This document addresses liver transplantation for individuals with end-stage liver disease. Donor livers are obtained from deceased donors, in which a whole or partial (split) liver may be transplanted. Living donors are another possible source from adult to child or adult to adult.

Note: Please see the following for additional information:

• CG-TRANS-02 Kidney Transplantation
• TRANS.00013 Small Bowel, Small Bowel/Liver and Multivisceral Transplantation

Position Statement

Note: Members must meet the disease specific criteria as well as the general Individual Selection Criteria below for the transplantation to be considered medically necessary.

Medically Necessary:

A whole or partial liver transplant using a deceased or living donor is considered medically necessary for selected individuals with end-stage organ failure due to irreversible liver damage that includes, but is not limited to, the following conditions:

1. Cholestatic liver diseases:
   1. Primary biliary cirrhosis
   2. Primary sclerosing cholangitis
   3. Biliary atresia
   4. Caroli's disease
   5. Familial cholestasis
   6. Arteriohepatic dysplasia (Alagaille's disease)
   7. Cystic Fibrosis
2. Hepatocellular injury:
   1. Viral-induced Hepatitis
   2. Drug induced
      1. Acetaminophen
      2. Associated with halothane, gold, disulfram, others
   3. Alcohol induced
   4. Toxin exposure: Amanita mushroom poisoning
   5. Autoimmune hepatitis
3. Inborn errors of metabolism:
   1. Wilson's disease
   2. Organic acidurias
   3. Hemochromatosis
   4. Alpha-1 antitrypsin deficiency
   5. Homozygous type II hyperlipoproteinemia
   6. Crigler-Najjar Syndrome type I
   7. Protoporphyria
   8. Some urea cycle deficiencies
   9. Glycogen storage diseases types I and IV
   10. Tyrosine deficiency
   11. Citrullinemia
   12. Ornithine transcarboxylase deficiency
   13. Familial amyloid polyneuropathy (requires transplantation - polyneuropathy and cardiac amyloidosis development due to the production of a variant transthyretin molecule by the liver)
   14. Oxalosis (primary)
4. Acute Diseases:
   1. Fulminant hepatic failure
5. Mass Occupying Lesions:
   1. Polycystic disease of the liver (requiring transplantation due to the anatomic complications of a hugely enlarged liver)
   2. Hepatoblastoma confined to the liver
   3. Primary hepatocellular carcinoma confined to the liver
   4. Hemangioendothelioma
   5. Hilar cholangiocarcinoma (CCA) with a cross-sectional diameter 3 cm or less in conjunction with neoadjuvant chemoradiation therapy and the tumor is unresectable or there is underlying liver disease such that the individual is not a candidate for resection
6. Vascular disease:
   1. Budd-Chiari Syndrome
7. Other:
   1. Cryptogenic cirrhosis

Liver Retransplantation

Retransplantation in individuals with graft failure of an initial liver transplant, due to either technical reasons or hyperacute rejection is considered medically necessary.

Retransplantation in individuals due to either chronic rejection or recurrent disease is considered medically necessary when the individual meets general selection criteria as defined below.

Investigational and Not Medically Necessary:

Liver transplants in individuals with extrahepatic malignancy, including, but not limited to non-hilar extrahepatic cholangiocarcinoma, intrahepatic cholangiocarcinoma or hepatocellular carcinoma when either condition extends beyond the liver, are considered investigational and not medically necessary.

Liver transplants for all other conditions that do not lead to end-stage organ failure due to irreversible liver damage are considered investigational and not medically necessary.

Xenotransplantation is considered investigational and not medically necessary.

Bioartificial liver devices are considered investigational and not medically necessary.

Note: For multi-organ transplant requests, criteria must be met for each organ requested. In those situations, a member may present with concurrent medical conditions which would be considered an exclusion or a comorbidity that would preclude a successful outcome, but would be treated with the additional organ transplant. Such cases will be reviewed on an individual basis for coverage determination to assess the member's candidacy for transplantation.

General Individual Selection Criteria

In addition to having end stage liver disease, the member must not have a contraindication as defined by the American Society of Transplantation in Guidelines for the Referral and Management of Patients Eligible for Solid Organ Transplantation (2001) listed below.

Absolute Contraindications- for Transplant Recipients include, but are not limited to, the following:

1. Metastatic cancer
2. Ongoing or recurring infections that are not effectively treated
3. Serious cardiac or other ongoing insufficiencies that create an inability to tolerate transplant surgery
4. Serious conditions that are unlikely to be improved by transplantation as life expectancy can be finitely measured
5. Demonstrated patient noncompliance, which places the organ at risk by not adhering to medical recommendations
6. Potential complications from immunosuppressive medications are unacceptable to the patient
7. Acquired immune deficiency syndrome (AIDS) (diagnosis based on Centers for Disease Control and Prevention [CDC] definition of CD4 count, 200 cells/mm³) unless the following are noted:
   1. CD4 count greater than 200 cells/mm³ for greater than 6 months
   2. HIV-1 RNA undetectable
   3. On stable anti-retroviral therapy greater than 3 months
   4. No other complications from AIDS (for example, opportunistic infection, including aspergillus, tuberculosis, coccidioidomycosis, resistant fungal infections, Kaposi’s sarcoma or other neoplasm)
   5. Meeting all other criteria for liver transplantation*

*Steinman, Theodore, et al. Guidelines for the Referral and Management of Patients Eligible for Solid Organ Transplantation. Transplantation. Vol. 71, 1189-1204, No. 9, May 15, 2001.