Achalasia of the esophagus in childhood: surgical treatment in 35 cases with special reference to familial cases and glucocorticoid deficiency association.

Achalasia of the esophagus is a relatively rare problem in children, but it can result in severe lung disease, growth retardation, or respiratory death in young infants. Surgical esophagocardiomyotomy remains the treatment of choice, and this report details a 25-year experience with 35 children with achalasia of the esophagus and their long-term postoperative follow-up. Occurrence of achalasia in the first 6 months of life, the existence of familial cases, and the association of achalasia with genetic diseases (familial dysautonomia, glucocorticoid insufficiency, Rozycki syndrome) suggest that achalasia in childhood may in certain cases represent a congenital problem.