During adolescence, patients with BCS present less commonly with ascites and may present with hepatomegaly alone. JAK-2 V617F mutation is the most common thrombophilic disorder during adolescence; though thrombophilic disorders are less common in adolescents than adults. Response to therapy is similar to adults, but better than children.
Intra-hepatic portal-venous collaterals are characteristic of Budd-Chiari syndrome (BCS) and are usually of small caliber and seen on Doppler. Creation of large portal-systemic shunt, either radiologically (Transjugular intrahepatic porto-systemic shunt) or surgically results in excellent long term outcomes in BCS. Here, we report a series of three rare cases of asymptomatic BCS, who had spontaneous large intra-hepatic portal-systemic shunts.
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