Emre Can Çelebioglu, Emre Utkan Buyukceran, Sede Kaynak Sahap, Fazilcan Zirek, Nazan Çobanoglu, Suat Fitoz, Sadik Bilgiç
Ankara University Faculty of Medicine. Igdır State Hospita.
Turkey
Science Progress
Sci Prog 2025;
DOI: 10.1177/00368504251333521
Abstract
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No