Successful coil embolization in an infant with congenital intrahepatic portosystemic shunts

Area of Science:

• Pediatric Gastroenterology
• Vascular Surgery
• Medical Imaging

Background:

• Congenital intrahepatic portosystemic shunts (IHSS) are rare vascular anomalies.
• IHSS can lead to severe metabolic derangements like hyperammonemia and hypergalactosaemia.
• Early diagnosis and intervention are crucial for favorable outcomes in affected infants.

Observation:

• A 7-month-old Japanese infant presented with hypergalactosaemia and hyperammonemia at 1 month of age.
• Diagnostic imaging, including ultrasonography and angiography, confirmed the presence of congenital IHSS.
• The infant's clinical presentation indicated significant metabolic disturbance secondary to the shunt.

Findings:

• Successful diagnosis of congenital intrahepatic portosystemic shunts was achieved using ultrasonography and angiography.
• Coil embolization was performed as a minimally invasive treatment for the vascular anomaly.
• The procedure resulted in the complete resolution of hyperammonemia and hypergalactosaemia.

Implications:

• Coil embolization is an effective treatment for congenital intrahepatic portosystemic shunts in infants.
• Timely intervention can prevent or reverse serious metabolic complications associated with IHSS.
• This case highlights the importance of advanced imaging and interventional techniques in managing rare pediatric vascular disorders.