Congenital porto-systemic shunts in children: preliminary results from the IRCPSS

Objectives and Study: Our aim was to use data from patients followed by centers participating in the first International Registry for Congenital Porto-Systemic Shunts (IRCPSS) to identify trends that might inform therapeutic strategies according to shunt anatomy. Methods: Data were collected retrospectively (descriptive patient data, mode of presentation, complications, management). Intrahepatic (IH) was defined as porto-hepatic and extrahepatic (EH) as a porto-systemic communication occurring either upstream of the portal vein or originating at the portal vein. Persistent ductus venosus were considered EH. Results: 246 children were identified with either intrahepatic (IH) or extrahepatic shunts (EH): 122 IH and 120 EH. 3 patients had both IH and EH. 24% of all CPSS were identified pre-natally. Among patients diagnosed pre-natally, a majority had IH shunts (75%). 189 (76%) patients were diagnosed post-natally at a mean age of 39.1 mo (0-200) for IH and 61.9mo (0-192) for EH. IH and EH shunts were equally frequent when diagnosed after birth. IH shunts were more often an incidental finding. Among patients diagnosed post-natally, symptoms were equally frequent among patients with IH (57%) or EH (61%) CPSS and are summarized in Table 1. In addition, patients with EH CPSS were more likely to have several symptoms than patients with IH CPSS. They were also more likely to have liver nodules on imaging (40.7% vs 26%). Closure: 184 children with CPSS were closed including 11 patients with 2 steps closure. Among these 184 patients 54 % of IH CPSS and 5% of EH CPSS closed spontaneously. 46% of IH CPSS required medical or surgical closure of which nearly 40% for a preventive indication. 94% of patients with EH CPSS were closed through a procedure, of which 41% were preventive. Conclusion: IH and EH shunts were equally frequent in this multicenter retrospective cohort of CPSS in children. CPSS are a cause of severe symptoms in children and should be sought in infants with hypoglycemia or cholestasis. In older children, they should be considered in the differential diagnosis of liver nodules, cardiopulmonary symptoms or neurocognitive deficits. Given the potential severity of complications, preventative closure was often performed, although timing and approach need further study, something which the IRCPSS aims to address.