Idiopathic myointimal hyperplasia of the mesenteric veins is an extremely rare non-thrombotic mesenteric veno-occlusive disease. The management of idiopathic myointimal hyperplasia of the mesenteric veins is not well-established, and although surgery is the mainstay of treatment, the optimal operation remains unclear. Therefore, we aimed to perform a systematic review to assess the various surgical procedures and associated outcomes for patients with idiopathic myointimal hyperplasia of the mesenteric veins.
A systematic search for articles published from 1946 to April 2022 in MEDLINE, EMBASE, Cinahl, Scopus, Web of Science, and Cochrane Library databases is reported. In addition, we report 4 cases of idiopathic myointimal hyperplasia of the mesenteric veins managed at our institution until March 2023.
A total of 53 studies and 88 patients with idiopathic myointimal hyperplasia of the mesenteric veins were included. Most (82%) were male patients, with a mean age of 56.6 years old. The majority (99%) of patients required surgery. Most reports described the involvement of the rectum and sigmoid colon (81%). The most common surgical procedures were Hartmann’s procedure (24%) and segmental colectomy (19%); completion proctectomy with ileal pouch-anal anastomosis was performed in 3 (3.4%) cases. In 6 (6.8%) cases, idiopathic myointimal hyperplasia of the mesenteric veins was suspected preoperatively and managed with elective surgery. Four (4.5%) complications were reported. Nearly all (99%) patients achieved remission with surgical intervention.
Idiopathic myointimal hyperplasia of the mesenteric veins is a rare pathologic entity infrequently suspected preoperatively and typically diagnosed after surgical resection. Surgical resection with Hartmann’s procedure or segmental colectomy was most commonly performed, with completion proctectomy and ileal pouch-anal anastomosis reserved for cases of extensive rectal involvement. Surgical resection was safe and effective, with a low risk of complications and recurrence. Surgical decision-making should be based on the extent of the disease at the time of presentation.

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