Intra-abdominal manifestations of von Recklinghausen′s neurofibromatosis

Neurofibromatosis type-1 (NF1), also known as von Recklinghausen disease, is a common autosomal dominant condition with an approximate incidence of one per 3000 births. NF1 involves multiple systems of the body. Abdominal involvement occurs in the form of neurofibroma and tumour growth in the liver,...

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Bibliographic Details
Main Author: Rastogi Rajul
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2008-01-01
Series:The Saudi Journal of Gastroenterology
Subjects:
Online Access:http://www.saudijgastro.com/article.asp?issn=1319-3767;year=2008;volume=14;issue=2;spage=80;epage=82;aulast=Rastogi
Description
Summary:Neurofibromatosis type-1 (NF1), also known as von Recklinghausen disease, is a common autosomal dominant condition with an approximate incidence of one per 3000 births. NF1 involves multiple systems of the body. Abdominal involvement occurs in the form of neurofibroma and tumour growth in the liver, mesentery, retroperitoneum, gastric and bowel. Gastrointestinal neoplasms have a reported occurrence of 2-25%. Two cases are reported herein as well as a review of the literature of the intra-abdominal manifestations of NF1, including a discussion on the radiological appearance and diagnosis. The article provides an insight into the intriguing variety of clinical problems that such patients may present.
ISSN:1319-3767
1998-4049