Liver transplantation indications
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohammed Abdelwahed M.D[2]
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Liver trasnsplantation Microchapters |
--Overview--
Indications Acute liver failure Acute liver failure is defined by the development of severe acute liver injury with encephalopathy and impaired synthetic function (international normalized ratio [INR] of ≥1.5) in a patient without cirrhosis or preexisting liver disease. viral and drug-induced hepatitis are the most common causes of acute liver failure in adults. [3]
Cirrhosis manifestation of compromised hepatic function [1]. Variceal hemorrhage, ascites, and encephalopathy are the primary manifestations of end-stage liver disease and are designated as markers of decompensation. development of hepatorenal syndrome, Patients with cirrhosis are typically candidates for liver transplantation once their biologic Model for End-stage Liver Disease (MELD) score is ≥15 (calculator 1 and calculator 2). However, some patients with Child B cirrhosis (table 2) with portal hypertension but a low MELD score may be candidates for liver transplantation. The transplantation evaluation is typically started once a patient has a MELD score >10. This permits the patient to meet the transplantation team prior to the development of end-stage liver disease and ensures adequate time for the patient to complete the pretransplantation evaluation.
Patients may also qualify for liver transplantation if they have a complication or condition that qualifies for standard MELD exception points. Conditions include ●Hepatocellular carcinoma. ●Hepatopulmonary syndrome. ●Portopulmonary hypertension (provided the mean arterial pressure can be maintained at <35 mmHg with treatment). ●Familial amyloid polyneuropathy. ●Primary hyperoxaluria. ●Cystic fibrosis. ●Hilar cholangiocarcinoma (provided the liver transplantation center has a UNOS-approved protocol detailing the work-up and management of patients with cholangiocarcinoma undergoing transplantation). ●Hepatic artery thrombosis (occurring within 14 days of liver transplantation but not meeting criteria for status 1A).
Liver neoplasms
Patients with some primary liver neoplasms may be candidates for liver transplantation, provided the neoplasms meet specific criteria (eg, for patients with hepatocellular carcinoma [HCC], a single lesion ≤5 cm or up to three separate lesions all <3 cm, no evidence of gross vascular invasion, and no regional nodal or distant metastases). In addition, there may be a role for liver transplantation in patients with neuroendocrine tumors that have metastasized to the liver, but experience in this setting is limited [4].
Some of the liver neoplasms that have been treated with liver transplantation include [4]: ●HCC
For patients with localized HCC who are candidates for surgical resection, we recommend resection rather than transplantation
●For patients who are not candidates for resection and who have a single lesion ≤5 cm, no more than three separate lesions, none larger than 3 cm, no evidence of gross vascular invasion, and no regional nodal or distant metastases, we recommend liver transplantation (Grade 1B). When these criteria are strictly applied, five-year survival rates of 75 percent or higher can be achieved. INDICATIONS FOR TRANSPLANTATION
For patients with localized HCC who are not candidates for resection, orthotopic liver transplantation is an appropriate strategy for patients with a single lesion ≤5 cm, up to three separate lesions, none larger than 3 cm, no evidence of gross vascular invasion, and no regional nodal or extrahepatic distant metastases. When these criteria are strictly applied, five-year survival rates 75 percent or higher can be achieved. Overall survival in carefully selected patients undergoing orthotopic liver transplantation (OLT) for HCC is similar to or only slightly worse than the survival of patients undergoing OLT for nonmalignant causes. Although randomized trials have not been carried out, uncontrolled series suggest that survival following OLT is as good or better than it is after alternative treatments for HCC in carefully selected patients.
The landmark study of Mazzaferro in 1996 established deceased-donor liver transplantation (OLT) as a viable option for the treatment of hepatocellular carcinoma (HCC) [5]. They showed that when transplantation was restricted to patients with early HCC (defined as single lesion ≤5 cm, up to three separate lesions, none larger than 3 cm, no evidence of gross vascular invasion, and no regional nodal or distant metastases), a four-year survival rate of 75 percent could be achieved. These outcomes are similar to expected survival rates for patients undergoing transplantation for cirrhosis without HCC. These criteria have become known as the Milan criteria and have been widely applied around the world in the selection of patients with HCC for liver transplantation [5]. Considerable interest has arisen in expansion of these transplant criteria in highly specialized centers, although such expanded criteria remain purely investigational at present. This subject is discussed in detail below. (See 'Expanded transplant criteria' below.) Extrahepatic staging in patients being considered for transplantation should include computed tomography (CT) of the chest and CT or magnetic resonance imaging (MRI) of the abdomen and pelvis [6]. Bone scan was previously required, but this was changed in December 2012. (See 'Requirements for listing and management while on the wait list' below.)
●Prelisting biopsy is not mandatory. However, in the United States, requirements for listing for liver transplantation from the United Network for Organ Sharing (UNOS) require that patients have imaging findings that are consistent with HCC; only class 5 nodules meet imaging criteria for listing for transplantation (table 4).
●Epithelioid hemangioendothelioma [5-7]
●Large hepatic adenomas
Metabolic disorders Liver-based metabolic conditions that have been treated with liver transplantation include: ●Familial amyloid polyneuropathy (qualifies for standard MELD exception points) [8-10].
●Primary hyperoxaluria (qualifies for standard MELD exception points)
●Cystic fibrosis (qualifies for standard MELD exception points)
●Alpha-1 antitrypsin deficiency [11,12].
●Some forms of glycogen storage disease (type I and type IV)
The optimal transplantation strategy for patients with PH type 1 remains uncertain [81]. Three different transplant options are available.
●Combined liver and kidney transplantation
●Isolated liver transplantation
●Isolated renal transplantation
●Tyrosinemia
OLT is performed in patients with persistent liver failure who do not respond to nitisinone therapy or have hepatic malignancy [39-41].
●Hemochromatosis [13,14]
●Wilson disease
Liver transplantation may be the only option for patients who present with acute liver failure and in those with decompensated liver disease who are unresponsive to drug therapy [82-86]. For those with advanced liver disease, a prognostic scoring system for children with Wilson disease presenting with failure was proposed by a group at Kings College and then later revised [87,88]. The revised system (based upon an index of serum bilirubin, the prothrombin time international normalized ratio [INR], aspartate aminotransferase level, and white blood cell count) had a sensitivity, specificity, and positive predictive value for determining the need for liver transplantation of 93, 98, and 88 percent, respectively [88]. This scoring system has been validated in pediatric and adult patients.
●Acute intermittent porphyria Reasonable indications for liver transplantation include frequent hospitalizations, unresponsiveness to hemin, and poor quality of life. However, it is often difficult to weigh the risks and benefits of liver transplantation because of the unpredictable natural course of the disease, which may progress or improve.
liver transplantation is usually reserved for patients who have developed end-stage liver disease or HCC.