Abstract
Introduction Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment
issues. The aim of this article is to show our experience in the heterogeneity of
this condition.
Material and Methods A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description
of the morphology, clinical impact, and treatment is given.
Results According to the imaging techniques (IT), the shunt was apparently intrahepatic in
14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children,
IT showed hepatic portal circulation. In total shunts in which radiological examination
was performed, invasive radiological techniques were able to demonstrate intrahepatic
portal vein. In other patients, it was not investigated as they are asymptomatic.
A child presented multiorgan failure with fulminant hepatic failure at birth. The
shunt was radiologically closed and clinical impairment reversed rapidly. He is now
asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven
children are asymptomatic at this time and are monitored periodically. Seven children
had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were
symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis,
gastrointestinal bleeding). Of these, in five we performed balloon test occlusion,
tolerated in all patients, followed by radiological closure. In our experience, the
advancement of interventional radiology techniques avoided surgery to close the shunt.
Conclusions Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections
established. CPSS has multiple clinical presentations, from asymptomatic patients
to acute liver failure. The therapeutic approach should be individualized and therefore
held in overspecialized centers.
Keywords
congenital portosystemic shunt - intrahepatic portal vein - hyperammonemia
