[Diffuse leiomyomatosis of the esophagus. Analysis of a case and review of the literature]

Abstract

Diffuse leiomyomatosis of the esophagus is a rare affection which is characterized by a diffuse hypertrophy of esophageal muscles, without involvement of the muscularis mucosae. This disease involves children and young adults, occasionally associated with other localisations of leiomyomatosis. The case of a 21 year old female, successfully treated by total esophagectomy, is reported herein. In this patient, proteinuria and hematuria led to the discussion of an aspect of Alport's syndrome which is characterized by association of leiomyomatosis, deafness, cataract, and hematuria.