Extrahepatic Portal Vein Thrombosis in Childhood: Risk Factors, Clinical Manifestations, and Management

Objective: Extrahepatic portal vein thrombosis (EHPVT) is a common cause of portal hypertension in children. The aim of the present study was to identify the clinical manifestations and the risk factors for development of EHPVT in pediatric patients. Subjects and Methods: This was a single-center retrospective cohort study. A total of 12 children (6 boys and 6 girls) took part in the study. We noted the clinical presentations and the predisposing risk factors for development of EHPVT in all patients. In addition, as all of them had undergone an esophagogastroduodenoscopy for detection and grading of esophageal varices as part of the treatment algorithm, we analyzed the endoscopic findings and the therapeutic approach. Results: The median age of subjects at diagnosis was 3.5 years (range: 1–17 years). The most frequent initial clinical manifestation was upper gastrointestinal bleeding (6 cases, 50.0%) followed by splenomegaly (3 cases, 25.0%). The most frequent systemic risk factor for EHPVT was presence of inherited prothrombotic disorder (10 cases, 83.3%), and the most common local risk factor for EHPVT was umbilical vein catheterization (5 cases, 41.7%). Esophageal varices were revealed in all the study participants, and in the most cases, they were grade ≥2. Propranolol was used as primary or secondary prophylaxis in 7 children (58.3%), and in 5 children (41.7%), a shunt was performed (Meso-Rex bypass in 3 children and splenorenal shunt in 2 children). Conclusion: Patients with known systemic or local risk factors for EHPVT are indicated for proactive ultrasound screening for early diagnosis and timely management.