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Canadian Journal of Gastroenterology
Volume 22, Issue 2, Pages 187-189
http://dx.doi.org/10.1155/2008/603105
Brief Communication

Esophageal Leiomyomatosis – An Unusual Cause of Pseudoachalasia

Sukanta Ray,1 Sundeep Singh Saluja,1 Ruchika Gupta,2 and Tushar Kanti Chattopadhyay1

1Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
2Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India

Received 9 June 2007; Accepted 28 June 2007

Copyright © 2008 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.