Purpose: Polycystic liver disease (PCLD) is a rare, but important disease whose mortality is not fully predicted by MELD score. Most patients with PCLD maintain normal liver synthetic function, but develop complications such as malnutrition and hepatic vein outflow obstruction. Since 2006, MELD exceptions have been available for PCLD based on a white paper, with a recent revision in 2016. Current MELD exception criteria focus on symptoms, cyst distribution, spared remnant volume, venous occlusion, and hepatic decompensation or renal failure. Methods: We used the UNOS database to assess differences between patients with PCLD who were transplanted versus patients who clinically deteriorated or died waiting for transplant to determine if the current MELD exception policy is appropriate. Results: Among patients who were transplanted, patients without a MELD exception (N=202) had lower initial albumin, 3.3 vs 3.6 (p<0.001), higher initial INR, 1.3 vs 1.1 (p<0.001), higher initial bilirubin, 2.6 vs 0.8 (p<0.004), worse initial creatinine, 4.4 vs 2.3 (p<0.001), higher MELD scores, 20.4 vs 12 (p<0.001), and worse functional status than patients who received a MELD exception (N=164). When comparing baseline characteristics of patients who became too ill or died before transplant (N=91) with patients who ultimately received a liver transplant (N=366), the sicker patients were older, 55.3 vs 51.1 (p<0.01), had lower albumin, 3.2 vs 3.6 (p<0.01), worse ascites, higher bilirubin, 2.2 vs 1.5 (p<0.01), worse functional status, worse INR, 1.4 vs 1.2 (p<0.01) and a greater percentage were listed for simultaneous kidney transplant , 63.7% vs 44.3% (p<0.01).There was no statistically significant difference in BMI, creatinine, or encephalopathy between the two groups. Of patients who received a MELD exception, only 5 patients (3%) died or clinically deteriorated while waiting. These 5 cases had significantly lower albumin and higher INR at baseline compared with MELD exception patients who received a transplant. MELD score was also higher, but did not reach statistical significance. Conclusions: Current MELD exception guidelines give all PCLD patients the same exception points, regardless of their lab MELD. Our study suggests that functional status is associated with clinical deterioration or death before transplant in PCLD patients with MELD exception and should be included in the assessment of these patients for liver transplantation.

CITATION INFORMATION: Chandradas S, Waalen J, Frenette C. Polycystic Liver Disease Patients May Not Be Appropriately Represented by MELD Exception Policy. Am J Transplant. 2017;17 (suppl 3).

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