We have located links that may give you full text access.
Colonic Adventitial Fibromuscular Dysplasia: A Nonspecific Arteriopathy Associated With Hirschsprung Disease and Other Obstructive Disorders.
Pediatric and Developmental Pathology 2018 July
Background Smooth muscle differentiation ("adventitial fibromuscular dysplasia," AFD) was purported as specific to arteries in the transition zone of Hirschsprung disease (HSCR) patients. We investigated AFD in an HSCR population and controls and consider the pathogenesis and significance of the vascular pathology. Design Vascular histology in sections from colonic HSCR resections (n = 55) was compared with age- and site-matched controls with (n = 19) and without (n = 28) non-HSCR obstructive conditions. Vascular pathology was mapped, and correlations were sought between the vascular findings and bowel distension, inflammation, neuromuscular anatomy, preoperative clinical variables, and postsurgical complications. Results One of 2 forms of AFD was identified in 42% (23/55) of the HSCR resections: the previously described "mature" form with adventitial bundles of differentiated smooth muscle cells (7/23, all submucosal) and a newly described "immature" AFD characterized by densely packed myofibroblasts in the arterial adventitia (16/23, 3 submucosal, 3 serosal, and 10 both). Adventitial inflammation and/or medial necrosis was present in the immature form (6/16). Mature submucosal AFD was present in 2/28 (7.1%) nonobstructive and 5/19 (26%) obstructive non-HSCR controls ( P = .10). Immature AFD was only found in less than 1-month-olds, and mature AFD only in older patients, including the 7 affected controls. AFD did not correlate with sex, syndromic status, length of the aganglionic segment, or postoperative complications. AFD was present in grossly dilated (17/23) and narrowed (10/23) regions and in the aganglionic (2/23), ganglionic (14/23), or both (7/23) segments. In several cases, AFD existed proximal to the histological transition zone. Conclusion AFD occurs in HSCR and other obstructive conditions but is significantly less common in the colons of patients with no history of dysmotility. The pathology likely progresses from a reversible accumulation of myofibroblasts in neonates to a stable population of mature smooth muscle cells. The distribution of vascular lesions does not correlate with neuropathological findings and suggests a nonspecific form of vascular injury, possibly related to bowel distension. AFD in HSCR resections has not been shown to be clinically significant and should not influence management.
Full text links
Related Resources
Trending Papers
Challenges in Septic Shock: From New Hemodynamics to Blood Purification Therapies.Journal of Personalized Medicine 2024 Februrary 4
Molecular Targets of Novel Therapeutics for Diabetic Kidney Disease: A New Era of Nephroprotection.International Journal of Molecular Sciences 2024 April 4
The 'Ten Commandments' for the 2023 European Society of Cardiology guidelines for the management of endocarditis.European Heart Journal 2024 April 18
A Guide to the Use of Vasopressors and Inotropes for Patients in Shock.Journal of Intensive Care Medicine 2024 April 14
Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association.Circulation 2024 April 19
Essential thrombocythaemia: A contemporary approach with new drugs on the horizon.British Journal of Haematology 2024 April 9
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.
By using this service, you agree to our terms of use and privacy policy.
Your Privacy Choices
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app