[Clinical profile and long-term outcome in children with esophageal achalasia]

Achalasia of the cardia is rare in children. We report our experience in the management of 20 children with achalasia.

Twenty children (seven girls including two sisters) with achalasia were studied, seven of them had a morbid association. Age at diagnosis ranged from eight months to 18 years (med: 6.4 yrs). Duration of symptoms prior to diagnosis ranged from one to 62 months (med: 8 months). Regurgitations, weight loss and recurrent pneumonias were the most common presenting symptoms. Diagnosis was established using esophageal manometry, which showed aperistalsis throughout the esophageal body with impaired relaxation of lower esophageal sphincter in all children, and chest x-ray and barium esophagram, which were abnormal in 11 and 18 children respectively.

Nifedipine used in nine children was ineffective. Two balloon dilatations were performed in one child with a poor result. Surgery with Heller's myotomy combined with an antireflux procedure was performed in 19 children. One child died six months later due to an hypoglycemic coma. During the follow-up (6 months-21 years) symptoms reappeared in five children. Seventy-two percent of the children had no symptoms one year after the surgery and 45%, five years after. Esophageal manometry performed after surgery in seven children showed a normal lower esophageal sphincter pressure but with impaired relaxation, and an aperistalsis throughout the esophageal body. These results justify prolonged follow-up of children with achalasia of the esophagus.