Esophageal Leiomyomatosis – An Unusual Cause of Pseudoachalasia. (2008)

• Record Type: Journal Article
• Authors: Ray, Sukanta; Saluja, Sundeep Singh; Gupta, Ruchika; Chattopadhyay, Tushar Kanti
• Is Part Of: Canadian Journal of Gastroenterology. Volume 22:Number 2(2008)
• Page Start: 187
• Page End: 189
• Publication Date: 2008
• Subjects: Dysphagia -- Esophagus -- Leiomyomatosis -- Pseudoachalasia
• DOI: 10.1155/2008/603105
• Languages: English
• ISSNs: 0835-7900

Abstract

Abstract : Esophageal leiomyomatosis is a rare hamartomatous disorder with varied presentation. In the literature, it is described mostly in children, and is associated with Alport's syndrome. A case of leiomyomatosis that presented as achalasia not associated with Alport's syndrome is described in a 35-year-old woman with a 16-year history of dysphagia. Barium swallow showed a smooth narrowing at the lower end of the esophagus with a longer than usual stricture length. Endoscopy showed a dilated esophagus with a submucosal nodule in the region of the cardia. A computed tomography scan revealed circumferential thickening of the esophagus involving the gastroesophageal junction, with fat planes maintained with the adjacent structure. Endoscopic ultrasound demonstrated a lesion arising from the muscularis propria. The manometry findings were suggestive of achalasia. She underwent transhiatal esophagectomy with gastric pull-up. Leiomyomatosis should be considered as a cause of psuedoachalasia in patients with symptoms suggestive of achalasia and atypical barium findings. Attempts should be made to confirm the diagnosis preoperatively using computed tomography and/or endoscopic ultrasound. Esophagectomy is the treatment of choice.