RIACHE Hafsa, BOUTALEB Joud, LOUBARIS Sarah, LOUGHZAIL Souhaila, HSSISSEN Laila, ALLALI Nazik, CHAT Latifa, EL HADDAD Siham
Journal: Medpeer Publisher
ISSN: 3066-2737
Volume: 2
Issue: 9
Date of Publication: 2025/09/09
Portal biliopathy is a rare complication of chronic portal vein thrombosis, exceptionally reported in sickle cell disease. We describe a 16-year-old girl with sickle cell thalassemia who developed portal cavernoma, collateral circulation, hepatomegaly, gallstones, splenosis, and biochemical cholestasis after splenectomy and extensive portal vein thrombosis. The condition results from extrinsic compression of the bile ducts by venous collaterals and ischemic changes of the peribiliary plexus, mechanisms exacerbated by the hypercoagulability of sickle cell disease. Imaging, particularly MRCP, is central to diagnosis, while management ranges from surveillance to endoscopic or surgical intervention depending on clinical severity. Awareness of this entity is essential to avoid confusion with malignant biliary obstruction and to guide appropriate multidisciplinary care.
Portal biliopathy, sickle cell disease, portal vein thrombosis, MRCP, case report
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