (2008).
Spergel, J. M. et al. Summary of the updated international consensus diagnostic criteria for eosinophilic esophagitis: AGREE conference. Ann. Allergy Asthma Immunol. 121, 281–284 (2018).
Hirano, I. et al. Endoscopic assessment of the oesophageal features of eosinophilic oesophagitis: validation of a novel classification and grading system. Gut 62, 489–495 (2013).
Koivusalo, A. I., Pakarinen, M. P., Lindahl, H. G. & Rintala, R. J. Endoscopic surveillance after repair of oesophageal atresia: longitudinal study in 209 patients. J. Pediatr. Gastroenterol. Nutr. 62, 562–566 (2016).
Eisenhuber, E. et al. Evaluation of swallowing disorders with videofluoroscopy in Austria: a survey. Eur. J. Radiol. 53, 120–124 (2005).
Miller, C. K. & Willging, J. P. Advances in the evaluation and management of pediatric dysphagia. Curr. Opin. Otolaryngol. Head Neck Surg. 11, 442–446 (2003).
Hörmann, M. et al. Videofluoroscopy of deglutition in children after repair of esophageal atresia. Acta Radiol. 43, 507–510 (2002).
Tutuian, R. & Castell, D. O. Esophageal function testing: role of combined multichannel intraluminal impedance and manometry. Gastrointest. Endosc. Clin. N. Am. 15, 265–275 (2005).
Rommel, N., Rayyan, M., Scheerens, C. & Omari, T. The potential benefits of applying recent advances in esophageal motility testing in patients with esophageal atresia. Front. Pediatr. 5, 137 (2017). This paper describes novel diagnostic tools for the assessment of dysphagia in EA.
Kahrilas, P. J. et al. The Chicago Classification of esophageal motility disorders, v3.0. Neurogastroenterol. Motil. 27, 160–174 (2015).
Gyawali, C. P. et al. Classification of esophageal motor findings in gastro-esophageal reflux disease: conclusions from an international consensus group. Neurogastroenterol. Motil. 29, e13104 (2017).
Bredenoord, A. J., Weusten, B. L. A. M., Timmer, R. & Smout, A. J. P. M. Intermittent spatial separation of diaphragm and lower esophageal sphincter favors acidic and weakly acidic reflux. Gastroenterology 130, 334–340 (2006).
Loots, C. et al. Gastroesophageal reflux, esophageal function, gastric emptying, and the relationship to dysphagia before and after antireflux surgery in children. J. Pediatr. 162, 566–573 (2013).
Myers, J. C. et al. Susceptibility to dysphagia after fundoplication revealed by novel automated impedance manometry analysis. Neurogastroenterol. Motil. 24, 812-e393 (2012).
Singendonk, M. M. J. et al. Pressure-flow characteristics of normal and disordered esophageal motor patterns. J. Pediatr. 166, 690–696 (2015).
Rommel, N., Oudenhove, L., Tack, J. & Omari, T. I. Automated impedance manometry analysis as a method to assess esophageal function. Neurogastroenterol. Motil. 26, 636–645 (2014).
López-Alonso, M. et al. Twenty-four-hour esophageal impedance-pH monitoring in healthy preterm neonates: rate and characteristics of acid, weakly acidic, and weakly alkaline gastroesophageal reflux. Pediatrics 118, e299–e308 (2006).
Mousa, H. M. et al. Esophageal impedance monitoring for gastroesophageal reflux. J. Pediatr. Gastroenterol. Nutr. 52, 129–139 (2011).
Fröhlich, T. et al. Combined esophageal multichannel intraluminal impedance and pH monitoring after repair of esophageal atresia. J. Pediatr. Gastroenterol. Nutr. 47, 443–449 (2008).
Omari, T. I. et al. Optimisation of the reflux-symptom association statistics for use in infants being investigated by 24-hour pH impedance. J. Pediatr. Gastroenterol. Nutr. 52, 408–413 (2011).
Woodley, F. W., Fernandez, S. & Mousa, H. Diurnal variation in the chemical clearance of acid gastroesophageal reflux in infants. Clin. Gastroenterol. Hepatol. 5, 37–43 (2007).
Singendonk, M. M. J., Benninga, M. A. & Wijk, M. P. Reflux monitoring in children. Neurogastroenterol. Motil. 28, 1452–1459 (2016).
Shieh, H. F. et al. Posterior tracheopexy for severe tracheomalacia associated with esophageal atresia (EA): primary treatment at the time of initial EA repair versus secondary treatment. Front. Surg. 4, 80 (2018).
Yalcin, S., Demir, N., Serel, S., Soyer, T. & Tanyel, F. C. The evaluation of deglutition with videofluoroscopy after repair of esophageal atresia and/or tracheoesophageal fistula. J. Pediatr. Surg. 50, 1823–1827 (2015).
Rosen, R. & Nurko, S. The importance of multichannel intraluminal impedance in the evaluation of children with persistent respiratory symptoms. Am. J. Gastroenterol. 99, 2452 (2004).
Mousa, H., Woodley, F. W., Metheney, M. & Hayes, J. Testing the association between gastroesophageal reflux and apnea in infants. J. Pediatr. Gastroenterol. Nutr. 41, 169–177 (2005).
Borrelli, O. et al. Role of gastroesophageal reflux in children with unexplained chronic cough. J. Pediatr. Gastroenterol. Nutr. 53, 287–292 (2011).
Krishnan, U., Paul, S., Messina, I. & Soma, M. Correlation between laryngobronchoscopy and pepsin in the diagnosis of extra-oesophageal reflux. J. Laryngol. Otol. 129, 572–579 (2015).
Rosen, R. et al. The edematous and erythematous airway does not denote pathologic gastroesophageal reflux. J. Pediatr. 183, 127–131 (2017).
Farrell, S., McMaster, C., Gibson, D., Shields, M. D. & McCallion, W. A. Pepsin in bronchoalveolar lavage fluid: a specific and sensitive method of diagnosing gastro-oesophageal reflux–related pulmonary aspiration. J. Pediatr. Surg. 41, 289–293 (2006).
Jat, K. R. Spirometry in children. Prim. Care Respir. J. 22, 221–229 (2013).
Zysman-Colman, Z. & Lands, L. C. Whole body plethysmography: practical considerations. Paediatr. Respir. Rev. 19, 39–41 (2016).
Zani, A. et al. International survey on the management of esophageal atresia. Eur. J. Pediatr. Surg. 24, 3–8 (2014).
Gottrand, F., Ley, D., Michaud, L. & Sfeir, R. Importance of an International Registry for and Collaborative Research on Esophageal Atresia. Front. Pediatr. 5, 81 (2017).
Pinheiro, P. F., Simoes e Silva, A. C. & Pereira, R. M. Current knowledge on esophageal atresia. World J. Gastroenterol. 18, 3662–3672 (2012).
Borruto, F. A. et al. Thoracoscopy versus thoracotomy for esophageal atresia and tracheoesophageal fistula repair: review of the literature and meta-analysis. Eur. J. Pediatr. Surg. 22, 415–419 (2012). This study reports that thoracoscopic oesophageal repair has the benefits of being minimally invasive and is as effective as open surgery in terms of operating time, postoperative ventilation time and postoperative leaks and strictures.
Rothenberg, S. Thoracoscopic repair of esophageal atresia and tracheo-esophageal fistula in neonates: the current state of the art. Pediatr. Surg. Int. 30, 979–985 (2014).
Teague, W. J. & Karpelowsky, J. Surgical management of oesophageal atresia. Paediatr. Respir. Rev. 19, 10–15 (2016).
Shieh, H. F. & Jennings, R. W. Long-gap esophageal atresia. Semin. Pediatr. Surg. 26, 72–77 (2017). This paper compares different surgical techniques in long-gap EA.
Lal, D., Miyano, G., Juang, D., Sharp, N. E. & St Peter, S. D. Current patterns of practice and technique in the repair of esophageal atresia and tracheoesophageal fistula: an IPEG survey. J. Laparoendosc. Adv. Surg. Tech. A 23, 635–638 (2013).
Gawad, N., Wayne, C., Bass, J. & Nasr, A. A chest tube may not be needed after surgical repair of esophageal atresia and tracheoesophageal fistula. Pediatr. Surg. Int. 34, 967–970 (2018).
Aslanabadi, S., Jamshidi, M., Tubbs, R. S. & Shoja, M. M. The role of prophylactic chest drainage in the operative management of esophageal atresia with tracheoesophageal fistula. Pediatr. Surg. Int. 25, 365–368 (2009).
Ferrand, A., Roy, S., Aspirot, A. & Faure, C. Post operative non-invasive ventilation and complications in oesophageal atresia-tracheoesophageal fistula. Paediatr. Child Health 22 (Suppl. 1), e25 (2017).
O’Connell, J. S. et al. Post-operative paralysis and elective ventilation reduces anastomotic complications in esophageal atresia: a systematic review and meta-analysis. Pediatr. Surg. Int. 35, 87–95 (2019).
Narayanan, S. K., Vazhiyodan, A. P., Somnath, P. & Mohanan, A. Is routine use of transanastomotic tube justified in the repair of esophageal atresia? World J. Pediatr. 13, 584–587 (2017).
Lal, D. R. et al. Challenging surgical dogma in the management of proximal esophageal atresia with distal tracheoesophageal fistula: outcomes from the Midwest Pediatric Surgery Consortium. J. Pediatr. Surg. 53, 1267–1272 (2017).
Yeung, A. & Butterworth, S. A. A comparison of surgical outcomes between in-hours and after-hours tracheoesophageal fistula repairs. J. Pediatr. Surg. 50, 805–808 (2015).
Manfredi, M. A. et al. Endoscopic esophageal vacuum therapy: a novel therapy for esophageal perforations in pediatric patients. J. Pediatr. Gastroenterol. Nutr. 67, 706–712 (2018).
Mathur, S., Vasudevan, S. A., Patterson, D. M., Hassan, S. F. & Kim, E. S. Novel use of glycopyrrolate (Robinul) in the treatment of anastomotic leak after repair of esophageal atresia and tracheoesophageal fistula. J. Pediatr. Surg. 46, e29–e32 (2011).
Tambucci, R. et al. Anastomotic strictures after esophageal atresia repair: incidence, investigations, and management, including treatment of refractory and recurrent strictures. Front. Pediatr. 5, 120 (2017). This article reviews oesophageal strictures in EA, including benign strictures in patients without EA and in adults.
Lang, T., Hümmer, H. P. & Behrens, R. Balloon dilation is preferable to bougienage in children with esophageal atresia. Endoscopy 33, 329–335 (2001).
Jayakrishnan, V. K. & Wilkinson, A. G. Treatment of oesophageal strictures in children: a comparison of fluoroscopically guided balloon dilatation with surgical bouginage. Pediatr. Radiol. 31, 98–101 (2001).
Lan, L. C. et al. Endoscopic balloon dilatation of esophageal strictures in infants and children: 17 years’ experience and a literature review. J. Pediatr. Surg. 38, 1712–1715 (2003).
Poddar, U. & Thapa, B. R. Benign esophageal strictures in infants and children: results of Savary-Gilliard bougie dilation in 107 Indian children. Gastrointest. Endosc. 54, 480–484 (2001).
van der Zee, D. & Hulsker, C. Indwelling esophageal balloon catheter for benign esophageal stenosis in infants and children. Surg. Endosc. 28, 1126–1130 (2014).
Dall’Oglio, L. et al. Endoscopic management of esophageal stenosis in children: new and traditional treatments. World J. Gastrointest. Endosc. 8, 212–219 (2016).
Newman, B. & Bender, T. M. Esophageal atresia/tracheoesophageal fistula and associated congenital esophageal stenosis. Pediatr. Radiol. 27, 530–534 (1997).
Romeo, E. et al. Endoscopic management of congenital esophageal stenosis. J. Pediatr. Surg. 46, 838–841 (2011).
Holland, A. J. & Fitzgerald, D. A. Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications. Paediatr. Respir. Rev. 11, 100–106 (2010).
Bruch, S. W., Hirschl, R. B. & Coran, A. G. The diagnosis and management of recurrent tracheoesophageal fistulas. J. Pediatr. Surg. 45, 337–340 (2010).
Lelonge, Y. et al. Chemocauterization with trichloroacetic acid in congenital and recurrent tracheoesophageal fistula: a minimally invasive treatment. Surg. Endosc. 30, 1662–1666 (2016).
Mahoney, L. & Rosen, R. Feeding problems and their underlying mechanisms in the esophageal atresia–tracheoesophageal fistula patient. Front. Pediatr. 5, 127 (2017).
Chan, L. J. et al. Treatment outcomes for eosinophilic esophagitis in children with esophageal atresia. Dis. Esophagus 29, 563–571 (2016).
Tytgat, S. H. A. J., van Herwaarden-Lindeboom, M. Y. A., van Tuyll van Serooskerken, E. S. & van der Zee, D. C. Thoracoscopic posterior tracheopexy during primary esophageal atresia repair: a new approach to prevent tracheomalacia complications. J. Pediatr. Surg. 53, 1420–1423 (2018).
Fitzgerald, D. A. Pro -con debate: “that antibiotic prophylaxis should be used in children with repaired oesophageal atresia and tracheo-oesophageal fistula”. Paediatr. Respir. Rev. 18, 58–59 (2016).
Peetsold, M. G., Heij, H. A., Deurloo, J. A. & Gemke, R. J. Health-related quality of life and its determinants in children and adolescents born with oesophageal atresia. Acta Paediatr. 99, 411–417 (2010).
Dingemann, C. et al. Long-term health-related quality of life after complex and/or complicated esophageal atresia in adults and children registered in a German patient support group. J. Pediatr. Surg. 49, 631–638 (2014).
Deurloo, J. A., Ekkelkamp, S., Hartman, E. E., Sprangers, M. G. & Aronson, D. C. Quality of life in adult survivors of correction of esophageal atresia. Arch. Surg. 140, 976–980 (2005).
Witt, S. et al. Quality of life in parents of children born with esophageal atresia. Eur. J. Pediatr. Surg. https://doi.org/10.1055/s-0038-1660867 (2018).
Le Gouëz, M. et al. Posttraumatic stress reactions in parents of children esophageal atresia. PLOS ONE 11, e0150760 (2016).
Yang, L. et al. De novo GLI3 mutation in esophageal atresia: reproducing the phenotypic spectrum of Gli3 defects in murine models. Biochim. Biophys. Acta 1842, 1755–1761 (2014).
Garcia, M., Downs, J., Russell, A. & Wang, W. Impact of biobanks on research outcomes in rare diseases: a systematic review. Orphanet J. Rare Dis. 13, 202 (2018).
Abrahamyan, L. et al. Alternative designs for clinical trials in rare diseases. 172, 313–331 (2016).
Lilford, R. J., Thornton, J. G. & Braunholtz, D. Clinical trials and rare diseases: a way out of a conundrum. BMJ 311, 1621–1625 (1995).
Institute of Medicine. Small Clinical Trials: Issues and Challenges (National Academies Press, Washington DC, 2001).
Urbani, L. et al. Multi-stage bioengineering of a layered oesophagus with in vitro expanded muscle and epithelial adult progenitors. Nat. Commun. 9, 4286 (2018).
Workman, M. J. et al. Engineered human pluripotent-stem-cell-derived intestinal tissues with a functional enteric nervous system. Nat. Med. 23, 49–59 (2017).
Jensen, T. et al. Polyurethane scaffolds seeded with autologous cells can regenerate long esophageal gaps: an esophageal atresia treatment model. J. Pediatr. Surg. (2018).
Cooper, J. E. et al. In vivo transplantation of fetal human gut-derived enteric neural crest cells. Neurogastroenterol. Motil. 29, e12900 (2017).
Czerkiewicz, I. et al. Biochemical amniotic fluid pattern for prenatal diagnosis of esophageal atresia. Pediatr. Res. 70, 199 (2011).
Chittmittrapap, S., Spitz, L., Kiely, E. M. & Brereton, R. J. Oesophageal atresia and associated anomalies. Arch. Dis. Child. 64, 364–368 (1989).
Conforti, A. et al. Laryngotracheal abnormalities in esophageal atresia patients: a hidden entity. Front. Pediatr. 6, 401 (2018).
Osei-Nketiah, S. et al. Management of oesophageal atresia in a developing country: is primary repair forbidden? Afr. J. Paediatr. Surg. 13, 114–119 (2016).
Rattan, K., Singh, J. & Dalal, P. Clinical profile and short-term outcome of neonates with esophageal atresia and tracheoesophageal fistula at tertiary care center in a developing country: a 25-year experience. J. Clin. Neonatol. 6, 225–230 (2017).
Morrow, T. Wilson-Cook’s flourish device uses magnets to fix pediatric esophageal atresia. Manag. Care 26, 32–33 (2017).
van der Zee, D. C. et al. Position paper of INoEA Working Group on Long-Gap Esophageal Atresia: for better care. Front. Pediatr. 5, 63 (2017).
Billmyre, K. K., Hutson, M. & Klingensmith, J. One shall become two: separation of the esophagus and trachea from the common foregut tube. Dev. Dyn. 244, 277–288 (2015).
Minoo, P., Su, G., Drum, H., Bringas, P. & Kimura, S. Defects in tracheoesophageal and lung morphogenesis inNkx2.1−/− mouse embryos. Dev. Biol. 209, 60–71 (1999).
Lawal, T. A., Gosemann, J.-H., Kuebler, J. F., Glüer, S. & Ure, B. M. Thoracoscopy versus thoracotomy improves midterm musculoskeletal status and cosmesis in infants and children. Ann. Thorac Surg. 87, 224–228 (2009).
Roberts, D. J. et al. Sonic hedgehog is an endodermal signal inducing Bmp-4 and Hox genes during induction and regionalization of the chick hindgut. Development 121, 3163–3174 (1995).
Perin, S., McCann, C. J., Borrelli, O., De Coppi, P. & Thapar, N. Update on foregut molecular embryology and role of regenerative medicine therapies. Front. Pediatr. 5, 91 (2017).
Nasr, A. & Langer, J. C. Mechanical traction techniques for long-gap esophageal atresia: a critical appraisal. Eur. J. Pediatr. Surg. 23, 191–197 (2013).
Esteves, E. et al. Laparoscopically assisted esophagectomy and colon interposition for esophageal replacement in children: preliminary results of a novel technique. J. Pediatr. Surg. 45, 1053–1060 (2010).
Bogusz, B., Patkowski, D., Gerus, S., Rasiewicz, M. & Górecki, W. Staged thoracoscopic repair of long-gap esophageal atresia without temporary gastrostomy. J. Laparoendosc. Adv. Surg. Tech. A 28, 1510–1512 (2018).
van der Zee, D. C., Gallo, G. & Tytgat, S. H. A. Thoracoscopic traction technique in long gap esophageal atresia: entering a new era. Surg. Endosc. 29, 3324–3330 (2015).
Gallo, G., Zwaveling, S., Groen, H., Van der Zee, D. & Hulscher, J. Long-gap esophageal atresia: a meta-analysis of jejunal interposition, colon interposition, and gastric pull-up. Eur. J. Pediatr. Surg. 22, 420–425 (2012).
T.I.O. holds an Australian National Health and Medical Research Council Senior Research Fellowship. The other authors report no external funding for this manuscript.
Nature Reviews Disease Primers thanks D. Van der Zee, and other anonymous reviewer(s), for their contribution to the peer review of this work.
The authors have no conflicts of interest relevant to this article to disclose.
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van Lennep, M., Singendonk, M.M.J., Dall’Oglio, L. et al. Oesophageal atresia. Nat Rev Dis Primers 5, 26 (2019). https://doi.org/10.1038/s41572-019-0077-0
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