Leiomyomatosis of the esophagus: experience over a decade

J Gastrointest Surg. 2009 Feb;13(2):206-11. doi: 10.1007/s11605-008-0701-x. Epub 2008 Sep 26.

Abstract

Purpose: To assess the clinical, radiological findings, and treatment strategies in patients with esophageal leiomyomatosis.

Background: Esophageal leiomyomatosis is a rare hamartomatous disorder with varied presentation. It is described mostly in children and is associated with Alport's syndrome.

Methods: A retrospective analysis of three cases managed in the Department of General Surgery at Chandigarh over a period of 10 years.

Results: The study involves three female patients of different generations within the same family with age range of 10-58 years. All presented with dysphagia of 2-7 years duration. Barium swallow revealed a long-segment stricture in two patients. Computed tomography (CT) demonstrated a circumferential mass lesion in the lower esophagus in all the patients. Esophageal resection was carried out in all the patients. All patients made an uneventful recovery.

Conclusions: Esophageal leiomyomatosis should be suspected in patients with long-standing dysphagia. Barium findings are suggestive but can mimic achalasia. CT scan shows a circumferential esophageal wall thickening. Surgical resection and reconstruction of the digestive passage is the optimal treatment.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Child
  • Esophageal Neoplasms / diagnosis*
  • Esophageal Neoplasms / genetics
  • Esophageal Neoplasms / surgery*
  • Esophagectomy
  • Female
  • Humans
  • Leiomyomatosis / diagnosis*
  • Leiomyomatosis / genetics
  • Leiomyomatosis / surgery*
  • Middle Aged