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神经肌肉血管错构瘤作为小肠梗阻的罕见病因

Neuromuscular and vascular hamartoma as an unusual cause of small bowel obstruction.

作者信息

Elstner Kristen E, Vickers Christopher R, Gett Rohan M, Qiu Min R, Killen Louise V

机构信息

Department of Colorectal Surgery, St. Vincent's Hospital, Darlinghurst, NSW, Australia.

Department of Gastroenterology, St. Vincent's Hospital, Darlinghurst, NSW, Australia.

出版信息

Int J Surg Case Rep. 2016;26:1-3. doi: 10.1016/j.ijscr.2016.06.042. Epub 2016 Jul 6.

Abstract

INTRODUCTION

Neuromuscular and vascular hamartoma (NMVH) is a rare, controversial lesion of the intestine, with only 23 cases reported in the English literature since its initial description in 1982.

PRESENTATION OF CASE

A 59year old female suffering from longstanding Crohn's disease with chronic stricture presented with symptoms of small bowel obstruction. Contrast studies demonstrated massive dilatation of the proximal small bowel. Laparotomy identified a 5cm long stenotic segment of ileum, with grossly distended jejunum and ileum proximally. Pathology determined the stricture's aetiology as a neuromuscular and vascular hamartoma of the small intestine.

DISCUSSION

NMVH is a benign lesion of hamartomatous origin. Its very existence is questionable due to histological similarities with several reactive pathologies, such as Crohn's and diaphragm diseases.

CONCLUSION

NMVH could be confused with a spectrum of chronic inflammatory bowel conditions, but this report establishes it as a distinct cause of chronic bowel obstruction.

摘要

引言

神经肌肉血管错构瘤(NMVH)是一种罕见的、有争议的肠道病变,自1982年首次描述以来,英文文献中仅报道了23例。

病例介绍

一名59岁患有长期克罗恩病并伴有慢性狭窄的女性出现小肠梗阻症状。造影检查显示近端小肠大量扩张。剖腹手术发现一段5厘米长的回肠狭窄段,近端空肠和回肠明显扩张。病理检查确定该狭窄的病因是小肠神经肌肉血管错构瘤。

讨论

NMVH是一种错构瘤源性的良性病变。由于其组织学与几种反应性病变(如克罗恩病和膈疾病)相似,其存在本身就存在疑问。

结论

NMVH可能与一系列慢性炎症性肠病相混淆,但本报告将其确立为慢性肠梗阻的一个独特病因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd87/4949502/6fb173fc336e/gr1.jpg

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