0041-1337/98/6604-529$03.00/0 TRANSPLANTATION Vol. 66, 529 –544, No. 4, August 27, 1998 Copyright © 1998 by Williams & Wilkins Printed in U.S.A. Transplantation  BRIEF COMMUNICATIONS LIVER AND KIDNEY TRANSPLANTATION FOR POLYCYSTIC DISEASE D. ROHAN JEYARAJAH,THOMAS A. GONWA, 1 GIULIANO TESTA,OSMAN ABBASOGLU, ROBERT GOLDSTEIN,BO S. HUSBERG,MARLON F. LEVY, AND GORAN B. KLINTMALM Baylor Institute of Transplant Sciences, Baylor University Medical Center, Dallas, Texas 75246 Background. With the poor results of resective and fenestration procedures for polycystic liver disease (PCLD), we present the first series of patients receiv- ing orthotopic liver transplantation for this condition. Methods. Five of our six patients with PCLD had polycystic kidney disease also. Three of these five re- ceived combined organ transplants, while the other two required subsequent kidney transplants. Results. Forty-eight and 52 months after orthotopic liver transplantation, all surviving patients had relief of their pain, distention, and anorexia. Two patients had succumbed to infectious complications and died at 15 and 24 months after transplant. Conclusions. We conclude that patients with PCLD can be transplanted safely for the relief of their dis- tention and anorexia, with good results. Those pa- tients with both PCLD and polycystic kidney disease who are not dialysis dependent can be managed for several years with isolated liver transplantation and then receive kidney transplantation if needed. Those who are dialysis dependent should receive combined liver-kidney transplantation. Unfortunately, patients with polycystic disease seem to be very susceptible to infectious complications after organ transplantation. Adult polycystic liver disease (PCLD*) can cause massive hepatomegaly leading to incapacitating pain, caval obstruc- tion, and hemorrhage (1–8). Many surgical techniques have been attempted in these patients, including cyst fenestration and resective procedures, but these have met with limited success (9 –11). With the improved results of organ trans- plantation, this may become a modality for definitive treat- ment of patients with PCLD. In addition, because many of these patients have polycystic kidney disease (PCKD) and renal insufficiency, combined liver-kidney transplantation (LKTx) can provide treatment for renal failure as well as liver pathology. Only two reports of liver transplantation for PCLD have been published. Kwok et al. (4) have reported a case of a 51-year-old female who had massive organomegaly from PCLD and PCKD. Her indication for liver transplantation (LTx) was intractable pain, and the indication for renal transplantation (KTx) was end-stage renal disease secondary to PCKD. The patient died intraoperatively due to massive hemorrhage. Starzl et al. have reported four patients with PCLD who received LTx (12). Two patients received com- bined LKTx, even though their renal function was adequate at the time of transplantation. The rationale for concomitant renal allografting was the fear that renal function would deteriorate with nephrotoxic immunosuppression. In addi- tion, the authors suggest that the liver allograft may protect the kidney from rejection, a view that has been shared by many (13). Three of the patients were doing well at the time of publication of this report. Between November 1987 and June 1994, we performed six LTx for the diagnosis of PCLD at Baylor University Medical Center. Three of these patients had concomitant KTx, whereas a further two patients required subsequent KTx. Patient characteristics are summarized in Tables 1 and 2 and presented below. Patient 1 was a 50-year-old female who had a long history of PCLD and PCKD. She presented with symptoms of in- creased abdominal girth, pain, inability to eat, and an epi- sode of hemorrhage into a liver cyst in the past. She had undergone three drainage procedures for both liver and renal cysts, which had provided temporary relief of her symptoms. Due to recurrent pain and anorexia from a liver that was palpable 20 cm below the right costal margin, she was re- ferred for transplantation. Her renal function was adequate at the time of evaluation, with a glomerular filtration rate of 1 Address correspondence to: Thomas A. Gonwa, MD, Baylor In- stitute of Transplant Sciences, Baylor University Medical Center, 3500 Gaston Avenue, Dallas, TX 75246. * Abbreviations: ACR, acute cellular rejection; CAH-B, chronic active hepatitis B; KTx, kidney transplant; LKTx, liver-kidney trans- plant; LTx, liver transplant; OLTx, orthotopic liver transplant; PCD, polycystic disease; PCKD, polycystic kidney disease; PCLD, polycys- tic liver disease. TABLE 1. Patient description Patient Gender Age (yr) Explant weight (g) Operation performed 1 F 50 4,510 LKTx 2 F 36 10,540 LTx/KTx a 3 M 48 11,560 LKTx 4 F 38 9,979 LTx/KTx 5 F 60 3,230 LKTx 6 F 48 2,700 LTx a LTx/KTx, liver transplant followed by kidney transplant. 529