title: Mesenteric Arteriovenous Dysplasia/Vasculopathy Is Distinct From Fibromuscular Dysplasia.
abstract: Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic vasculopathy that usually affects the carotid and renal arteries. We have observed FMD-like vascular changes in specimens resected for ischemia or Crohn's disease (CD). On the basis of a systematic clinicopathologic review of these 11 cases identified between 1982 and 2014, we describe a distinct mesenteric vasculopathy that involves both arteries and veins [mesenteric arteriovenous dysplasia/vasculopathy (MAVD/V)] and is characterized by (1) concentric/eccentric smooth muscle collarette around the tunica media of both the artery and the vein in ≥2 foci, (2) varying degrees of intimal and medial hyperplasia and adventitial fibrosis, and (3) lack of inflammation or thrombi. MAVD/V cases were clinically diagnosed as CD (45%), mass/lesion (27%), ischemia (9%), obstruction (9%), or rectal prolapse (9%). Abdominal pain for >1 year was the most common symptom. Most patients were women (M:F=1:2.7; mean age, 63 y). Mucosal changes mimicking CD, such as architectural distortion (55%), multifocal ulcers (73%), and pyloric gland metaplasia (64%), were common; however, no granulomas or transmural lymphoid aggregates were identified. Ischemic pattern of injury was seen in 4 cases. Upon follow-up (mean, 31.2 mo), 8 patients were found to be asymptomatic, 2 had died of unrelated causes, and 1 was lost to follow-up. We propose the name MAVD/V for a distinct noninflammatory, nonatherosclerotic, localized form of mesenteric vasculopathy that involves both arteries and veins, distinct from FMD. Unlike FMD, surgical resection appears to be curative, with a favorable clinical outcome. Awareness of this vascular entity is important as patients may be potentially misdiagnosed as having CD and ischemic bowel disease.
authors.affiliation: Departments of *Anatomic Pathology ‡Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH †Department of Anatomic Pathology, University of Utah, Salt Lake City, UT.
authors.forename: Deepa T
authors.initials: DT
authors.lastname: Patil
authors.forename: Juliana
authors.initials: J
authors.lastname: Kissiedu
authors.forename: E Rene
authors.initials: ER
authors.lastname: Rodriguez
authors.forename: Erinn
authors.initials: E
authors.lastname: Downs-Kelly
authors.forename: Xiuli
authors.initials: X
authors.lastname: Liu
authors.forename: Lisa A
authors.initials: LA
authors.lastname: Rybicki
authors.forename: Carmela D
authors.initials: CD
authors.lastname: Tan
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D005260:Female; D005352:Fibromuscular Dysplasia / Q000175:diagnosis* / Q000473:pathology; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008638:Mesenteric Arteries / Q000473:pathology; D008641:Mesenteric Vascular Occlusion / Q000175:diagnosis* / Q000473:pathology; D008642:Mesenteric Veins / Q000473:pathology; D008875:Middle Aged; D012189:Retrospective Studies