Bloch P, Quijada J
Hôpital Américain de Paris, Unité de Chirurgie Digestive, Neuilly.
Gastroenterol Clin Biol. 1992;16(11):890-3.
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.
食管弥漫性平滑肌瘤病是一种罕见疾病，其特征为食管肌肉弥漫性肥大，不累及黏膜肌层。该病累及儿童和年轻人，偶尔与平滑肌瘤病的其他部位病变相关。本文报道了一名21岁女性患者，经全食管切除术成功治疗。在该患者中，蛋白尿和血尿引发了对阿尔波特综合征某一方面的讨论，该综合征的特征是平滑肌瘤病、耳聋、白内障和血尿同时存在。
