- 1Chongqing Medical University, Chongqing, China
- 2Department of Pathology, The Chongqing General Hospital, Chongqing, China
Background: Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare and poorly understood disease, typically affecting the rectosigmoid colon of young, otherwise healthy men. Clinically, it is often mistaken for inflammatory bowel disease, as biopsies show ischemic mucosal changes without classic inflammatory features. Surgical resection is both diagnostic and curative, although the etiology of IMHMV remains unclear.
Case presentation: We report the case of a female patient with IMHMV involving the right hemicolon, concomitant with Clostridium difficile infection. Her symptoms persisted despite targeted treatment for C. difficile. She subsequently underwent a laparoscopic right hemicolectomy, which revealed mesenteric vein occlusion due to myointimal hyperplasia, confirmed by elastin staining and desmin immunohistochemistry. Histopathological examination established the diagnosis of IMHMV. The patient recovered well postoperatively, with no recurrence observed during follow-up.
Conclusion: This is the first documented case of IMHMV involving the right hemicolon and complicated by Clostridium difficile infection. In addition, we reviewed 82 previously reported cases from 1991 to 2024, highlighting the clinical, imaging, and pathological characteristics of IMHMV. Recognition of this rare entity is essential to avoid unnecessary pharmacotherapy, prevent misdiagnosis as inflammatory bowel disease, and facilitate timely surgical management.
1 Introduction
Idiopathic Myointimal Hyperplasia of the Mesenteric Veins (IMHMV) is a rare and poorly understood condition that presents a diagnostic challenge for clinicians and pathologists. It is classified as an ischemic bowel disease characterized by venous occlusion due to smooth muscle proliferation in the tunica intima of the mesenteric veins, without the presence of a thrombus (1). IMHMV is often misdiagnosed as inflammatory bowel disease (IBD) (Supplementary Table S1), as definitive diagnosis of bowel ischemia and venous thrombotic disease relies on pathological changes that are not distinguishable through preoperative radiological or clinical assessments. Consequently, a definitive diagnosis can only be achieved postoperatively, since biopsies are unable to differentiate ischemic abnormalities from those associated with known IBD manifestations (2). Since the first case was reported in the United States in 1991, approximately 81 cases of IMHMV have been documented in the literature, with the present case representing the 82nd reported instance. Majority of these cases ultimately end up with different degrees of bowel resection (3).
Idiopathic myointimal hyperplasia of the mesenteric veins mostly involves the thickening of small and medium-sized mesenteric veins with the hallmark manifestation of intimal smooth muscle proliferation resulting in luminal occlusion and mucosal ischemic changes (2). Pathologists may miss the diagnosis unless elastin stains are performed, as the affected veins can easily be mistaken for arteries. The etiology of IMHMV remains unclear. One hypothesis suggests that the disease may result from an arteriovenous fistula. Another Hypothesis proposes that IMHMV represents the end stage of ‘phlebitis,’ as lesions resembling IMHMV have been observed in cases of lymphocytic, granulomatous, and necrotizing phlebitis (4, 5).
We herein describe the case of a female patient with idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) at right colon with Clostridium difficile infection (CDI).
2 Case presentation
A 68-year-old female patient, with a history of chronic abdominal pain, was admitted to the hospital with chief complaint of severe abdominal pain for the past 2 months. The abdominal pain initially started in the left hypochondrium as a vague discomfort, but later migrated and localized as a persistent distending pain in the lower abdomen, accompanied by vomiting, bloody stools, and low-grade fever. There was history of loss of appetite. She has consumed only porridge in the past 2 months. There was no history of joint pain, skin lesions, chest pain, cough, or contact with contaminated water. One month prior, she was admitted with a diagnosis of hypertensive heart disease, grade 3 hypertension, abnormal coagulation function, ischemic enteropathy, and chronic atrophic gastritis. She has previously undergone resection of a lung abscess. She self-reports an allergy to penicillins and abdominal pain after taking aspirin. No significant family history.
The physical examination revealed tenderness in the upper middle abdomen, left flank, and lower abdomen, more prominent in the upper middle and lower abdomen. No rebound tenderness or palpable masses were noted. Normal bowel sounds. The following Laboratory findings were reported: CRP = 20.51 mg/L, Hg = 106 g/L, D-Dimer = 0.78 mg/L, VIII factor activity = 267.90%, TP = 63.7 g/L, ALB = 31.8 g/L, positive fecal occult blood test, and Clostridium difficile antigen test.
A colonoscopy revealed congested and inflamed mucosa in the colon (Figures 1D–I). Salt and pepper appearance was seen in the region. The transverse colon exhibited a whitish appearance with loss of vasculature. Biopsy findings revealed a non-specific ulcer.
Figure 1. Colonic imaging and computed tomography suggested IMHMV. (A,B) Axial CT images showing thickened colonic wall with edema (red arrowhead) and no mesenteric artery stenosis. (C) Abdominal CTA revealed mixed plaques at the origin of the abdominal aorta and bilateral common iliac arteries, suggesting the possibility of mesenteric microthrombosis. Flexible sigmoidoscopy images demonstrated severely inflamed mucosa (D–J) with significant luminal narrowing (H,I).
Computed tomography angiography (CTA) of the abdomen showed mixed plaques in the abdominal aorta and at the origin of the bilateral common iliac arteries (Figure 1C). Computed tomography (CT) scan of the abdomen results showed a swollen colon wall with diminished enhancement and surrounding exudate that was suggestive of ischemic bowel disease (Figures 1A,B). Based on the presentation and investigative findings, she was diagnosed with a suspected case of ischemic bowel disease and was started on scopolamine hydrobromide for spasm and pain relief, fasting, omeprazole for gastric protection, and atorvastatin calcium tablets to stabilize plaques. The patient’s abdominal pain slightly subsided, but intermittent abdominal pain persisted, primarily around the navel, with slight relief after activity or oxygen inhalation. The stools were yellow and pasty. Papaverine was additionally used to relieve spasm and improve intestinal blood supply, while mesalazine was administered to repair the intestinal mucosa. Metronidazole tablets were added to treat the infection, but the patient experienced nausea and acid reflux after administration. Additionally, no pseudomembranous enteritis was found in the colonoscopy, and there was no mucus or pseudomembranes in the stool. Metronidazole was discontinued after 1 week. A conservative management approach was followed, but the symptoms did not resolve. A decision was made to proceed with a laparoscopic right hemicolectomy.
Postoperative pathology revealed the removal of 1 cm of ileum and 48 cm of colon. A local intestinal stricture was present 41 cm from the ileal resection margin, with a length of approximately 2 cm. The intestinal mucosa at this site was grayish-brown with granular hyperplasia. An ulcer was observed 28 cm from the ileocecal valve, adjacent to the stricture, measuring 10 × 3 cm. At this site, the intestinal mucosal folds were absent and the intestinal wall was hardened. The remaining proximal colon exhibited multiple scattered ulcers with a maximum diameter of 1–3 cm. The mesenteric fat at the lesion site was hyperplastic and encircled the intestinal wall, with the stricture being most prominent. Three lymph nodes were found around the intestine, with a maximum diameter of 0.2–0.3 cm (Figure 2).
Figure 2. (A–D) Hematoxylin–eosin (H&E) staining of the resected surgical specimen (original magnification ×200); (E) elastic fiber staining (200x magnification); (F) immunostaining for smooth muscle actin shows the endoluminal nature of the venous proliferation. (200x magnification); (G) EBER in situ hybridization (ISH) test. (200x magnification); (H) CMV staining is negative. (I&J) Surgical resection specimen from IMHMV. Large ulcer (white arrow); scattered ulcers (blue arrow); stenosis (yellow arrow); (K–M) Hematoxylin–eosin (H&E) staining of the mucosal biopsy (original magnification ×200) shows ischemic-type changes, including epithelial sloughing, ulceration, and atrophy, with dense neutrophilic infiltration and prominent capillary proliferation but without perivascular hyalinization. The muscularis mucosae is thickened with associated fibrosis.
2.1 Integrated histopathology description
Histopathological examination revealed that the intestinal mucosa and submucosa were replaced by fibrous tissue, with prominent proliferation of small- to medium-sized veins, some extending from the lamina propria to the serosa. The mucosal villi were absent at the stenotic segments, while the crypt architecture was largely preserved, without significant distortion or branching. Inflammatory infiltrates were present but limited, predominantly composed of neutrophils, with minimal lymphoplasmacytic component. Notably, there was no basal plasmacytosis, crypt abscesses, or Paneth cell metaplasia, features that typically indicate chronic inflammatory bowel disease, supporting the exclusion of IBD. The hyperplastic veins showed extensive intimal proliferation, resulting in slit-like lumina, with thickened walls and protruding endothelial cells. Capillaries in the affected areas also exhibited slit-like lumina and thickened walls. No histopathological features characteristic of Clostridium difficile infection, such as pseudomembranes, mucosal necrosis, or prominent exudates, were observed.
Immunohistochemical staining with Desmin confirmed that the smooth muscle in the venous intima was proliferating in a semicircular pattern. Concurrently, Verhoeff-Van Gieson (VVG) elastic fiber staining demonstrated noticeable intimal thickening in the arteriole-type vessels. No vasculitis was present; EBER RNA and CMV expression were negative, excluding EBV colitis and CMV colitis (Figure 2). No evidence of thrombosis or malignancy was observed. The final diagnosis was idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV). Follow-up over 1 year after surgery, she was doing well, without issue.
We reviewed the patient’s previous biopsy specimens and noted dense neutrophilic infiltration of the mucosal layer with prominent capillary proliferation but without perivascular hyalinization. The muscularis mucosae was thickened with associated fibrosis and lacked features of chronic active inflammation, such as lymphoplasmacytic infiltrates, basement membrane disruption, or crypt abscesses. Although these findings were not diagnostic, they did not support inflammatory bowel disease or Clostridium difficile infection (Figures 2K–M).
3 Discussion
Idiopathic myointimal hyperplasia is a rare cause of intestinal ischemia. Compared to other inflammatory conditions of the gastrointestinal tract, IMHMV is not caused by arterial thromboembolism, venous thrombus or vasculitis, and its etiology remains poorly understood.
The most common symptoms reported in previous cases include abdominal pain, perforation, and hematochezia (Supplementary Table S1). The patient was admitted with chief complaint of severe abdominal pain. Patients with C. difficile enteritis generally present with diarrhea, crampy abdominal pain, and leukocytosis (6). Given that the patient presented with Clostridium difficile infection (CDI) and the clinical manifestations of IMHMV and pseudomembranous colitis caused by CDI were similar, the histological findings were distinctly different. The former often results in mucosal changes due to non-specific ischemic injury, rather than a toxin-mediated inflammatory process, with the absence of pseudomembrane formation and interstitial necrosis (7). CDI may lead to microvascular dysfunction and thrombosis of the superior mesenteric artery (8, 9) and affect the development of ischemic bowel disease (10). Ischemic colitis may be a complication of CDI (11). As a rare cause of ischemic bowel disease, CDI may offer insight into the pathogenesis of IMHMV. This case is notable as the first reported instance of IMHMV combined with CDI. CT imaging of our patient revealed focal colonic wall thickening and submucosal edema with preserved mesenteric arterial patency. However, the characteristic radiographic findings of IMHMV described in previous reports—enlarged and tortuous pericolonic vessels with rich, dilated peripheral veins—were not observed in this case (Figure 1). This absence may reflect the early stage and limited extent of venous involvement in our patient, as well as differences in imaging protocol compared with prior studies (Table 1).
Our patient has hypertension and hypertensive heart disease, consistent with previous reports noting cardiovascular risk factors in many IMHMV cases (4, 12–14). IMHMV is characterized by myointimal hyperplasia leading to mesenteric vein occlusion. Experimental studies have shown that elevated blood pressure can induce intimal thickening and endothelial changes in small vessels (15–26), which may suggest a potential role of hypertension in the pathogenesis of IMHMV. Further research is warranted to clarify this association.
The absence of concrete histopathological criteria for a definitive diagnosis of IMHMV makes biopsy-based diagnosis challenging for pathologists, leading to the early initiation of treatment with anti-inflammatory drugs rather than surgery, which, at present, remains the only effective treatment for this condition and has been reported to be completely curative. However, only six cases of IMHMV have been diagnosed preoperatively (27–29). Arteriolized capillary, subendothelial fibrin deposits, and perivascular hyalinization are the most recent specific pathological features that facilitate the identification of IMHMV in mucosal biopsy samples (1). Biopsy specimens from this patient were reviewed to summarize findings indicating the absence of the muscular layer in the intestinal wall, a gap-like appearance of the intestinal wall due to capillary hyperplasia, thickening of the wall, and prominent endothelial cells. Notably, while the mesenteric vein exhibited characteristic smooth muscle proliferation in the intima (consistent with IMHMV pathology), the arterial changes presented a diagnostic paradox: although these vessels retained their intimal architecture, significant non-myointimal thickening was observed, which differed fundamentally from the venous pathology. we describe these as vessels with arterial-type wall thickening of uncertain significance, confirmed by immunohistochemistry to be non-myointimal in nature (Figure 2). This histologic feature has not been documented in prior IMHMV cases and warrants particular attention, as it may represent (1) a previously unrecognized disease variant, (2) concurrent vascular pathology, or (3) a broader disease spectrum or secondary vascular remodeling, given that small-vessel involvement of this type has not been reported in the existing literature. The presence of definitive venous pathology meets the current diagnostic criteria for IMHMV, while the observed alterations in other small vessels may indicate secondary vascular remodeling, a coexisting process, or a previously unrecognized disease spectrum that requires further investigation.
Currently, all cases are diagnosed based on pathological results, and the lack of methods for early diagnosis means that the diagnosis of IMHMV is often delayed. The clinical manifestations and endoscopic findings are nonspecific and closely resemble those of IBD and IMP. Similar imaging manifestations have also been observed, with IMP showing more similarity in this regard. Below, we summarize the differential diagnosis of IMHMV, IBD and IMP (Table 2). In particular, IBD, with nearly 53.1% of IMHMV cases being diagnosed as IBD before surgery (Supplementary Table S2), will be subdivided into the two subtypes of IBD, namely UC and CD, to differentiate them from IMHMV. This approach can enhance clinicians’ understanding of the disease and improve the diagnostic rate.
4 Conclusion
This case report describes the first documented instance of Idiopathic Myointimal Hyperplasia of the Mesenteric Veins (IMHMV) with right colonic involvement complicated by Clostridium difficile infection in a female patient, and also provides the inaugural description of small vessels exhibiting arterial-type wall thickening of uncertain significance in IMHMV.
Histopathological analysis demonstrated a paradoxical coexistence of characteristic venous myointimal hyperplasia and a subset of small vessels showing arterial-type wall thickening that distinctly lacked Desmin-positive smooth muscle cell proliferation—an essential feature of the venous lesions—thus creating a diagnostic paradox given the established venocentric pathology of IMHMV. Clinicians should maintain a high index of suspicion for IMHMV when encountering venocentric myointimal hyperplasia in biopsy specimens from elderly patients with suspected inflammatory bowel disease or distal colorectal ischemia, with the caveat that concurrent arterial-type wall thickening of uncertain significance should prompt thorough clinicopathological correlation to exclude alternative vasculopathies.
Data availability statement
The original contributions presented in the study are included in the article/Supplementary material, further inquiries can be directed to the corresponding author/s.
Ethics statement
The studies involving humans were approved by Medical Ethics Committee of Chongqing People’s Hospital. The studies were conducted in accordance with the local legislation and institutional requirements. The human samples used in this study were acquired from a by- product of routine care or industry. Written informed consent for participation was not required from the participants or the participants’ legal guardians/next of kin in accordance with the national legislation and institutional requirements. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article. Written informed consent was obtained from the participants for the publication of this case report.
Author contributions
WW: Conceptualization, Data curation, Formal analysis, Writing – original draft, Writing – review & editing. XD: Investigation, Resources, Writing – review & editing. XJ: Formal analysis, Visualization, Writing – review & editing. MY: Methodology, Supervision, Writing – review & editing. XT: Investigation, Validation, Writing – review & editing.
Funding
The author(s) declare that financial support was received for the research and/or publication of this article. XT is currently receiving grants (2022TIAD-KPX0243 and 2023MSXM034) from Organization Technological Innovation and Application Development of Chongqing. XJ is currently receiving a grant (2024MSXM162) from the project of the Chongqing Municipal Health Commission.
Conflict of interest
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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Supplementary material
The Supplementary material for this article can be found online at: https://www.frontiersin.org/articles/10.3389/fmed.2025.1674469/full#supplementary-material
Abbreviations
IMHMV, Idiopathic myointimal hyperplasia of mesenteric veins; CDI, Clostridium difficile infection; IBD, Inflammatory bowel disease; UC, Ulcerative colitis; CD, Crohn’s disease; IC, Ischemic colitis; IMP, idiopathic mesenteric phlebosclerosis; CT, computed tomography; CTA, computed tomography angiography.
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Keywords: idiopathic myointimal hyperplasia of mesenteric veins, Clostridium difficile infection, inflammatory bowel disease, idiopathic mesenteric phlebosclerosis, vessels with arterial-type wall thickening of uncertain significance
Citation: Wang W, Deng X, Jiang X, Yang M and Tang X (2025) Case Report: Idiopathic myointimal hyperplasia of mesenteric veins mimicking inflammatory bowel disease: a case report with literature review. Front. Med. 12:1674469. doi: 10.3389/fmed.2025.1674469
Edited by:
Monjur Ahmed, Thomas Jefferson University, United StatesReviewed by:
Sudhakar Ramamoorthy, All India Institute of Medical Sciences, Mangalagiri, IndiaKristen Rumer, Mayo Clinic, United States
Copyright © 2025 Wang, Deng, Jiang, Yang and Tang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Xuefeng Tang, dHhmYXR5QDE2My5jb20=
Xue Deng1,2