Open AccessPseudoachalasia as First Manifestation of a Malignancy
Author(s) -
Elisabeth Fabian,
Andreas Eherer,
Carolin Lackner,
Christian Urban,
FreyjaMaria SmolleJuettner,
Guenter J. Krejs
Publication year - 2019
Publication title -
digestive diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.879
H-Index - 66
eISSN - 1421-9875
pISSN - 0257-2753
DOI - 10.1159/000495758
Subject(s) - medicine , achalasia , malignancy , sarcoidosis , adenocarcinoma , esophageal motility disorder , amyloidosis , radiology , esophagogastric junction , cancer , surgery , esophagus
Pseudoachalasia is a condition in which symptoms, radiologic, endoscopic, and manometric findings mimick idiopathic achalasia. About 4% of patients with a typical constellation for idiopathic achalasia will turn out to have pseudoachalasia, posing a major diagnostic challenge. A large spectrum of underlying causes of pseudoachalasia has been described. However, in about 70% of affected patients, this condition is caused by a malignancy (mostly adenocarcinoma of the esophagogastric junction or cardia). We describe a 16-year-old high school student referred for management of achalasia who turned out to have pseudoachalasia due to adenocarcinoma of the cardia. He was cured with preoperative chemotherapy followed by radical surgery. Therapy of pseudoachalasia secondary to neoplasia is directed against the tumor or may be palliative to keep the lumen open. Other causes of pseudoachalasia include esophageal motility disturbances as a paraneoplastic phenomenon (e.g., with small cell lung cancer), post fundoplication or post bariatric surgery, in association with a thoracic aortic aneurysm, or with sarcoidosis or amyloidosis. Therapy is directed accordingly to eliminate or correct the underlying cause.