Congenital portosystemic shunts

To date, the prevalence of congenital portosystemic shunts is not known, but the severity of its repercussions is recognized.

Complications of congenital portocaval shunts include benign and malignant hepatic tumors, impaired concentration and attention (encephalopathy), and severe cardiopulmonary complications, all of which have already been remported in infancy. It is therefore a true systemic disease with paediatric presentation and severe repercussions since these can lead to cancer, a liver transplant.

Complications can be diagnosed at any age. To this end we have created an international registry aimed to include all patients from childhood to adulthood.

Our goal is to better characterize malformations and their complications. Above all, our goal is to provide patients with the best care as soon as the diagnosis is made.

We offer multidisciplinary management involving interventional radiology, cardiology if necessary, neurology, and other specialties according to the needs of the child.

Links:

The International Registry of Porto-Systemic shunts

IRCPSS

ESPGHAN