Sclerosing peritonitis post liver transplantation: a rare condition where surgery is an important treatment option.

BACKGROUND: Sclerosing peritonitis (SP) is a rare but potentially fatal complication following orthotopic liver transplantation (OLT). The definitive surgical management is via a laparotomy peritonectomy and enterolysis procedure, but this carries risks particularly in the immunosuppressed transplant patient population. The natural history of SP is known from a handful of case reports and series, which mostly report de novo cases arising early on following OLT. The aim of this study was to identify all cases of de novo SP following OLT and the outcomes of management. METHODS: Cases of SP post OLT were identified from the Australian National Liver Transplantation Unit (New South Wales) database of all 1393 adult patients. RESULTS: Three cases of SP were diagnosed between 2 and over 9 years post-transplantation. Two patients proceeded to laparotomy and a peritonectomy and enterolysis procedure of the cocooned bowel. The third was managed conservatively due to a relatively indolent course and their medical co-morbidities. CONCLUSION: SP should be considered in the differential diagnosis in patients post OLT presenting with symptoms of bowel obstruction, even years following transplantation. Surgery may be required in order to achieve a satisfactory outcome in some cases.

Nature and outcomes of the increased incidence of colorectal malignancy after liver transplantation in Australasia.

OBJECTIVES: To examine whether incidence of colorectal malignancy is increased in Australasian liver transplant recipients compared with the general population of Australia, and to assess the characteristics and outcomes of colorectal malignancy in this patient group. DESIGN, SETTING AND PATIENTS: Data on patients who underwent orthotopic liver transplantation (OLTx) and had a diagnosis of de-novo colorectal malignancy after transplantation during the period 1985-2011 were obtained from the Australia and New Zealand Liver Transplant Registry, and these data were compared with colorectal malignancy data from the Australian Institute of Health and Welfare. MAIN OUTCOME MEASURES: Time from OLTx to diagnosis of colorectal malignancy, stage of colorectal malignancy at diagnosis, patient survival, and standardised incidence ratio (SIR) for colorectal malignancy. RESULTS: Forty-eight of 3735 recipients (1.3%) were diagnosed with colorectal malignancy at a median of 7.3 years after OLTx. More advanced colorectal malignancy (regional or metastatic disease) was evident at diagnosis in 20 of the 48 patients; these patients tended to be younger than patients with less advanced malignancy (P = 0.01) and diagnosed sooner after OLTx (P = 0.005). Despite treatment predominantly with surgery, 19 of the 48 patients died from the malignancy. The overall SIR for colorectal malignancy liver transplant recipients compared with the general population of Australia was 2.80 (95% CI, 2.06-3.71). CONCLUSIONS: The incidence of colorectal malignancy is increased in liver transplant recipients in comparison with the general population. Of concern is the tendency for advanced malignancy to be diagnosed in younger patients. These data highlight the importance of considering whether specific guidelines for colorectal malignancy screening in the Australasian adult liver transplant population are needed.

Initial outcomes of using allografts from donation after cardiac death donors for liver transplantation in New South Wales.

OBJECTIVES: To report the early outcomes of the initial selection and use of donation after cardiac death (DCD) donor livers for transplantation in New South Wales, following a guidelines implementation process. DESIGN AND SETTING: Review of database and medical records from the Australian National Liver Transplantation Unit and the NSW Organ and T2 Donation Service for DCD activity including organ donor offers and retrievals, from 1 July 2007 to 31 December 2010. MAIN OUTCOME MEASURES: Acceptance and utilisation rates of livers from DCD donors, and patient and graft outcomes after liver transplantation. RESULTS: Of the potential 84 DCD donor offers, 45 were declined, and 15 of the 39 attempted retrievals provided livers for transplantation. The most common reason for non-retrieval of the liver was the time to declaration of death exceeding 30 minutes after withdrawal of treatment (14 donors), followed by abnormality in the donor liver (eight donors). Data on early outcomes for liver transplant recipients showed a median peak aspartate aminotransferase of 3667 U/L (range, 919-11 264 U/L), but no delayed graft function. Four patients developed biliary complications (two within 3 months and two later). Patient and graft survival were 100% at a median follow-up of 15 months. CONCLUSIONS: As a result of the re-establishment of multiorgan donation through the DCD pathway, 15 (18%) of the selected DCD donors provided livers for transplantation. Patient and graft survival rates were excellent, and the rate of intra- and postoperative complications was acceptable. Hence, the selective transplantation of DCD donor liver allografts will continue to be pursued and the outcomes followed.

Grade of deceased donor liver macrovesicular steatosis impacts graft and recipient outcomes more than the Donor Risk Index.

BACKGROUND AND AIM: Donor liver steatosis can impact on liver allograft outcomes. The aim of the present study was to comprehensively report on the impact of type and grade of donor steatosis, as well as donor and recipient factors, including the reported Donor Risk Index (DRI), on liver allograft outcomes. METHODS: A review of unit data for all adult liver transplant procedures from 2001 to 2007, as well as donor offers. Donor liver biopsies were regraded for steatosis by an experienced histopathologist. RESULTS: Steatosis was detected in 184/255 (72%) of biopsies, of which 114 (62%) had microvesicular steatosis (MiS; 68 mild, 22 moderate, 24 severe) and 70 (38%) macrovesicular steatosis (MaS; 59 mild, 7 moderate, 4 severe). The majority (66/70, 94%) of biopsies with MaS also contained MiS. Allograft steatosis was associated with increasing donor body mass index (P = 0.000), plus donor male sex (P < 0.05). Primary non function (P = 0.002), early renal failure (P = 0.040), and requirement for retransplantation (P = 0.012) were associated only with severe MaS. Early biliary complications were associated with moderate MaS (P = 0.039). Only severe MaS was significantly associated with inferior allograft survival at 3 months (relative risk = 12.09 [8.75-19.05], P = 0.000) and 1 year (P = 0.000). CONCLUSIONS: MiS is a common finding and frequently coexists with MaS on liver allograft biopsy, while isolated MaS is uncommon. Only the presence of moderate to severe MaS is associated with inferior early allograft outcomes. The impact of severe MaS on allograft survival appears greater than other donor factors, including the calculated DRI.

Isolated liver transplantation in children with cystic fibrosis--an Australian experience.

CF liver disease is an uncommon indication for pediatric LT. Determining optimal timing and type (isolated liver versus multi-organ) of transplantation for those with severe liver disease can be challenging and involves consideration of the extent of liver disease (PHT, synthetic dysfunction) and extrahepatic factors such as pulmonary function. We present the experience of isolated LT for CF at our center. Eight children received one allograft each (3.9% of all grafts). One- and four-yr survivals are both 75%. The two deaths occurred within the first two months after LT, and in both cases, invasive fungal infections were implicated, one following treatment for acute severe rejection. All had significant PHT, and six had synthetic dysfunction. All had roux-en Y biliary anastomoses and none developed long-term biliary complications. Seven had pulmonary colonization with Pseudomonas aeruginosa and six with fungus at time of transplantation. Mean pre-LT FEV1 was 80% (range 59-116%) predicted, and lung function post-LT was stable. Isolated LT in children with CF is successful in those with relatively preserved pulmonary function, which does not appear to deteriorate as a consequence. Roux-en Y biliary anastomosis and antifungal prophylaxis should be a part of management of these patients.

Donor and recipient selection leads to good patient and graft outcomes for right lobe split transplantation versus whole graft liver transplantation in adult recipients.

The outcomes of right lobe split (RLS) liver transplantation are variable in adult recipients. This report is an analysis of outcomes of our initial 5-year experience with the right lobe trisegment split graft. A retrospective analysis was performed of the recipient and graft outcomes from July 2002 to March 2007 of all adult recipients of RLS grafts versus recipients of whole grafts (WGs). All data were analyzed with Stata version 8 (Stata Corp., Texas). There were 43 (19.1%) RLS recipients and 182 (80.9%) WG recipients. The median Model for End-Stage Liver Disease score was 13 (7-23) in the RLS group and 18 (6-50) in the WG group (P < 0.001). Hepatocellular carcinoma and primary sclerosing cholangitis were more common in the RLS group (P < 0.05), whereas alcoholic cirrhosis and chronic hepatitis C were more common in the WG group. The median donor age was lower in the RLS group at 39 (13-61) years versus the WG group at 47 (12-79) years (P < 0.001). Primary nonfunction occurred in 1.6% of the WG patients only. Biliary complications occurred in 28% of the RLS patients versus 28% of the WG patients. Vascular complications occurred in 18% of the RLS patients versus 14% of the WG patients. The retransplantation rate was similar at 2.3% in the RLS group versus 4.9% in the WG group (P = not significant). Overall 3-year recipient survival was 92.7% in the RLS group versus 82.7% in the WG group (P = 0.284). Graft survival was 88.4% in the RLS group at 3 years versus 78.5% in the WG group (P = 0.304). In conclusion, good outcomes can be achieved with RLS liver transplantation in adult recipients without a detrimental effect on recipient or graft survival.

Poorer survival in patients whose explanted hepatocellular carcinoma (HCC) exceeds Milan or UCSF Criteria. An analysis of liver transplantation in HCC in Australia and New Zealand.

BACKGROUND: Milan and University of California San Francisco (UCSF) Criteria have been used for selection of patients with hepatocellular carcinoma (HCC) for liver transplantation (LTx). The aims of this study were to analyse the results of LTx for HCC in Australia and New Zealand with emphasis on the effects of discordance between pre-LTx radiological and post-LTx pathological staging. METHODS: A total of 186 LTx for HCC carried out between July 1985 and August 2003 were included. Patients were categorized according to the Milan and UCSF Criteria. RESULTS: The median follow-up was 6.55 years (range 2.96-20.93 years). Pre-LTx factors associated with better survival include tumour size < or = 5 cm, number of tumours < or = 3, staging within Milan and UCSF Criteria and more recent transplantation (1996-2003). In all, 14 patients had a pre-LTx stage outside the Milan but within the UCSF Criteria. One- and 5-year patient survival rates were, respectively, 88% and 74% within the Milan Criteria, and 87% and 73% within the UCSF Criteria. Vascular invasion, capsular invasion, lymph node invasion and pathological stage outside UCSF Criteria were associated with poor outcome. Of patients within the Milan and UCSF Criteria pre-LTx, 24% and 18%, respectively, were outside the same criteria post-LTx. These patients had poorer survival rates. CONCLUSIONS: The use of the UCSF Criteria in this cohort increased the number of patients eligible for LTx without compromising 5-year survival rates. Patients whose explant tumours were outside the Milan or UCSF Criteria had poorer outcomes compared with those whose explants remained within these criteria.

Early high peak hepatitis C viral load levels independently predict hepatitis C-related liver failure post-liver transplantation.

The aim of this study was to examine the importance of the serum hepatitis C viral load within the first year post-liver transplant in determining posttransplant survival. A retrospective analysis of 118 consecutive hepatitis C virus-positive liver transplant recipients who received an allograft from January 1997 to September 2005 was undertaken with a median duration of follow-up of 32.4 months. Univariate and multivariate analyses were used to examine the effects of recipient, donor, surgical, and viral factors on posttransplant outcomes. A total of 620 viral load estimations were undertaken in the first 12 months following transplantation. Patient and graft survival rates at 1, 3, and 5 years were 87.8%, 79.9%, and 70.1% and 87.0%, 79.2%, and 68.2%, respectively. According to multivariate analysis, a peak viral load > or = 10(7) IU/mL (P = 0.004; hazard ratio, 8.68; 95% confidence interval, 2.04-37.02) and exposure to antirejection therapy (P = 0.05; hazard ratio, 2.26; 95% confidence interval, 1.01-5.38) were both independent predictors of diminished patient and graft survival and hepatitis C-related allograft failure. The only other independent predictor of hepatitis C virus-related outcome after transplant was azathioprine use, which was associated with improved outcomes (P = 0.04; hazard ratio, 0.25; 95% confidence interval, 0.07-0.91). A peak viral load in the first year after transplant of >10(8), 10(7) to 10(8), and <10(7) IU/mL was associated with a mean survival of 11.8, 70.6, and 89.1 months respectively (P < or = 0.03). The results emphasize the importance of high viral loads in the early posttransplant period as an independent predictor of recipient outcomes.

Cadaveric liver transplant from older donors.

OBJECTIVE: To examine the effects of cadaveric donor age on outcomes following orthotopic liver transplantation OLT. METHODS: Data were collected on all patients who underwent OLT between January 1997 and December 2004 at the Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. During this period, 313 OLTs were performed: 51 patients 16% received older donor livers OD; 60 or more years old, and 262 84% received younger donor livers YD; less than 60 years old. RESULTS: In the study group 313 patients, we found significantly more recipients of OD liver with blood group O: 51% versus 33% p=0.025 and with fulminant hepatic failure: 9.8% versus 5% p=0.018 compared to YD recipients. No difference between OD and YD liver recipients was found in initial poor graft function: 16/51 31% versus 74/262 28%, primary non-functioning: 6.5% versus 6.5%, the overall graft loss: 15/51 29% versus 62/262 24%, post-revascularization liver biopsy steatosis: 14/40 35% versus 82/232 36% or hepatic artery thrombosis: 1/51 2% versus 8/262 3%. There was no difference in graft actuarial survival between OD and YD recipients at 1, 3, and 5 years, 82% versus 87%, 75% versus 81%, and 75% versus 77% p=0.27 log rank or patient actuarial survival, 86% versus 89%, 79% versus 83%, and 79% versus 80% p=0.336 log rank. CONCLUSION: Orthotopic liver transplantation can be achieved with acceptable outcomes using selected livers from older deceased donors.

Outcome of patients with hepatocellular carcinoma referred to a tertiary centre with availability of multiple treatment options including cadaveric liver transplantation.

UNLABELLED: Hepatocellular carcinoma (HCC) is a primary cancer of the liver with an established causal link to viral hepatitis and other forms of chronic liver disease. AIMS: The aim of this study was to analyse the determinants of outcome in patients with HCC referred to a tertiary centre for management. METHOD: Two hundred and thirty-five prospective patients with HCC and minimum 12-month follow-up were studied. RESULTS: The cohort was heterogeneous, with 52% Caucasian, 40% Asian and 5% of Middle-Eastern origin. Independent predictors of outcome included tumour size and number, the presence of ascites or portal vein thrombosis, alpha-foetoprotein >50 U/L and an impaired performance status. Treatment was determined on an individual case basis by a multidisciplinary tumour team. Surgical resection was primary treatment in 43 patients, liver transplantation in 40 patients, local ablation (percutaneous radiofrequency ablation or alcohol injection) in 33 patients, transarterial chemoembolisation in 33 patients, chemotherapy or other systemic therapy in 30 patients and no treatment in 56 patients. After adjustment for significant covariates, both liver transplantation (P<0.001) and surgical resection (P=0.029) had a significant effect on patient survival compared with no treatment, but local ablation (P=0.410) and chemoembolisation (P=0.831) did not. Liver transplantation resulted in superior overall and, in particular, disease-free survival compared with surgical resection (disease-free survival 84 vs 15% at 5 years). CONCLUSION: In conclusion, both surgical resection and liver transplantation significantly improve the survival of patients with HCC, but improvements need to be made to the delivery of loco-regional therapy to enhance its effectiveness.

Hereditary lysozyme amyloidosis: spontaneous hepatic rupture (15 years apart) in mother and daughter. role of emergency liver transplantation.

Hepatic rupture is a rare condition, and treatment options are very limited. We report a case of hepatic rupture secondary to hereditary lysozyme amyloidosis that was successfully treated by liver transplantation. The mother of this patient had presented in an identical fashion 15 years earlier in the pretransplant era and died very rapidly.