PB0291 - Clinical Manifestations, Treatment and Outcomes of Pediatric Portal Vein Thrombosis

Background: Portal vein thrombosis (PVT) is an uncommon thromboembolic event that causes portal hypertension (PH) leading to splenomegaly and varices, resulting in upper gastrointestinal bleeding.

Aims: To study the characteristics and clinical courses of PVT

Methods: A retrospective study recruited 49 children < 18 years old, diagnosed with PVT by imaging studies during 2011-2022. The demographic data, clinical course, and outcome including PH were recorded.

Results: The median (range) age was 7 months (0.08–209 months), with a female: male ratio of 26:23. Twenty-six of the total 49 PVT patients were neonates with cholestatic jaundice. Seventeen (65%) neonates with PVT had a history of umbilical catheterization. Comorbidities of neonates with PVT were prematurity, asphyxia, maternal gestational diabetes mellitus, and sepsis. Beyond the neonatal period, the comorbidities were hepatic tumors, recurrent ascending cholangitis from the choledochal cyst or biliary atresia post-Kasai operation, chromosomal abnormalities, and liver abscess. Six children presented with UGIB and splenomegaly, leading to a diagnosis of PVT. The location of PVT was a single site in 28 patients, which was more common in the left PV. Multiple sites of PVT were identified in 21 patients. Twenty-three patients received enoxaparin, but only two of the 23 patients developed PH. Thrombophilic studies on eighteen patients revealed a high factor VIII level of 5, a positive APS of 1, and a FXII deficiency of 1. The outcomes of PVT were portal hypertension = 10, complete resolution = 23, left lobe atrophy = 7, and partial resolution = 7. Six patients (12.2%) died, one from a genetic disease and five from cancer.

Conclusion(s): Neonates were the majority of PVT caused by umbilical catheterization. Longitudinally follow-up doppler ultrasound might be essential for early detection and treatment of PH. Children with hepatobiliary tract infections should be aware of PVT.