Aetiology and management of extrahepatic portal vein obstruction in children: King's College Hospital experience

Abstract

Objective: To study a single-centre experience of the management of extrahepatic portal vein obstruction (EHPVO) in children during the last 3 decades.

Materials and methods: The medical records of 108 children (67 male, median age 4.75 years, range = 1 day-16.3 years) presenting with EHPVO between 1979 and 2005 were reviewed retrospectively.

Results: Extended prothrombotic screening performed in 30 patients revealed low protein C activity (6 patients), low free protein S (2), and a positive lupus anticoagulant (1); factor V Leiden mutations and the JAK2V617F mutation were not identified. Associated congenital anomalies were found in 26 of the 108 children (24%). Clinical presentation included splenomegaly in 98 (91%) and ascites in 3 (3%). Elevation of liver enzymes and prolonged international normalized ratio were seen in 13 (12%) and 14 (13%) children, respectively. Haematological parameters of hypersplenism were present in 13 (12%). Bleeding occurred in 83 (77%) patients with a median age of 4.58 (0.02-16.37) years. On first endoscopy, oesophageal varices were present in 92 patients; of those subjects, 70 (76%) received sclerotherapy, 5 (5%) had band ligation, and 16 (17%) received both. Complications of endoscopy occurred in 34 (37%) patients: oesophageal ulcers in 16, oesophageal stricture in 10, both in 7, and erosive gastritis in 1. Seventeen (16%) children underwent shunt surgery for uncontrolled bleeding at a median age of 9.7 (5.2-23.7) years.

Conclusions: The aetiology of EHPVO in the majority of patients remains unknown. Sclerotherapy and banding are effective treatments for bleeding varices with good long-term outcome. Procoagulant state is an infrequent cause of EHPVO in children.