Liver transplantation for adult polycystic liver disease

Abstract

Polycystic liver disease, commonly associated with polycystic kidney disease, can result in massive hepatomegaly and debilitating symptoms. Surgical intervention for symptomatic polycystic liver disease has been associated with significant morbidity and inconsistent long-term palliation; it is more appropriate in patients with a single dominant cyst or cysts which is/are confined to one lobe. At our institution, nine patients have undergone orthotopic liver transplantation for symptomatic hepatic cysts with excellent long-term results and minimal morbidity and mortality. Surgical candidates were selected based on severe limitations in daily activities and on sequelae of hepatic cystic involvement. Other factors considered were the extent and pattern of hepatic cystic disease, the degree of hepatic and renal dysfunction, and prior surgical intervention. Three patients (33%) required combined liver and kidney transplantation because of renal cystic involvement with renal insufficiency. The one-year survival rate was 89% with excellent symptomatic relief and improved quality of life in all the surviving patients. One death occurred in a significantly malnourished 62-year-old female. Complications included one case each of hepatic artery thrombosis requiring retransplantation, biliary leak necessitating biliary reconstruction, and postoperative bleeding requiring re-exploration. The mean hospital stay was 23 days and the mean intraoperative blood transfusion requirement was 18 units. Our experience demonstrates that appropriately selected patients with extensive hepatic involvement with adult polycystic liver disease can have an excellent outcome with transplantation, with morbidity comparable with other surgical options. (HEPATOLOGY 1998;28:412-415.)

Results of combined and sequential liver-kidney transplantation

“…Ideal candidates for combined liver-kidney transplantation are those patients with end-stage liver disease in association with irreversible renal disease. Therefore, acquired viral hepatitis in dialysis patients 10,33 and hereditary disorders such as polycystic liver-kidney disease [34][35][36][37] currently represent the most frequent indications for such a procedure. 18 Opinions differ regarding primary hyperoxaluria type 1 (PH1).…”

Development and validation of a polycystic liver disease complaint-specific assessment (POLCA)

“…Using search terms referring to the disease, its treatment forms and aspects of health-related quality of life, 15 articles were identified for data extraction [4,5,7,8,[17][18][19][20][21][22][23][24][25][26][27].…”

Surgical management of polycystic liver disease

“…Although a majority of PCLD patients have normal liver function, orthotopic and living donor liver transplantation have been successfully utilized in the treatment of symptomatic PCLD [53][54][55][56][57][58][59][60][61][62][63][64] . Aspiration, fenestration, or surgical resection can provide adequate palliation to those patients with large single cysts or dominant disease in one lobe, but the treatment of small, truly diffuse, cystic type PCLD may well require transplantation.…”

“…Indications for transplantation include cachexia, weight loss, recurrent cyst infections, portal hypertension, and ascites. Early reports have proposed these patients not wait until endstage complications of their PCLD become manifest before offering the option of transplantation [56,58,59] . Transplantation in those with end-stage PCLD, exhibited by severe disability, weakness, and malnutrition, has been shown to have higher infection-related mortality in early liver transplant series [56,59] .…”

“…Performing earlier transplantation in appropriate candidates would seem to offer a g reater chance of improved outcomes, meaningful recovery, and return to their prior functional status and quality of life. Furthermore, patients who have undergone prior more conservative therapies (aspiration, sclerosis, fenestration, or resection) may have postsurgical changes that make transplantation much more difficult [53,58,59] . T h e o p t i o n o f t r a n s p l a n t a t i o n s h o u l d b e balanced ag ainst the risks of surg er y, long-ter m immunosuppression, and the need for concur rent or subsequent renal transplantation in those with ADPKD.…”