Myointimal Hyperplasia Of Mesenteric Veins Research Articles

CT findings in idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) and comparison to other colitides.

The purpose of this study is to determine computed tomography (CT) findings that aid in differentiating idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) from other colitides. Retrospective review of histiologic proven cases of IMHMV (n = 12) with contrast enhanced CT (n = 11) and/or computed tomography angiography (CTA) (n = 9) exams. Control groups comprised of CT of infectious colitis (n = 13), CT of inflammatory bowel disease (IBD) (n = 12), and CTA of other colitides (n = 13). CT exams reviewed by 2 blinded gastrointestinal radiologists for maximum bowel wall thickness, enhancement pattern, decreased bowel wall enhancement, submucosal attenuation value, presence and location of IMV occlusion, peripheral mesenteric venous occlusion, dilated pericolonic veins, subjective IMA dilation, maximum IMA diameter, maximum peripheral IMA branch diameter, ascites, and mesenteric edema. Presence of early filling veins was an additional finding evaluated on CTA exams. Statistically significant CT findings of IMHMV compared to control groups included greater maximum bowel wall thickness, decreased bowel enhancement, IMV occlusion, and peripheral mesenteric venous occlusion (p < 0.05). Dilated pericolonic veins were seen more frequently in IMHMV compared to the infectious colitis group (64% versus 15%, p = 0.02). Additional statistically significant finding on CTA included early filling veins in IMHMV compared to the CTA control group (100% versus 46%, p = 0.008). IMHMV is a rare chronic non-thrombotic ischemia predominantly involving the rectosigmoid colon. CT features that may aid in differentiating IMHMV from other causes of left-sided colitis include marked bowel wall thickening with decreased enhancement, IMV and peripheral mesenteric venous occlusion or tapering, and early filling of dilated veins on CTA.

Enterocolic phlebitis: a rare cause of bowel ischemia and review of the literature.

Enterocolic phlebitis (EP) is a rare cause of bowel ischemia due to isolated venulitis of the bowel wall and mesentery without arterial involvement. EP is often misdiagnosed as inflammatory bowel disease, carcinoma, or diverticulitis due to non-specific symptoms as well as non-specific clinical and radiological findings. While unresponsive to pharmacotherapy, surgical resection of the affected bowel appears to be the only successful therapy with a very low recurrence rate. Etiology of EP remains unknown. We report a case of EP with rare presentation in the left hemicolon and unusual histological findings emphasizing the heterogeneity of this cause of enterocolic ischemia. The review and comparison of the three entities-EP, mesenteric inflammatory veno-occlusive disease (MIVOD), and idiopathic myointimal hyperplasia of mesenteric veins (IMHMV), all describing patterns of bowel ischemia due to isolated pathology of mesenteric veins-reveal that the current terminology is unclear. EP and MIVOD are very similar and may be considered the same disease. IMHMV, though, differs in localization, symptom duration, and histological findings but also shares features with EP and MIVOD. Further studies and harmonized terminology are inevitable for better understanding of the disease, prevention of unnecessary pharmacotherapy, and reduction in time to diagnosis.

S2039 Idiopathic Myointimal Hyperplasia of Mesenteric Veins Disguised as IBD: A Diagnostic Challenge

S2039 Idiopathic Myointimal Hyperplasia of Mesenteric Veins Disguised as IBD: A Diagnostic Challenge

IBD MIMICS PRESENTED AT IBD LIVE: WHEN IT IS NOT INFLAMMATORY BOWEL DISEASE

Abstract BACKGROUND A group of Inflammatory Bowel Disease (IBD) specialists have been utilizing a weekly live video conference “IBD Live” to discuss multidisciplinary management of challenging cases. Since 2009 the conference has featured robust interactive case discussions among 150-200 gastroenterologists and colorectal surgeons from multiple academic institutions. Although the majority of cases presented are IBD, there have been a number of cases where IBD was not the ultimate diagnosis. Hence, we sought to characterize these “IBD mimics” to provide a structure for differential diagnosis. METHODS All 106 hours of case conferences recorded and archived between May 2018 and June 2021 were reviewed. A total of 183 of the 215 cases discussed had an IBD diagnosis including 124 Crohn’s disease (CD), 54 ulcerative colitis (UC) and 5 IBD unclassified. RESULTS Many of the remaining 32 (14.9%) cases (Table 1) were referred to a specialist with a prior diagnosis of IBD. There were 10 cases of colonic inflammation initially diagnosed as UC. Etiologies included drug-induced colitis secondary to checkpoint inhibitors for cancers, ocrelizumab for multiple sclerosis, and nintedanib for pulmonary fibrosis. There were cases of ischemic colitis, idiopathic myointimal hyperplasia of mesenteric veins, segmental colitis associated with diverticulosis, and diversion colitis. There were 8 cases of systemic diseases, diagnosed as Common Variable Immunodeficiency, sarcoidosis and vasculitis, including cases of Henoch–Schönlein purpura and CTLA4 haploinsufficiency with autoimmune infiltrates. There were 5 cases of ileal disorders, ultimately diagnosed as lymphoma, tuberculosis, Meckel’s diverticulum, and appendiceal carcinoid all previously thought to be CD. There were small bowel mucosal disorders including collagenous sprue and autoimmune enteropathy, and 2 polyposis cases secondary to Cronkhite-Canada and juvenile polyposis syndromes. There were also cases thought to be penetrating complications of CD, including a case of abdominal actinomyces infection. Finally, there were 3 cases where no consensus diagnosis was made, with broad differential diagnoses including cryptogenic multifocal ulcerous stenosis enteritis. CONCLUSIONS IBD mimics were found in 15% of cases presented at a global IBD webinar in a 3-year period. Clinicians should have a high level of suspicion and actively pursue alternative etiologies when patients do not respond as anticipated to IBD therapy and/or when the disease characteristics are not completely consistent with IBD. These non-IBD diagnoses will lead to a significant change in medical, surgical and pharmacological treatment approaches, which will impact total cost, unplanned care and quality. Our IBD Live conference has positively impacted the care of our patients, and magnified the importance of considering a broad differential for IBD "mimics."

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

Introduction and importanceIdiopathic 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 presentationA 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 discussionThe 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.ConclusionThe 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.

1546 Idiopathic Myointimal Hyperplasia of Mesenteric Veins: A Rare Cause of Treatment Refractory Colitis

INTRODUCTION: Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare disease which mimics inflammatory bowel disease (IBD) clinically and endoscopically while showing ischemic changes without classic features of IBD on pathology. Delay in diagnosis is common; definitive diagnosis is usually only feasible after surgical resection. CASE DESCRIPTION/METHODS: A 62-year-old male with a past medical history of hypertension and hyperlipidemia presented with a 1-year history of recurrent abdominal pain, bloody diarrhea, and rectal pain. He was initially evaluated at another hospital and diagnosed with pneumatosis intestinalis and left-sided colitis. Left colon biopsies were suggestive of ischemia but computerized tomography angiogram (CTA) revealed patent mesenteric arteries. Extensive workup for thrombophilia, infectious, inflammatory, and autoimmune etiologies were negative. His symptoms did not respond to antibiotics and oral steroids. He presented to our institution for a second opinion. At presentation, he was diaphoretic, afebrile and in distress. Physical exam was significant for lower abdominal tenderness without peritoneal signs. Laboratory investigations revealed elevated inflammatory markers (Table 1). Repeat CTA showed patent mesenteric arteries. Colonoscopy revealed severe inflammation and congestion extending from the mid-transverse colon to the rectum. The terminal ileum and ascending colon were normal. Biopsies from inflamed mucosa revealed ischemic colitis with fibrinoid microvascular wall necrosis and fibrin thrombi (Figure 1). He was started on IV Methylprednisolone 40 mg daily but symptoms progressed despite IV steroids. He underwent total colectomy with end ileostomy which was curative. Histopathological analysis of the resected colon confirmed IMHMV. DISCUSSION: Current opinion suggests that IMHMV is caused by non-inflammatory, non-thrombotic obliteration of medium-to-large size mesenteric veins. IMHMV is a difficult diagnosis to make preoperatively. However, preoperative diagnosis is suggested by the presence of arterialized capillaries, subendothelial fibrin deposits and fibrin thrombi on endoscopic biopsies. IMHMV is typically refractory to steroids and biologics. Gastroenterologists and pathologists should have a high index of suspicion for IMHMV in patients with treatment refractory colitis, negative mesenteric angiography and colon biopsies showing ischemic changes without classic features of IBD. Colectomy is usually curative without recurrence of symptoms.

Enterocolic lymphocytic phlebitis with marked myointimal hyperplasia and perivenous concentric fibrosis

Enterocolic lymphocytic phlebitis (ELP) is a rare enteropathy characterized by lymphocytic phlebitis of the mesenteric veins without arteritis. Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare disease similar to ELP, characterized by myointimal hyperplasia that constricts the lumen of veins, causing mucosal injury. A 62-year-old man with chief complaint of abdominal pain was treated by partial resection of the ileum after 3 months of conservative therapy. The pathologic diagnosis was ELP with prominent myointimal hyperplasia. Histologically, the lesion consisted of lymphocytic infiltration into the vein accompanied by prominent myointimal hyperplasia and perivenous concentric fibrosis, which are characteristics shared by ELP and IMHMV. The observations in this case suggest that some of ELP and IMHMV may belong to the same disease spectrum. Furthermore, perivascular concentric fibrosis was a remarkable observation that may contribute to differential diagnosis between ELP and "true" IMHMV.

Ischaemic colitis secondary to idiopathic myointimal hyperplasia of mesenteric veins: a case report

Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare cause of chronic ischaemic colitis. We present the case of a 63-year-old man with no prior history of inflammatory bowel disease, who presented with four months of watery diarrhoea with associated weight loss. No evidence of infection was found on microbiological studies. The distal descending colon appeared circumferentially thickened on CT scanning. Endoscopy showed severe superficial ulceration with a cobblestone-like appearance in the sigmoid colon. Three sets of biopsies taken over six weeks showed ulceration and features of ischaemic colitis.

Idiopathic Myointimal Hyperplasia of Mesenteric Veins: An Uncommon Cause of Ischemic Colitis With Distinct Mucosal Features.

Idiopathic myointimal hyperplasia of mesenteric veins causes chronic ischemic mucosal injury with segmental strictures that mimic inflammatory bowel disease and nonocclusive ischemic colitis. It is characterized by myointimal proliferative changes that narrow the lumina of veins combined with ischemic injury and ulcers. Most cases reported to date have been diagnosed following surgical resection. The aim of this study was to determine whether mucosal changes of idiopathic myointimal hyperplasia of mesenteric veins are sufficiently sensitive and specific to allow its recognition in biopsy material. The study group consisted of 10 patients with idiopathic myointimal hyperplasia of mesenteric veins who underwent surgical resection of the affected colon, 7 of whom had available prior endoscopic biopsies. The control group included 10 patients each with radiation, nonocclusive ischemia, Crohn disease, diverticulitis, and mucosal amyloidosis, and 5 cases of small vessel (leukocytoclastic) vasculitis. Study patients were mostly older men with distal colorectal disease. All resection specimens showed mucosal ischemia with numerous thick-walled (arteriolized) capillaries and glassy subendothelial fibrin deposits; numerous hyalinized, eosinophilic thrombi were detected in 90% of colectomy specimens. Biopsies showed arteriolized capillaries (100%), subendothelial fibrin deposits (86%), fibrin thrombi (43%), and perivascular hyalinization (43%). Fibrin thrombi were observed in only one case each of ischemic colitis and small vessel vasculitis, and none of the other abovementioned features were seen in any of the controls. We conclude that arteriolized capillaries, subendothelial fibrin deposits, and perivascular hyalinization are frequent and specific features that can facilitate recognition of idiopathic myointimal hyperplasia of mesenteric veins in biopsy samples.

Un cas d’hyperplasie myointimale idiopathique des veines mésentériques du côlon

The idiopathic myointimal hyperplasia of mesenteric veins is a rare pathology, affecting recto-sigmoid and mimicking clinically an inflammatory chronic disease of the bowel. Only about fifteen cases have been reported in the literature. This lesion is characterized by a myointimal thickening of the mesenteric veins, without inflammatory infiltrate of the vascular wall, differentiating it from vasculitis. We present here the case of a 48-year-old man, in whom the diagnosis of ulcerative colitis then digestive vasculitis had first been raised.

Idiopathic Myointimal Hyperplasia Is a Distinct Cause of Chronic Colon Ischemia

Introduction: Colon ischemia (CI) is typically an acute manifestation of altered colonic blood flow resulting in mucosal ischemia and resolves spontaneously within days. Some patients, however, may evolve into chronic segmental colitis. This is a controversial topic, given the varied definitions of this entity and lack of distinguishing histologic features. We sought to further examine the clinicopathologic features of chronic CI in an attempt to distinguish it from acute CI. Methods: Cases were identified from our electronic medical record between 1/1/94 and 8/15/15. Only cases having symptom chronicity (>1 month of abdominal symptoms) and persistent ischemic histology in patients 18-99 years of age were included for review. Histologic evaluation was performed by two gastrointestinal pathologists. Results: From 15 CI patients with clinical and histologic chronicity, 8 had unequivocal venous intimal hyperplasia characterized by thickened submucosal, mural and extramural veins which defines the rare condition known as idiopathic myointimal hyperplasia of mesenteric veins (IMHMV). The remaining 7 patients had non-specific histologic findings of ischemia. The median age at diagnosis for patients with IMHMV was 62.5 years (range 22-75) compared to 61.6 years (range 29-88) in those without. By sex, 2 of the IMHMV and 3 non-IMHMV patients were female. Vascular imaging in all IMHMV cases demonstrated mesenteric arterial and venous patency. Abdominal pain and diarrhea were the two most common presenting symptoms, occurring in 7 (87.5%) and 5 (62.5%) cases, respectively. The sigmoid colon was most commonly involved in 75% of IMHMV versus only 29% of non-IMHMV cases. Surgical resection was performed in 7 IMHMV patients as part of management for CI or suspected inflammatory bowel disease, resulting in symptomatic resolution. In retrospect, all 7 IMHMV patients with resection had characteristic changes in pre-resection biopsies, including thickened, tortuous mucosal venules and, in some cases, myointimal hyperplasia in superficial submucosal veins. Conclusion: The histologic changes of IMHMV are distinctive in colonic resections and are not detected by vascular imaging. Pre-operative diagnosis of IMHMV is possible from endoscopically obtained biopsies, which show a combination ischemic injury and altered mucosal venules. As segmental colon resection is curative of IMHMV, improved awareness of this entity is needed to avoid misdiagnosis and potentially harmful treatment such as immunosuppression.

Idiopathic myointimal hyperplasia of mesenteric veins in the elderly

Idiopathic myointimal hyperplasia of mesenteric veins in the elderly

Idiopathic myointimal hyperplasia of mesenteric veins and pneumatosis intestinalis: A previously unreported association

Idiopathic myointimal hyperplasia of mesenteric veins is a very rare disease occurring in young male patients, with no more than eight cases reported in the world literature. It causes venous ischemia in the sigmoid colon and rectum that clinically resembles inflammatory bowel disease. Pneumatosis intestinalis is also a rare condition usually associated to a wide range of diseases including bowel ischemia. We herein report on a case of pneumatosis intestinalis associated to idiopathic myointimal hyperplasia of mesenteric veins. To our knowledge, this is the first report of such an association, and the first one of idiopathic myointimal hyperplasia of mesenteric veins occurring in a female patient as well.

Focal myointimal hyperplasia of mesenteric veins is associated with previous trauma in surgical specimens

Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare and poorly understood disease that occurs in the rectosigmoid colon of predominantly young, previously healthy, male patients. This disease is often confused with chronic idiopathic inflammatory bowel disease clinically, and pathologists may miss the diagnosis unless elastin stains are performed because diseased veins may readily be mistaken for arteries. The etiology of IMHMV is unclear, but a traumatic pathomechanism resulting in arterialization of the veins has been proposed. Review of bowel resection specimens ( n=68) for non-neoplastic disease within a 1-year period in patients younger than 50 years of age revealed 10 cases with focal mesenteric vein myointimal hyperplasia. Significantly more cases with focal myointimal hyperplasia of mesenteric veins (MHMV) were associated with pre-resection trauma to the involved bowel segment (5/11 vs. 5/57; p = 0.0016 ). A significant association of MHMV with pre-resection trauma supports the hypothesis that idiopathic myointimal hyperplasia of the mesenteric veins may be the result of trauma through torsion/stretching of the sigmoid colon and, subsequently, increased mesenteric venous pressure through arterialization.

Idiopathic Myointimal Hyperplasia of Mesenteric Veins

Idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) is a rare and poorly understood disease that occurs in the rectosigmoid colon of predominantly young, previously healthy male patients. IMHMV typically requires segmental resection due to complications after a relatively protracted clinical course. This disease presents a challenging diagnostic dilemma for the clinician because it is initially often confused with chronic idiopathic inflammatory bowel disease. We report a case of IMHMV, illustrate endoscopic and histopathologic features, and review key characteristics of this rare entity.

Intestinal ischemia secondary to idiopathic myointimal hyperplasia of mesenteric veins: a case report and review of the literature

Intestinal ischemia secondary to idiopathic myointimal hyperplasia of mesenteric veins: a case report and review of the literature

Unexpected Sudden Death During Propranolol Therapy in a Patient with Mild Mesenteric Venous Myointimal Hyperplasia

Propranolol, a beta-adrenergic blocking agent commonly used in the treatment of hypertension, decreases mesenteric blood flow during exercise, at rest, and in cirrhosis. Widespread idiopathic myointimal hyperplasia of mesenteric veins produces intestinal ischemia in otherwise healthy individuals. This report describes a 42-year-old woman who died suddenly and unexpectedly while attending a ball game. She had hypertension treated with propranolol and no other clinically apparent disorders. Autopsy revealed mild mesenteric venous myointimal hyperplasia and segmental jejunal infarction. Recent clinical and experimental studies are used to propose possible mechanisms for this death which combine the effects of propranolol and mesenteric venous myointimal hyperplasia.

Mesenteric inflammatory veno-occlusive disease. A seldom recognized cause of intestinal ischemia.

We describe seven patients (three men, four women; ages 27-78) who presented with signs of intestinal ischemia requiring surgical intervention. In each case, the resected colon, small bowel, or both showed striking phlebitis and venulitis affecting veins of the bowel and mesentery and resulting in ischemic injury of the bowel. In each case, this vasculopathy was the only demonstrable cause of ischemia. Arteritis involving the bowel or the mesentery was not found in any patient, and none had clinical evidence or a history of extraintestinal vasculitis. The composition of the inflammatory infiltrate was variable; in four patients, it was predominantly lymphocytic, in two necrotizing and in one lymphocytic/granulomatous. In addition, three patients also had myointimal hyperplasia of the affected mesenteric veins. Six of seven patients recovered uneventfully after surgery, suggesting a self-limited or indolent process, and the seventh died of an unknown cause. We propose the name mesenteric inflammatory veno-occlusive disease (MIVOD) to describe this unusual and previously unrecognized cause of intestinal ischemia. Its etiology is unknown, and MIVOD may represent a precursor of the recently described idiopathic myointimal hyperplasia of mesenteric veins.

Enterocolic Lymphocytic Phlebitis

Venous compromise as a cause of ischemic colitis and enteritis is infrequent. Enterocolic lymphocytic phlebitis, a rare cause of such compromise, to our knowledge has been reported in only three patients. We describe a patient with this condition who had hematochezia and a cecal mass that was mistaken endoscopically and radiologically for carcinoma and required resection. The mass, which was ulcerated, was due to intense submucosal edema. The patient had no history of taking hydroxyethylrutoside, a drug used in Europe in treating varicose veins that has been implicated in all three previously reported cases. This entity is characterized by a mixed T- and B-cell vasculitis affecting veins exclusively in the wall and mesentery of the colon and small bowel, without systemic involvement, leading to fibrointimal proliferation, thrombosis, venous occlusion, and subsequent edema and ischemic necrosis. It should be distinguished from other enterocolic phlebitides, including Behçet disease, systemic lupus erythematosus, and myointimal hyperplasia of mesenteric veins, and can be treated by local excision of the affected bowel.