Introduction: Due to organ scarcity, many methods have been developed for liver transplantation (LT) to use existing donors more efficiently. Auxiliary liver transplantation and domino liver transplantation (DLT) are among these methods. Familial hypercholesterolemia (FH) is a genetic mutation resulting in a deficiency of the low density lipoprotein (LDL) receptor. As a result of this receptor deficiency, sufficient levels of cholesterol cannot be removed from the circulation and fatal consequences such as myocardial infarction can occur. In this case report, we present the results of whole graft domino cross auxiliary liver transplantation in a patient with FH.
Methods: A 2-year-old boy was admitted to our center with a diagnosis of mapple syrup urine disease (MSUD). The patient was diagnosed on postnatal day 5 and was being followed up. The patient had to follow a completely protein restricted diet. No problems were detected in the examinations performed for LT. Liver function tests were normal. Liver biopsy showed a completely normal liver histopathologically. We planned to perform LT from the patient's father and use the liver from the patient as a graft.
The recipient of DLT was a 6-year-old boy with FH. Xanthomas occurred in the presacral area and elbows and total cholesterol level was found to be 799 mg/dl. There was no response to medical treatment and xanthomas increased. We planned DLT for the patient who did not have a suitable donor. The patients liver function tests and liver biopsy were completely normal. Considering any vascular complications that may occur in the graft we will transplant to the FH patient, we decided to perform left hepatectomy instead of complete hepatectomy.
Results: We performed left lateral liver transplantation in a patient with MSUD from his father. We first performed left hepatectomy on the FH patient. During hepatectomy, the left portal vein, left hepatic artery, left bile duct and left hepatic vein were transected with as much stump as possible. After the whole graft was perfused, portal vein (PV) anastomosis was performed end-to-end with the left PV of the recipient, hepatic artery (HA) anastomosis was performed end-to-end with the left HA of the recipient, and hepatic vein (HV) anastomosis was performed end-to-end with the left HV. The graft choledochal duct was anastomosed end to end with the recipient's left bile duct. On postoperative day 4, total cholesterol value decreased from 799 mg/dl to 242 mg/dl. LDL cholesterol value decreased from 717 mg/dl to 182 mg/dl. Liver function tests were completely normal in both recipients. Both patients were successfully discharged on postoperative day 9.
Conclusion: Domino cross auxiliary liver transplantation is an effective treatment for selected patients with metabolic diseases. Performing only left lobectomy on the native liver of the DTL recipient is an effective method to keep the patient in a safe area against complications that may occur in the graft.