P008: CLINICAL OUTCOMES OF LIVER TRANSPLANTATION FOR POLYCYSTIC DISEASE: EVIDENCE FROM A SINGLE-CENTER SERIES
Mariana P Acosta, MD; Nicolas P Caram, MD; Daniel Schmidt, MD; Marianfeli Landino, MD; Behrouz Ashrafi, MD; Shatz Vadim, MD; Fouad Souki, MD; Ramona Nicolau-Raducu, MD, PhD
Jackson Memorial Hospital/University of Miami
Background: Polycystic liver disease (PLD) is an inherited disorder marked by progressive hepatic cyst growth. While liver function is usually preserved, severe cyst burden can cause mass effect complications, sometimes requiring liver transplantation (LT). Perioperative challenges include surgical constraints, significant bleeding, thrombosis risk, and difficult extubation due to airway compression and atelectasis.
Methods: A retrospective study was conducted using the LT database from the Liver Transplant Unit at Jackson Memorial Hospital, University of Miami, USA. Data were extracted for patients who underwent LT between January 2016 and December 2024, with follow-up until February 2025. Kaplan-Meier log-rank curves were calculated.
Results: Among 1,077 LT recipients, 18 patients (1.6%) had PLD and met the inclusion criteria. Of these, 12 (67%) were female. The median physiologic Model for End-Stage Liver Disease (MELD-Na) score at transplantation was 17 (IQR: 7–19), and the mean age at transplantation was 60 years (SD 6). All patients had concurrent autosomal dominant polycystic kidney disease (ADPKD). The median preoperative creatinine was 3.87 mg/dL (IQR: 1.31-6.67), and 8 patients (44%) required hemodialysis prior to LT.
Regarding kidney transplantation, 1 patient was excluded due to intraoperative mortality; Among the remaining 17, 4 (23%) had undergone kidney transplantation before LT, 2 (12%) received a simultaneous liver-kidney transplant, 11 (65%) underwent kidney transplant in the days following LT.
Vena cava preservation (piggyback technique) was used in 83% of cases. The median operative time was 4.81 hours (range: 2.35–9.57), and the mean liver explant weight was 4,623 g (range:562–9,954). Estimated blood loss was 2.5 L (IQR: 1.37–12.25), with massive transfusion (≥10 units of PRBCs within 24 hours) required in 50% of cases. Prophylactic heparin of 30 units/kg was administered prior to portal clamping in 78% of patients. Intraoperative cardiac thrombosis occurred in 5 patients (28%) with 4 out of 5 not receiving prophylactic heparin. The events occurred during dissection (1), anhepatic (1) and after portal reperfusion (3). The one patient who received prophylactic heparin, experienced massive hemorrhage due to surgical complexity immediately post reperfusion, intraoperative cardiac arrest, and required AV-ECMO, resulting in intraoperative death. The median post-transplant extubation time was 21 hours (IQR: 14–58), with reintubation required in 3 patients (17%). The median intensive care unit (ICU) stay was 10 days (range: 3–227), while the median hospital stay was 14 days (range: 6–231). No patient required retransplantation. Kaplan Meier 1-year survival was 83%, while overall patient and graft survival was 68%.
Conclusions: Simultaneous liver and kidney transplantation (SLKT) is a definitive treatment for ADPKD with severe polycystic liver disease, addressing organ failure and hepatomegaly complications. Despite surgical complexity and perioperative risks, survival outcomes remain favorable, emphasizing the need for meticulous management, patient selection, and tailored surgical approaches.
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