Achalasia is a primary esophageal motility Motility The motor activity of the gastrointestinal tract. 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 Lower Esophageal Sphincter Esophagus: Anatomy relaxation 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: 25 Aug, 2021
Image of
megaesophagus
Megaesophagus
Trypanosoma cruzi/Chagas disease (loss of esophageal
motility
Motility
The motor activity of the gastrointestinal tract.
Gastrointestinal Motility and diffuse esophageal dilation)
A: Posteroanterior
X-ray
X-ray
Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source.
Pulmonary Function Tests showing widening of the
superior mediastinum
Superior mediastinum
Mediastinum and Great Vessels: Anatomy (arrows)
B:
Barium swallow
Barium Swallow
Imaging of the Intestines study demonstrating a dilated and tortuous
esophagus
Esophagus
The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part.
Esophagus: Anatomy (arrowheads)
Types of achalasia
All types exhibit impaired
esophagogastric junction
Esophagogastric junction
The area covering the terminal portion of esophagus and the beginning of stomach at the cardiac orifice.
Esophagus: Anatomy relaxation.
Type I: On swallow, the
upper esophageal sphincter
Upper esophageal sphincter
The structure at the pharyngoesophageal junction consisting chiefly of the cricopharyngeus muscle. It normally occludes the lumen of the esophagus, except during swallowing.
Esophagus: Anatomy relaxed, but is followed by 100% failed contractions and no esophageal pressurization (absent
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).
Type II has panesophageal pressurization in at least 20% of swallows.
Type III is defined by the presence of preserved fragments of distal
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 or
premature
Premature
Childbirth before 37 weeks of pregnancy (259 days from the first day of the mother’s last menstrual period, or 245 days after fertilization).
Necrotizing Enterocolitis contractions for at least 20% of the swallows (spastic).
Esophagogastroduodenoscopy showcasing a dilated esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy with food residue in a patient presenting with achalasia
Image: “Gastrointestinal 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) findings” by US National Library of Medicine. License: CC BY 4.0Achalasia in a barium swallow Barium Swallow Imaging of the Intestines study: Narrowing is seen at the distal esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy (“bird’s beak”) due to inadequate relaxation of the lower esophageal sphincter Lower Esophageal Sphincter Esophagus: Anatomy.
Image: “ Barium swallow Barium Swallow Imaging of the Intestines” by Department of Internal Medicine, Nashville, TN, USA. License: CC BY 4.0