achalasia
Pronunciation: ak-uh-LAY-zhah
A primary esophageal motility disorder characterized by failure of the lower esophageal sphincter to relax.
Full Definition
Achalasia is a rare primary esophageal motility disorder characterized by failure of the lower esophageal sphincter (LES) to relax appropriately and loss of normal esophageal peristalsis. In pediatric patients, the condition typically presents with progressive dysphagia to both solids and liquids, regurgitation, chest pain, and weight loss. The pathophysiology involves degeneration of the enteric nervous system within the esophageal wall, leading to impaired neuromuscular coordination. Diagnosis is established through esophageal manometry, which demonstrates elevated LES pressure and absent or abnormal peristalsis. Barium swallow may show the classic 'bird's beak' appearance. Treatment options include pneumatic dilation, laparoscopic Heller myotomy, or peroral endoscopic myotomy (POEM).
Usage
Usage note: Distinguish from pseudoachalasia; specify subtype when classified by manometry.
In Context
- "High-resolution manometry confirmed the diagnosis of achalasia with absent peristalsis." — Motility study report
- "Laparoscopic Heller myotomy with fundoplication was performed for pediatric achalasia." — Operative report