Domino liver transplantation: How far can we push the paradigm?

Abstract

Domino liver transplantation (DLT) has emerged as a strategy for increasing the number of liver grafts available: morphologically normal livers from donors with metabolic diseases can be used for select recipients with hepatocellular carcinoma (usually outside the Milan criteria). Familial amyloidotic polyneuropathy (FAP) is the most common indication for DLT. When FAP patients are involved in DLT, the indications and outcomes are clear and good, although de novo FAP development within various periods of time has been described in DLT recipients of FAP livers. With the increasing need for organs, livers explanted from patients with rare metabolic diseases, such as primary hyperoxaluria (PH), acute intermittent porphyria (AIP), maple syrup urine disease (MSUD), and homozygous familial hypercholesterolemia (HFHC), are being used for DLT. However, insufficient data about the use of livers from patients with these rare metabolic diseases are available. In this review, we focus on the latter disorders. PH is not a good indication for DLT because recipients of PH livers develop hyperoxaluria and early acute renal failure. AIP also seems to be a debatable indication for DLT because of the rapid development of neurotoxicity in AIP liver recipients. However, the outcomes of DLT with HFHC and MSUD liver grafts (which include the risk of the de novo development of these genetic diseases) are promising. For rare metabolic liver diseases to be established as indications for DLT, more reports and studies are needed. Liver Transpl 18:22-28, 2012. V C 2011 AASLD. Because of the organ shortage and the increasing number of patients on the waiting list for liver transplantation (LT), alternative techniques such as split LT and living donor LT have been developed. Another possible way of increasing the pool of liver grafts is the use of marginal livers. Domino liver transplantation (DLT) has emerged as a strategy for increasing the number of liver grafts available: explanted organs are used for select patients. Except for the production of an abnormal protein or enzyme, these livers are morphologically normal and fully functional. Additionally, the metabolic diseases of the donors usually should not produce symptoms in the DLT recipients for many years. In comparison with deceased donor liver transplantation (DDLT), DLT should not expose the recipients or the donors to any significant additional operative risks. The survival of the recipients should be as good as it would be with DDLT, and the morbidity and mortality of the donors must be kept to a minimum. 1 So that as much information as possible could be gathered about DLT, an international registry (the Domino Liver Transplant Registry) was created in 1999 as an extension of the already existing Familial Amyloidotic Polyneuropathy World Transplant Registry. 2 According to this registry, DLT had been performed 790 times by December 31, 2009. 3

Curative Intent Treatment of Hepatocellular Carcinoma - 844 Cases Treated in a General Surgery and Liver Transplantation Center

“…However, Milan criteria (MC) remain the most frequently used for patient selection (2). LT was performed using whole graft livers (from cadaveric donor or from living donor in case of domino technique), and partial livers (living donor LT or split liver transplantation in case of cadaveric donors) (17,(29)(30)(31). In order to expand the donor pool marginal donors could be accepted with good results.…”

A metabolic chimera: Two defective genotypes make a normal phenotype

“…APOLT preserves the possibility of withdrawal of immunosuppression in case gene therapy becomes clinically viable during the lifetime of the patient. Domino liver transplantation (DLT) 5 involves use of a genetically defective liver as a graft for a second recipient, usually an elderly person or one with hepatocellular carcinoma, in whom the anticipated interval to manifest the disease is longer than their life expectancy (familial amyloid polyneuropathy). DLT is also used when the extrahepatic organs of the recipient are able to compensate for the deficient gene in the transplanted liver (maple syrup urine disease) or as a bridge to retransplantation.…”

Successful combined liver/kidney transplantation from a donor with Pompe disease

“…Therefore, the potential impact of organ specific genetic defects that may be transmitted to the recipient must be considered [16][17][18]. In our case and increasingly in the future, the assistance of medical geneticists was and can be an invaluable aid for the evaluation of potential donor candidates.…”