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Esophageal congenital stenosis in children

F. Couttenier (1), R. Sfeir (1), G. Podevin (2), P. Chomette (3), A. Maurel (4), M. Dassonville (5), C. Borderon (6), A. Dabadie (7), D. Weil (8), A. Breton (9)

A. Leke Lokombe (10), A. Morali (11), L. Michaud (1)

Departments of Surgery and Pediatrics of Lille (1) , Nantes (2), Paris(3), Saint Pierre de la RĂ©union (4), Bruxelles (5), Clermont Ferrand (6), Rennes (7), Angers (8), Toulouse (9), Amiens (10), (11)Nancy Reference Centre for Esophageal Diseases and Congenital Malformations, Pediatric Unit, CHRU, Lille, France



Introduction: The congenital oesophageal stenosis (CES) is a rare malformation, with few existing information on the revelation mode, management and evolution. The goal of this retrospective, multicentric study was to describe the clinical and evolutionary characteristics of CES.

Material and methods: A questionnaire was addressed to each surgical team that treated this pathology. All the patients followed for a CES over an 18 years period were included.

Results: 35 observations (19 boys) were collected. The CES was associated with an esophageal atresia in 16 cases. The age at diagnosis was between 1 day an 14 years (an average of 22 months), more precocious if the CES was associated with EA (5 months versus 37 months, p< 0,05). 13 CES were diagnosed during the treatment for EA. The revealing symptoms were dysphagia (58%) or food blocking (63%). Respiratory symptoms were observed at 17%. The esogastroduodenal barium study (33/35) and the esogastroduodenal fibroscopy (29/35) allowed the diagnosis in all cases. The CES was located in the inferior 1/3 of the esophagus in 2/3 of the cases. The anatomopathology examination revealed in 5 cases tracheo-bronchial heterotopy, in other 5 cases mucous diaphragms and in 25 cases fibromuscular hypertrophy. The treatment was endoscopic dilatations alone (n=23), dilatations and surgery (n=7), surgery alone (n=5). Digestive symptoms persisted in 60% of the cases at distance after the treatment.

Conclusion: These results reveal the need for a multidisciplinary management that can be standardized considering the rarity of this pathology. The long-term follow-up is also essential.


Key words: congenital esophageal stenosis