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小肠扭转作为冯雷克林霍增氏病的并发症:一例报告

Small bowel volvulus as a complication of von Recklinghausen's disease: a case report.

作者信息

Werner Thomas Artur, Kröpil Feride, Schoppe Martin Olaf, Kröpil Patric, Knoefel Wolfram Trudo, Krieg Andreas

机构信息

Thomas Artur Werner, Feride Kröpil, Wolfram Trudo Knoefel, Andreas Krieg, Department of Surgery (A), Heinrich-Heine-University and University Hospital Duesseldorf, 40225 Duesseldorf, Germany.

出版信息

World J Gastroenterol. 2014 Jun 28;20(24):7979-83. doi: 10.3748/wjg.v20.i24.7979.

Abstract

We report the case of a 25-year-old male with Neurofibromatosis type I (NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstruction. Computerized tomography showed a volvulus of the terminal ileum with mesenteric swirling as the cause of the patient's symptoms. Consecutive exploratory laparotomy confirmed the diagnosis and 70 cm of the small intestine was resected due to an affection of the mesentery by multiple neurofibromas. The gastrointestinal tract is affected in approximately 10% of patients with NF-1, however the mesentery is almost always spared. Here we describe the unique case of a patient with a volvulus caused by mesenteric manifestation of von Recklinghausen's disease, emphasizing the role of surgery in a team of multidisciplinary specialists to treat this multiorganic disease.

摘要

我们报告了一例25岁的I型神经纤维瘤病(NF-1)男性患者,入院时表现为机械性肠梗阻导致的急腹症临床症状。计算机断层扫描显示末端回肠扭转并伴有肠系膜漩涡,这是患者症状的原因。连续的剖腹探查术确诊了诊断,由于多个神经纤维瘤累及肠系膜,切除了70厘米的小肠。约10%的NF-1患者胃肠道会受到影响,然而肠系膜几乎总是幸免。在此我们描述了一例由冯·雷克林豪森病肠系膜表现引起肠扭转的独特病例,强调了手术在多学科专家团队治疗这种多器官疾病中的作用。

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本文引用的文献

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The pathoetiology of neurofibromatosis 1.神经纤维瘤病 1 的发病机制。
Am J Pathol. 2011 May;178(5):1932-9. doi: 10.1016/j.ajpath.2010.12.056. Epub 2011 Mar 31.
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Neurofibromatosis type 1 revisited.1型神经纤维瘤病再探讨。
Pediatrics. 2009 Jan;123(1):124-33. doi: 10.1542/peds.2007-3204.

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