Achalasia in childhood: surgical treatment and outcome

Achalasia is a rare disorder of the esophagus in children. From 1971 to 1999, 20 children with achalasia of the esophagus have been treated at our institution including two patients who were referred to us after esophagomyotomy. There were 13 boys and 7 girls (average age, 8.2 years; range 2 to 15 years). Presenting symptoms were vomiting (n = 18), dysphagia (n = 11), loss of weight (n = 5), recurrent respiratory infections (n = 3), cough (n = 2) and noisy respiration (n = 1). Barium swallow established diagnosis in all patients. Esophagoscopy was used as a supportive investigation in some patients (n = 10). Nineteen patients underwent Heller-Zaiger operation (modified Heller esophagomyotomy) either by transabdominal (n = 16) or transthoracic approach (n = 3) with (n = 6) or without concomitant antireflux procedure. The postoperative period was uneventful in all patients. Follow-up ranged from 2 months to 16 years. Decreased or absent peristalsis persisted in initial control esophagograms in all patients. Gastroesophageal reflux was encountered in only one patient. Complete relief of symptoms was noted in 14 patients. Mild to moderate dysphagia was encountered in 5 patients and all of them were evaluated by endoscopy and upper gastrointestinal series. Dysphagia resolved spontaneously in one child and following two dilations in another child. One child has moderate dysphagia after a short follow-up period. Esophageal stenosis was seen in the remaining two and subsequently treated by esophagocardioplasty (Heyrowsky and Wendel operations). Achalasia should be considered in the differential diagnosis in any children with persistent dysphagia, recurrent respiratory tract infections and vomiting, including children treated for clinically suspected gastroesophageal reflux. The obvious mode of treatment is surgical myotomy in children. Modified Heller esophagomyotomy is the procedure of choice, which can be performed either by an abdominal or a thoracic approach. The need to carry out a concomitant antireflux procedure remains controversial. The most frequent postoperative problem is persistent dysphagia. It may be self-limited in some cases and disappear during follow-up. Resistant stenosis following esophagocardiomyotomy can be treated by esophagocardioplasty procedures such as Heyrowsky and Wendel operations.