Polycystic liver disease: a clinical review.

Polycystic liver disease rarely occurs in isolation as part of autosomal dominant polycystic liver disease, but more commonly, it exists as an extra-renal manifestation of autosomal dominant polycystic kidney disease. The pathogenesis of polycystic liver disease involves defects in the primary cilium of the cholangiocyte, with genetic mutations that impair key proteins integral to the complex functioning of cilia. While most patients are asymptomatic and require no intervention aside from reassurance and genetic counseling, in a minority of patients, polycystic liver disease creates a myriad of symptoms from the compressive effects of enlarged cysts, and can even cause malnutrition and liver decompensation in the severest of cases. In patients with symptomatic disease, a variety of interventional radiology or surgical techniques can be considered, including aspiration with sclerotherapy of a dominant cyst, fenestration, segmental hepatic resection, and even liver transplantation. Although there are no curative medical options for polycystic liver disease, somatostatin analogs hold promise and have shown minimal efficacy in human studies. However, further research is needed to develop more efficacious medical treatments.

Massive avascular malformations causing life threatening portal hypertension.

 We present an unusual case of extensive avascular malformations (AVMs) causing non-cirrhotic portal hypertension. This phenomenon, though previously described, is a rare clinical entity which, in the setting of life threatening portal hypertension, may require vascular decompression either by surgery or a transjugular intrahepatic portosystemic shunt.

An unusual cause of cancer mimicry following liver transplantation.

Sirolimus is an approved anti-rejection agent following liver or kidney transplantation that works through inhibition of the mammalian target of rapamycin (mTOR). As sirolimus functions through a pathway independent of calcineurin inhibition, it may have less potential for nephrotoxicity and carcinogenesis. That being said, there are a myriad of potential adverse effects reported with sirolimus, many of which are severe and unknown or poorly understood. Herein we present a case of sirolimus causing a serious but uncommon adverse event in an adult liver transplant recipient; the adverse event in this instance unfortunately resulted in significant medical testing and morbidity. The adverse event profile of sirolimus is summarized through review of available evidence.

Do older patients utilize excess health care resources after liver transplantation?

INTRODUCTION: Liver transplantation is a highly effective treatment for end-stage liver disease. However, there is debate over the practice of liver transplantation in older recipients (age ≥ 60 years) given the relative shortage of donor grafts, worse post-transplantation survival, and concern that that older patients may utilize excess resources postoperatively, thus threatening the economic feasibility of the procedure. AIM: To determine if patients ≥ 60 years of age utilize more health resources following liver transplantation compared with younger patients. MATERIAL AND METHODS: Consecutive adult patients who underwent primary liver transplantation (n = 208) at a single center were studied over a 2.5-year period. Data were collected on clinico-demographic characteristics and resource utilization. Descriptive statistics, including means, standard deviations, or frequencies were obtained for baseline variables. Patients were stratified into 2 groups: age ≥ 60 years (n = 51) and < 60 years (n = 157). The Chi-Square Test, Mantel-Haenszel Test, 2-sample test and odds ratios were calculated to ascertain associations between age and resource utilization parameters. Regression analyses were adjusted for model for end-stage liver disease score, location before surgery, diabetes mellitus, donor age, cold ischemia time, albumin, and diagnosis of hepatitis C. RESULTS: Recipients ≥ 60 years of age have similar lengths of hospitalization, re-operative rates, need for consultative services and readmission rates following liver transplantation, but have longer lengths of stay in the intensive care (hazard ratio 1.97, p = 0.03). CONCLUSION: Overall, liver transplant recipients ≥ 60 years of age utilize comparable resources following LT vs. younger recipients. Our findings have implications on cost-containment policies for liver transplantation.

Clinical outcomes of liver transplantation for polycystic liver disease: a single center experience.

Polycystic liver disease (PLD) is a celiopathy characterized by progressive growth of multiple hepatic cysts. In a minority of patients, severe symptomatic hepatomegaly necessitates liver transplantation (LT). The purpose of this study is to describe the postoperative and long-term outcomes of all patients transplanted for PLD at our center. All patients who underwent LT for PLD were identified through our database. Using patient charts, data were extracted on patient demographics and medical history, postoperative surgical and medical complications, length of hospitalization, prevalence of chronic kidney failure, and patient and graft survival. Subjects were contacted in April 2010 to verify their survival and confirm their need, if any, for hemodialysis and/or kidney transplantation. Descriptive statistics for patient and graft survival were performed. From 1993 to 2010, 14 subjects underwent LT and 1 subject underwent combined kidney and LT; all subjects were female and the mean age was 49.0 years. 10 (66.7%) subjects had polycystic kidney disease. Patients experienced a high rate of vascular complications, including hepatic artery thrombosis (HAT) or stenosis in 3 (20%) and 2 (13.3%) subjects, respectively. One subject had early graft loss due to HAT and underwent re-transplantation. The mean length of hospitalization was 18.8 days. After a mean of 66.8 months of follow-up (3-200), 13 (86.7%) subjects are alive with satisfactory graft function, and no patients had renal failure. In conclusion, patients who underwent LT for PLD had a high rate of postoperative vascular complications. However, long-term patient and graft survival, and kidney function, is excellent.