特发性肠系膜静脉肌内膜增生症临床病理特征观察及文献回顾

Clinical and pathological characteristics of idiopathic myointimal hyperplasia of mesenteric vein with review of literature

目的探讨特发性肠系膜静脉肌内膜增生症临床病理特征。方法收集1例少见的特发性肠系膜静脉肌内膜增生症,通过回顾临床信息、组织形态特点,复习国内外相关文献,总结其临床病理特征。结果患者女性,30岁,间断性右下腹痛6个月余。以克罗恩病治疗无效,随后手术切除右半结肠。术前活检见固有层纤维素沉积及黏膜下层“动脉化”毛细血管;手术标本发现肠系膜侧显著的中小静脉肌内膜增生,部分静脉腔显著狭窄或闭塞,弹力纤维染色提示内膜下弹力纤维增生紊乱,考虑特发性肠系膜静脉肌内膜增生。结论特发性肠系膜静脉肌内膜增生症是一种少见的缺血性结肠炎,确诊依赖手术标本肠系膜中小静脉肌内膜的增生,活检标本内皮下纤维蛋白沉积和毛细血管“动脉化”或可帮助诊断。

Objective To investigate the clinicopathological characteristics of idiopathic mesentery vein intimal hyperplasia. Methods A case of rare idiopathic mesentery vein myointimal hyperplasia was collected, and its clinical and pathological characteristics were summarized by reviewing the clinical information, histological characteristics, and relevant literature at home and abroad. Results The patient was a 30-year-old female with intermittent right lower abdominal pain for over 6 months. The treatment for Crohn's disease was ineffective, and the right colon was subsequently surgically removed. Preoperative biopsy showed cellulose deposition in the lamina propria and “arterialized” capillaries in the submucosa. The surgical specimens found significant intimal hyperplasia of the middle and small veins on the mesentery side, and significant stenosis or occlusion of some venous cavities. Elastic fiber staining suggested that the elastic fiber hyperplasia under the intima was disordered. Idiopathic mesentery vein intimal hyperplasia was considered.Conclusion Idiopathic myointimal hyperplasia of mesentric vein is a rare ischemic colitis. The diagnosis depends on the hyperplasia of the myointima of the small and medium-sized mesentery veins in the surgical specimens, the deposition of fibrin under the endothelium of the biopsy specimens and the “arterialization” of capillaries, which may help the diagnosis.