Achalasia is a primary esophageal motility Esophageal Motility Gastrointestinal Motility disorder that develops from the degeneration of the myenteric plexus Myenteric plexus One of two ganglionated neural networks which together form the enteric nervous system. The myenteric (Auerbach's) plexus is located between the longitudinal and circular muscle layers of the gut. Its neurons project to the circular muscle, to other myenteric ganglia, to submucosal ganglia, or directly to the epithelium, and play an important role in regulating and patterning gut motility. Gastrointestinal Neural and Hormonal Signaling. This condition results in impaired lower esophageal sphincter relaxation Sphincter relaxation Gastrointestinal Motility and absence of normal esophageal peristalsis Peristalsis A movement, caused by sequential muscle contraction, that pushes the contents of the intestines or other tubular organs in one direction. Gastrointestinal Motility. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship typically present with dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming "stuck." Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia to solids and liquids along with regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD). Diagnosis is established by high-resolution manometry. To rule out malignancy Malignancy Hemothorax as a cause of achalasia, upper endoscopy Endoscopy Procedures of applying endoscopes for disease diagnosis and treatment. Endoscopy involves passing an optical instrument through a small incision in the skin i.e., percutaneous; or through a natural orifice and along natural body pathways such as the digestive tract; and/or through an incision in the wall of a tubular structure or organ, i.e. Transluminal, to examine or perform surgery on the interior parts of the body. Gastroesophageal Reflux Disease (GERD) is performed. Barium swallow Barium Swallow Imaging of the Intestines study helps evaluate the esophageal morphology. Management options include pneumatic balloon dilation, surgical myotomy, and botulinum toxin Botulinum toxin Toxic proteins produced from the species Clostridium botulinum. The toxins are synthesized as a single peptide chain which is processed into a mature protein consisting of a heavy chain and light chain joined via a disulfide bond. The botulinum toxin light chain is a zinc-dependent protease which is released from the heavy chain upon endocytosis into presynaptic nerve endings. Once inside the cell the botulinum toxin light chain cleaves specific snare proteins which are essential for secretion of acetylcholine by synaptic vesicles. This inhibition of acetylcholine release results in muscular paralysis. Botulism injection. Choice of treatment is dependent on the type of achalasia and surgical risk. Medications are available for those who fail initial intervention; however, they provide the least benefit.
Last updated: Oct 21, 2022
Image of megaesophagus (loss of esophageal motility and diffuse esophageal dilation)
A: Posteroanterior X-ray showing widening of the superior mediastinum (arrows)
B: Barium swallow study demonstrating a dilated and tortuous esophagus (arrowheads)
Types of achalasia
All types exhibit impaired esophagogastric junction relaxation.
Type I: On swallow, the upper esophageal sphincter relaxed, but is followed by 100% failed contractions and no esophageal pressurization (absent peristalsis).
Type II has panesophageal pressurization in at least 20% of swallows.
Type III is defined by the presence of preserved fragments of distal peristalsis or premature contractions for at least 20% of the swallows (spastic).
Esophagogastroduodenoscopy showcasing a dilated esophagus with food residue in a patient presenting with achalasia
Image: “Gastrointestinal endoscopy findings” by US National Library of Medicine. License: CC BY 4.0Achalasia in a barium swallow study: Narrowing is seen at the distal esophagus (“bird’s beak”) due to inadequate relaxation of the lower esophageal sphincter.
Image: “Barium swallow” by Department of Internal Medicine, Nashville, TN, USA. License: CC BY 4.0