3D-printed model for surgical planning in congenital porto-systemic shunt: A case report

Introduction

Hepatobiliary surgery can be challenging due to the complex nature of variations in liver anatomy, particularly in small children. Congenital portosystemic shunts (CPS – Abernethy syndrome) are increasingly recognized with a range of clinical presentations and associated complications and can present anatomical challenges for operative shunt closure. Three-dimensional printing (3DP) has gained popularity due to its ability to produce accurate models with the specific anatomical characteristics for the individual patient. We used a 3DP model from original image sources for planning of surgical shunt closure in a child with CPS and for educating the family and surgical team preoperatively.

Case presentation

A 5-year-old male with autism and hyperammonemia of 80 μmol/L and was found to have an intrahepatic CPS. No tumors were seen within the liver. Physical examination was unremarkable. After treatment with Rifaximin to lower blood ammonia levels, his ability to concentrate and achieve milestones improved. Angiography with shunt occlusion showed an intrahepatic shunt between the left portal and the middle hepatic vein into the IVC. Intrahepatic portal branches were demonstrated with the implication that a one stage closure could be performed. A 3DP model was printed from the cross sectional imaging which allowed for more detailed planning of shunt ligation and to explain the surgical details to the parents. Surgical closure was performed with no complications.

Conclusion

3DP models can be a useful tool for planning complex congenital porto-systemic shunt closure surgery.