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Smith-Lemli-Opitz syndrome is an autosomal recessive metabolic disease characterized by mental retardation and multiple congenital anomalies. The main pathology is the lack of the enzyme 3β-hydroxysterol Δ7-reductase, which is the last enzymatic step in cholesterol synthesis, ending with a low cholesterol level. Cholesterol is vitally important in cell membranes and myelination of the nervous system. The cholesterol level affects many systems of the body, especially the nervous system. The cause of liver involvement in Smith-Lemli-Opitz syndrome is unclear, and many hypotheses have been suggested. Here, we present the early results of a patient with Smith-Lemli-Opitz syndrome who underwent living-donor liver transplant due to cirrhosis. As a result of liver transplant, normal cholesterol levels were shown, as well as improvements in the patient's neurodevelopment and behavior. Early liver transplant may be considered for patients with a defect of cholesterol biosynthesis, even in the absence of cirrhosis, and may be a future treatment option to prevent risks of neurologic deterioration.

Objective: living liver and kidney donor surgeries are major surgical procedures applied to healthy people with mortality and morbidity risks not providing any direct therapeutic advantage to the donor. In this study, we aimed to share our simultaneous and sequential living liver-kidney donor experience under literature review in this worldwide rare practice. Material and methods: between January 2007 and February 2018, a total of 1109 living donor nephrectomies and 867 living liver donor hepatectomies were performed with no mortality to living-related donors. Eight donors who were simultaneous or sequential living liver-kidney donors in this time period were retrospectively reviewed and presented with their minimum 2-year follow-up. Results: of the 8 donors, 3 of them were simultaneous and 5 of them were sequential liver-kidney donation. All of them were close relatives. Mean age was 39 (26-61) years and mean BMI was 25.7 (17.7-40). In 3 donors, right lobe, in 4 donors, left lateral sector, and in 1 donor, left lobe hepatectomy were performed. Median hospital stay was 9 (7-13) days. Two donors experienced early and late postoperative complications (Grade 3b and Grade 1). No mortality and no other long-term complication occurred. Conclusion: expansion of the donor pool by utilizing grafts from living donors is a globally-accepted proposition since it provides safety and successful outcomes. Simultaneous or sequential liver and kidney donation from the same donor seems to be a reasonable option for combined liver-kidney transplant recipients in special circumstances with acceptable outcomes. / Giriş ve amaç: canlı karaciğer ve böbrek verici ameliyatları tamamen sağlıklı bireylere uygulanan cerrahi işlemlerdir. Bu cerrahiler vericiye doğrudan bir faydası olmayan, ölüm ve komplikasyon riski taşıyan büyük bir işlemdir. Bu çalışmamızda dünya genelinde çok yaygın olmayan eş zamanlı veya birbirini takip eden canlı karaciğer ve böbrek verici ameliyatı deneyimimizi literatür irdemesi ile birlikte paylaştık. Gereç ve yöntem: Ocak 2007-Şubat 2018 tarihleri arasında merkezimizde, alıcısı ile yakınlık ilişkisi olan vericilere toplam 1109 canlı böbrek verici ameliyatı ve 867 canlı karaciğer verici ameliyatı verici kaybı yaşanmadan gerçekleştirilmiştir. Bunlardan eş zamanlı veya birbirini takip edecek şekilde canlı karaciğer ve böbrek verici ameliyatı olan 8 verici minimum 2 yıllık takipleri ile incelenmiştir. Bulgular: bu 8 vericiden 3 tanesi eş zamanlı ve 5 tanesi birbirini takip edecek şekilde canlı karaciğer ve böbrek verici ameliyatı olmuşlardır. Hepsi alıcının yakın akrabasıydı. Ortalama yaş 36 (26-61) ve ortalama BMI 25,7 kg/m (17,7-40) idi. Vericilerden 3’üne sağ lob verici hepetektomisi, 4’üne verici sol lateral sektör hepatektomisi ve 1’ine sol lob verici hepetektomisi gerçekleştirilmiştir. Median hastanede kalış süresi 9 (7-13) gündü. Vericilerden 2’sinde erken dönemde komplikasyon gelişmiştir (Dindo Grade 3b ve Grade 1). Verici ölümü ve başka bir geç dönem komplikasyonu gelişmiştir. Sonuç: verici havuzunun genişletilmesinde canlı vericilerin güvenli olarak başarılı sonuçlar ile kullanılması dünya genelinde kabul görmektedir. Aynı vericinin eş zamanlı veya takip eden ameliyatlar ile karaciğer ve böbrek vericisi olması özel durumlarda kombine karaciğer ve böbrek alıcıları için güvenli bir seçenek olabilmektedir., NA

Liver surgery is one of the most complex surgical interventions with high risk and potential for complications. Posthepatectomy liver failure (PHLF) is a serious complication of liver surgery that occurs in about 10% of patients undergoing major liver surgery. It is the main source of morbidity and mortality. Appropriate surgical techniques and intensive care management are important in preventing PHLF. Early start of the liver support systems is very important for the PHLF patient to recover, survive, or be ready for a liver transplant. Nonbiological and biological liver support systems should be used in PHLF to prepare for treatment or organ transplantation. The definition of the state, underlying pathophysiology and treatment strategies will be reviewed here.

Knowledge of living donor liver transplantation (LDLT) for autoimmune liver diseases (AILDs) is scarce. This study analyzed survival in LDLT recipients registered in the European Liver Transplant Registry with autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis (PSC) and the non-autoimmune disorder alcohol-related cirrhosis. In total, 29 902 individuals enrolled between 1998 and 2017 were analyzed, including 1003 with LDLT. Survival from >90 days after LDLT for AILDs in adults was 85.5%, 74.2%, and 58.0% after 5, 10, and 15 years. Adjusted for recipient age, sex, and liver transplantation era, adult PSC patients receiving LDLT showed increased mortality compared to donation after brain death (DBD) (hazard ratio [HR] = 1.95, 95% confidence interval [CI] = 1.36-2.80, p < .001). Pediatric PSC patients showed also increased mortality >90 days after LDLT compared to DBD (HR = 3.00, 95% CI 1.04-8.70, p = .043). Multivariate analysis identified several risk factors for death in adult PSC patients receiving LDLT including a male donor (HR = 2.49, p = .025). Adult PSC patients with LDLT versus DBD conferred increased mortality from disease recurrence (subdistribution hazard ratio [subHR] = 5.36, p = .001) and biliary complications (subHR = 4.40, p = .006) in multivariate analysis. While long-term outcome following LDLT for AILD is generally favorable, PSC patients with LDLT compared to DBD might be at increased risk of death., Astellas Pharma; Novartis; Institut Georges Lopez; Sandoz, The Organ Sharing Organizations: the French ABM (Sami Djabbour), the Dutch NTS (Maaike de Wolf), the Eurotransplant Foundation (Marieke Van Meel), the Spanish RETH (Gloria de la Rosa), the UK-Ireland NHSBT (Michael Daynes), and the Scanditransplant (Ilse Duus Weinreich) are acknowledged for the data cross-check and sharing with the ELTR. The European Liver Transplant Registry is supported by a grant from Astellas Pharma, Novartis, Institut Georges Lopez, and Sandoz and receives logistical support from the Paul Brousse Hospital (AP-HP).

Background/aim: the progression of chronic kidney disease (CKD) in recipients of living-donor liver transplant (LDLT) compared to deceased-donor liver transplant (DDLT) has not been studied in the literature. We hypothesize that CKD stage progression in LDLT recipients is reduced compared to that of their DDLT counterparts. Materials and methods: a retrospective study was undertaken including 999 adult, single-organ, primary liver transplant recipients (218 LDLT and 781 DDLT) at 2 centers between January 2003 and December 2012, in which CKD progression and regression were evaluated within the first 3 years after transplantation. Results: waiting time from evaluation to transplantation was significantly lower in LDLT patients compared to recipients of DDLT. CKD stage progression from preoperative transplant evaluation to transplantation was significantly greater in DDLT. Deceased-donor liver transplant recipients continued to have higher rates of clinically significant renal disease progression (from stage I-II to stage III-V) across multiple time points over the first 3 years posttransplant. Furthermore, a greater degree of CKD regression was observed in recipients of LDLT. Conclusion: it can be concluded that LDLT provides excellent graft and patient survival, significantly reducing the overall incidence of clinically significant CKD stage progression when compared to DDLT. Moreover, there is a significantly higher incidence of CKD stage regression in LDLT compared to DDLT. These observations were maintained in both high and low model for end-stage liver disease(MELD)populations. This observation likely reflects earlier access to transplantation in LDLT as one of the contributing factors to preventing CKD progression., NA

Objective: living liver donor surgery is a major surgical procedure applied to healthy people with mortality and morbidity risks and does not provide any direct therapeutic advantage to the donor. We retrospectively analyzed the postoperative complication of our living liver donors to figure out the risks of donation. Material and methods: between November, 2006 and December, 2018, a total of 939 living liver donor hepatectomies were performed with no mortality to the living-related donors. Eight hundred and ninety donors with a minimum 1-year follow-up were analyzed retrospectively. Results: Of the 890 donors, 519 (58.3%) were males and 371 (41.7%) were females. Mean age was 35 years (18-64) and mean body mass index was 25.7 kg/m2(17.7-40). Right donor hepatectomy was performed to 601 (67.5%), left donor hepatectomy to 28 (3.2%) and left lateral sector hepatectomy to 261 (29.3%) of the donors. Of the 890 donors, 174 (19.5%) donors experienced a total of 204 early and late complications including life- threatening and nearly life- threatening complications in 26 (2.9%) of them. Intraoperative complication occurred in 4 (0.5%) donors. Right donors hepatectomy complication rate (23.3%) was higher than left donor (14.3%) and left lateral sector donor hepatectomy (11.5%). Conclusion: all donor candidates should be well-informed not only on the details of early and late complications of living liver donation, also possible outcomes of the recipient. In addition to detailed physical evaluation, preoperative psychosocial evaluation is also mandatory. Comprehensive donor evaluation, surgical experience, surgical technique, close postoperative follow-up and establishing a good dialog with the donor allows better outcomes. / Giriş ve amaç: canlı karaciğer verici ameliyatı tamamen sağlıklı bireylere uygulanan, vericinin doğrudan terapötik bir kazanç elde etmediği, ölüm ve ciddi komplikasyon riskleri taşıyan büyük bir cerrahi işlemdir. Bu çalışmamızda; canlı karaciğer vericisi olmanın riskini ortaya koymak amacıyla retrospektif olarak canlı karaciğer verici ameliyatı geçirmiş vericilerimizin ameliyat sonrası komplikasyonlarını ve sonuçlarını inceledik. Gereç ve yöntem: Kasım 2006-Aralık 2018 tarihleri arasında merkezimizde alıcısı ile yakınlık ilişkisi bulunan toplam 939 karaciğer vericisine, canlı karaciğer verici hepatektomisi mortalitesiz olarak gerçekleştirildi. Bu olgulardan minimum bir yıl takipli 890’ı retrospektif olarak incelendi. Bulgular: incelen 890 vericiden, 519 (%58,3)’u erkek, 371 (%41,7)’i kadındı. Ortalama yaş 35 (18-64) ve ortalama beden kütle indeksi 25,7 kg/m2 (17.7-40) idi. Canlı sağ verici hepatektomisi 601 (%67,5), sol verici hepatektomisi 28 (%3,2) ve verici sol lateral sektör hepatektomisi 261 (%29,3) vericide gerçekleştirilmiştir. Vericilerden 174 (%19,5)’ünde toplam 204 komplikasyon görülmüştür. Bunların 26 (%2,9)’sında hayatı tehdit edici komplikasyonlar gelişmiştir. Cerrahi işlem sırasında 4 (%0,5) vericide komplikasyon gelişmiştir. Canlı sağ verici hepatektomisinde (%23,3) komplikasyon oranları, sol (14,3%) ve sol lateral sektör hepatektomisine (%11,5) göre daha fazla gözlenmiştir. Sonuç: tüm verici adayları sadece verici ameliyatının detayları, erken ve geç komplikasyonları ile ilgili olarak değil ayrıca alıcının olası sonuçları hakkında da ayrıntılı olarak bilgilendirilmelidir. Ayrıntılı klinik muayene, tetkik ve değerlendirmelere ek olarak psiko-sosyal değerlendirme de kaçınılmazdır. Kapsamlı verici değerlendirilmesi, cerrahi deneyim ve teknik, yakın cerrahi sonrası takip, verici ve yakınları ile kurulacak iyi diyalog daha iyi sonuçları elde etmemizi sağlayacaktır., NA

Background Kaposi`s sarcoma (KS) is a complication of immunosuppressive therapy for transplant recipients. Unlike adult recipients, KS in pediatric organ transplantation is quite rare. Treatment is usually withdrawal of immunosuppression; non-responders often receive chemotherapy. Case We have reported a child with post-liver transplant visceral KS which has progressed despite withdrawal of immunosuppressive therapy, who has been treated with Paclitaxel for three weeks. KS has regressed completely after four cycles of Paclitaxel. Conclusion Paclitaxel should be considered as an effective first line treatment option for patients with posttransplant KS.

Liver metastasis is the main cause of death in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs), but only 10%-20% of metastasis in these cases is resectable at the time of diagnosis. In some cases, medical and interventional radiological treatments may not be effective. Liver transplantation, although controversial, may be an option. Worldwide organ-sharing systems do not provide exception points, but give recommendations for liver transplantation in cases of hepatic metastasis from GEP-NETs due to the issue of fair access to donor organs. Living donor liver transplantation is an option in select cases. Presented here are 2 cases in which living donor liver transplantation was performed in emergency situations as a life-saving procedure, with acceptable survival and without donor complications.

Situs inversus totalis (SIT) is a rare congenital anomaly, and liver transplantation (LT) in an adult SIT patient is extremely rare. Liver transplantation in a SIT patient is also technically challenging due to reversed anatomical structures. Here we present the case of an 18-year-old female with SIT in whom left lobe living donor LT was performed. The patient suffered from cirrhosis due to autoimmune hepatitis. The recipient and donor are doing well without complications 20 months after LT. Situs inversus totalis should not be considered a contraindication for LT. If possible, use of a living donor left lobe graft for LT is more feasible than a living donor right lobe graft. It is also technically easier than using deceased donor full-size liver graft in SIT patients who require liver transplantation.

Objectives In this paper, the results of liver transplant due to hepatoblastoma in 10 pediatric patients at Istanbul Sisli Memorial Hospital Transplantation Center are presented. Materials and methods We retrospectively evaluated medical records of pediatric patients diagnosed with hepatoblastoma and who underwent liver transplant at our clinic between January 2009 and March 2014. We examined age, weight, chemotherapy regimen, graft type for liver transplant, duration of hospital stay, complications, follow-up duration, and survival information. Results The median age of the 10 patients included in our study was 13.5 months (range, 8-120 mo), and the median weight was 10 kg (range, 6.5-30 kg). Two of the patients were twins. Five patients had pretreatment extent of disease III (centrally placed cases), and five had pretreatment extent of disease IV hepatoblastoma. Preoperative chemotherapy was given to 7 patients as cisplatin plus doxorubicin and to 3 patients per the International Childhood Liver Tumors Strategy Group 3 High-Risk Protocol at external centers. These protocols were administered according to treatment center preference. Nine patients received transplants from living donors. Two grafts were right lobes, and 7 were left lateral segments. In the remaining patient, a whole liver was received from a deceased donor. The histopathologic subgroups were epithelial in 5 patients, with others being of mixed type. Postoperative complications occurred in 3 patients as infection, intra-abdominal fluid collection, and acute rejection. The median follow-up was 32 months. One patient died because of lung metastasis within 9 months after transplant. Conclusions Centers should offer liver transplant to patients with centrally located tumors. For centers that have an insufficient number of deceased donors, living-donor liver transplant with optimal planning and early treatment can be performed.

Background The concept of beliefs could provide a basis for how donors may perceive recipients' end-stage liver failure (ESLF) and surgery for organ donation. However, there is no such quantitative study. Therefore, the objective of this study was to explore beliefs of living donors about recipients' ESLF and surgery for organ donation. Methods The sample comprised 16 living donors who donated a part of their liver to a patient who had ESLF. The data were analyzed by following established procedures for inductive qualitative analysis. Results Analysis showed that donors' beliefs can be viewed in a number of groups. Beliefs about recipients' ESLF included diverse explanations for ESLF (blaming oneself and physicians) and physical symptoms (developmental slowing down). Beliefs about being a donor included reasons for being a donor (performing a good deed, being healed), barriers to being a donor (other people being ignorant and selfish), ways to manage these barriers (following one's gut feeling), and factors facilitating being a donor (the feeling that one does not have many people to leave behind). Beliefs about surgery for organ donation included physical effects (pain, feeling stiff). Beliefs about organ donation included views that general organ donation should be encouraged and that people's awareness should be raised. Conclusions Existing psychological perspectives could help to interpret some beliefs. Nevertheless, other beliefs, not previously reported, could be considered as targets for individual consultations/psycho-educational programs for fostering emotional well-being.

Anatomic variation of the hepatobiliary system is often related to the biliary tract and vascular supply of the liver. We present here one of the smallest accessory hepatobiliary system. The case of a 30-year-old male who was a living liver donor is presented. During the dissection of the portal hilum, 1.5 cm of accessory liver (AL) tissue was noted below the left lobe of the liver. This AL tissue had a gallbladder of 1.5 cm and which had a cystic duct opening to the bile duct of the accessory liver. The AL bile duct opened to the left bile duct of the liver. The arterial and portal supply of the AL came from the left artery and left portal vein of the liver. The accessory gallbladder also had a cystic artery coming from the arterial branch of the AL. It was noted that the hepatic vein of the AL opened directly to left lobe tissue. The hepatobiliary system has many anatomic variations, but this case is rare and original in the literature in that it may be a cause of confusion and even a false diagnosis.

OBJECTIVES Here, we present the outcomes of 100 consecutive pediatric liver transplant patients in our center. MATERIALS AND METHODS Five hundred fifteen adult and pediatric liver transplants were performed at Organ Transplantation Center, Sisli Memorial Hospital, Istanbul, Turkey, between August 2006 and November 2012. Of these, the first 100 consecutive pediatric liver transplant patients were retrospectively analyzed. RESULTS One hundred three liver transplants were performed in 100 children (mean age, 4.7 y; age range, 4.4 mo to 17.3 y; 53% female, 47% male; mean body weight, 17.2 kg; range, 4.5 to 75 kg). Biliary atresia (27%) and progressive familial intrahepatic cholestasis (18%) were the most common causes of liver disease. Of all the cases, 88.4% were living-donor liver transplants. Arterial reconstruction was performed under an operating microscope in most cases. Duct-to-duct biliary anastomoses were preferred in anatomically favorable cases. Mean hospital stay was 17.5 days. Median follow-up was 19.9 months (range, 6 to 66.1 mo). The main complication after surgery was infection (34%). Postoperative technical complications included hepatic arterial thrombosis (3.9%), portal venous thrombosis (6.8%), and biliary leak (6.8%). One-, 3-, and 5-year patient survivals were 89.8%, 89.8%, and 83.8%. There were no serious postoperative complications in the living donors. CONCLUSIONS Living-donor liver transplant in pediatric patients is a safe alternative to deceased-donor transplant. It is becoming the most frequent treatment option for end-stage liver disease in pediatric patients in our center, given the paucity of pediatric deceased-donor organs.

Diaphragmatic hernia is a rare complication after pediatric liver transplant. This report presents occurrences of diaphragmatic hernia after living-donor liver transplants in 2 children.In 1 of the 2 patients, a right-sided diaphragmatic hernia developed after a living-donor liver transplant due to progressive familial intrahepatic cholestasis where a left lateral segment graft was used. In the other patient, a left-sided diaphragmatic hernia developed after a living-donor liver transplant due to biliary atresia following Kasai portoenterostomy where a left lateral segment graft was used.After diaphragm repair, the postoperative course was uneventful and there were no recurrences.A literature review identified nearly 30 cases of diaphragmatic hernia following liver transplants; diaphragmatic hernia should be considered a potential surgical complication after liver transplant.

Acute liver failure is a rapidly progressive and life-threatening disease in children, whose clinical features differ from those of adults.This is a review of a single center's experience with pediatric acute liver failure in a region with insufficient deceased donor support. The study is a retrospective review and analysis of 22 pediatric patients with acute liver failure between January 2007 and May 2013.The cause of acute liver failure was indeterminate in 45.4% of cases. Listing for liver transplant was required in 72.7% of patients, whereas 27.3% developed spontaneous remission. In the patients placed on the liver transplant wait list, 75% underwent liver transplant and 25% died before undergoing liver transplant. The presence of ascites, high-grade encephalopathy, and laboratory findings including high lactate dehydrogenase and phosphorous levels and international normalized ratio were significant parameters in selecting patients needing liver transplants. All liver transplants were from living donors. One- and 3-year patient survival rates after liver transplant were 75% and 75%. No serious donor complications occurred.Living-donor liver transplant may be the only option to save the lives of pediatric patients with acute liver failure, especially in regions with insufficient deceased-donor support. Timely referral to a multidisciplinary transplant center, expedient evaluation of living donors, and appropriate timing of transplant are crucial for a successful outcome.

Argininosuccinic aciduria is a urea cycle disorder caused by an argininosuccinate lyase enzyme deficiency that ends with nitrogen accumulation as ammonia. Argininosuccinic aciduria patients are at risk for long-term complications including poor neurocognitive outcome, hepatic disease, and systemic hypertension despite strict pharmacologic and dietary therapy. As the liver is the principle site of activity of the urea cycle, it is logical that a liver transplant should be an option, with careful patient selection, even in the absence of cirrhosis. We present 2 pediatric argininosuccinic aciduria patients who underwent a living-donor liver transplant from their mothers. After the liver transplant, the general well-being of the patients and their quality of life improved significantly. Liver transplant should be an option for argininosuccinic aciduria patients to prevent further neurologic deterioration and improve the patient's quality of life.

Aim: Liver transplantation from living donors affects not only recipients’ but also donors’ lives. The aim of this study was to explore living donors’ experience of life. Methods: The sample consisted of 16 living donors who donated a part of their liver to a patient who had end-stage liver failure. Anonymised interview transcripts were analyzed following established conventions. Results: The analysis showed that participants evaluated their life in terms of limitations brought by organ donation surgery, awareness of the need for lifestyle changes, emotional changes, changes in character, and mixed relationships. Emotional changes involved the experience of both negative and positive emotions (feeling reputable, feeling like being born again). Changes in character included both worsening of character (becoming half human, turning into an aggressive person) and positive changes in character (becoming more of a believer and a humanist). Mixed relationships included feeling supported by loved ones and doctors, reduction of burden of care, formation of a special bond, not feeling supported by potential supporters like mothers, or spouses and worsening of close relationships. Conclusions: Some findings (experience of negative emotions, lack of support from others) could be interpreted in terms of existing psychological theory. Other findings (worsening aspects of character, experience of positive emotions, improvement in aspects of character, formation of a special bond, worsening of close relationships) extended the literature and could be viewed as targets for educational programs for donors. © 2016 The Author(s). This open access article is distributed under a Creative Commons Attribution (CC-BY) 4.0 license.

The LDLT option in the pediatric population allows recipients to be transplanted early. A total of 202 consecutive pediatric liver transplants from two different institutions--108 (LDLT) and 94 (DDLT)--were retrospectively compared. Overall, one- and three-yr patient and graft survival were similar between DDLT and LDLT. ACR was greater in recipients of DDLT at one and three yr (50.8% and 61.0%) compared to LDLT (30.8% and 32.2%) (p = 0.002). When the data were stratified according to PELD/MELD score, LDLT with a low score had better one- and three-yr graft survival (96.2% and 96.2%) compared to DDLT (88.2% and 85.2%) (p = 0.02), with comparable patient survival (p = 0.75). Patient and graft survival were similar between DDLT and LDLT in the high PELD/MELD group. Lower incidence of ACR in both low and high PELD/MELD groups was (29.6% and 34.3%) for LDLT compared to DDLT (50.3% and 53.3%, p = 0.002 and p = 0.028, respectively). Regardless of PELD/MELD score, status, age group, and recipient weight, LDLT provides excellent patient and graft survival with a lower incidence of rejection compared to DDLT.

Leiomyosarcoma of the vena cava is a rare malignant tumor. A 61-year-old woman was admitted with right upper quadrant abdominal pain. Computed tomography revealed a retrohepatic vena cava tumor originating 2 cm below the confluence of the hepaic veins and ending 2 cm above the renal veins. The tumor was resected with 1 cm clear surgical margins, without requiring liver resection. Polytetrafluoroethylene vascular graft was used for reconstruction of the vena cava. Now 32 months postoperatively, there has been no recurrence or metastasis. Radical resection with negative surgical margins is the best curative therapy for leiomyosarcoma. Polytetrafluoroethylene vascular graft can be used in extensive tumors located at the vena cava.

Aim Liver transplantation affects not only recipients and living donors' lives, but also the nature and quality of their relationship. Moreover, the ways in which recipients of liver transplant experience life and views of living donors on how recipients experience life may differ. These differences may account for relational changes. It is also important to understand how recipients and their living donors' views differ if the aim is to devise psychoeducational programs for recipients and living donors. Therefore, the present study examined the recipients' experience of life after a diagnosis of end-stage liver failure (ESLF) and transplantation surgery from donors' perspective. Methods The sample consisted of 16 living donors who donated a part of their liver to a patient with ESLF. Thematic analysis was undertaken in parallel with interviews during which an interview guide was followed. Findings Donors felt that recipients evaluated life after the diagnosis of ESLF and transplantation surgery in terms of limitations, mixed relationships, emotional changes, and improvement in life. Conclusion Experience of social limitations, negative emotions, and the feeling that one is supported by others could be interpreted in terms of existing psychological theory. Some ways of adjusting that have not been reported before within the context of ESLF extended the literature. These included others being frightened of being infected by ESLF and being insensitive, experience of positive emotions, and ways of improving. Overall, compared with findings of previous qualitative work among recipients, our findings suggest that donors' evaluation of recipients' lives converge with that of recipients.

We present here the outcomes of our first 500 liver transplants and discuss the lessons learned during this time.We retrospectively analyzed the first 500 consecutive transplants within the listing criteria of the United Network for Organ Sharing, with recipients and donors with minimum 1-year follow-up. Patient survival and donor complications were analyzed for 31 liver transplant recipients with hepatocellular carcinoma beyond the Milan criteria who had transplant performed during the same time.Between August 2006 and March 2013, there were 519 liver transplants performed in 500 patients (365 adult, 135 pediatric). There were 394 living-donor and 125 deceased-donor liver transplants. In addition, 31 adult liver transplants were performed in patients with hepatocellular carcinoma beyond Milan criteria (22 living-donor and 9 deceased-donor transplants). The main cause of chronic liver failure was biliary atresia in pediatric patients (30.4%) and chronic hepatitis B infection in adults (35.6%). The complication rate for primary nonfunction was 3.8%, overall biliary complications 24.0% (significantly higher after adult living-donor liver transplant, 30.3%), hepatic artery thrombosis 1.6%, portal vein thrombosis 3.0%, retransplant 3.8%, acute cellular rejection 29.6%, and bacterial infection 39.4%. Overall 1-, 3-, and 5-year patient survival rates in the first 500 consecutive transplants performed on recipients within United Network for Organ Sharing listing criteria were 87.8%, 85.0%, and 78.6%; for hepatocellular carcinoma patients beyond the Milan criteria, survival rates were 71.9%, 52.5%, and 38.2%.In regions without a sufficient number of deceased donors, living-donor liver transplant, with its associated problems, is the only alternative to deceased-donor liver transplant. Liver transplant requires teamwork, with all players working well together for a successful outcome. The important keys to success in liver transplant include decision-making, timing, surgical skills, experience, and close follow-up.

Major bile duct injury during cholecystectomy represents potentially severe complications with unpredictable long-term results. If these lesions are not treated adequately, they can lead to hepatic failure or secondary biliary cirrhosis therefore requiring liver transplantation. We report a patient who required liver transplantation 15 years after open cholecystectomy. A l0-year old girl underwent open cholecystectomy and duodenal repair for cholelithiasis and cholecystoduodenal fistula. She required two surgical interventions, hepaticojejunostomy which was performed in another center and portoenterostomy for biliary stricture at our institution seven years after the cholecystectomy. Eight years after the third operation, she required recurrent hospitalization for treatment of hepatic abscesses. The extremely short intervals between the three life threatening episodes and the rapid progression to severe sepsis were taken into consideration and liver transplantation was performed at the age of 25. She is leading a healthy life at 4 years post transplantation. Although iatrogenic biliary injury can usually be treated successfully by a combination of surgery, radiological and endoscopic techniques, patients with severe injuries develop irreversible liver disease. This case report and review of the literature suggest that liver transplantation is a treatment modality for a selected group of patients with end-stage liver disease secondary to bile duct injury.

Background Iatrogenic factors became the leading mechanisms of severe cholangitis in a referral center. Patients and methods The records of the 58 patients treated for severe cholangitis between 1996 and May 2004 (inclusive) were evaluated. Results The most frequent underlying diseases were periampullary tumors and mid-bile duct carcinomas (22), followed by proximal cholangiocarcinomas (14). The triggering mechanism was an incomplete endoscopic retrograde cholangiopancreatography (ERCP) in 32 patients, incomplete or inappropriate percutaneous transhepatic biliary drainage (PTBD) in 6, apparently successful ERCP and stenting in 1, and percutaneous transhepatic cholangiography in 1. PTBD was the treatment of choice (38). Mortality was 29% (17/58); the major causes were refractory sepsis (8) and incomplete biliary drainage (advanced tumor, technical failure, or hemobilia) (8). Conclusions In this series composed predominantly of patients referred after development of sepsis, ERCP and PTBD complications were the leading mechanisms of severe cholangitis. Nonoperative biliary manipulations are invasive procedures with potentially fatal complications. The decisions to perform such procedures and periprocedural management are responsibilities of an experienced multidisciplinary team.

Background: Radical resection is the only potentially curative treatment for hepatic alveolar echinococcosis (AE). Although Turkey is an endemic region, population screening is not performed and early diagnosis is rare. Consequently, surgeons are compelled to explore possibilities such as near-total resection and biliodigestive anastomosis for palliation of jaundice. Methods: Surgery was performed in 32 patients with hepatic AE with the following indications: (1) resection; (2) palliation of jaundice; (3) definite assessment of operability; (4) failure in the management of cavity infection by percutaneous methods. Curative resection (R0 = complete resection of all parasitic mass [n = 9], and R1 = a resection in which a small remnant was left on a vital structure [n = 8]) were performed in 17 patients, intrahepatic cholangiojejunostomy in 7, laparotomy-external drainage in 7, and debulking in 1. Results: Perioperative mortality rates were 2/17, 0/7, 2/7 and 1/1, respectively. Twelve patients in the curative resection group are alive without recurrence/progression of the small remnant during a median follow-up of 59 (range 27–116) months. One patient developed an inoperable recurrence that was treated with albendazole. One patient was lost to follow-up. Long-term albendazole treatment was effective in all R1 patients except a patient who had slow asymptomatic progression. Successful palliation of jaundice was achieved in 5 of the 7 intrahepatic cholangiojejunostomy patients. Conclusions: The results of R1 resection in alveolar hydatid disease are similar to those of R0 resection; a small remnant is successfully controlled by albendazole. In patients with jaundice due to hilar invasion, biliary diversion from segment 3 or 5 is effective for palliation of the jaundice and facilitates albendazole treatment.

Background A majority of coagulation factors are synthesized in the liver. Factor XI (FXI) deficiency (Rosenthal syndrome) is one of the rare inherited coagulation disorders with an extremely low risk of transmission by liver transplantation (LT). Case Report We report here the case of a 50-year-old man who unknowingly acquired FXI deficiency by LT. During 1 year of post-transplant follow-up, his activated partial thromboplastin time (aPTT) remained prolonged, but he did not develop bleeding complications. The patient required retransplantation due to chronic rejection and is currently doing well 4 years after his first liver transplantation. Conclusions The presence of a prolonged aPTT in a deceased donor should raise suspicion for the presence of rare coagulation factor deficiencies. During urgent, lifesaving procedures such as LT, it may be impossible to avoid transmission. Awareness of this possibility will allow early detection and management.

In patients with biliary atresia, portal vein problems may cause challenges for liver transplant. Interposition grafts have been used for vascular anastomoses in transplant recipients with varied success. A cryopreserved iliac artery graft was used for the reconstruction of the portal vein in a 29-month-old infant with biliary atresia. At 17 months after transplant, she developed upper gastrointestinal bleeding that was caused by portal vein occlusion because of vascular calcifications in the graft. Upper gastrointestinal endoscopy showed esophageal varices with fresh bleeding, and the varices were band ligated. At 3 months after the bleeding episode, the patient was asymptomatic and biochemical tests were normal. In summary, liver transplant with cryopreserved iliac artery graft may be complicated by calcifications and portal vein occlusion, and caution is advised in using this graft material for portal vein anastomoses.

We describe herein a female patient with non-Hodgkin's lymphoma of the liver and present a review of the related literature. The patient was referred with the diagnosis of malignant hemangiopericytoma (with an open biopsy). The physical examination, standard laboratory test results and tumor marker levels were all normal. A nonstandard left lobectomy was performed. Histopathological and immunohistochemical examinations revealed non-Hodgkin's lymphoma of B-cell type. The findings of a peripheral blood smear and bone marrow biopsy were normal. There was no other site of involvement based on physical or radiological examinations. These findings established the diagnosis of primary hepatic lymphoma. Fewer than 100 cases have been reported in the world literature. The best treatment results have been obtained by a resection followed by chemotherapy when feasible.

A 9-month-old female infant with biliary atresia underwent cadaveric liver transplantation due to progressive cholestatic hepatitis following a Kasai operation. She had biliary atresia splenic malformation syndrome (BASM) composed of an absent retrohepatic inferior vena cava with an azygous connection, preduodenal portal vein, polysplenia, and intestinal malrotation. A portal vein thrombosis developed on the 4th postoperative day requiring immediate treatment by thrombectomy. The patient is well with normal liver function at 3 months follow-up. Although BASM may render the transplantation more difficult, the presence of BASM is no longer a contraindication to liver transplantation.

Liver hamartoma is a rare type of benign tumor which usually occurs in the first few years of life. Although it is the second most common benign tumor in childhood, only about 100 cases have been reported in the English literature. Thus, it is extremely rare to encounter liver hamartoma in adults. This report details the clinical presentation and surgical management of a 24-year-old woman with a rapidly growing liver hamartoma. A right hepatic lobectomy was successfully performed and the patient was discharged in good health on the 10th postoperative day.

Two patients operated on for solid and cystic tumors of the pancreas are presented. One of them had a history of a benign cerebral tumor (astrocytoma) for which a ventriculo-peritoneal shunt had been done 3 years previously. The operations performed were a type-I regional pancreatectomy in one patient and a Whipple's procedure in the other. The patients were discharged well without complications and no recurrence has been detected 1 year after the operation

Surgery is the main modality in the treatment of hepatic hydatid disease. In this report, a laparoscopic surgical method is described, and the results in the first 16 cases are presented. The method involves the use of an aspirator-grinder apparatus that achieves effective evacuation of viable cyst contents with the patient benefiting from the laparoscopic approach. Cavity infection occurred in two patients and was treated conservatively. In another patient, postoperative ultrasonography revealed a thick-walled cavity containing a dense fluid. Because the patient was symptomatic, pericystectomy was performed during the sixth postoperative month. Early postoperative parameters and the early follow-up results in other patients (2-17 months) are encouraging. The method is particularly suitable for uncomplicated, early-stage cysts located in laparoscopically accessible locations.

The purpose of this study was to assess the usefulness of a new staging scheme for alveolar echinoccocosis un of the liver. A total of 28 patients with hepatic alveolar echinococcosis underwent CT examinations and were staged according to the proposed staging scheme (stage 1: lesion measuring less than 3 cm in diameter; stage 2: lesion greater than 3 cm, confined to less than three hepatic segments; stage 3 a: lesion with invasion of more than three hepatic segments; stage 3 b: invasion of liver hilum or suprahepatic vena cava; stage 4: invasion of surrounding organs and/or distant metastasis). Of the patients 16 underwent laparotomy, and results of the radiologic staging were correlated with surgical data. The CT findings correlated well with surgical findings. Patients were followed-up for a period of 6 months to 7 years. We concluded that the proposed staging scheme seemed to be useful for the therapeutic planning of patients with alveolar echinococcosis of the liver.

Immunosuppressive treatment generally increases the severity of active infection. Therefore, liver transplant is contraindicated in the presence of active tuberculosis. Despite the importance of supportive treatment, liver transplant is the only treatment for fulminant hepatic failure.We report a case of successful liver transplant for fulminant hepatic failure in the presence of active tuberculosis infection.We immediately performed a liver transplant from a live donor. The patient received low-dose immunosuppressive treatment and antituberculosis treatment. The patient was cured and discharged on the 25th day after surgery. We stopped antituberculosis treatment 10 months after discharge. The patient has been followed for 32 months after transplant with normal graft function and has been free of pulmonary tuberculosis infection.Liver transplant can be performed in cirrhotic patients with active infections, such as tuberculosis, as a life-saving procedure.

Intraoperative dysrhythmias commonly occur in the surgical management of congenital heart diseases. It may also be seen in other surgical procedures. The initiating factors for an arrhythmia during surgery is usually a transient insult such as hypoxemia, cardiac ischemia, catecholamine excess, electrolyte abnormality and acidosis. CAVB is a life-threatening dysrhythmia in all ages. We herein report a case of transient CAVB in a 30-month-old boy during living-related liver transplantation for bile duct paucity-associated liver cirrhosis. Moreover, we discuss the probable etiology and treatment of CAVB in liver transplantation.

An 11-month-old female infant underwent living-donor liver transplantation for secondary biliary cirrhosis 8 months after Kasai operation. The portal vein was hypoplastic, and its diameter was only 4 mm at the level of the splenomesenteric confluence. End-to-end anastomosis of the recipient suprarenal vena cava to the graft portal vein (a left lateral section from the patient's mother) was performed. An end-to-side portocaval shunt with the recipient portal vein was constructed to mitigate portal hypertension. The early postoperative course was relatively uneventful. However, persistent hepatitis caused by infection with Cytomegalovirus and chronic rejection resulted in progressive hepatic dysfunction. Nine months after the initial operation, a living-donor retransplantation (a left lateral section from the patient's grandmother) was performed. One month after retransplantation, severe acute rejection that eventually required OKT3 treatment developed. The patient was in excellent health until 4 months after retransplantation, when another acute rejection episode (for which she was successfully treated) developed. Cavoportal hemitransposition should be included in the armamentarium of the transplant surgeon for the management of extensive portal system thrombosis and portal vein hypoplasia. An additional shunt may be useful in mitigating portal hypertension.

Introduction Liver transplantation affects not only recipients ’but also donors’ lives. Little is known on the effect of liver failure and transplantation surgery on the donors’ lives. Aims The aim of this study was to explore the donors’ experienceof life. Methods The sample consisted of 16 living donors of liver transplant. Thematic analysis was undertaken in parallel with interviews during which an interview guide was followed. Anonymised interview transcripts were analysed following established conventions. Results Findings showed that participants evaluated their life in terms of limitations, awareness of the need for lifestyle changes, emotional changes, changes in character and mixed relationships. Emotional changes included both negative and positive emotions (feeling reputable, feeling like being born again, rewarded in after life). Changes in character included both worsening of character (becoming half human, turning into an aggressive person) and positive changes in character (becoming more of a believer, a stronger person, a humanist). Mixed relationships included feeling supported by loved ones and doctors, reduction of burden of care because of lack of support, formation of a special bond between donors and recipients, not feeling supported by potential supporters and worsening of close relationships. Discussion Some findings (experience of negative emotions, lack of support from others) could be interpreted in terms of existing psychological theory. Other findings (worsening aspects of character, experience of positive emotions, improvement in aspects of character, formation of a special bond, worsening of close relationships) not reported before could be viewed as targets for educational programs for donors.

Introduction Not only recipients but also donors can beaffected by liver transplantation. Only a small number of qualitative studies have explored donors' beliefs. Objectives The aim of this study was to explore donors' beliefs about liverfailure and transplantation surgery. Methods The sample consisted of 16 living donors of livertransplant. An interview guide was followed during the interviews. Thematicanalysis was undertaken in parallel with interviews. Established conventions were followed foranalyzing anonymised interview transcripts inductively. Results Participants’ accounts can be grouped into anumber of beliefs including beliefs about liver failure, being a donor, transplantationand organ donation. Beliefs about renal failure included diverse explanationsfor liver failure (blamingoneself and doctors, senseless drug use, stress, God’s will) and physical symptoms(yellowing, itching). Beliefs about being a donor includedreasons for being a donor (performing a good deed, being healed, not committing a sin), barriers to being a donor (beingcriticized by others, other people beingsenseless, ignorant and selfish), ways to manage these barriers (gettingloved ones’ consent, following one’s gut feeling), factors facilitating being adonor (having a few responsibilities and not having many people to leave behind). Beliefs about transplantation surgery included physical effects (pain, feeling stiff). Beliefs about organ donation included the views that organ donationshould beencouraged and awarenessshould be raised. Discussion Somebeliefs could be interpreted in terms of existing psychologicaltheory. However, other beliefs which werenot reported before could be viewed as targetsfor psycho-educational programs.

Between 1977 and 1995, 495 patients were operated on for hepatic hydatidosis. Total pericystectomy was performed in 26 patients (closed technique in 21 and open technique in 5). Twenty-one patients had single cysts and 5 had two cysts. The median cyst diameter was 8cm (range; 3–20cm). The requirements for total pericystectomy were: (1) the cyst(s) should be located away from the hepatic veins, large bile ducts, or major branches of the portal vein and hepatic artery and (2) the patient should be fit to undergo a major operation. There was no procedure-related morbidity. One patient developed a biliary fistula that closed after endoscopic sphincterotomy. The median hospital stay was 7 days (range; 3–22 days). The median follow up was 24 months (range; 9–114 months). There was disease recurrence in a non-adjacent segment in 1 patient at 4 years. Pericystectomy is a potentially dangerous operation, but it avoids problems with cavity management and has low rates of biliary fistula, spillage, and recurrence. Its success stems primarily from careful patient selection.

Obstruction of a major hepatic vein, or major portal vein, or biliary tree branch causes atrophy of the related hepatic region, and frequently, hypertrophy in the remaining liver-the atrophy-hypertrophy complex (AHC). Whether hydatid cysts can cause AHC is controversial. The records of 370 patients who underwent surgery for hepatic hydatid disease between August 1993 and July 2002 were evaluated retrospectively. Excluding six patients with previous interventions on the liver, AHC had been recorded in the operative notes of 16 patients (4.4%); for all patients, a cyst located in the right hemiliver had caused atrophy of the right hemiliver and compensatory hypertrophy of the left hemiliver. The computed tomography images of seven patients were suitable for volumetric analysis. The median (range) right and left hemiliver volumes were 334 (0-686) ml and 1084 (663-1339) ml, respectively. The median (range) cyst volume was 392 (70-1363) ml. AHC due to Echinococcus granulosus was confirmed by objective volumetric analysis. The presence of AHC should alert the surgeon to two implications. First, pericystectomy may be hazardous due to association with major vascular and biliary structures. Second, in patients with AHC, the hepatoduodenal ligament rotates around its axis; this should be considered to avoid vascular injury if a common bile duct exploration is to be performed.

Background: Ten patients with postoperative external biliary fistula treated by endoscopic sphincterotomy are reported. Methods: Nine of these patients were operated for hepatic hydatid disease and one for a liver abscess. Mean daily output of bile through the fistulae which were present for 5–39 days was approximately 500 cc. Results: Treatment was successful in nine patients with closure of the fistulae in 2–15 days (mean, 7 days). No response was obtained in one patient who was reoperated, and an intrahepatic biliary duct was found to be completely eroded by the cyst wall. Conclusions: Endoscopic sphincterotomy should be the first-line treatment for postoperative external biliary fistulae related to hepatic hydatid disease.

We presented our experience with definitive treatment of traumatic biliary injuries.Six male patients (mean age 13 years; range 2 to 32 years) who were referred to our unit for definitive treatment of traumatic biliary injuries were retrospectively evaluated. Data were analyzed in terms of demographic characteristics, mechanisms of injuries, associated injuries, previous treatments, symptoms on admission, treatment at our unit, and the results of treatment. Outcome was assessed using modified Schweiser and Blumgart criteria.The injuries were due to blunt abdominal trauma in all the patients but one who had a gunshot wound. In three patients, biliary injuries were missed at the initial operation. On admission, three patients had external biliary fistulas, two had biliary strictures. One patient was sent following inadvertent ligation of the hepatoduodenal ligament during attempts to control hemorrhage. Roux-en-Y hepaticojejunostomy was performed in three patients. Percutaneous biloma drainage was performed in two patients, resulting in fistula closure in 13 and 40 days, respectively. One patient was treated by endoscopic retrograde cholangiopancreatography and papillotomy, which enabled fistula closure in three days. One patient was lost to follow-up. One patient died from hepatic failure 11 years after the trauma. At the end of a mean follow-up of 49 months (range 15 to 75 months), three patients were in excellent condition, while one patient experienced occasional attacks of cholangitis.In patients with undetected biliary injuries and in those with unsuccessful repair attempts, biliary reconstruction should be performed in experienced hepatopancreatobiliary surgery units.

Background Laparoscopic treatment of hydatid disease of the liver can be performed safely in selected patients. Methods Six hundred and fifty patients were treated for hydatid disease of the liver between 1980 and 2003 at the Hepatopancreatobiliary Surgery Unit of Istanbul Medical Faculty, Istanbul University. Of these, 60 were treated laparoscopically between 1992 and 2000. A special aspirator-grinder apparatus was used for the evacuation of cyst contents. Ninety-two percent of the cysts were at stages I, II or III according to the ultrasonographic classification of Gharbi. Results Conversion to open surgery was necessary in eight patients due to intra-abdominal adhesions or cysts in difficult locations. There was no disease- or procedure-related mortality. Most of the complications were related to cavity infections (13.5%) and external biliary fistulas (11.5%) resulting from communications between the cysts and the biliary tree. There were two recurrences in a follow-up period ranging between 3.5 and 11 years. Discussion Laparoscopic treatment of hydatid disease of the liver is an alternative to open surgery in well-selected patients. Important steps are the evacuation of the cyst contents without spillage, sterilization of the cyst cavity with scolicidal agents and cavity management using classical surgical techniques. Our specially designed aspirator-grinder apparatus was safely used to evacuate the cyst contents without causing any spillage. Knowledge of the relationship of the cyst with the biliary tree is essential in choosing the appropriate patients for the laparoscopic technique. In our experience of 650 cases, the biliary communication rate was as high as 18%; half of these can be detected preoperatively. In the remaining, biliary communications are usually detected during or after surgery. Endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy are helpful to overcome this problem. As hydatid disease of the liver is a benign and potentially recurrent disease, we advocate the use of conservative techniques in both laparoscopic and open operations.

To evaluate treatment results in iatrogenic biliary injuries with concomitant vascular injuries.Between January 1998 and May 2002 (inclusive), angiography was performed in 45 of the 105 patients treated for iatrogenic biliary tract injury. The charts of these 45 patients and 5 other patients in whom vascular injury was diagnosed at operation were evaluated retrospectively. Twenty-nine patients had concomitant vascular injury, the biliovascular injury group (BVI), and the remaining 21 patients had isolated biliary tract injury (IBTI).The most frequent initial operation was a cholecystectomy. The frequency of high-level (Bismuth III or IV) strictures was 90% in the BVI group and 62% in the IBTI group ( P0.05). Perioperative mortality was 7% in the BVI group and 5% in the IBTI group ( P0.05). The morbidity in the BVI group was significantly higher ( P0.05). Two patients in each group were lost to follow up. During a median (range) follow up of 31 months (5-51 months), a successful functional outcome was achieved in 96% of the BVI group and 100% of the IBTI group with a multimodal approach ( P0.05).The frequency of high-level biliary injury and morbidity were significantly higher in the BVI group. However, concomitant vascular injury had no significant effect on mortality and medium-term outcome of biliary reconstruction. Thus, routine preoperative angiography is not recommended.

A 54-year-old woman was referred with the diagnosis of hepatic angiosarcoma, made by percutaneous biopsy under ultrasonographic guidance. Ultrasonography (US) had revealed a 48 × 42 × 35 mm mass in the right lobe. Standard biochemical tests and whole blood count had yielded normal results. At our institution, magnetic resonance imaging demonstrated a hypervascular mass in the right lobe. Alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 levels were normal. Serological tests were negative for hepatitis B and C viruses. There was no evidence of metastasis. A right hepatectomy was performed. Histopathological examination confirmed the diagnosis of angiosarcoma. However, there was a suspicion of microscopically positive margins. Relaparotomy and resection of a 1-cm-thick slice of hepatic parenchyma was performed. Histopathological examination revealed necrotic tumor cells at the previous margin. The new surgical margin was tumor free. Due to the expected poor prognosis, prophylactic chemoembolization of the remnant liver (lipiodol + adriamycin + mitomycin) was performed at 3 and 7 months postoperatively. She has been alive without recurrence for 5 years and 4 months. Hepatic angiosarcoma has two distinct presentations: multiple tumors and a solitary tumor. The reported poor results largely stem from the predominance of the multiple tumors and consequent unresectability. Long-term survival is possible in solitary resectable hepatic angiosarcomas.

Radical resection is the only potentially curative treatment for hepatic alveolar echinococcosis (AE). Although Turkey is an endemic region, population screening is not performed and early diagnosis is rare. Consequently, surgeons are compelled to explore possibilities such as near-total resection and biliodigestive anastomosis for palliation of jaundice.Surgery was performed in 32 patients with hepatic AE with the following indications: (1) resection; (2) palliation of jaundice; (3) definite assessment of operability; (4) failure in the management of cavity infection by percutaneous methods. Curative resection (R0 = complete resection of all parasitic mass [n = 9], and R1 = a resection in which a small remnant was left on a vital structure [n = 8]) were performed in 17 patients, intrahepatic cholangiojejunostomy in 7, laparotomy-external drainage in 7, and debulking in 1.Perioperative mortality rates were 2/17, 0/7, 2/7 and 1/1, respectively. Twelve patients in the curative resection group are alive without recurrence/progression of the small remnant during a median follow-up of 59 (range 27-116) months. One patient developed an inoperable recurrence that was treated with albendazole. One patient was lost to follow-up. Long-term albendazole treatment was effective in all R1 patients except a patient who had slow asymptomatic progression. Successful palliation of jaundice was achieved in 5 of the 7 intrahepatic cholangiojejunostomy patients.The results of R1 resection in alveolar hydatid disease are similar to those of R0 resection; a small remnant is successfully controlled by albendazole. In patients with jaundice due to hilar invasion, biliary diversion from segment 3 or 5 is effective for palliation of the jaundice and facilitates albendazole treatment.