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CASE REPORT Table of Contents  
Ahead of print publication
Mesenteric inflammatory veno-occlusive disease of the colon: An under-recognized entity


1 Department of Histopathology, Apollo Hospitals, Chennai, Tamil Nadu, India
2 Department of Gastroenterology, Apollo Hospitals, Greams Road, Chennai, Tamil Nadu, India

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Date of Submission01-Jun-2022
Date of Decision20-Jul-2022
Date of Acceptance25-Jul-2022
Date of Web Publication22-Aug-2022
 

  Abstract 


Isolated nonthrombotic venulitis of the colon with concomitant ischemic injury, also known as mesenteric inflammatory veno-occlusive disease (MIVOD), is a rare inflammatory vascular disorder of unknown etiology. First described in 1994, there are only about 34 cases in the literature. We report a 25-year-old woman who presented with loose stools and abdominal pain of 3-week duration. Investigations revealed eosinophilia. Endoscopy showed erosion and ulcers of the gastrointestinal tract, with mucosal biopsies showing features of ischemic injury. Computed tomography showed an edematous and thickened colonic wall with impending perforation, hence, a subtotal colectomy was performed. The patient, however, had a cardiac arrest and expired despite adequate measures. The resection specimen was consistent with a diagnosis of MIVOD. As the clinical and radiological features of MIVOD are nonspecific and overlap with inflammatory bowel disease or mesenteric ischemic disorders, histopathological examination of the resection specimen is the mainstay for its diagnosis. We believe this is the only other case in the literature of MIVOD with eosinophil predominance.

Keywords: Eosinophil predominance, isolated phlebitis, mesenteric inflammatory veno-occlusive disease


How to cite this URL:
Shah SN, Bhayani P, Parameswaran SA, Parameswaran A. Mesenteric inflammatory veno-occlusive disease of the colon: An under-recognized entity. Apollo Med [Epub ahead of print] [cited 2022 Sep 27]. Available from: https://apollomedicine.org/preprintarticle.asp?id=354227





  Introduction Top


Mesenteric inflammatory veno-occlusive disease (MIVOD) is a rare inflammatory vascular disorder of unknown etiology, with ischemic injury secondary to isolated venulitis/phlebitis in the bowel wall or the mesentery. Vascular disorders of the gastrointestinal tract usually occur secondary to systemic vasculitis, autoimmune disorders, hypercoagulable states, trauma, and rarely with inflammatory bowel disease (IBD). Unlike these conditions, MIVOD is predominantly a phlebitic disorder, which is rarely suspected clinically and mostly diagnosed on resection specimens. The clinical and radiological features of MIVOD are nonspecific and overlap with IBD or mesenteric ischemic disorders. Mucosal biopsy rarely raises the possibility of the entity, with features of only ischemic injury. Histopathology of the resected specimen shows isolated lymphocytic and/or granulomatous phlebitis of the mesenteric and bowel wall veins, with resultant ischemic injury and hence is the only way to establish the diagnosis.[1],[2] Eosinophilic venulitis presenting as MIVOD is another extremely rare[3] and anecdotal reaction pattern which is seen in the current case.


  Case Report Top


A 25-year-old woman presented with complaints of nonfoul-smelling loose stools associated with blood and mucus of 3-week duration. Diabetes mellitus, hypertension, hypothyroidism, and polycystic ovaries have been managed for the past 10 years. Clinical examination showed tachycardia. Investigations revealed peripheral eosinophilia. Stool examination showed no parasites. The vasculitis panel showed a low C3 complement. Anti-ds DNA and anti-neutrophil cytoplasmic antibodies were negative. Eosinophilia fluorescence in situ hybridization panel was negative for PDGFRA, PDGFRB, and FGFR1 gene rearrangements. Endoscopy revealed erosions in the stomach and first part of the duodenum, ulcers of varying sizes and depth throughout the colon, with normal appearing intervening mucosa. Colonic mucosal biopsies revealed ischemic-type ulcers with features suspicious of vasculitis. Pulse therapy of methylprednisolone was given for 3 days, followed by oral prednisolone at 60 mg daily, which improved the patient's condition.

Six days later, she again had increased stool frequency and abdominal pain. An emergency contrast-enhanced computed tomography abdomen was suggestive of pancolitis with transverse colon gangrene and features of impending perforation [Figure 1]a, [Figure 1]b, [Figure 1]c. An emergency laparotomy was performed. Intraoperatively, the transverse colon was gangrenous with edematous ascending and hyperemic descending. Subtotal colectomy with an end ileostomy was performed. Unexpectedly, 4 h later, she had a cardiac arrest with pulseless ventricular tachycardia. She was anuric and unresponsive. Echocardiography excluded pulmonary thromboembolism. Cardiopulmonary resuscitation was performed, and postarrest measures were initiated. Continuous renal replacement therapy was recommended, which was not consented to by the family. She expired 6 h later.
Figure 1: (a-c) Contrast-enhanced CT scan of the abdomen shows a thinned-out, non-enhancing wall of the mid and distal transverse colon, with the rest of the colon revealing wall thickening with increased enhancement and submucosal edema. CT: Computed tomography

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Grossly, cecum and colon showed cobble stoning with focal areas of ulceration [Figure 2]a and [Figure 2]b with gangrenous transverse colon. Histology revealed multiple foci of ischemic necrosis with ulceration of the colonic mucosa with fissuring and fibrovascular reaction [Figure 3]a and [Figure 3]b. The colonic wall showed patchy severe inflammation, predominantly eosinophils admixed with some lymphocytes and plasma cells [Figure 3]c and [Figure 3]d. Mucosa showed no evidence of eosinophilic cryptitis or crypt abscesses. Veins of varying sizes demonstrated segmental necrosis of their walls with thrombi, mural, and perivascular infiltrates of eosinophils, with conspicuously uninvolved arteries adjacent to them [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d, [Figure 4]e, [Figure 4]f. One regional node showed extensive necrosis, with focal eosinophilic infiltrates at the periphery. The colonic morphology was compatible with an eosinophilic variant of MIVOD.
Figures 2: (a and b) Resected colon showing nodular thickening of the wall, ulceration of the mucosa and cobble stoning. Note the abrupt transition zone with the necrotic yellow colon

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Figure 3: (a and b) Colonic wall showing ulceration with fissuring, exudation and severe transmural ischemic necrosis, (H and E, ×40). (c and d) Colonic wall with marked submucosal edema and perivenous inflammation with eosinophil predominance, (H and E, ×100 and × 400), respectively

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Figure 4: (a-e) Veins with necrosis and mural and perivenous inflammation of thrombi (c). Note paired unaffected arteries, (H and E, ×100) (d) High power view of the inflamed vein with luminal and eosinophil-predominant inflammation, (H and E, ×400). (f) Isolated venulitis with adjacent uninvolved arteries, Elastic stain, ×100

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  Discussion Top


MIVOD was first described by Flaherty in 1994,[4] with the criteria for the diagnosis being the presence of phlebitis causing ischemic injury with the absence of arteritis or systemic vasculitis. There are about 34 cases of MIVOD in English literature[1] and one of the so-called “eosinophilic variants.”[3] The age of these patients ranged from 24 to 78 years, and the duration of symptoms varied from 1 day to 4 months.[1] Symptoms consisted of bloody stools with increased frequency, crampy abdominal pain, nausea, and vomiting. The organs involved were the jejunum, colon, gallbladder, omentum,[1] and spleen.[5] Radiologically, thickening of the colonic wall, poor mural enhancement, and prominent pericolic vessels with the irregular appearance of the inferior mesenteric vein should raise a suspicion of MIVOD.[6]

As the diagnostic pathologic features are not usually appreciated on endoscopic biopsy, histological examination on resection specimens is a must for a definitive diagnosis. The characteristic histological findings include lymphocytic and/or granulomatous venulitis in the submucosa with arterial sparing, ischemic injury of the mucosa with ulceration, exudation, and occasional pseudomembrane formation, with myointimal hyperplasia of the venous channels and secondary thrombus formation indicating chronicity.[1],[2] The present case had transmural eosinophil-predominant inflammation with eosinophilic venulitis, which was described earlier in a 41-year-old woman who presented with friable polypoidal eosinophil-rich inflammatory mass with venulitis in the cecum. One pericolic lymph node showed extensive necrosis,[3] also seen in the current case. The significance of this peculiar association is unclear and may be serendipitous.

The etiology of MIVOD remains unknown, although its occurrence has been associated with infections, autoimmune disorders, drugs, etc., and has been documented in a patient following stem cell transplantation. These varied scenarios suggest immune dysregulation.[2] Patients with MIVOD appear to have a reasonably good prognosis if prompt surgery is performed, with only two cases of recurrences and one case of death due to postoperative complications.[1] Our patient enigmatically expired in the postoperative period, possibly related to cardiac rhythm disturbances secondary to hypereosinophilia involving the heart, as has been described in Loeffler's disease. MIVOD should be differentiated from other clinical mimics, as medical therapy is ineffective.[2] A high index of suspicion combined with a detailed radiological and clinical assessment may lead to early diagnosis and timely surgical intervention.


  Conclusion Top


MIVOD is a rare intestinal vascular disorder which may mimic IBD clinically and radiologically in the early stages. Surgical resection is the definitive treatment, with pathological examination being the only way to confirm the diagnosis. We report a rare case of MIVOD in a young woman to increase awareness of this uncommon entity. Of note, the dominant inflammatory cell was the eosinophil, and this appears to be consistent with the so-called “eosinophilic variant” of MIVOD described in an earlier case in the literature.

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Conflicts of interest

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  References Top

1.
Matsuda K, Hashiguchi Y, Kikuchi Y, Asako K, Ohno K, Okada Y, et al. Successful surgical management of mesenteric inflammatory veno-occlusive disease. Surg Case Rep 2020;6:27.  Back to cited text no. 1
    
2.
Finan M, Browne E. Mesenteric inflammatory veno-occlusive disease: A rare mimic of venous thrombosis and fulminant colitis. Hum Pathol Case Rep 2017;8:18-9.  Back to cited text no. 2
    
3.
Forouhar F, Rustagi T, Lamea L. Eosinophilic venulitis of colon presenting as ileocecal mass. Ann Clin Lab Sci 2011;41:373-8.  Back to cited text no. 3
    
4.
Flaherty MJ, Lie JT, Haggitt RC. Mesenteric inflammatory veno-occlusive disease. A seldom recognized cause of intestinal ischemia. Am J Surg Pathol 1994;18:779-84.  Back to cited text no. 4
    
5.
Allali D, Puppa G, Chizzolini C. Mesenteric inflammatory veno-occlusive disease of the spleen metasynchronous with two arterial thrombotic events in systemic lupus erythematosus. Lupus 2018;27:150-3.  Back to cited text no. 5
    
6.
Miracle AC, Behr SC, Benhamida J, Gill RM, Yeh BC. Mesenteric inflammatory veno-occlusive disease: Radiographic and histopathologic evaluation of 2 cases. Abdom Imaging 2014;39:18-24.  Back to cited text no. 6
    

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Correspondence Address:
Saloni Naresh Shah,
Department of Histopathology and Cytology, 21 Greams lane, Off Greams road, Thousand lights, Chennai - 600 006, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_85_22



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    -  Bhayani P
    -  Parameswaran SA
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