食管神经纤维瘤病

Neurofibromatosis of the esophagus.

作者信息

Sica Giuseppe S, Sujendran Vejay, Warren Brian, Maynard Nicholas D

机构信息

Department of Surgery, John Radcliffe Hospital, Headington, Oxford, United Kingdom.

出版信息

Ann Thorac Surg. 2006 Mar;81(3):1138-40. doi: 10.1016/j.athoracsur.2005.02.022.

DOI:10.1016/j.athoracsur.2005.02.022

PMID:16488750

Abstract

There have been previous reports suggesting an association between von Recklinghausen's neurofibromatosis and esophageal dysmotility. We report the first case of true Recklinghausen's neurofibromatosis of the esophagus leading to end-stage pseudoachalasia. The diagnosis and management of this condition is discussed together with the pathogenesis and pathology of this rare entity.

摘要

此前已有报道表明冯雷克林霍增氏神经纤维瘤病与食管运动障碍之间存在关联。我们报告首例导致终末期假性贲门失弛缓症的食管真性雷克林霍增氏神经纤维瘤病病例。本文讨论了该疾病的诊断、治疗以及这一罕见病症的发病机制和病理。