Trans-splenic catheterization for congenital extrahepatic portosystemic shunt embolization treating hepatopulmonary syndrome: A case report

Congenital extrahepatic portosystemic shunt (CEPS) is a rare vascular malformation, classified into type I (complete absence of the portal vein) and type II (presence of a hypoplastic portal vein). This anomaly may lead to severe complications, including liver tumors, hepatopulmonary syndrome (HPS), and pulmonary hypertension. We report the case of a girl in her early teens with type II CEPS and a prior history of hepatocellular carcinoma (HCC), who subsequently developed HPS. Due to the complexity of the vascular anatomy, conventional femoral vein catheterization was unsuccessful. A trans-splenic approach, performed under ultrasound guidance, enabled successful embolization of the shunt using an 18 mm vascular plug. The patient's symptoms resolved completely within 3 months postprocedure, with normalization of oxygen saturation and resolution of orthodeoxia. This case underscores the efficacy of trans-splenic access in complex CEPS cases and highlights the importance of portal pressure monitoring prior to shunt closure to minimize the risk of complications such as superior mesenteric vein thrombosis.