{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T04:58:09Z","timestamp":1781240289687,"version":"3.54.1"},"reference-count":34,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Esophageal atresia (EA) is the most common congenital esophageal malformation. An improvement in survival led to a focus on functional outcomes and quality of life (QoL). We analyzed the long-term outcomes and QoL of patients submitted to surgery for EA. Perinatal characteristics, surgical procedures, gastrointestinal and respiratory current symptoms and QoL were investigated. Thirty-nine patients were included. Long Gap patients had a higher rate of prematurity and low birth weight. The prevalent surgical procedure was primary esophageal anastomosis, followed by gastric pull-up. Twenty-four patients had post-operative stenosis, while gastroesophageal reflux (GER) required fundoplication in eleven cases. Auxological parameters were lower in Long Gap patients. The lowest scores of QoL were in the Long Gap group, especially in younger patients, which was the group with the highest number of symptoms. In the long term, the QoL appeared to be more dependent on the type of esophageal atresia rather than on associated malformations. Surgical management of GER was indicated in all patients with Long Gap EA, supposedly due to the prevalence of gastric pull-up for this type of EA. The assessment of QoL is part of surgeon\u2019s management and needs to be performed in each phase of a child\u2019s development.<\/jats:p>","DOI":"10.3390\/children9030331","type":"journal-article","created":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T08:17:03Z","timestamp":1646122623000},"page":"331","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Congenital Esophageal Atresia Long-Term Follow-Up\u2014The Pediatric Surgeon\u2019s Duty to Focus on Quality of Life"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1008-6632","authenticated-orcid":false,"given":"Carlotta","family":"Ardenghi","sequence":"first","affiliation":[{"name":"Pediatric Surgery Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elettra","family":"Vestri","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Costanzo","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Lanfranchi","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0012-1366","authenticated-orcid":false,"given":"Maurizio","family":"Vertemati","sequence":"additional","affiliation":[{"name":"CIMaINa (Interdisciplinary Centre for Nanostructured Materials and Interfaces), University of Milano, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0206-2892","authenticated-orcid":false,"given":"Francesca","family":"Destro","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ugo Maria","family":"Pierucci","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2137-5974","authenticated-orcid":false,"given":"Valeria","family":"Calcaterra","sequence":"additional","affiliation":[{"name":"Pediatric Department, Children\u2019s Hospital \u201cVittore Buzzi\u201d, 20154 Milan, Italy"},{"name":"Pediatrics and Adolescentology Unit, Department of Internal Medicine, University of Pavia, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gloria","family":"Pelizzo","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Department, \u201cVittore Buzzi\u201d Children\u2019s Hospital, 20154 Milan, Italy"},{"name":"Department of Biomedical and Clinical Science \u201cLuigi Sacco\u201d, University of Milano, 20157 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,3,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1002\/bdra.23067","article-title":"Prevalence of Esophageal Atresia among 18 International Birth Defects Surveillance Programs","volume":"94","author":"Nassar","year":"2012","journal-title":"Birth Defects Res. Part A\u2014Clin. Mol. Teratol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1136\/archdischild-2011-300597","article-title":"Oesophageal Atresia: Prevalence, Prenatal Diagnosis and Associated Anomalies in 23 European Regions","volume":"97","author":"Pedersen","year":"2012","journal-title":"Arch. Dis. Child."},{"key":"ref_3","unstructured":"(2021, May 15). Prevalence Charts and Tables|EU RD Platform. Available online: https:\/\/eu-rd-platform.jrc.ec.europa.eu\/eurocat\/eurocat-data\/prevalence_en."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1016\/j.jpedsurg.2015.01.004","article-title":"A Cross-Sectional Nationwide Survey on Esophageal Atresia and Tracheoesophageal Fistula","volume":"50","author":"Carlucci","year":"2015","journal-title":"J. Pediatric Surg."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1002\/bdr2.1891","article-title":"Survival of Infants Born with Esophageal Atresia among 24 International Birth Defects Surveillance Programs","volume":"113","author":"Bell","year":"2021","journal-title":"Birth Defects Res."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1055\/s-0030-1249610","article-title":"Early and Long Term Outcome in Children with Esophageal Atresia Treated over the Last 22 Years","volume":"222","author":"Lacher","year":"2010","journal-title":"Klin. Padiatr."},{"key":"ref_7","first-page":"63","article-title":"Position Paper of INoEA Working Group on Long-Gap Esophageal Atresia: For Better Care","volume":"5","author":"Bagolan","year":"2017","journal-title":"Front. Pediatrics"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1007\/s00383-012-3142-2","article-title":"Delayed Primary Anastomosis for Management of Long-Gap Esophageal Atresia: A Meta-Analysis of Complications and Long-Term Outcome","volume":"28","author":"Friedmacher","year":"2012","journal-title":"Pediatric Surg. Int."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1007\/s00383-007-1964-0","article-title":"Prognosis of Congenital Tracheoesophageal Fistula with Esophageal Atresia on the Basis of Gap Length","volume":"23","author":"Upadhyaya","year":"2007","journal-title":"Pediatric Surg. Int."},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Destro, F., Maestri, L., Meroni, M., Rebosio, F., Re, G.D., Mantegazza, C., Calcaterra, V., and Pelizzo, G. (2021). Usefulness of Gastrojejunostomy Prior to Fundoplication in Severe Gastro-Esophageal Reflux Complicating Long-Gap Esophageal Atresia Repair: A Preliminary Study. Children, 8.","DOI":"10.3390\/children8010055"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1016\/0022-3468(93)90029-K","article-title":"Motility Function of the Esophagus before Primary Anastomosis in Esophageal Atresia","volume":"28","author":"Shono","year":"1993","journal-title":"J. Pediatric Surg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1055\/s-2008-1071074","article-title":"Motility Studies of the Esophagus in a Case of Esophageal Atresia before Primary Anastomosis and in Experimental Models","volume":"7","author":"Shono","year":"1997","journal-title":"Eur. J. Pediatric Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1097\/MPG.0000000000001401","article-title":"ESPGHAN-NASPGHAN Guidelines for the Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children with Esophageal Atresia- Tracheoesophageal Fistula","volume":"63","author":"Krishnan","year":"2016","journal-title":"J. Pediatric Gastroenterol. Nutr."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1055\/s-0039-3400284","article-title":"ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework","volume":"30","author":"Coppi","year":"2020","journal-title":"Eur. J. Pediatric Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1055\/s-0039-1693729","article-title":"Clinical Factors Affecting Condition-Specific Quality-of-Life Domains in Pediatric Patients after Repair of Esophageal Atresia: The Swedish-German EA-QOL Study","volume":"30","author":"Quitmann","year":"2020","journal-title":"Eur. J. Pediatric Surg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1111\/1753-0407.13110","article-title":"Glycemic Control in Type 1 Diabetes Mellitus and COVID-19 Lockdown: What Comes after a \u201cQuarantine\u201d?","volume":"12","author":"Ceconi","year":"2020","journal-title":"J. Diabetes"},{"key":"ref_17","unstructured":"W.B. Saunders Company (1953). The Surgery of Infancy and Childhood: Its Principles and Techniques, W.B. Saunders Company."},{"key":"ref_18","unstructured":"(2021, December 12). BMI Z-Score and Percentile Calculator. Available online: https:\/\/www.bcm.edu\/bodycomplab\/BMIapp\/BMI-calculator-kids.html."},{"key":"ref_19","unstructured":"(2021, June 12). Growth Charts\u20142000 CDC Growth Charts\u2014United States, Available online: https:\/\/www.cdc.gov\/growthcharts\/cdc_charts.html."},{"key":"ref_20","first-page":"1","article-title":"Development and Pilot-Testing of a Condition-Specific Instrument to Assess the Quality-of-Life in Children and Adolescents Born with Esophageal Atresia","volume":"30","author":"Abrahamsson","year":"2017","journal-title":"Dis. Esophagus"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"3662","DOI":"10.3748\/wjg.v18.i28.3662","article-title":"Current Knowledge on Esophageal Atresia","volume":"18","author":"Pinheiro","year":"2012","journal-title":"World J. Gastroenterol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.jss.2014.03.068","article-title":"Comparing Characteristics and Outcomes in Infants with Prenatal and Postnatal Diagnosis of Esophageal Atresia","volume":"190","author":"Fallon","year":"2014","journal-title":"J. Surg. Res."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1016\/0022-3468(94)90354-9","article-title":"Oesophageal Atresia: At-Risk Groups for the 1990s","volume":"29","author":"Spitz","year":"1994","journal-title":"J. Pediatric Surg."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/j.surg.2009.01.017","article-title":"Esophageal Atresia: Prognostic Classification Revisited","volume":"145","author":"Okamoto","year":"2009","journal-title":"Surgery"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1016\/j.jpedsurg.2016.11.046","article-title":"Perioperative Management and Outcomes of Esophageal Atresia and Tracheoesophageal Fistula","volume":"52","author":"Lal","year":"2017","journal-title":"J. Pediatric Surg."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1016\/j.amjsurg.2014.09.019","article-title":"Esophageal Atresia and Transitional Care\u2014Step 1: A Systematic Review and Meta-Analysis of the Literature to Define the Prevalence of Chronic Long-Term Problems","volume":"209","author":"Connor","year":"2015","journal-title":"Am. J. Surg."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1136\/archdischild-2012-301730","article-title":"Long-Term Outcome of Children with Oesophageal Atresia Type III","volume":"97","author":"Legrand","year":"2012","journal-title":"Arch. Dis. Child."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.1016\/j.jpedsurg.2014.03.021","article-title":"Measured Gap Length and Outcomes in Oesophageal Atresia","volume":"49","author":"Thakkar","year":"2014","journal-title":"J. Pediatric Surg."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1053\/j.sempedsurg.2008.10.009","article-title":"The Surgical Approach to Esophageal Atresia Repair and the Management of Long-Gap Atresia: Results of a Survey","volume":"18","author":"Ron","year":"2009","journal-title":"Semin. Pediatric Surg."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1053\/j.sempedsurg.2017.02.009","article-title":"Long-Gap Esophageal Atresia","volume":"26","author":"Shieh","year":"2017","journal-title":"Semin. Pediatric Surg."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1055\/s-0033-1347911","article-title":"Gastroesophageal Reflux after Repair of Esophageal Atresia","volume":"23","author":"Tovar","year":"2013","journal-title":"Eur. J. Pediatric Surg."},{"key":"ref_32","first-page":"161","article-title":"Infants Born with Esophageal Atresia with or without Tracheo-Esophageal Fistula: Short-and Long-Term Outcomes","volume":"20","author":"Leibovitch","year":"2018","journal-title":"Isr. Med. Assoc. J."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1055\/s-0038-1672144","article-title":"Generic Health-Related Quality of Life after Repair of Esophageal Atresia and Its Determinants within a German-Swedish Cohort","volume":"29","author":"Flieder","year":"2019","journal-title":"Eur. J. Pediatric Surg."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"e423","DOI":"10.1097\/INF.0000000000002845","article-title":"Effect of Social Distancing Due to the COVID-19 Pandemic on the Incidence of Viral Respiratory Tract Infections in Children in Finland during Early 2020","volume":"39","author":"Kuitunen","year":"2020","journal-title":"Pediatric Infect. Dis. J."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/3\/331\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:30:14Z","timestamp":1760135414000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/3\/331"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,1]]},"references-count":34,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2022,3]]}},"alternative-id":["children9030331"],"URL":"https:\/\/doi.org\/10.3390\/children9030331","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,1]]}}}