Portosystemic shunt in children: Outcomes from a pediatric referral center

Abstract

Background

Congenital portosystemic shunts are rare vascular malformations in children that can result in complications such as abnormal liver function, liver masses, pulmonary hypertension, and hepatopulmonary syndrome.

Methods

Patients with portosystemic shunts were managed according to the protocol established at the center. The characteristics and treatment outcomes of patients over a 3-year period (from January 2021 to December 2023) were retrospectively reviewed and reported.

Results

There were 21 pediatric patients, including 13 intrahepatic cases and 8 extrahepatic cases. All intrahepatic cases were detected at birth. The extrahepatic cases were diagnosed at an average age of 6 years. Among the 13 intrahepatic cases, clinical presentations included cholestatic jaundice (7/13) and hyperammonemia (8/13). During follow-up, 10 out of 11 intrahepatic shunts spontaneously closed after 2–10 months of monitoring, while 1/11 shunt remained open after 18 months. Two patients died due to severe pneumonia and heart defects. Among the 8 extrahepatic cases, clinical presentations included liver masses in 3/8 cases, hepatopulmonary syndrome in 1/8 case, hyperammonemia in 5/8 cases, and pulmonary hypertension in 1/8 case. Four cases without intrahepatic portal system were monitored regularly, awaiting liver transplantation if necessary. One case was lost to follow-up. Three cases underwent surgery or endovascular interventions, performed in one or two stages to close the shunt, all resulting in positive outcomes (1 case with hepatopulmonary syndrome fully recovered, 1 case with hyperammonemia and coagulation disorder returned to normal, 1 case with pulmonary hypertension and hyperammonemia did not show improvement in pulmonary hypertension after 6 months, but NH3 levels returned to normal).

Conclusions

Intrahepatic portosystemic shunts detected at birth often close spontaneously. In contrast, extrahepatic shunts are often detected in older children with a variety of symptoms. Surgical or endovascular interventions to close the shunt are the preferred treatment options when possible, yielding good outcomes.