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肠系膜静脉特发性肌内膜增生症:因姑息性手术不足而在切除术后确诊

Idiopathic Myointimal Hyperplasia of the Mesenteric Veins: Diagnosed After Resection due to Insufficient Palliative Surgery.

作者信息

Hoshino Natsuki, Yamamoto Jun, Obara Nao, Takei Shogo, Nakao Eiichi, Shimizu Yasuhiro, Tanaka Yusaku, Yabuno Taichi, Hayashi Hiroyuki, Mochizuki Yasuhisa

机构信息

Department of Gastrointestinal Surgery, Yokohama Municipal Citizen's Hospital, Yokohama, Kanagawa, Japan.

Department of Inflammatory Bowel Disease, Yokohama Municipal Citizen's Hospital, Yokohama, Kanagawa, Japan.

出版信息

Surg Case Rep. 2025;11(1). doi: 10.70352/scrj.cr.25-0129. Epub 2025 Jun 7.

DOI:10.70352/scrj.cr.25-0129
PMID:40511447
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12162148/
Abstract

INTRODUCTION

Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare, non-thrombotic ischemic bowel disease caused by the proliferation of smooth muscle cells in the venous intima. Most patients are initially diagnosed with typical ischemic colitis or inflammatory bowel disease (IBD) and are treated nonsurgically. There is no established treatment for IMHMV, and surgery is the mainstay of treatment, although the optimal surgical approach remains unclear.

CASE PRESENTATION

A 66-year-old man visited the previous hospital with abdominal pain and was clinically diagnosed with ischemic colitis. Diagnostic colonoscopy showed inflammation from the sigmoid colon to the lower rectum. The biopsy did not reveal malignancy or IBD. His clinical condition did not improve after 2 months of conservative treatment, and he underwent a diverting colostomy. The symptoms temporarily improved, and he was referred to our hospital. However, 1 month postoperatively, computed tomography showed a rupture of the sigmoid colon and fluid collection around the sigmoid colon. He was diagnosed with perforation of the sigmoid colon due to relapse of ischemic colitis and underwent Hartmann's procedure. Histopathological examination showed stenosis and obstruction of the venous lumen at the perforation site with whole-layer necrosis, and proliferation of smooth muscle cells in the venous intima. The pathological diagnosis was IMHMV. He was discharged from our hospital 23 days after surgery without major complications and has had no symptoms or recurrence 8 months after surgical resection.

CONCLUSIONS

IMHMV is a rare disease, infrequently suspected preoperatively and typically diagnosed after surgical resection. Palliative surgery, such as colostomy, may not be a sufficient treatment for IMHMV.

摘要

引言

肠系膜静脉特发性肌内膜增生(IMHMV)是一种罕见的非血栓性缺血性肠病,由静脉内膜平滑肌细胞增殖引起。大多数患者最初被诊断为典型的缺血性结肠炎或炎症性肠病(IBD),并接受非手术治疗。IMHMV尚无既定的治疗方法,手术是主要的治疗手段,尽管最佳手术方式仍不明确。

病例介绍

一名66岁男性因腹痛前往之前的医院就诊,临床诊断为缺血性结肠炎。诊断性结肠镜检查显示从乙状结肠到直肠下段有炎症。活检未发现恶性肿瘤或IBD。经过2个月的保守治疗后,他的临床状况没有改善,于是接受了转流性结肠造口术。症状暂时改善,随后他被转诊至我院。然而,术后1个月,计算机断层扫描显示乙状结肠破裂及乙状结肠周围有积液。他因缺血性结肠炎复发被诊断为乙状结肠穿孔,并接受了哈特曼手术。组织病理学检查显示穿孔部位静脉腔狭窄和阻塞,伴有全层坏死,静脉内膜平滑肌细胞增殖。病理诊断为IMHMV。他术后23天从我院出院,无重大并发症,手术切除8个月后无症状且未复发。

结论

IMHMV是一种罕见疾病,术前很少被怀疑,通常在手术切除后确诊。姑息性手术,如结肠造口术,可能不足以治疗IMHMV。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/82da2d52e910/scr-11-01-25-0129-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/908373e52e37/scr-11-01-25-0129-g001.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/38200883026a/scr-11-01-25-0129-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/82da2d52e910/scr-11-01-25-0129-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/908373e52e37/scr-11-01-25-0129-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/5f44b47211dd/scr-11-01-25-0129-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/46b7378aed1c/scr-11-01-25-0129-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c06c/12162148/38200883026a/scr-11-01-25-0129-g004.jpg
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本文引用的文献

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Surgery. 2023 Sep;174(3):473-479. doi: 10.1016/j.surg.2023.04.014. Epub 2023 Jun 8.
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Idiopathic Myointimal Hyperplasia of the Mesenteric Veins: A Case Report and Scoping Review of Previously Reported Cases From Clinical Features to Treatment.
肠系膜静脉特发性肌内膜增生症:一例报告及既往报道病例从临床特征到治疗的范围综述
Front Med (Lausanne). 2022 Apr 13;9:855335. doi: 10.3389/fmed.2022.855335. eCollection 2022.
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Idiopathic Myointimal Hyperplasia of Mesenteric Veins (IMHMV) with two spontaneous bowel perforations: A case report and literature review.伴有两次自发性肠穿孔的肠系膜静脉特发性肌内膜增生(IMHMV):病例报告及文献综述
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