Achalasia: diagnosis, management, and clinical course in 16 children

• Published in: Pediatrics (Evanston) 1983-05, Vol.71 (5), p.798-805
• Main Authors: Berquist, W E, Byrne, W J, Ament, M E, Fonkalsrud, E W, Euler, A R
• Format: Article
• Language: English
• Subjects: Adolescent; Child; Child, Preschool; Dilatation; Esophageal Achalasia - diagnosis; Esophageal Achalasia - surgery; Esophageal Achalasia - therapy; Esophagogastric Junction - physiopathology; Esophagoscopy; Female; Humans; Male; Manometry; Pressure
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.71.5.798

Clinical features, radiographic and esophageal manometry findings, and treatment results in 16 patients less than 15 years old with achalasia are described. Esophageal manometry performed in 15 patients showed results similar to those found in adults: (1) increased resting lower esophageal sphincter pressure, (2) incomplete or failure of relaxation of the lower esophageal sphincter on swallowing, and (3) ineffective or absence of peristalsis in all. The most common symptoms in the 16 patients were: dysphagia in 15, postprandial vomiting in 13, and retrosternal pain in five. The average duration from onset of symptoms to diagnosis was 28 months. The esophagram was diagnostic in all patients. Pneumatic dilation was the initial treatment in eight and was successful for more than 1 year in five. Two patients required two dilations and were then symptom-free for more than 1 year, but required a Heller myotomy. The remaining patients underwent Heller myotomy following failure of the second dilation. Three patients underwent myotomy and two patients had myotomy with fundoplication as initial treatment; only one remained symptomatic. Esophageal dilation using a pneumatic dilator should be the initial treatment of choice in school-aged children. However, if more than two dilations are required within 1 year, surgical management is recommended.