抄録
Achalasia of the esophagus, although commonly encountered in adults, is rare in infants and children. Esophageal achalasia in a 4 year old female with Down's syndrome is reported and pathophysiology of the lower esophagus in this patient is discussed in this paper. The patient is a 4 year old female. She was noted to vomit since the age of 7-months, and regurgitation occured after every meal. Dilatation and marked narrowing at the gastro-esophageal junction was found by an upper gastro-intestinal series. Esophageal manometric study showed that lower esophageal sphincter (LES) pressure was elevated (66cmH_2O), and LES relaxation corresponding to swallowing was not found. After a modified esophagomyectomy had been performed, partial fundoplication was added for preventation of gastro-esophageal reflux. After esophagomyectomy, LES pressure has significantly decreased. Post-operative fluoroscopic examination revealed good passage of barium. No ganglion cell was found in the biopsied specimen.
