Living-Donor Liver Transplantation in a Carbamoyl Phosphate Synthetase I Deficiency Recipient With Scoliosis: A Case Report.

Background: Neuromuscular scoliosis is associated with cerebral palsy caused by metabolic diseases. Patients with scoliosis require meticulous consideration in abdominal surgery, as scoliosis can reduce the abdominal cavity volume, compress abdominal organs, and cause abdominal complications. Special attention should be paid to the graft position, especially in the setting of liver transplantation (LT). We herein describe a pediatric case of LT for carbamoyl phosphate synthetase I (CPS1) deficiency with severe scoliosis.

Case Presentation: A 13-year-old girl with CPS1 deficiency was transferred to our department as a candidate for liver transplantation. She underwent living donor liver transplantation with a left lobe from her mother. Following LT, portal vein (PV) complications occurred due to the kinking anastomosis, requiring several rounds of graft repositioning, PV reconstruction, thrombectomy, and finally stent placement due to severe scoliosis. Technical efforts were made to ensure PV blood flow with stent placement via the umbilical vein. Three months after LT, she was discharged from our hospital with sufficient PV flow.

Conclusions: This report suggests the need for a careful surgical approach in patients with skeletal abnormalities, such as the management of complications arising from anatomical abnormalities and selection of the appropriate graft size. Preoperative assessment and surgical planning of both donors and recipients according to patient characteristics should be carefully conducted.