Rama Kumari Badyal, Jasmina Ahluwalia* and Kim Vaiphei
Departments of Histopathology and Hematology*, Postgraduate Institute of Medical Sciences and Research, Chandigarh, India7>
Received: 01 August, 2017; Accepted: 01 September, 2017; Published: 04 September, 2017
Kim Vaiphei, Professor, Department of Histopathology, Anand Block, Postgraduate Institute of Medical Sciences and Research, Chandigarh, India, Pin – 160012, Tel: +91 9815912943; Fax: 0172 2744401; E-mail:
Rama Kumari B, Ahluwalia J, Vaiphei K (2017) Myointimal hyperplasia of mesenteric vein- an experience in a tertiary care centre. Arch Clin Gastroenterol 3(3): 074-079. 10.17352/2455-2283.000044
© 2017 Rama Kumari B, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Myointimal hyperplasia; Mesenteric veins; Intestinal ischemia; Inflammatory bowel disease; Colitis; Tuberculosis; ymphoma
IMHMV: Idiopathic Myointimal Hyperplasia Of Mesenteric Vein; IBD: Inflammatory Bowel Disease; MIVOD: Mesenteric Inflammatory Veno-Occlusive Disorder; EVG: Elastica Van Giesen; MT: Masson’s Trichrome; IHC: Immunohistochemistry; SMA: Smooth Muscle Actin; PT: Prothrombin Time; APTT: Activated Partial Thromboplastin Time; PC: Protein C; PS: Protein S; ATIII: Anti-Thrombin III; FVL: Factor V Leiden Mutation; ACA: Anti-Cardiolipin Antibody; CECT: Contrast Enhanced Computed Tomography; IPSID: Immunoproliferative Small Intestinal Disease
Background: Idiopathic myointimal hyperplasia of mesenteric vein (IMHMV) is an extremely rare cause of venous mesenteric ischemia. It is defined as non-thrombotic occlusion of the mesenteric veins secondary to myointimal hyperplasia. IMHMV is a poorly understood condition involving the rectosigmoid colon predominantly young previously healthy male individuals
Purpose - to analyze surgically resected bowel specimens reported routinely as intestinal ischemia for the presence of features of IMHMV. There were 30 IMHMV of 118 cases that had been reported as intestinal ischemia.
Results: Clinical and laboratory findings in these 30 cases were re-evaluated. Mean age was 41.83±21.67 years and male female ratio was 5:1. Common presenting symptoms were abdominal pain (93.3%), diarrhoea (70%) and rectal bleeding (40%). Left colon was commonest site of involvement followed by right colon and ileum. Unique histological features observed were variable grades of fibrosis with subserosal and submucosal small to medium size veins showing myointimal proliferation. Thirteen of these 30 patients had associated intestinal stress conditions like strictures, torsions, volvulus and intussusceptions. Majority of these patients were investigated further for hypercoagulable states and were documented to be negative.
Conclusion: IMHMV is a selflimited condition where surgical resection of the affected segment is sufficient for a definitive treatment and needs to be recognized to avoid further unwarranted “management”.
Idiopathic myointimal hyperplasia of mesenteric vein (IMHMV) is a rare disease described by Genta and Haggitt in 1991 . It is a poorly understood disease frequently involving rectosigmoid usually in healthy young males. Often it is mistaken for inflammatory bowel disease (IBD) or mesenteric inflammatory veno-occlusive disorder (MIVOD) . IMHMV usually presents with rectal bleeding or bloody stool resembling IBD. The diagnosis is established at histopathological examination of the surgical resected specimen in which the veins show intimal smooth muscle proliferation with normal accompanying arteries. IMHMV has been described mainly in the territory of inferior mesenteric venous drainage . It has a benign clinical course and does not relapse after surgical resection of the affected intestinal segment . Incidence of IMHMV is not known, likely to be under recognized and is important to differentiate from other conditions like IBD and intestinal tuberculosis. The present study was undertaken to determine prevalence of IMHMV in a tertiary care centre in surgical resected specimens retrospectively in cases that had been reported as ischemic enterocolitis, and to evaluate clinical profiles and laboratory parameters.
Material and Methods
We retrospectively evaluated cases that had been routinely reported as intestinal ischemia, by examining the hematoxylin and eosin (H&E) stained slides. We studied the major veins and arteries and the smaller sub-mucosal vessels. We also evaluated the clinical profiles and the laboratory parameters form the patient records. There were 118 cases which had been reported as ischemic enterocolitis over five years i.e. from 2011 to 2016. Site wise distributions of these 118 cases included left (34 cases) and right (48 cases) segmental colonic resections, total colectomy (10 cases), segmental resections of small bowel (20 cases), and resection of colostomy site (6 cases).
In the present study the term pre-resection trauma refers to the bowel segment that had been exposed to significant mechanical pressure and stress like volvulus, intussusceptions, obstruction due to stricture (inflammatory and/or neoplastic) and revision surgery. Clinical diagnoses included subacute and acute intestinal obstruction, bowel gangrene, gastrointestinal bleed, IBD, bowel perforation, volvulus, intussusceptions, diverticulitis, malignant stricture and stomal take down (Tables 1,2). A particular section was selected and the same was subjected further to histochemistry stainings like elastica Van Giesen (EVG), Masson’s trichrome (MT) and immunohistochemistry (IHC) for smooth muscle actin [SMA; clone 1A4, Dako, Denmark; dilution 1:100]. Blood vessels present at different bowel segment and layers of bowel wall were evaluated for myointimal smooth muscle hyperplasia, thrombosis, inflammation and necrosis. Of the 118 cases evaluated, 30 showed myointimal hyperplasia in varying sized veins at varying layer in the bowel wall. Clinicopathological features of these 30 cases were evaluated and all were subjected to further investigation for prothrombotic state which included prothrombin time (PT), activated partial thromboplastin time (APTT), protein C (PC) and S (PS), anti-thrombin III (ATIII), Factor V Leiden mutation (FVL), anti-cardiolipin antibody (ACA) and anti-ẞ2GP1 testing (B2GPI). Pro-thrombotic work-ups were carried out after a minimum gap of six weeks following the surgical procedure and four weeks after completion of oral anticoagulation therapy.
Frequencies of myointimal hyperplasia observed in veins were evaluated with respect to previous history of pre-resection trauma in different clinical conditions and were compared using Pearson Chi-square test. All calculations were performed using SPSS® version 17 (Statistical Packages for the Social Sciences, Chicago, IL).
Thirty (25%) of 118 cases showed histological features of myointimal hyperplasia of the mesenteric veins (MIHMV) in minor and major branches. Distributions of the involved segments in these 30 resected specimens were - nine left colon, six right colon, five total colectomy, nine small bowel, and one colonic stomal takedown. Nineteen (63%) patients were between 10 to 50 years, and 11 (37%) were between 50 to 90 years, mean age = 41.83±21.67. There were 25 males and 5 females (M:F=5:1). Table 3 shows demographic, clinical and laboratory parameters and histopathological features. Presenting symptoms in these 30 patients were abdominal pain in 28 (93.3%), diarrhea in 21 (70%), lower gastrointestinal bleeding in 12 (40%), constipation in nine (30%) and abdominal distension in eight (26.6%). Two were known patients of diabetes mellitus and one patient of essential hypertension.
Pre-resection colonoscopy in 20 patients revealed - i) diffuse mucosal ulceration involving left colon in 12 patients with rectal sparing and ii) multiple pseudopolyps and short segment strictures in sigmoid colon in eight. Contrast enhanced computed tomography scan (CECT) of abdomen available in 11/30 patients showed large bowel segmental mural thickening in six (20%) and thickened jejunal loops in two patients (6%). Three (10%) of them showed thrombosis of the portal and superior mesenteric veins (Figure 1) along with thrombosis of the superior mesenteric artery in two (6.6%). One more patient had presented with sub-acute intestinal obstruction. He was found to have jejunal stricture on endoscopy and the biopsy of which showed features of advanced stage of immunoproliferative small intestinal disease (IPSID). He was put on CHOP regimen. After one year of completion of CHOP regimen, he presented with fresh complaints of abdominal distension, diarrhoea and pedal oedema. Abdominal CECT showed pneumoperitoneum with evidence of sealed perforation and thickened small bowel loops. He deteriorated progressively and sustained cardiac arrest and died. At autopsy proximal jejunum showed two cm long stricture with serosal fibrosis and adhesion. Sections from this site showed extensive transmural fibrosis with mild degree of chronic inflammatory cell infiltration. There was no residual tumor. Sub-serosal and sub-mucosal veins were grossly occluded by myointimal and fibrointimal proliferations. There was another case who was clinically diagnosed as Tubercular stricture of ileo-caecal region. Histopathology of the resected specimen showed features of MIH in sub-serosal and sub-mucosal veins with no evidence of Tuberculosis. Tables 1 and 2 highlighted case distributions in different clinical conditions.
- Genta RM, Haggitt RC (1991) Idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterology 101: 533-539. Link: https://goo.gl/42x9uX
- Abu-Alfa AK, Ayer U, West AB (1996) Mucosal biopsy findings and venous abnormalities in idiopathic myointimal hyperplasia of the mesenteric veins. Am J Surg Pathol 20: 1271-1278. Link: https://goo.gl/sn2afs
- Blaszyk H, West AB (2005) Idiopathic myointimal hyperplasia of the mesenteric veins: a rare diagnostic pitfall. Am J Gastroenterol 100: 1423-1424. Link: https://goo.gl/kuHxXa
- Shermana J, Kaob PC, Westc AB, Blaszyka H (2006) Focal myointimal hyperplasia of mesenteric veins is associated with previous trauma in surgical specimens. Pathol Practice Research 202: 517-522. Link: https://goo.gl/kxtVy4
- Wangensteen KJ, Fogt F, Kann BR, Osterman MT (2015) Idiopathic myointimal hyperplasia of the mesenteric veins diagnosed preoperatively. J Clin Gastroenterol 49: 491–494. Link: https://goo.gl/bxRbMf
- Flaherty MJ, Lie JT, Haggitt RC (1994) Mesenteric inflammatory veno-occlusive disease: a seldom recognized cause of intestinal ischemia. Am J Surg Pathol 18: 779-784. Link: https://goo.gl/KeUjsu
- Saraga E, Bouzourenne H (2000) Enterocolic (lymphocytic) phlebitis: a rare cause on intestinal ischemic necrosis. A series of six patients and a review of the literature. Am J Surg Pathol 24: 824-829. Link: https://goo.gl/ZMwEaF
- Hu JC, Forshaw MJ, Thebe P, Stewart M (2005) Mesenteric inflammatory venoocclusive disease as a cause of acute abdomen: report of five cases. Surg Today 35: 961-964. Link: https://goo.gl/svxeFK
- Ngo N, Chang F (2007) Enterocolis lymphocytic phlebitis: clinicopathologic features and review of the literature. Arch Pathol Lab Med 131: 1130-1134. Link: https://goo.gl/Mg2YdF
- Costa MN, Saiote J, Pinheiro MJ, Duarte P, Bentes T, et al. (2016) Segmental colitis caused by idiopathic myointimal hyperplasia of mesenteric veins. Rev Esp Enferm Dig 108: 821-826. Link: https://goo.gl/9GU2zc
- Bryant J (1998) Unexpected sudden death during propranolol therapy in a patient with mild mesenteric venous myointimal hyperplasia. J Forensic Sci 43: 905-907. Link: https://goo.gl/3McMYZ
- Savoie LM, Abrams AV (1999) Refractory proctosigmoiditis caused by myointimal hyperplasia of mesenteric veins: report of a case. Dis Colon Rectum 42: 1093-1096. Link: https://goo.gl/iQvpxE
- Kao PC, Vecchio JA, Hyman NH, West AB, Blaszyk H (2005) Idiopathic myointimal hyperplasia of mesenteric veins: a rare mimic of idiopathic inflammatory bowel disease. J Clin Gastroenterol 39: 704-708. Link: https://goo.gl/rKwjWY
- De Hertogh G, Van Eyken P, Stessens L, Caenepeel P, Geoboes K (2005) Myointimal hyperplasia of mesenteric veins secondary to heterozygous factor V Leiden mutation. Histopathol 47: 322-334. Link: https://goo.gl/ECv1ga
- García-Castellanos R, Lopez R, de Vega VM , Ojanguren I, Pinol M, et al. (2011) Idiopathic myointimal hyperplasia of mesenteric veins and pneumatosis intestinalis: A previously unreported association. J Crohns Colitis 5: 239-244. Link: https://goo.gl/EUKtai
- Lanitis S, Kontovounisios C, Karaliotas C (2012) An extremely rare small bowel lesion associated with refractory ascites. Idiopathic myointimal hyperplasia of mesenteric veins of the small bowel associated with appendiceal mucocoele and pseudomyxoma peritonei. Gastroenterology 142: e5-e7. Link: https://goo.gl/3oid1W
- Korenblit J, Burkart A, Frankel R, Klinge M, Greenbaum L, et al. (2012) Refractory pancolitis: a novel presentation of idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterol Hepatol (NY) 8: 696-700. Link: https://goo.gl/ifNsnK
- Feo L, Cheeyandira A, Schaffzin DM (2013) Idiopathic myointimal hyperplasia of mesenteric veins in the elderly. Int J Colorectal Dis 28: 433-434. Link: https://goo.gl/ryg8uu
- Chiang CK, Lee CL, Huang CS, Huang SH, Wu CH (2012) A rare cause of ischemic proctosigmoiditis: idiopathic myointimal hyperplasia of mesenteric veins. Endoscopy 44: E54-E55. Link: https://goo.gl/arPvMp
- Thomas BS (2013) Myointimal hyperplasia of the mesenteric veins mimicking infectious colitis. Int J Colorectal Dis 28: 727. Link: https://goo.gl/4mGSBs
- Kern WH, Wells WJ, Meyer BW (1981) The pathology of surgically excised aortocoronary saphenous vein bypass grafts. Am J Surg Pathol 5: 491-496. Link: https://goo.gl/9xe779
- Chang KM (2007) New histologic findings in idiopathic mesenteric phlebosclerosis: clues to its pathogenesis and etiology-probably ingested toxic agent-related. J Chin Med Assoc 70: 227-235. Link: https://goo.gl/ymFk3n
- Platz J, Hyman N (2012) Idiopathic Myointimal Hyperplasia of Mesenteric Veins. Gastroenterol Hepatol (NY) 8: 700-702. Link: https://goo.gl/8q8svA
- Gan SI, Urbanski S, Coderre SP, Panaccione R (2004) Isolated visceral small artery fibromuscular hyperplasia-induced ischemic colitis mimicking inflammatory bowel disease. Am J Gastroenterol 99: 2058-2062. Link: https://goo.gl/9pXMSe
- Lavu K, Minocha A (2003) Mesenteric inflammatory veno-occlusive disorder: a rare entity mimicking inflammatory bowel disorder. Gastroenterology 125: 236-239. Link: https://goo.gl/g86JGh
- Jevon GP, Ravikumara M (2010) Endoscopic and histologic findings in pediatric inflammatory bowel disease. Gastroenterol Hepatol 6: 174-180. Link: https://goo.gl/nFnqNj
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