THE CLINICAL SPECTRUM OF PORTO-SINUSOIDAL VASCULAR DISEASE IN PAEDIATRICS: A SINGLE CENTRE REVIEW.
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All Authors
Lucas, S.F.
Jayaprakash, K.
Mtegha, M.
Rajwal, S.
Karthikeyan, P.
LTHT Author
Lucas, Sandra Fernandes
Jayaprakash, Kavitha
Mtegha, Marumbo
Rajwal, Sanjay
Karthikeyan, Palaniswamy
Jayaprakash, Kavitha
Mtegha, Marumbo
Rajwal, Sanjay
Karthikeyan, Palaniswamy
LTHT Department
Leeds Children's Hospital
Doctors' Rotation
Children's Liver Unit
Doctors' Rotation
Children's Liver Unit
Non Medic
Publication Date
2025
Item Type
Conference Abstract
Language
Subject
Subject Headings
Abstract
Porto-sinusoidal vascular disease (PSVD) is a condition which affects the portal venules and sinusoids in the absence of cirrhosis. 1 Knowledge about PSVD natural history is still limited. 2-4 We aimed to describe the characteristics of children affected with PSVD, as well as their histological findings and clinical outcomes. We performed a retrospective review of children with native liver biopsy samples suggestive of PSVD from January 2019 to October 2024. Forty-eight patients fit the diagnostic criteria of PSVD as described by Gottardi et al.1 with 64.6% (n=31) having associated portal hypertension (PHT) and 35.4% (n=17) without PHT. The most common reason for referral to hepatology was isolated elevated transaminases in the non-PHT group, hypersplenism and portal hypertensive bleed in the PHT group (table 1). There were no significant differences between the histological features found on both groups, apart from a higher prevalence of perisinusoidal fibrosis in those with PHT (p=0.018). The non-PHT group had a higher median ALT of 61 (IQR 35, 213) at presentation when compared to the PHT group (p=0.021). During the follow up period no significant adverse events were observed in the non-PHT group, while 2 children developed hepatopulmonary syndrome and 3 underwent liver transplantation in the PHT group. Of those with PHT, 16 had evidence of portal vein thrombosis (PVT) with cavernous transformation at presentation and 2 developed PVT during the follow up period. In summary, PSVD is a condition with a variable phenotypic presentation and disease course. Those without PHT, mostly presenting with isolated elevated transaminases, appear to have a milder disease course, while those with PHT can have poor outcomes. Prospective studies are required to improve our understanding of the disease course and identify those at risk of progressing to PHT and developing adverse outcomes.
Journal
Frontline Gastroenterology