Zachary C Vinton, Carter A Schulz, Stephanie J Melquist, Clark C Kulig
Isolated polycystic liver disease (PLD) is rare and may cause debilitating mass-effect symptoms despite relatively preserved hepatic synthetic function. Because the Model for End-Stage Liver Disease (MELD) score does not capture nutritional, functional, and mechanical disease burden, affected patients may have low calculated MELD scores despite advanced symptomatic disease. Updated MELD exception guidance broadened consideration for PLD to include malnutrition and sarcopenia. We describe a patient with isolated PLD who was initially considered too well for transplantation but subsequently developed progressive weight loss, sarcopenia, and functional decline, prompting repeat evaluation, wait-list registration, and MELD exception approval. Several weeks after listing, he sustained traumatic hepatic cyst rupture, resulting in fatal hemoperitoneum and abdominal compartment syndrome. This case highlights the evolving clinical course of symptomatic PLD, the importance of longitudinal reassessment, and the potential severity of rare cyst-related complications.