To report a case of a three-month-old infant with a significant intrahepatic portosystemic shunt and review existing literature on congenital portosystemic shunts, highlighting the clinical significance of timely intervention.
Key Findings:
The infant presented with hyperbilirubinemia (316 µmol/L) and elevated transaminase levels (ALT 944 U/L, AST 405 U/L).
Doppler ultrasonography and contrast-enhanced CT confirmed a large intrahepatic portosystemic shunt (up to 25 mm in diameter) with hypoplasia of the right hepatic lobe.
Postoperative complications included coil migration into the pulmonary artery, which was successfully managed with endovascular retrieval.
The patient showed rapid normalization of liver function tests and catch-up growth during follow-up, restoring portal blood flow.
Interpretation:
The case demonstrates the feasibility and effectiveness of endovascular closure of large intrahepatic congenital portosystemic shunts in very young infants, suggesting implications for future clinical practice.
Limitations:
The procedure was technically challenging due to the patient's young age and small vessel caliber, which may limit applicability to similar cases.
Intraoperative portal pressure measurement and balloon occlusion testing could not be performed, potentially affecting the assessment of shunt closure.
Conclusion:
Endovascular intervention for large intrahepatic CPSS is a viable option in infants, emphasizing the need for thorough anatomical evaluation, careful postoperative monitoring, and the importance of multidisciplinary planning.
