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Journal of ZheJiang University(Medical Science)  2016, Vol. 45 Issue (3): 297-301    DOI: 10.3785/j.issn.1008-9292.2016.05.13
    
A case of neurofibromatosis complicated with small bowel obstruction
MAO Lingna1, GUO Qingqu2, WANG Guifeng1, SONG Zhenya1
1. Department of General Practice, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310009, China;
2. Department of General Surgery, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310009, China
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Abstract  

Neurofibromatosis type 1(NF-1) with gastrointestinal stromal tumor (GIST) is a rare case in clinical practice. But it is even rarer that the GIST tumor in this case is so large that it cause small intestinal obstruction. Here we report such a male case of NF-1,with positive family history of NF-1 and nodules all over the patient's skin and abdominal cavity. The patient came to hospital with a sudden upper abdominal pain and nausea and vomiting over 5 d. Abdominal computerized tomography (CT) showed that there were numerous nodules of different size in his abdominal cavity. And the largest one is about 10 cm in diameter,which oppressed the small bowel and caused the small bowel mesenteric volvulus and obstruction of the bowel. Finally,the tumor was pathologically proved to be a GIST tumor but not NF nodule. The patient stays healthy until now after operation.



Key wordsNeurofibromatosis 1/diagnosis      Neurofibromatosis 1/surgery      Intestinal obstruction/etiology      Intestine, small      Gastrointestinal stromal tumors/surgery      Case reports     
Received: 17 February 2016     
CLC:  R739  
Cite this article:

MAO Lingna, GUO Qingqu, WANG Guifeng, SONG Zhenya. A case of neurofibromatosis complicated with small bowel obstruction. Journal of ZheJiang University(Medical Science), 2016, 45(3): 297-301.

URL:

http://www.zjujournals.com/xueshu/med/10.3785/j.issn.1008-9292.2016.05.13     OR     http://www.zjujournals.com/xueshu/med/Y2016/V45/I3/297


1型神经纤维瘤病并发小肠梗阻一例

1型神经纤维瘤病(NF-1)合并胃肠道间质瘤(GIST)临床罕见,同时GIST肿块巨大且引起肠梗阻的病例更鲜有报道。本文报道一例有家族史的NF-1病例,患者全身皮肤以及腹腔内遍布结节,突发上腹部胀痛伴恶心、呕吐5 d。入院检查腹部CT检查提示腹腔内有巨大肿块并压迫小肠导致肠系膜扭转、小肠梗阻。最终手术证实腹腔内巨大肿块为GIST。手术后至今半年,患者恢复良好。


关键词: 神经纤维瘤病1型/诊断,  神经纤维瘤病1型/外科学,  肠梗阻/病因学,  小肠,  胃肠道间质肿瘤/外科学,  病例报告 
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