Song Sharon J, Shroff Stuti G
Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA.
Case Rep Pathol. 2017;2017:6793031. doi: 10.1155/2017/6793031. Epub 2017 Aug 15.
Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare disease characterized by intimal smooth muscle proliferation, leading to the thickening of small to medium-sized mesenteric veins. This vascular disease mimics inflammatory bowel disease (IBD) clinically and endoscopically, while showing ischemic mucosal changes without the classic features of IBD on biopsy. Given the mixed picture, this entity is frequently misdiagnosed. Surgical resection of the diseased bowel segment reveals the true etiology of the pathology and is curative. We describe a case of a 59-year-old man with a long-standing history of Crohn's disease refractory to medical therapy and status after multiple small bowel resections. The patient underwent a subtotal abdominal colectomy with pathology showing dense, indurated mesenteric adipose tissue, significant muscularis propria hypertrophy, and myointimal hyperplasia of the mesenteric veins in a peri-ileal and pericolic distribution, as confirmed by elastin stain. No evidence of mucosal ischemic changes or findings of chronicity or acuity were seen. IMHMV, a rare disease with a mixed presentation, poses a significant diagnostic challenge to clinicians and pathologists.
肠系膜静脉特发性肌内膜增生(IMHMV)是一种罕见疾病,其特征为内膜平滑肌增殖,导致中小肠系膜静脉增厚。这种血管疾病在临床和内镜检查中类似炎症性肠病(IBD),但活检时显示缺血性黏膜改变,却无IBD的典型特征。鉴于这种复杂情况,该病症常被误诊。手术切除病变肠段可揭示病理的真正病因,且具有治愈性。我们描述了一例59岁男性患者,有长期克罗恩病病史,药物治疗无效,且经历多次小肠切除术后的情况。患者接受了次全腹结肠切除术,病理显示致密、硬结的肠系膜脂肪组织、显著的固有肌层肥大以及回肠周围和结肠周围分布的肠系膜静脉肌内膜增生,弹性蛋白染色证实了这一点。未见黏膜缺血改变或慢性或急性病变的迹象。IMHMV是一种表现复杂的罕见疾病,给临床医生和病理学家带来了重大的诊断挑战。