Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study.

Patients with end-stage isolated polycystic liver disease (PCLD) suffer from incapacitating symptoms because of very large liver volumes. Liver transplantation (LT) is the only curative option. This study assesses the feasibility of LT in PCLD. We used the European Liver Transplant Registry (ELTR) database to extract demographics and outcomes of 58 PCLD patients. We used Kaplan-Meier survival analysis for survival rates. Severe abdominal pain (75%) was the most prominent symptom, while portal hypertension (35%) was the most common complication in PCLD. The explantation of the polycystic liver was extremely difficult in 38% of patients, because of presence of adhesions from prior therapy (17%). Karnofsky score following LT was 90%. The 1- and 5-year graft survival rate was 94.3% and 87.5%, while patient survival rate was 94.8% and 92.3%, respectively. Survival rates after LT for PCLD are good.

[Mega-duodenum and constipation after surgery for congenital atresia of the jejunum]. / Megaduodenum og obstipation efter operation for kongenit tyndtarmsatresi.

A 33 year-old female presented with constipation and a right-sided palpable abdominal mass, suspected to be the colon. The colonic transit time was prolonged, but the dilated organ was a mega duodenum that had developed after surgery for three jejunal atresies performed one day after birth. The colon was malrotated, being situated to the left of the columna. Renewed resection surgery was uneventful, and after such surgery the constipation receded and the colonic transit time returned to normal.

[Liver surgery in Denmark 2002-2007]. / Leverresektioner i Danmark 2002-2007.

INTRODUCTION: We evaluated the organisation, management and outcome for patients undergoing elective liver resection in Denmark in the period 2002-2007. MATERIAL AND METHODS: Nationwide data based on the National Patient Registry and discharge information from hospital departments in the period 1 January 2002 to 31 December 2007 were analysed. RESULTS: Twenty-three departments performed 818 resections with five departments performing 96% and 18 departments performing 4% of the operations. The amount of non-anatomical resections constituted 30% (248 of 818) of the resections. The median postoperative stay was nine days, and the hospital mortality rate was 3.9%, distributed between 2.4% for non-anatomical resections, 2.9% for segmental resections and 5.2% for right-sided hepatectomy. CONCLUSION: The number of treated patients was too small as was the number referred to highly specialised liver surgery units. Moreover, the amount of non-anatomical resections was too high as was the average postoperative stay and the hospital mortality rate. In future, we propose that liver resections be centralised in 2-3 hospitals each capable of providing all the following services: surgery, hepatology, oncology and interventional radiology.

Liver transplantation in polycystic liver disease: a relevant treatment modality for adults?

OBJECTIVE: Polycystic liver disease (PLD) is a rare, hereditary, benign disorder. Hepatic failure is uncommon and symptoms are caused by mass effects leading to abdominal distension and pain. Liver transplantation (LTX) offers fully curative treatment, but there is still some controversy about whether it is a relevant modality considering the absence of liver failure, relative organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD and to compare the survival with the overall survival of patients who underwent LTX from 1992 to 2005. MATERIAL AND METHODS: A retrospective study of the journals of 440 patients, who underwent 506 LTXs between 1992 and 2005, showed that 14 patients underwent LTX for PLD. All patients had normal liver function. Three were receiving haemodialysis and thus underwent combined liver/kidney transplantation. One patient had undergone kidney transplantation 10 years earlier. RESULTS: Median follow-up was 55 months. One patient who underwent combined transplantation died after 5.4 months because of multiorgan failure after re-LTX, and one patient, with well-functioning grafts, died of lymphoma after 7 months. At present 12 patients are alive, relieved of symptoms and with good graft function. CONCLUSIONS: We conclude that patients treated for PLD by LTX have a good long-term prognosis and excellent relief of symptoms and that LTX might be considered in severe cases of PLD, where conventional surgery is not an option.

[Liver resection over the last decade]. / Leverresektioner gennem ti år.

AIMS: The results after liver resection have improved over the last decade with an operative mortality rate of less than 5% in high-volume centres. The aim of the present study was to assess the perioperative outcome after hepatic resection and to assess the long-term survival after liver resection of hepatic metastases from colorectal cancer and hepatocellular carcinoma in our institution. MATERIALS AND METHODS: The patients who underwent their primary liver resection from 1.1.1995-31.12.2004 in our institution were included. The surgical outcome was reviewed retrospectively and the five-year survival after resection of hepatic metastases from colorectal cancer and hepatocellular carcinoma was estimated. RESULTS: 141 patients (71M/70F), median age 58 years (1-78), underwent a liver resection in the ten-year period. The number of resections increased from two in 1995 to 32 in 2004. Median hospital stay was 9 days (3-38). The most frequent complication was biliary leakage (7.8%), haemorrhage (2.8%) and hepatic insufficiency (2.8%). 30-days mortality was 1.4%. The actuarial 5-survival after hepatic resection for colorectal liver metastases and hepatocellular carcinoma was 39% and 42%, respectively. CONCLUSION: The morbidity and mortality rate after hepatic resection and the long-term survival for patients undergoing resection for hepatic metastases from colorectal cancer and hepatocellular carcinoma in our institution are comparable with the best high-volume centres.

[Cysts of the liver]. / Levercyster.

Cysts of the liver are discovered in connection with a scope of diseases ranging from simple, infectious, or parasitic to neoplastic cysts. Symptoms, paraclinical, radiological and diagnostic characteristics are described with emphasis on ruling out malignancy. The treatment options from ultrasound guided drainage to resections and liver transplantation are discussed. It is concluded that up to 25% of cysts must be treated surgically, because recurrence after percutaneous or laparoscopic treatment is between 5% and 71%, and only resection or liver transplantation are curative.

[Surgical treatment of children with hepatic tumours]. / Kirurgisk behandling af levertumorer hos børn.

INTRODUCTION: In this paper we review the results of surgical treatment of children with hepatic tumours. MATERIALS AND METHODS: The study comprises 33 children who have undergone lever resection or liver transplantation since 1990. 26 patients had hepatoblastoma, 3 had hepatocellular carcinoma, 2 had rhabdomyosarcoma, 1 had a mesenchymal tumour, and 1 had a giant haemangioma. RESULTS: Because of the number of patients, we only analyzed the results of the treatment in the hepatoblastoma group. The survival was the same after resection (77.3%) and liver transplantation (75%). There was no difference in survival dependent on the type of resection, and there was no impact of the extension of tumour growth at the time of diagnosis. CONCLUSION: The combination of neoadjuvant chemotherapy followed by liver resection or liver transplantation is the treatment of choice in all children with hepatoblastoma. The results have improved dramatically over the last decades. The results in Denmark compare well with international results. Since 2000, very effective chemotherapy has downstaged all referred patients, so subsequent liver resection have been possible.

Retransplantation of the liver in adults: long-term outcome and prognostic modeling.

BACKGROUND/AIMS: The purpose of this study was to assess the outcome of liver retransplantation in adults at our institution and identify the subset of patients in which the outcome was too poor to justify retransplantation. METHODOLOGY: Over a 12-year period, we performed 346 liver transplantations in 305 adult patients, and of these, 41 (11.8%) were retransplantations. Survival data were stratified and multivariate analysis was conducted to identify variables associated with poor outcome after retransplantation. RESULTS: The 90-day, 1-year, and 5-year survival rates after liver retransplantation were 65.9%, 62.6%, and 48.2%, respectively. These rates were significantly inferior compared to those following single liver transplantation. Major adverse events affecting patient survival after retransplantation occurred within the first 90 days after surgery. Three independent prognostic variables of patient survival after retransplantation were identified: preoperative coagulation factor, total bilirubin, and the need for preoperative dialysis. Using these variables, we defined a simplified mathematical model available at the time of decision of retransplantation. CONCLUSIONS: The present study indicated three important prognostic factors associated with a poor outcome after retransplantation. Based on our newly developed scoring-system estimating patient survival, we suggest that retransplantation must be indicated before the deterioration of more than two organs. These findings should assist in the decision process for liver retransplantation in adult patients.

Long-term graft outcome of pediatric liver transplantation in Copenhagen: analysis of the first 51 cases.

BACKGROUND: Graft loss after liver transplantation remains a significant problem, especially in pediatric patients. The aim of this study was to assess our initial series of pediatric liver transplantation and to identify the risk factors that influence graft outcome. METHODS: The first 51 transplantations were analyzed retrospectively. All transplantations were stratified into three groups according to graft type (full-size, reduced-size, and living-related-donor graft). Survival data of the grafts were stratified and multivariate analysis conducted with respect to preoperative and surgical factors. RESULTS: Seventeen of all the transplants were full-size grafts and 34 technical-variant grafts (27 reduced-size grafts from cadavers and 7 living-related-donor grafts). The overall graft survival rates were 65, 62 and 53% at 1, 3 and 5 years, respectively. Twenty-three of 51 grafts (45%) were lost. Poor status of the recipients (hospitalization or intensive care unit care before surgery), a retransplanted graft, and a reduced-size graft were independent risk factors for graft failure. With experience, overall graft survival has improved significantly and the differences in graft survival between graft types have disappeared. CONCLUSIONS: To improve graft survival after pediatric liver transplantation, the timely referral of potential recipients to the transplant team and employing a meticulous technique during the operation, particularly for the technical-variant graft, are required.

[Extrapancreatic tumour-induced hypoglycaemia]. / Ekstrapankreatisk tumorinduceret hypoglykaemi.

A 53-year-old non-diabetic man was admitted with hypoglycaemia, neuroglycopenic symptoms and acromegaloid facial swelling. Serum insulin concentration was suppressed, but the free concentration of insulin-like growth factor (IGF-II) was markedly elevated. CT scan demonstrated a large tumour in the liver. The histology showed a benign, solid, fibrous tumour. A hemihepatectomy was performed, and a 3.6 kg tumour was removed. Postoperatively, the blood glucose concentration and the concentration of free IGF-II returned to normal and the acromegaloid facial features disappeared.

Liver transplantation for Budd-Chiari syndrome: A European study on 248 patients from 51 centres.

BACKGROUND/AIMS: The results of liver transplantation for Budd-Chiari syndrome (BCS) are poorly known and the role and timing of the procedure are still controversial. The aim of this study was to investigate the results of transplantation for BCS, focusing on overall outcome, on prognostic factors and on the impact of the underlying disease. METHODS: An enquiry on 248 patients representing 84% of the patients transplanted for BCS in the European Liver Transplantation Registry between 1988 and 1999. RESULTS: Of the 248 patients, 70.4% were female and 29.6% male. The mean age was 35.7 years. The overall actuarial survival was 76% at 1 year, 71% at 5 years and 68% at 10 years. 77% of deaths occurred in the first 3 months: 47% were due to infection and multiple organ failure, and 18% to graft failure or hepatic artery thrombosis. Late mortality (>1 year) occurred in nine patients, due to BCS recurrence in four of them. The only pre-transplant predictors of mortality on multivariate analysis (Cox) were impaired renal function and a history of a shunt. CONCLUSIONS: Liver transplantation for BCS is an effective treatment, irrespective of the underlying cause, and should be considered before renal failure occurs.

Obesity increases mortality in liver transplantation--the Danish experience.

Obesity is increasing in the western world at an epidemic rate. The USA results in obese patients undergoing orthotopic liver transplantation (OLT) are divergent, and so far no European experience has been reported. This study was designed to determine if obesity is a risk factor for mortality and morbidity in OLT in a medium-size European center. In a retrospective study of the records of 365 consecutive patients who had undergone OLT from 1990 to 2003, 20 obese patients [body mass index (BMI) > 30 kg/m2] were identified. Their data were compared with those of the nonobese (BMI < 30 kg/m2) patients operated immediately before. There were no differences in demographic data, diagnosis leading to OLT, United Network of Organ Sharing (UNOS) classification, Child-Pugh score, or preoperative morbidity. The groups were also comparable concerning donor data, duration of operation, use of blood products, intensive care unit (ICU), or hospital admission. Mortality was, however, significantly increased in the obese group (P = 0.01). Our study clearly demonstrates an increased mortality in obese patients undergoing OLT and the relative scarcity of organs taken into account, it seems reasonable to consider obesity as a relative contraindication to OLT.

Idiopathic extensive peliosis hepatis treated with liver transplantation.

A 50-year-old Danish man, who neither had wasting disease nor was taking steroid-containing drugs, complained of abdominal distension, due to a markedly enlarged liver. Percutaneous needle biopsies were taken from the liver, and the findings gave suspicion of a neoplastic tumor. Because of reduced liver function and treatment-resistant ascites, he underwent liver transplantation without a definite preoperative diagnosis. The resected liver weighed 2900 g, and almost all of the parenchyma was destroyed and replaced by multicystic blood-filled spaces, diagnosed as extensive peliosis hepatis complicating liver cirrhosis. Extensive peliosis with liver cirrhosis is a rare condition. Only two cases, caused by contraceptives and treated by liver transplantation, are reported in the English-language literature. We could find no cause other than alcohol abuse lasting several years in this patient, and classified the present case as idiopathic extensive peliosis hepatis. Although scarce subjective findings and misleading liver biopsies made an exact diagnosis difficult, an orthotopic liver transplantation was the only treatment for such complicated peliosis hepatis.

Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy.

BACKGROUND/AIMS: Hepatobiliary malignancies are frequently seen in primary sclerosing cholangitis (PSC) and they complicate the evaluation of patients and timing of liver transplantation. METHODS: Data from all Nordic PSC patients listed for liver transplantation during 1990-2001 were recorded prospectively. Predictors of hepatobiliary malignancy and patient survival rates have been analysed. RESULTS: Hepatobiliary malignancy was found in 52/255 (20%) patients accepted to the waiting list. Recent diagnosis of PSC, no ursodeoxycholic acid (UDCA) treatment, clinical suspicion and previous colorectal-cancer were predictors of malignancy. Among 89 patients with a strong suspicion of malignancy prior to acceptance, 35 (39%) had confirmed malignancy. A clinical suspicion had been raised in 35/52 (67%) patients with malignancy. Malignancy was found in 31/223 patients who received a liver allograft. The 1-, 3- and 5-year patient survival rates following transplantation for patients with PSC and cholangiocarcinoma were 65, 35 and 35%, respectively. CONCLUSIONS: Hepatobiliary malignancy is suspected in 1/3 of the PSC patients and found in 1/5. Although cholangiocarcinoma is regarded as a contraindication to liver transplantation (LTX), PSC patients with cholangiocarcinoma had a 35% 5-year survival following transplantation.

Liver transplantation for primary sclerosing cholangitis in the Nordic countries: outcome after acceptance to the waiting list.

Primary sclerosing cholangitis (PSC) is a common indication for liver transplantation, but evaluation of patients and timing of liver transplantation remain as major problems. Data from PSC and control patients listed for liver transplantation from 1990 through 2000 in the Nordic countries were recorded prospectively. Outcomes from the waiting list and after transplantation have been recorded for both groups. For PSC patients, regression analyses have been performed to analyze predictors of outcome. A total of 255 PSC and 610 control patients were accepted on the liver transplantation waiting list from 1990 to 2000. In the PSC group, 223 patients (87%) received a first liver allograft, and 32 patients (13%) died without transplantation. The corresponding figures for the control group were 89% and 10%. For PSC patients, the 5- and 10-year survival from the time of acceptance was 68% and 58%, respectively. A higher Model for End-Stage Liver Disease score and a shorter duration of PSC predicted death on the waiting list for PSC patients. PSC is a frequent indication for liver transplantation. In our material, serum bilirubin or Model for End-Stage Liver Disease score and PSC duration are predictors of outcome including survival of the waiting list.