Volume 3, Issue 4 | July 2016
Image | Colon

Idiopathic Myointimal Hyperplasia of the Mesenteric Veins

Download PDF

Agam D. Patel, MD1, Yecheskel Schneider, MD1, Monica Saumoy, MD1, Charles Maltz, MD, PhD1, Heather Yeo, MD2, Jose Jessurun, MD3, and David Wan, MD1

1Department of Gastroenterology, New York Presbyterian Hospital, Weill Cornell Medical Center, New York, NY
2Department of Surgery, New York Presbyterian Hospital, Weill Cornell Medical Center, New York, NY
3Department of Pathology, New York Presbyterian Hospital, Weill Cornell Medical Center, New York, NY

ACG Case Rep J 2016;3(4):e84. http://dx.doi.org/10.14309/crj.2016.57. Published online: July 27, 2016.

Case Report

A 65-year-old man with no significant past medical history was admitted to an outside hospital with 1.5 months of crampy, lower abdominal pain associated with rectal urgency and tenesmus. Initial computed tomography showed thickening of his rectosigmoid and mid to distal descending colon; follow-up colonoscopy revealed inflammation of the affected areas with focal lamina propria vascular congestion and sloughing epithelium in the rectosigmoid colon. A course of antibiotics followed by a course of oral steroids did not improve his symptoms.

CG-CGCR160001F001

Figure 1. Sigmoid colon demonstrating severely friable, ulcerated, and erythematous mucosa.

He was admitted with worsening pain. Initial laboratory studies were unremarkable except for a positive Clostridium difficile polymerase chain reaction, although oral vancomycin did not lead to improvement. Computed tomography showed similar findings with pericolonic inflammation. Subsequent flexible sigmoidoscopy revealed cobblestoning and skip inflammation of the mucosa in the sigmoid colon with rectal ulcers concerning for Crohn’s disease, but his symptoms worsened despite aggressive treatment with steroids and mesalamine. Biopsies returned revealing dilated mucosal capillaries without active colitis. His symptoms continued to worsen, so a repeat sigmoidoscopy 9 days later demonstrated stricturing of the sigmoid colon and worsening of the mucosal ulceration (Figure 1). Repeat biopsies in the rectosigmoid colon showed severe ischemic injury concerning for idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV). The patient was recommended for surgical resection multiple times. Soon thereafter he developed a perforation of the sigmoid colon and underwent an emergent resection with a left end colostomy. Final pathology revealed muscular thickening of the intramural veins consistent with IMHMV (Figure 2).

CG-CGCR160001F002

Figure 2. Final histopathology of colon at 20x magnification demonstrating muscular thickening of the intramural veins (white arrow) with histologically unremarkable accompanying arteries (black arrow).

Idiopathic myointimal hyperplasia of the mesenteric veins is a rare disease entity typically affecting young, healthy males often initially misdiagnosed as inflammatory bowel disease because of the similarity in patient demographics, clinical symptoms, and endoscopic findings. However, biopsies usually reveal nonspecific ischemic changes.1,2 Usually the final diagnosis is made in histopathological analysis after surgical resection, which reveals nonthrombotic and noninflammatory occlusion of the mesenteric veins secondary to myointimal hyperplasia.3 One report has been published in which the diagnosis was made preoperatively through endoscopic biopsy; our case was similarly diagnosed, suggesting that a preoperative diagnosis may be routinely possible if there is a high degree of suspicion for IMHMV.4 Recurrences have not been reported after surgical resection, raising the idea that it is likely curative. Because our patient’s sigmoid perforation was likely related to IMHMV, early treatment may be of benefit to reduce morbidity.


Disclosures

Author contributions: AD Patel, Y. Schneider, M. Saumoy, C. Maltz, and H. Yeo wrote the manuscript. J. Jessurun provided and analyzed the pathological images. D. Wan supervised the manuscript writing and is the article guarantor.

Financial disclosure: None to report.

Informed consent was obtained for this case report.

Previous Presentation: This case was presented at the ACG Annual Scientific Meeting; October 16-21, 2105; Honolulu, Hawaii.

Correspondence: David Wan, Department of Gastroenterology and Hepatology, 1305 York Ave, 4th Floor, New York, NY 10021 (Daw9044@med.cornell.edu).

Received December 10, 2015; Accepted January 19, 2016


References

  1. Abu-Alfa AK, Ayer U, West AB. Mucosal biopsy findings and venous abnormalities in idiopathic myointimal hyperplasia of the mesenteric veins. Am J Surg Pathol. 1996;20(10):1271–8. PubMed
  2. Kao PC, Vecchio JA, Hyman NH, et al. Idiopathic myointimal hyperplasia of mesenteric veins: A rare mimic of idiopathic inflammatory bowel disease. J Clin Gastroenterol. 2005;39(8):704–8. PubMed
  3. Platz J, Hyman N. Idiopathic myointimal hyperplasia of mesenteric veins. Gastroenterol Hepatol. 2012;8(10):700–2. PubMed
  4. Wangensteen KJ, Fogt F, Kann BR, Osterman MT. Idiopathic myointimal hyperplasia of the mesenteric veins diagnosed preoperatively. J Clin Gastroenterol. 2015;49(6):491–4. Article | PubMed

CC Icon

© 2016 Patel et al. This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0.