International Registry of CongenitalPorto-Systemic Shunts (IRCPSS)
A multi-center, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts
Congenital portosystemic shunts (CPSS) are a rare malformation in humans the systemic repercussions of which are potentially severe. They include signs and symptoms such as hepatic encephalopathy, pulmonary vascular diseases, benign or malignant liver tumors and biological and metabolic disorders. The true incidence of CPSS is not known. CPSS can be diagnosed at any age either because the patient is symptomatic or as an incidental finding.