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

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 o...

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Autores principales: Michaud, Laurent, Coutenier, Frédéric, Podevin, Guillaume, Bonnard, Arnaud, Becmeur, François, Khen-Dunlop, Naziha, Auber, Frédéric, Maurel, Aude, Gelas, Thomas, Dassonville, Martine, Borderon, Corinne, Dabadie, Alain, Weil, Dominique, Piolat, Christian, Breton, Anne, Djeddi, Djamal, Morali, Alain, Bastiani, Florence, Lamireau, Thierry, Gottrand, Frédéric
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4222067/
https://www.ncbi.nlm.nih.gov/pubmed/24289834
http://dx.doi.org/10.1186/1750-1172-8-186
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author Michaud, Laurent
Coutenier, Frédéric
Podevin, Guillaume
Bonnard, Arnaud
Becmeur, François
Khen-Dunlop, Naziha
Auber, Frédéric
Maurel, Aude
Gelas, Thomas
Dassonville, Martine
Borderon, Corinne
Dabadie, Alain
Weil, Dominique
Piolat, Christian
Breton, Anne
Djeddi, Djamal
Morali, Alain
Bastiani, Florence
Lamireau, Thierry
Gottrand, Frédéric
author_facet Michaud, Laurent
Coutenier, Frédéric
Podevin, Guillaume
Bonnard, Arnaud
Becmeur, François
Khen-Dunlop, Naziha
Auber, Frédéric
Maurel, Aude
Gelas, Thomas
Dassonville, Martine
Borderon, Corinne
Dabadie, Alain
Weil, Dominique
Piolat, Christian
Breton, Anne
Djeddi, Djamal
Morali, Alain
Bastiani, Florence
Lamireau, Thierry
Gottrand, Frédéric
author_sort Michaud, Laurent
collection PubMed
description 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 < 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.
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spelling pubmed-42220672014-11-07 Characteristics and management of congenital esophageal stenosis: findings from a multicenter study Michaud, Laurent Coutenier, Frédéric Podevin, Guillaume Bonnard, Arnaud Becmeur, François Khen-Dunlop, Naziha Auber, Frédéric Maurel, Aude Gelas, Thomas Dassonville, Martine Borderon, Corinne Dabadie, Alain Weil, Dominique Piolat, Christian Breton, Anne Djeddi, Djamal Morali, Alain Bastiani, Florence Lamireau, Thierry Gottrand, Frédéric Orphanet J Rare Dis Research 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 < 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. BioMed Central 2013-12-01 /pmc/articles/PMC4222067/ /pubmed/24289834 http://dx.doi.org/10.1186/1750-1172-8-186 Text en Copyright © 2013 Michaud et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an open access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research
Michaud, Laurent
Coutenier, Frédéric
Podevin, Guillaume
Bonnard, Arnaud
Becmeur, François
Khen-Dunlop, Naziha
Auber, Frédéric
Maurel, Aude
Gelas, Thomas
Dassonville, Martine
Borderon, Corinne
Dabadie, Alain
Weil, Dominique
Piolat, Christian
Breton, Anne
Djeddi, Djamal
Morali, Alain
Bastiani, Florence
Lamireau, Thierry
Gottrand, Frédéric
Characteristics and management of congenital esophageal stenosis: findings from a multicenter study
title Characteristics and management of congenital esophageal stenosis: findings from a multicenter study
title_full Characteristics and management of congenital esophageal stenosis: findings from a multicenter study
title_fullStr Characteristics and management of congenital esophageal stenosis: findings from a multicenter study
title_full_unstemmed Characteristics and management of congenital esophageal stenosis: findings from a multicenter study
title_short Characteristics and management of congenital esophageal stenosis: findings from a multicenter study
title_sort characteristics and management of congenital esophageal stenosis: findings from a multicenter study
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4222067/
https://www.ncbi.nlm.nih.gov/pubmed/24289834
http://dx.doi.org/10.1186/1750-1172-8-186
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