Achalasia in childhood: a 20-year experience.

The incidence of achalasia in children is lower than that in adults, but the disturbances in growth and development, and the severity of pulmonary symptoms are more profound. We have treated 20 children with the disease over the past 20 yr. All children were first treated with dilatation of the cardioesophageal junction either with filoform and followers, or in older children, pneumatic dilatation under fluoroscopic control. Five children, all older than 9 yr of age and all female, had prolonged relief of symptoms following two dilatations and never required a surgical procedure. Fifteen children responded unsatisfactorily to repeated dilatation and 12 of these accepted a surgical procedure. A modifier Heller procedure was performed in 11 children and one patient was treated with a transthoracic cardioplasty. The operations resulted in dramatic relief of symptoms, satisfactory weight gain, and the disappearance of pulmonary symptoms in every case. We recommend that all children under 9 yr of age should have a modified Heller procedure as primary therapy. In children older than 9 yr of age, dilatation is an appropriate initial therapy but should not be repeated unless significant improvement results.