Leiomyomatosis of the Esophagus: Experience over a Decade
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To assess the clinical, radiological findings, and treatment strategies in patients with esophageal leiomyomatosis.
Esophageal leiomyomatosis is a rare hamartomatous disorder with varied presentation. It is described mostly in children and is associated with Alport’s syndrome.
A retrospective analysis of three cases managed in the Department of General Surgery at Chandigarh over a period of 10 years.
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.
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.
KeywordsLeiomyomatosis Esophageal resection Alport’s syndrome Esophageal leiomyomatosis Familial
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