Achalasia of the cardia in children: a worldwide survey.

This report summarizes a worldwide survey of achalasia in childhood. The data were obtained by circulating a questionnaire to pediatric surgeons throughout the world. Information was received regarding 175 patients, and the analysis is presented. Childhood achalasia appears to be more common in boys, and familial cases are rare. Regurgitation of food and dysphagia are the most frequent symptoms in affected children. Although 18% of patients had symptom onset during infancy, only 6% of the patients were identified as having achalasia during infancy. The survey suggests only a fair clinical response to transthoracic esophagocardiomyotomy and a good to excellent clinical response to transabdominal esophagocardiomyotomy. A cooperative effort between several institutions will permit the development of prospective treatment protocols for children with achalasia.