HOW MUCH LIVER TISSUE IS NECESSARY TO COMPENSATE THE DEFECT IN TYPE 1 HYPEROXALURIA?

Abstract

A170 Aims: Liver transplantation (LTX) corrects the enzymatic defect responsible for primary hyperoxaluria. It has been advocated in combination with kidney transplantation (KTX) in patients with renal failure, since KTX alone can result in early graft loss. Methods: Case presentation of a patient with primary hyperoxaluria type 1. Pre- and posttransplant data were collected prospectively and reviewed in a retrospective fashion. Results: We present in a 58-year-old male patient with primary hyperoxaluria type 1, confirmed by genetic testing, on hemodialysis, who underwent resection of the left lateral segment of the liver followed by an orthotopic auxiliary left lateral segment liver transplantation and kidney transplantation from a deceased donor. The larger part of the donor liver was transplanted in another patient. The postoperative course was marked by an episode of biliary leakage from both the cut surface of the liver and the biliary duct-to-duct left hepatic anastomosis, which was converted to a Roux-en-Y 8 days posttransplant. The serum oxalate dropped from 34.8 before transplant to 3.6-8.3 μmol/L in the first months after transplant to less than 1 μmol/L (normal range 0.4-3.0 μmol/L). One year after transplantation the patient has good renal function, with an iothalamate GFR of 58 ml/min. Conclusions: Orthotopic auxiliary LTX is a good alternative to whole LTX in primary hyperoxaluria. By using a split deceased donor liver, it does not deprive the cadaveric donor pool and protects the recipient from liver failure in case of graft loss. This approach is also attractive in the living donor setting.