title: Achalasia of the esophagus in childhood. Surgical treatment in 35 cases, with special reference to familial cases and glucocorticoid deficiency association.
abstract: Achalasia of the esophagus is a relatively rare problem in children, but it may be the cause of severe lung disease, growth retardation and respiratory death in young infants. Surgical esophago-cardio-myotomy remains the treatment of choice, and this article details 25 years of experience with 35 children with achalasia of the esophagus and their late post-operative follow-up. The occurrence of achalasia in the first six months of life, the existence of a familial factor, the prevalent possible association with genetic diseases (familial dysautonomia, glucocorticoid insufficiency, Rozycki syndrome) suggest that achalasia in childhood may in certain cases represent a congenital problem, somewhat different from the adult form, which is considered to be an acquired disease.
authors.affiliation: Department of Pediatric Surgery, Hopital des Enfants Malades, Paris.
authors.forename: C
authors.initials: C
authors.lastname: Nihoul-Fékété
authors.forename: F
authors.initials: F
authors.lastname: Bawab
authors.forename: S
authors.initials: S
authors.lastname: Lortat-Jacob
authors.forename: P
authors.initials: P
authors.lastname: Arhan
mesh_terms: D002648:Child; D004402:Dysautonomia, Familial / Q000150:complications; D004931:Esophageal Achalasia / Q000453:epidemiology / Q000235:genetics / Q000601:surgery*; D004947:Esophagus / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000172:deficiency; D006801:Humans; D008297:Male; D013997:Time Factors
chemical_list: D005938:Glucocorticoids