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.
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.
Authors: P Guillem; F Delcambre; L Cohen-Solal; J P Triboulet; C Antignac; L Heidet; P Quandalle Journal: Gastroenterology Date: 2001-01 Impact factor: 22.682
Authors: Martina C Anker; Joachim Arnemann; Katrin Neumann; Peter Ahrens; Helga Schmidt; Rainer König Journal: Am J Med Genet A Date: 2003-06-15 Impact factor: 2.802