Characteristics and management of congenital esophageal stenosis: findings from a multicenter study

Orphanet Journal of Rare Diseases
L MichaudF Gottrand

Abstract

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. 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. 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 < 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 (T...Continue Reading

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Citations

May 5, 2016·Avicenna Journal of Medicine·Alsafadi AhmadImad Absah
Apr 18, 2015·Gastroenterology Research and Practice·Louise RothEinar Arnbjörnsson
Jul 30, 2016·Pediatric Surgery International·Kazuki YokotaKosuke Chiba
Nov 28, 2017·Current Gastroenterology Reports·Hayat MousaChristophe Faure
Jan 23, 2020·Asian Journal of Endoscopic Surgery·Kyoko MochizukiHidehito Usui
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Sep 19, 2019·The New England Journal of Medicine·Carlo Di LorenzoRicard Masia
Aug 11, 2019·Pediatric Surgery International·Chiyoe ShirotaHiroo Uchida
Jan 3, 2021·NeoReviews·Jillian TaylorVilmaris Quinones Cardona
Aug 31, 2021·Frontiers in Pediatrics·Laura Antonia RitzJochen Hubertus

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Methods Mentioned

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surgical resection

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