https://www.ncbi.nlm.nih.gov/pubmed/31729328
Indian Pediatr. 2019 Nov 15;56(11):965-967.
Pediatric Hepatic Venous Outflow Tract Obstruction: Experience from a Transplant Center.

Malik I1, Bhatia V2, Kumar K3, Sibal A2, Goyal N4.

Abstract

We performed a review of case records of children diagnosed with hepatic venous outflow tract obstruction at our center in last 10 years. Out of 11 cases identified, 6 had variable blocks in the hepatic venous system and 4 had combined hepatic venous and inferior vena cava (IVC) block. One child with paroxysmal nocturnal hemoglobinuria (PNH) had isolated IVC involvement. Angioplasty was attempted in 3 patients; among them 2 had successful outcome. Seven children with advanced liver disease underwent transplantation, which was successful in six. With availability of modalities like interventional radiology and transplantation, the overall prognosis of hepatic venous outflow tract obstruction seems to be good when managed in a well-equipped center.