Idiopathic Myointimal Hyperplasia of Mesenteric Veins

Kao, Patricia C; Vecchio, James; Hyman, Neil; West, A. Brian; Blaszyk, Hagen

doi:10.1097/00004836-200509000-00011

Cited by 43 publications

(22 citation statements)

References 9 publications

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“…The lack of improvement despite multiple conventional therapies should, in such cases, suggest that the proctocolitis may not be due to IBD or infectious causes, and prompt a search for rare conditions. In the face of severe disease and morbidity, and failing diagnostic clarification, surgical resection may be curative, as it was in this case, an outcome supported by the published experience with IMHMV [1][2][3][4][5].…”

Section: Discussionsupporting

confidence: 65%

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An Intimate Cause of Proctocolitis

et al. 2015

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Section: Discussionsupporting

confidence: 65%

“…A previously reported rare cause, IMHMV, has also been identified as an etiology of chronic ischemic proctocolitis [1][2][3][4]. IMHMV is characterized by myointimal thickening and obliteration of the mesenteric veins usually only evident in surgical specimens.…”

Section: Discussionmentioning

confidence: 99%

An Intimate Cause of Proctocolitis

et al. 2015

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“…The precise incidence of IMHMV is unknown, as only 21 cases have been reported in the literature to date (Table I). This disease is known to occur predominantly in young, healthy men, with a sudden onset of crampy abdominal pain accompanying repeated episodes of diarrhea and bloody stools (4,8). The pathophysiologic mechanism seems to be an abnormal arteriovenous shunting with elevated venous pressure and consecutive congestion and ischemia of the involved bowel segment (9).…”

Section: Discussionmentioning

confidence: 99%

Segmental colitis caused by idiopathic myointimal hyperplasia of mesenteric veins

Costa¹,

Saiote²,

Pinheiro³

et al. 2016

Rev Esp Enferm Dig

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Diseases causing colonic ischemia may be mistaken with other causes of segmental colitis such as inflammatory bowel disease, especially in young patients. The authors present the case of a 47-year-old male with severe proctosigmoiditis. Assessment excluded infectious causes, thrombophilia and systemic vasculitis. The initial histological specimen was suggestive of inflammatory bowel disease and therapy was initiated with intravenous steroids and, at day 5, infliximab, with no response. The patient was proposed for surgery. Pathological examination of the surgical specimen revealed an idiopathic myointimal hyperplasia of mesenteric veins, a rare entity exhibiting necrotizing phlebitis with rapid progression to segmental necrosis in the rectosigmoid colon. In this paper the authors discuss the differential diagnosis of proctosigmoiditis in young ages and the approach to this exceptionally rare ischemic entity. INTRODUCTIONDiseases causing colonic ischemia may have similar presentation symptoms to other causes of segmental colitis such as inflammatory bowel disease, especially in young patients. Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare condition causing segmental colonic ischemia that should be considered in the differential diagnosis of severe colonic inflammatory bowel disease refractory to intensive medical treatment. Preoperative diagnosis of IMHMV could be difficult as intimal thickening venules are in the submucosa and deeper layers. Even when a full-thickness biopsy is performed the diagnosis may be inconclusive. Standard treatment is surgical resection and there are no reports of postoperative disease recurrence. The following case illustrates this rare clinical condition in a young adult male patient. CASE REPORTA 47-year-old Caucasian male patient was admitted to our medical department on February 2012 with bloody diarrhea (more than 20 stools per day), lower abdominal cramping pain, proctalgia and malaise.The patient reported a previous 9 month history of hypogastric cramp-like pain, 4-5 small volume stools, without blood, mucus or pus, as well as a compelling urge to defecate and fecal incontinence. One month before, the patient had been assessed on the emergency department of another hospital due to clinical deterioration with anal pain, persistent urge to defecate, tenesmus and straining at stool. There, he had undergone a colonoscopy with biopsy and a pelvic CT scan. The colonoscopy showed edema of the rectal mucosa and sigmoid colon. The biopsies did not reveal any significant alterations. The pelvic CT scan identified parietal thickening of the rectum. At that time, the patient was treated with an antispasmodic. No improvement was observed.Patient's personal and family histories were irrelevant and epidemiological context was unremarkable. He had not been undergoing any other course of medication and denied known allergies, smoking, alcohol and history of injected or inhaled drug use.Upon physical examination, he was afebrile with lower abdominal tenderness w...

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“…Drugs, cytomegalovirus, bone marrow transplants, and antiphospholipid antibody syndrome are all involved in the disease, but a detailed pathogenesis remains unknown [3–6]. MIVOD is mostly resistant to medical treatment, as anticoagulants and immunoregulatory drugs have proven ineffective for patients with MIVOD [7, 8]. In most cases, surgical resection of the involved bowel is required and prognosis is typically excellent [7, 8].…”

Section: Introductionmentioning

confidence: 99%

“…MIVOD is mostly resistant to medical treatment, as anticoagulants and immunoregulatory drugs have proven ineffective for patients with MIVOD [7, 8]. In most cases, surgical resection of the involved bowel is required and prognosis is typically excellent [7, 8]. Here we report a very rare case in which MOVID occurred during the course of ulcerative colitis (UC).…”

Section: Introductionmentioning

confidence: 99%

Mesenteric inflammatory veno-occlusive disease occurring during the course of ulcerative colitis: a case report

et al. 2018

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BackgroundMesenteric inflammatory veno-occlusive disease (MIVOD) is difficult to diagnose because of its rarity, nonspecific clinical findings, and frequent confusion with other diseases including inflammatory bowel disease. This report presents a very rare case of MIVOD that occurred during the course of ulcerative colitis (UC).Case presentationA 32-year-old man, who had been diagnosed with UC at the age of 29 and was in remission maintained by oral administration of 5-aminosalicylic acid (5-ASA), showed exacerbation of diarrhea and was admitted to the hospital. Since it was deemed an exacerbation of UC, intravenous steroid therapy and oral administration of tacrolimus were initiated, but his condition continued to worsen. Abdominal computed tomography (CT) was performed and showed intraperitoneal free air, leading to a diagnosis of gastrointestinal perforation and the performance of emergency surgery (subtotal colectomy and ileostomy). Histopathological examination of the resected colon of the patient showed mucosal inflammatory findings that were not typical of UC, including multiple organized thrombi with recanalization in the veins existing in the submucosal layer to the subserosal layer and an increased infiltration of inflammatory cells. These findings led to the pathological diagnosis of MIVOD.ConclusionWe report a very rare case in which MIVOD occurred during the course of UC.

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Idiopathic Myointimal Hyperplasia of Mesenteric Veins

Cited by 43 publications

References 9 publications

An Intimate Cause of Proctocolitis

An Intimate Cause of Proctocolitis

Segmental colitis caused by idiopathic myointimal hyperplasia of mesenteric veins

Mesenteric inflammatory veno-occlusive disease occurring during the course of ulcerative colitis: a case report

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