Idiopathic Myointimal Hyperplasia of Mesenteric Veins (IMHMV) with two spontaneous bowel perforations: A case report and literature review

Abstract

Introduction and importance Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a poorly understood disorder which poses a diagnostic challenge to clinicians and pathologists. Here we have described the case of a male patient with IMHMV along with a presumed history of ulcerative colitis for 1 year. Case presentation A 55-year-old male presented to the OPD with history of chronic abdominal pain. Clinical and radiological examination coupled with endoscopic findings resulted in the patient being wrongly diagnosed to be a case of ulcerative colitis and was managed accordingly. Throughout his multiple hospital visits following treatment for ulcerative colitis, the patient was persistently symptomatic. He presented with 10 days history of increasing abdominal pain and constipation following which he developed spontaneous colonic perforation for which he underwent exploratory laparotomy left colectomy and Hartman's procedure. The final pathology of the resected colon found to be consistent of Idiopathic myointimal hyperplasia of the mesenteric veins and ischemic bowel changes. Clinical discussion The absence of clear-cut endoscopic biopsy findings of ulcerative colitis made radiological picture to be the mainstay for diagnosis, which was inaccurate and exposed the patient to unnecessary treatment with immuno-modulators thus resulting in poor response to treatment. As the disease progressed, further narrowing of the vessels made the clinical picture to look closer to ischemic bowel pathology as the patient developed a top surgical emergency (i.e. bowel perforation). Such pathological finding (IMHMV) can only be diagnosed in a fully prepared tissue histology, but rather be considered when no other consistent alternative diagnosis was found. Conclusion The treating physicians must definitely consider the possibility of idiopathic myointimal hyperplasia of mesenteric veins when similar manifestations are encountered in biopsy specimens of old cases with suspected inflammatory bowel disease or non-occlusive ischemia of the distal colorectum.

Idiopathic Myointimal Hyperplasia of the Mesenteric Veins: A Case Report and Scoping Review of Previously Reported Cases From Clinical Features to Treatment

“…The etiology of IMHMV remains unclear. The underlying diseases in the reported cases include hypertension, aortic valve replacement, diabetes mellitus, cerebral infarction, abdominal surgery, pulmonary embolism, hyperlipidemia, primary biliary cirrhosis, and renal transplantation ( 3 – 11 ). However, most patients were previously healthy with no significant drug history, and due to the rarity of IMHMV, it is still uncertain whether these underlying diseases correlate with the occurrence of vessel abnormalities.…”

“…The clinical course is usually chronic, involving weeks or months. However, there were eight cases with acute onset of the disease documented in the literature with complaints of watery diarrhea and abdominal pain ( 7 , 11 , 17 , 25 – 29 ). The mean age at diagnosis was 58 (range, 21–83) years.…”

“…At the early diseasestage, edematous, erythematous, and friable colonic mucosa may be seen, while ulceration and inflammatory exudates develop later with disease progression ( 11 , 31 ). A case of mucosal edematous and erythematous lesions at onset was reported that progressed to more severe patchy ulcerations and inflammatory exudates within several weeks ( 31 ).…”

“…A case of mucosal edematous and erythematous lesions at onset was reported that progressed to more severe patchy ulcerations and inflammatory exudates within several weeks ( 31 ). A recent case study also described a patient with endoscopic findings of mild inflammation and congestion of the colon on admission which progressed to severely inflamed mucosa with significant narrowing of the lumen 2 weeks later ( 11 ). This indicates that the obvious mucosal changes may lag behind the onset of clinical symptoms, which manifest as a mild pattern at the early stage of the diseaseand that ulcers or luminal stricture may appear rapidly in weeks depending on the severity of vascular stenosis.…”

Endovascular approach in the management of idiopathic myointimal hyperplasia of the inferior mesenteric vein

“…Patients present with nonspecific symptoms that mimic IBD, including severe abdominal pain, weight loss, rectal bleeding, and diarrhea or constipation (Song and Shroff 2017 ). Patients may also present with subtle bowel perforations (Almumtin et al 2021 ). Initial radiologic evaluation of IMHMV patients may yield findings suggestive of colitis, and endoscopy can produce nonspecific inflammatory findings, both of which were seen in this patient.…”