A controversy that has been tough to swallow: is the treatment of achalasia now digested?

Abstract

Esophageal achalasia is a rare neurodegenerative disease of the esophagus and the lower esophageal sphincter that presents within a spectrum of disease severity related to progressive pathological changes, most commonly resulting in dysphagia. The pathophysiology of achalasia is still incompletely understood, but recent evidence suggests that degeneration of the postganglionic inhibitory nerves of the myenteric plexus could be due to an infectious or autoimmune mechanism, and nitric oxide is the neurotransmitter affected. Current treatment of achalasia is directed at palliation of symptoms. Therapies include pharmacological therapy, endoscopic injection of botulinum toxin, endoscopic dilation, and surgery. Until the late 1980s, endoscopic dilation was the first line of therapy. The advent of safe and effective minimally invasive surgical techniques in the early 1990s paved the way for the introduction of laparoscopic myotomy. This review will discuss the most up-to-date information regarding the pathophysiology, diagnosis, and treatment of achalasia, including a historical perspective. The laparoscopic Heller myotomy with partial fundoplication performed at an experienced center is currently the first line of therapy because it offers a low complication rate, the most durable symptom relief, and the lowest incidence of postoperative gastroesophageal reflux.

Figure 1

Examples of progressive dilation of the esophagus in different patients with achalasia. a Normal diameter esophagus leading to a bird’s beak at the LES. b Minimal esophageal dilation (from 4 to 7 cm). c Progressive esophageal dilation (from 7 to 10 cm) with preserved esophageal axis. d Greater dilation (>10 cm) and initial sigmoidal course of the distal esophagus.

Figure 2

A barium esophagram showing a normal caliber esophagus with a large epiphrenic diverticula in a patient with achalasia.

Figure 3

A barium esophagram in a patient with a gastric band causing pseudoachalasia. The esophagus is dilated and empties barium slowly.

Figure 4

The anterior vagus nerve (arrow) is identified and preserved. Then two graspers are used to begin the myotomy just above the GEJ.

Figure 5

A completed 8-cm myotomy is shown. Arrow 1 points to the anterior vagus nerve. Arrow 2 points to the left edge of the myotomy. Arrow 3 points to the exposed esophageal submucosa.

Figure 6

The creation of a Dor fundoplication. The first suture from the anterior portion of the fundus to the left edge of the myotomy (arrow 1). The second suture will anchor the fundoplication and part of the myotomy in the abdomen by apposing the fundus (arrow 2) to the left crus (not shown) and then to left edge of the myotomy (arrow 3).

Figure 7

The completed Dor fundoplication. Arrows 1 and 2 show the suture line that anchors the fundoplication to the right edge of the myotomy. Arrow 2 incorporates the fundus, the right crus to the right edge of the myotomy. Arrow 3 points the suture that secures the fundoplication to the diaphragm. Arrow 4 points to the divided short gastric vessels that are brought into an anterior position as the fundoplication is created. This reinforces the need for a complete mobilization of the gastric fundus for the proper configuration of the fundoplication.