Imaging features, classification and clinical features of intrahepatic congenital portosystemic shunts

Congenital portosystemic shunts (CPSS) are a rare developmental anomaly diverting blood flow from the portal venous system and the liver to the systemic venous system. This case series examines the sonographic imaging findings, shunt classification, ultrasound shunt ratios, and outcomes in nine children (5 females, 4 males) admitted to our institution between 2015 and 2022 were included in this study. The shunts were initially categorized by the Parks classification and were followed by serial ultrasounds. Clinical presentation, clinical course, laboratory data, shunt ratios, and time to shunt closure were all followed on subsequent ultrasounds. The most common type of CPPS was the Type 3 shunt. In cases where shunt ratios were measured, the shunt ratio gradually decreased in tandem with decreasing ammonia levels until spontaneous closure was achieved. Predictors of lack of shunt closure included high shunt ratios and Type 4 shunts. Patients with CPPS can be followed with the shunt ratio calculation obtained from sonographic imaging, which may correlate to ammonia levels and indicate risk of hepatic encephalopathy as well as predict speed and timing of closure.