P0384 THREE CASES OF CONGENITAL INTRAHEPATIC PORTOSYSTEMIC VENOUS SHUNT IN NEONATES.

Abstract

Introduction: Congenital intrahepatic portosystemic venous shunt (CIHPSS) are very unusual abnormalities. Only 50 publications have been counted. They are defined as macroscopic communications between the portal and the systemic venous circulation. They are entirely or partially located within a heathy liver that functions without portal blood. When discovered early in life, they are in favour of a malformative origin of the shunt. HEREIN, we report three neonate observations with their icon-çography. For the case n°1, it was a color Doppler and ultrasound scanning for a fever checkup,, which diagnosed the CIHPSS. between the right portal vein and the right-sus hepatic vein. In the two other one, it was the antenatal color Doppler and ultrasound scanning which diagnosed shunts during the third term of pregnancy. Those three shunts were considered as type II, according to the PARK’s classification system for IHPSS anormalies, because they link up a portal branch to an hepatic vein in a same parenchymal segment. Methods: Case n°1 is now 6 years old and is still asymptomatic. A clinical, biological and ultrasonographic examination, once a year is sufficient. The vascular fistula resolved spontaneously when case n°2 was 28 months old and when case n°3 was 8,5 months old. Results: DISCUSSION: Normally, toxic metabolites of nutrients and residual activity of pancreatic enzymes are degraded by the liver. Here, due to the shunt, they bypass directly into the systemic circulation. Then, hyperammoniemia, trimethylaminuria, hyperinsulinemia, pulmonary hypertension or congestive heart failure can appear. THERAPY: Because of the rarity of this disorder, the choice of treatment is controversial. It depends on the natural course of the disease, but the majority of patients did not have symptoms until their 6th decade. Initially, the spontaneous resolution is expected. If hepatic encephalopathy or heart failure progress, some treatment may indicate: angiographic intervention with percutaneous coil embolisation, surgical intervention such as shunt ligation and hepatic resection or realisation of an alternative portosystemic shunt. Conclusion: CIHPSS have been thought to be uncommon, but improvement of imaging (US, CT, MRI) have shown even asymptomatic intrahepatic shunts in an increasing number of patients. Spontaneous resolution is frequent. Metabolic and hemodynamic repercussions will guide the management in other cases.