引用文本：董旻昱, 龚晓蓉. 特发性肠系膜静脉肌内膜增生症的临床研究进展[J/CD]. 消化肿瘤杂志（电子版）, 2025, 17(4): 557-563.

作者：董旻昱，龚晓蓉

单位：广州医科大学附属第一医院消化内科，广东 广州 510120

Authors：Dong Minyu，Gong Xiaorong

Unit：Department of Gastroenterology, the Key Laboratory of Advanced Interdisciplinary Studies, the First Affiliated Hospital of Guangzhou Medical University, Guangzhou 510120, Guangdong, China

摘要：

特发性肠系膜静脉肌内膜增生症（idiopathic myointimal hyperplasia of the mesenteric veins, IMHMV）是一种罕见的肠道血管疾病，其特征为肠系膜静脉壁肌内膜异常增生，导致血管腔狭窄甚至闭塞，进而引起肠道缺血性损伤。该疾病临床表现多样，常见腹痛、腹泻、便血等非特异性症状，与炎症性肠病（inflammatory bowel disease, IBD），特别是克罗恩病或溃疡性结肠炎的临床表现高度相似。这种相似性导致IMHMV极易被误诊为IBD，从而接受不恰当的免疫抑制剂或抗炎治疗，最终延误病情，增加并发症的发生风险。IMHMV由于其罕见性、临床表现的非特异性以及诊断的困难性，使得多数患者在确诊时已出现肠梗阻、穿孔等严重并发症，需要急诊手术干预。本文旨在通过回顾和梳理国内外相关文献，重点探讨IMHMV的病因假说、临床特征、内镜及影像学表现、组织病理学特点以及治疗进展，以期为临床实践提供参考。

关键词：特发性肠系膜静脉肌内膜增生症；肠道血管疾病；毛细血管动脉化

Abstract：

Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare intestinal vascular disorder characterized by abnormal myointimal hyperplasia of the mesenteric venous wall, leading to luminal narrowing or even occlusion and subsequent ischemic intestinal injury. Its clinical presentation is highly variable; nonspecific symptoms such as abdominal pain, diarrhea, and hematochezia predominate and closely mimic those of inflammatory bowel disease (IBD)—particularly Crohn disease or ulcerative colitis. This phenotypic overlap frequently results in misdiagnosis of IMHMV as IBD, prompting inappropriate immunosuppressive or anti-inflammatory therapy and ultimately delaying correct management while increasing the risk of complications. Owing to its rarity, nonspecific manifestations, and diagnostic challenges, most patients are not correctly identified until advanced stages marked by bowel obstruction or perforation, necessitating emergent surgical intervention. This article systematically examines domestic and international literature to summarize current hypotheses regarding etiology, clinical features, endoscopic and imaging findings, histopathological hallmarks, and therapeutic progress, thereby providing practical guidance for clinical practice.

Key words：Idiopathic myointimal hyperplasia of the mesenteric veins; Intestinal vascular disease; Capillarization of arterioles

注：网络优先发布