Intestinal obstruction in von Recklinghausen's disease. (October 2014)

• Record Type: Journal Article
• Authors: Trilling, B.; Faucheron, J.‐L.
• Is Part Of: Colorectal disease. Volume 16:Number 10(2014)
• Page Start: 762
• Page End: 768
• Publication Date: 2014-10
• Subjects: Colon (Anatomy) -- Diseases -- Periodicals; Rectum -- Diseases -- Periodicals; 616.34
• DOI: 10.1111/codi.12649
• Languages: English
• ISSNs: 1462-8910

Abstract

<abstract abstract-type="main" id="codi12649-abs-0001"> <title>Abstract</title> <sec id="codi12649-sec-0001" sec-type="section"> <title>Aim</title> <p>Gastrointestinal manifestations occur in up to 25% of patients with neurofibromatosis type 1. This review reports all published cases of acute intestinal obstruction due to neurofibromatosis type 1 and identifies mechanisms of obstruction, the nature of the tumour and the outcome.</p> </sec> <sec id="codi12649-sec-0002" sec-type="section"> <title>Method</title> <p>A systematic review of the literature on acute intestinal obstruction due to neurofibromatosis type 1 was performed by searching the major electronic databases. All relevant references were reviewed for possible inclusion. All the references of the relevant articles were screened for any further articles that were missed in the initial search.</p> </sec> <sec id="codi12649-sec-0003" sec-type="section"> <title>Results</title> <p>We identified 25 articles from 1972 to 2013 reporting 25 patients with von Recklinghausen's disease who underwent laparotomy for acute intestinal obstruction. Three further patients were operated on in our institution. The mean age of the patients was 44.2 years and the male/female ratio 15/13. The mechanisms of obstruction were intrinsic obstruction (16), extrinsic obstruction (8) and intussusception (4). Histology was reported to show neurofibroma (19), gastrointestinal stromal tumour (5) and adenocarcinoma (4). Among patients whose outcome was mentioned (17), 10 were asymptomatic after a mean follow‐up of 1.5 years.</p> </sec> <sec id="codi12649-sec-0004" sec-type="section"> <title>Conclusion</title> <p>Acute bowel obstruction is a rare manifestation of neurofibromatosis type 1. Surgery is often necessary to treat the complication and to determine the exact nature of the tumour and the prognosis.</p> </sec> </abstract>