Achalasia in childhood: Surgical treatment and outcome

Achalasia is a rare disorder of the esophagus in children. From 1971 to 199 9, 20 children with achalasia of the esophagus have been treated at our ins titution including two patients who were referred to us after esophagomyoto my. There were 13 boys and 7 girls (average age, 8.2 years; range 2 to 15 y ears). 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. E sophagoscopy was used as a supportive investigation in some patients (n=10) . Nineteen patients underwent Heller-Zaiger operation (modified Heller esop hagomyotomy) 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 1 6 years. Decreased or absent peristalsis persisted in initial control esoph agograms in all patients. Gastroesophageal reflux was encountered in only o ne patient. Complete relief of symptoms was noted in 14 patients. Mild to m oderate dysphagia was encountered in 5 patients and all of them were evalua ted by endoscopy and upper gastrointestinal series. Dysphagia resolved spon taneously in one child and following two dilations in another child. One ch ild has moderate dysphagia after a short follow-up period. Esophageal steno sis was seen in the remaining two and subsequently treated by esophagocardi oplasty (Heyrowsky and Wendel operations). Achalasia should be considered in the differential diagnosis in any childre n with persistent dysphagia, recurrent respiratory tract infections and vom iting, including children treated for clinically suspected gastroesophageal reflux. The obvious mode of treatment is surgical myotomy in children. Mod ified Heller esophagomyotomy is the procedure of choice, which can be perfo rmed 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 so me cases and disappear during follow-up. Resistant stenosis following esoph agocardiomyotomy can be treated by esophagocardioplasty procedures such as Heyrowsky and Wendel operations.