Segmental colitis caused by idiopathic myointimal hyperplasia of mesenteric veins

Abstract

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...

Idiopathic myointimal hyperplasia of the mesenteric veins: Case report and review of the literature

“…Of the 34 cases of IMHMV that occurred in men, usually in late middle age (median age at diagnosis 62 years) . Many of these patients, including our patient, have cardiovascular risk factors or disease .…”

“…The etiology of this nonthrombotic occlusion of mesenteric veins is poorly understood. First described by Genta and Haggitt in 1991, 33 reported cases of IMHMV have now been reported (Table ), and it is likely there are many more misdiagnosed or not reported.…”

“…The non‐specific presenting symptoms include diarrhea, usually bloody; lower abdominal pain; and weight loss, resulting in the frequent misdiagnosis of IBD. Patients are often treated with corticosteroids or other IBD therapies such as 5‐aminosalicylates, immunomodulators, or biologic agents …”

“…The radiological appearance of IMHMV on computed tomography (CT) is suggestive of colitis, with thick, edematous bowel and fat stranding . Strictures in the sigmoid colon have also been reported .…”

Segmental intestinal necrosis in a young patient

“…Segmental ischemic colitis in young patients is an uncommon disease and the main cause is cocaine abuse (2). However, there are other less frequent causes such as intestinal inflammatory disease, autoimmune vasculitis, myointimal hyperplasia of mesenteric vein, infectious colitis and neuroleptic drugs (1,3). This patient was under treatment with four antiepileptic drugs although; intestinal ischemia is not one of its side effects.…”