A Case of Congenital Portosystemic Shunt Treated with Shunt Embolization

Abstract

Congenital portosystemic shunt (CPS) is an anomaly of splanchnic venous flow which bypasses the liver and drains directly into the systemic circulation. It is associated with complications like neonatal cholestasis, hepatopulmonary syndrome, portopulmonary hypertension and encephalopathy. A female with microdeletion of chromosome 5q14.3, atrial septal defect, ventral septal defect and chronic oxygen requirement presented at 8 months with elevated AST/ALT (50) and irritability. Ammonia was elevated to 90. Abdominal CT showed a portosystemic shunt connecting portal vein to inferior vena cava. She was treated with lactulose/rifaxamin without any benefit. Due to concern for encephalopathy, she underwent interventional radiology (IR) procedure to close the shunt. A 9 mm shunt joining the main portal vein to inferior vena cava was found. Left portal vein, left hepatic vein and middle hepatic vein were draining into the shunt. A vascular plug was deployed. After four months, she had recurrence of hyperammonemia. Repeat IR procedure showed two intrahepatic portosystemic shunts emanating from left hepatic vein and persistent communication between enlarged left portal vein and the dominant shunt. The shunts were embolized with coil and dominant shunt was re-occluded with vascular plug. Follow up ultrasound showed successful closure of the shunts and patient has done well with decreased oxygen requirement and normalization of ammonia and AST. CPS are vascular malformations that lead to severe complications. These may not be diagnosed early due to presence of other comorbid conditions like congenital heart defects. Chronic hyperammonemia may lead to encephalopathy which can cause cognitive disorders, drowsiness, delirium, mental retardation, behavioral problems and attention hyperactivity disorders. Doppler ultrasonography (US) is the key imaging modality for diagnosis and monitoring. Computed tomography (CT) with contrast helps in documentation of location and anatomy of the shunt. The management is based on the size of the shunt. Small intrahepatic shunts may close spontaneously and may be monitored till age of 2 years. Large intrahepatic shunts, those with persistent ductus venosus and extrahepatic shunts need to be considered for closure, by endovascular methods or surgery. Our patient improved remarkably following IR guided embolization of her CPS shunt.Figure 1Figure 2Figure 3