Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction.

Abstract

Meso-Rex bypass is the surgical intervention of choice for children with extrahepatic portal vein obstruction (EHPVO). Patency of Rex vein, umbilical recessus of the portal vein, is a prerequisite for this surgery. Conventional diagnostic modalities poorly detect patency, while transjugular wedged hepatic vein portography (WHVP) accurately detects patency in 90%. We aimed to assess Rex vein patency and portal vein branching pattern in children with EHPVO using transjugular WHVP and to identify factors associated with Rex vein patency. Transjugular WHVP was performed in 31 children with EHPVO by selective cannulation of left and right hepatic veins. Rex vein patency, type of intrahepatic portal venous anatomy (Types A-E), and factors associated with patency of Rex vein were studied. The patency of Rex recess on transjugular WHVP was 29%. Complete obliteration of intrahepatic portal venous radicles was the commonest pattern (Type E, 38.7%) while Type A, the favorable anatomy for meso-Rex bypass, was seen in only 12.9%. Patency of the Rex vein, but not the anatomical pattern, was associated with younger age at evaluation (patent Rex: 6.6 ± 4.9 years vs. nonpatent Rex: 12.7 ± 3.9 years, p = 0.001). Under-5-year children had a 12 times greater chance of having a patent Rex vein (odds ratio: 12.22, 95% confidence interval: 1.65-90.40, p = 0.004). Patency or pattern was unrelated to local factors like umbilical vein catheterization, systemic thrombophilia, or disease severity. Less than one-third of our pediatric EHPVO patients have a patent Rex vein. Younger age at evaluation is significantly associated with Rex vein patency.