ACG Clinical Guidelines: Diagnosis and Management of Achalasia

Abstract

Achalasia is an esophageal motility disorder characterized by aberrant peristalsis and insufficient relaxation of the lower esophageal sphincter. Patients most commonly present with dysphagia to solids and liquids, regurgitation, and occasional chest pain with or without weight loss. High-resolution manometry has identified 3 subtypes of achalasia distinguished by pressurization and contraction patterns. Endoscopic findings of retained saliva with puckering of the gastroesophageal junction or esophagram findings of a dilated esophagus with bird beaking are important diagnostic clues. In this American College of Gastroenterology guideline, we used the Grading of Recommendations Assessment, Development and Evaluation process to provide clinical guidance on how best to diagnose and treat patients with achalasia.

Figure 1.

(a) Endoscopic appearance of foam and saliva in the esophagus in achalasia. (b) Puckering of gastroesophageal junction requiring more than usual pressure to traverse in achalasia. (c) Barium swallow showing dilated esophagus with retained barium and “bird beaking.”

Figure 2.

High resolution manometry of achalasia phenotypes: type I-absent pressurization (left), type II-pan pressurization (middle), and type III-spastic contractions (right). Lower esophageal sphincter relaxation is impaired for all subtypes.

Figure 3.

Pneumatic dilator sizes 3.0 cm (bottom), 3.5 cm (middle), and 4.0 cm (top) used in treating patients with achalasia. Graded approach of starting with the smaller 3.0-cm balloon and progressing to the larger sizes if failed therapy is recommended in all except younger male patients in whom initial approach with 3.5-cm balloon may be used.

Figure 4.

(a) Fluoroscopic image of using a 3.0-cm pneumatic dilator in a patient with achalasia showing balloon positioning of 2 rings (middle of balloon; blue arrow) at the esophagogastric junction with subsequent dilation to obliterate the balloon waist. (b) Pneumatic dilation via direct endoscopic approach showing positioning of the 2 rings at the esophagogastric junction during endoscopy with inflation of the balloon to the maximum pressure of 13 mm Hg.

Figure 5.

Surgical view of the distal esophagus during a myotomy.

Figure 6.

Dilated sigmoid esophagus representing end-stage achalasia with retained saliva and barium.

Figure 7.

Timed barium swallow (a) before and (b) after pneumatic dilation showing retention of barium in the former and complete emptying posteffective therapy.

Figure 8.

Diagnostic and treatment algorithm for patients with suspected achalasia. FLIP, functional lumen imaging probe; GERD, gastroesophageal reflux disease; HRM, high resolution manometry; PPI, proton pump inhibitor.