Mesenteric inflammatory veno-occlusive disease occurring during the course of ulcerative colitis: a case report
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Mesenteric 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).
A 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.
We report a very rare case in which MIVOD occurred during the course of UC.
KeywordsMesenteric inflammatory veno-occlusive disease Enterocolic lymphocytic phlebitis Intramural mesenteric venulitis Chronic intestinal lymphocytic micro phlebitis Ulcerative colitis
Acute respiratory distress syndrome
Granulocyte Monocyte Apheresis
Mesenteric inflammatory veno-occlusive disease
Mesenteric inflammatory veno-occlusive disease (MIVOD) is a rare disease characterized by lymphocytic inflammation of the intestinal wall and mesenteric veins and venules, without arterial involvement and without evidence of systemic vasculitis, which results in intestinal ischemia . MIVOD is difficult to diagnose because of its rarity, nonspecific clinical findings, and frequent confusion with other diseases, along with the necessity for histopathological verification . Drugs, cytomegalovirus, bone marrow transplants, and antiphospholipid antibody syndrome are all involved in the disease, but a detailed pathogenesis remains unknown [3, 4, 5, 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]. Here we report a very rare case in which MOVID occurred during the course of ulcerative colitis (UC).
The first case of isolated mesenteric venous inflammation and associated thrombosis leading to mesenteric ischemia was described in 1976 . Since then, various terminologies have been used to describe these pathological findings, including necrotizing and enterocolic lymphocytic phlebitis , idiopathic myointimal hyperplasia , intramural mesenteric venulitis, and most recently, MIVOD.
The clinical symptoms of patients with MIVOD are abdominal pain and mucus per rectum or bloody diarrhea of variable duration. Endoscopic findings are not typical. Several reports show that contrast-enhanced CT and angiography are useful [7, 12]. However, in our case, contrast-enhanced CT showed colonic wall thickening, but was unable to detect the abnormalities of the mesenteric veins. The diagnosis of MIVOD is based on histopathological findings combined with the exclusion of other systemic diseases such as Buerger’s disease, Behçet’s disease, rheumatoid arthritis, and systemic lupus erythematosus .
Histopathological findings of MIVOD show isolated vasculitis of the small mesenteric veins and their intramural tributaries with thrombosis as a secondary manifestation, but arterial involvement is not seen . Some subacute cases show granulomatous phlebitis and myointimal hyperplasia, as observed in our case.
In addition to the similarity of the clinical symptoms and endoscopic images between MIVOD and UC, endoscopic biopsies from patients with MIVOD show nonspecific mucosal inflammation consistent with UC because these endoscopic biopsies may not include the submucosal vessels that are necessary for an accurate diagnosis . Therefore, MIVOD is often misdiagnosed and treated as UC, and when the appropriate treatment for UC is unsuccessful, colectomy must be performed, resulting in the pathological diagnosis of MIVOD .
Although our case was clinically and pathologically consistent with UC at the disease onset, his clinical course suddenly worsened 3 years later, resulting in a subtotal colectomy and the pathologically proven diagnosis of MIVOD. Because UC and MIVOD have similar endoscopic findings and there are no endoscopic findings specific to MIVOD, it is very difficult to diagnose MIVOD with endoscopic findings alone. In addition, since the postoperative specimen revealed that the surface mucosal structure of the colon at the site where phlebitis and venous thrombus existed was relatively maintained, it seemed unlikely that secondary thrombus was formed by exacerbation of UC. Therefore, we contend that this case of MIVOD developed during the course of UC.
Generally, the postoperative course of MIVOD is good; however, in our case, certain issues remained, such as residual rectal inflammation and persistent small bleeding. Although it is advantageous to remove the residual rectum and close the ileostomy, the possibility of anastomotic leakage must be sufficiently evaluated. At the present time, we currently resort to conservative observation, with the need for careful, continued evaluation of the residual rectum and lower small intestine in the future.
MIVOD has no specific clinical findings and the final diagnosis is based on the histologic diagnosis of resected intestinal tract, resulting in a small number of reported cases of MIVOD. Furthermore, cases of MIVOD developing during the course of UC appear to be very rare. Further investigation of the mechanism of MIVOD onset and its relationship with UC is necessary through the accumulation of more cases in the future.
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YY1 and KS wrote the paper; YY2, YS and YH contributed to the paper design and coordination; YA, YO and TA contributed to the pathological examination. All authors have read and approved the manuscript.
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Written consent was obtained from the patient. As a case report, approval from the institutional review board was not needed.
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