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Pretransplant malignancy unrelated to hepatocellular carcinoma is a challenging condition in liver transplantation. Standard of care requires the completion of treatments and a disease-free period before the transplant. However, in the setting of a fulminant hepatic failure, these steps cannot be achieved. A 46-year-old woman with a recent diagnosis of stage 2 breast cancer presented to our center with a fulminant hepatic failure of unknown origin. Because of the rapid worsening of her clinical status, she was listed as eligible for transplant after a multidisciplinary evaluation. Because of a shortage of available donors, a deceased donor ABO-incompatible liver transplant with a synchronous mastectomy and first-level axillary lymphadenectomy was performed. To prevent antibody-mediated rejection, a triple immunosuppression therapy and a postoperative therapeutic plasmapheresis were performed. The patient remains without cancer recurrence at 18 months of follow-up. Recent studies have shown that cancer recurrence in recipients with pretransplant malignancy is considerably lower than suggested in previously published studies. However,this data is not sufficient to establish evidence-based guidelines on the indications and timing of transplant. In selected cases, the presence of a pretransplant malignancy does notrepresent a contraindication for a rescue liver transplant. Further studies are needed to stratify the risk and to help clinicians to choose the best strategy in an urgent context such as this.

Auxiliary partial orthotopic liver transplant is a complex technique whereby a partial liver graft is transplanted in the orthotopic position, leaving behind a portion of the native liver. In acute liver failure, auxiliary partial orthotopic liver transplant serves as a rescue therapy and bridge for the native liver to regenerate. Auxiliary partial orthotopic liver transplant was initially considered a technically challenging procedure with inferior results versus orthotopic liver transplant. However, advancements in surgical techniques have led to improved results with auxiliary partial orthotopic liver transplant. Auxiliary partial orthotopic liver transplant is now increasingly accepted as a valid treatment option for acute liver failure. We present 2 cases of acute liver failure treated with auxiliary partial orthotopic liver transplant using an extra-small-for-size graft. The first case was a 12-year-old female patient who presented with druginduced acute liver failure and required an auxiliary partial orthotopic liver transplant with an extra-small left lobe graft (graft-to-recipient weight ratio of 0.45). The second case was a 23-year-old male patient with acute liver failure of unknown etiology who underwent an auxiliary partial orthotopic liver transplant with a small right lobe graft (graft-torecipient weight ratio of 0.5). In both cases, computed tomography liver volumetry was performed to determine the appropriate graft size for the recipient. Both patients underwent successful auxiliary partial orthotopic liver transplants, with good postoperative recovery. Follow-up examinations showed satisfactory liver function without evidence of graft failure or rejection. Auxiliary partial orthotopic liver transplant using extra-small grafts can be an effective treatment option for acute liver failure when no other suitable option exists, including as a rescue procedure for small grafts. However, careful patient selection and surgical planning are essential to ensure successful outcomes.

Sevoflurane is a volatile anesthetic agent that does not tend to cause clinically significant hepatotoxicity, but there are some reported hepatotoxicity cases in the literature. In the case presented here, adenotonsillectomy was performed during influenza infection, and sevoflurane was administered, after which acute fulminant hepatitis developed. At hour 24 of hospitalization after fulminant hepatic failure, liver transplant was performed in a 3.5-year-old patient without any known diseases. In such cases, etiology investigations should be planned, life support therapy should be administered, and information should be given to the patient to avoid exposure to sevoflurane in the future.

Drug-induced liver injury resulting in fulminant liver failure is a well-known condition, and many drugs have been documented in the literature as possible etiologies. However, hydralazine has seldom been reported as the offending agent. Our case report is about one such rare scenario of fulminant liver failure due to hydralazine use as an antihypertensive. A 65-year-old female patient presented with signs of fulminant liver failure 2 months after starting hydralazine for hypertension. She underwent extensive workup for the cause of acute liver failure. Other possible medications were ruled out, and workup for autoimmune and other etiologies were also negative. The patient underwent a deceased donor liver transplant and has been doing well since then. Her liver was found to be atrophic, with microscopically confirmed drug-induced liver injury. Hydralazine is used orally to treat essential hypertension and intravenously to emergently lower blood pressure. Hydralazineinduced acute liver failure is extremely rare. However, in this rare case where hydralazine-related drug-induced liver injury worsened to the extent of requiring liver transplant, we felt obliged to document and highlight this complication as a form of reminder to our colleagues of this serious outcome.

Heat stroke is a multiple organ dysfunction syndrome of poorly understood pathogenesis. Exertional heat stroke with acute liver failure is a rarely reported condition. Liver transplant has been recommended as treatment in cases of severe liver dysfunction; however, there are only 5 described cases of long-term survival after this procedure in patients with heat stroke. Here, we present 2 cases of young athletes who developed heat stroke. Both patients developed acute liver failure and were listed for liver transplant. Liver function tests of one patient improved, and he was discharged on postoperative day 13. The other patient showed no signs of improvement and liver biopsy showed massive necrosis. The patient underwent combined kidney-liver transplant and was discharged on postoperative day 17. After a follow-up of longer than 6 years, both patients are doing well with normal liver function and no neurologic sequelae. We also reviewed all published cases of hepatic failure associated with heat stroke and found 9 published cases of liver transplant for heat stroke in the English literature. Conservative management appears to be justified in heat stroke-associated liver failure, even in the presence of accepted criteria for emergency liver transplant. If the liver does not show signs of recovery and hepatic decompensation progresses, liver transplant should be performed.

Objectives: We analyzed liver tissue morphology dynamics in experimental animals with acute liver failure during treatment with a combination of transplanted cultured embryonic hepatocytes and the hepatoprotective drug Erbisol., Materials and Methods: We studied 30 white outbred male rats weighing 250 to 350 g, divided into 6 groups with 5 animals in each group. Acute liver damage was induced by a hepatotoxic model of acute liver failure for which carbon tetrachloride was applied (200 μg CCl4/100 g body weight with toxin-to-oil volume ratio of 1:1). To obtain cultured embryonic hepatocytes, we used an enzymatic-mechanical process that caused only minimal cell damage. This method is known to improve the output of viable cultured embryonic hepatocytes, improve the morphological-functional properties of the hepatocytes, and reduce the process time during procurement, and thereby reduce the overall time from procurement to the subsequent culturing of the obtained cells. Transplant of cultured embryonic hepatocytes was performed intrape-ritoneally at a dose of 50 million (0.5 × 108) cells. Morphological studies were performed on day 7 and day 21 of the experiment., Results and Conclusions: There were significant morphological changes in livers from animals with acute liver failure, caused by widespread necrosis and an elevated inflammatory response. Treatment with injections of cultured embryonic hepatocytes induced regeneration of the liver parenchyma cells and reduced the inflammatory response, both of which were further reduced in rats that received combined treatment of transplant with Erbisol. Combined application of cultured embryonic hepatocytes and Erbisol potentiated the effects of both treatments, which produced intensive proliferation of hepatocytes, hypertrophy and polyploidization of hepatocyte nuclei, and an early restoration of liver structure and organ mass.

Dengue is a common viral infection, especially in tropical countries, and it is a well-known cause of acute liver failure. The effects on the liver range from a mild illness to acute liver failure. Dengue-related acute liver failure has a high mortality rate, and the role of liver transplant in such cases has been less studied, due to the rapid progression and often associated multiorgan dysfunction in severe cases. Here, we report the first living donor liver transplant for an acute liver failure due to dengue. Although liver transplant is not the choice of treatment for sick patients, timely intervention in specific patients may have a role.

Objectives: Acute drug-induced liver failure is a rare indication for liver transplant. There is only one case of flupirtine-induced liver failure requiring transplant in the literature. In February 2018, the European Medicines Agency issued a withdrawal of approval for flupirtine medication in European countries as a result of the risk of acute liver failure., Materials and Methods: The aim of this study was a German-wide collection of data regarding patients with liver transplant as a result of flupirtine-associated liver failure., Results: A total of 9 patients received transplants. All patients were women with a mean age of 43 years. Indication for flupirtine medication was musculoskeletal symptoms and migraine headache. The medication was taken over a period of approximately 3 months. All patients developed progressive acute liver failure, and no patient had previous chronic liver disease or cirrhosis. The mean laboratory Model for End Stage Liver Disease score for the patients was 31 ± 7 at time of transplant. Eight of the 9 patients were listed as "high urgency" for transplant. After transplant, they had an uneventful course with a prolonged mean intensive care unit stay of 13 ± 8.7 days. The whole hospitalization time was 43 ± 21 days., Conclusions: This is the largest published series of patients who received liver transplant after a drug-induced acute liver failure from flupirtine medication.

Hemophagocytic lymphohistiocytosis is a rare and life-threatening systemic disease that can cause hepatic infiltration and present as acute liver failure. Here, we report a case of a 3-year-old pediatric patient who presented with acute liver failure and hepatic encephalopathy secondary to hemophagocytic lymphohistiocytosis. She had left lateral segment liver transplant from her father. After 27 months, she had bone marrow transplant from her sister. At the time of reporting (36 months after liver transplant), she showed normal liver function and blood peripheral counts. We found that liver transplant can be a curative treatment for this type of rare disorder, not only to improve the quality of life but also to prolong survival.

Albendazole is often used as adjunctive therapy in the treatment of echinococcal infection to reduce cyst viability before and prevent recurrence after surgical treatment. In this report, we present a 38-year-old Iraqi woman with Echinococcus initiated on albendazole therapy who developed acute liver failure 6 weeks after treatment. Investigation for common viral and autoimmune causes of liver injury was unremarkable, and a liver biopsy revealed changes consistent with severe, drug-induced liver injury. Despite discontinuation of albendazole, liver function continued to deteriorate, prompting rescue with an orthotopic liver transplant. Often used perioperatively in the management of Echinococcus infection, albendazole can induce idiosyncratic severe liver injury, mandating close monitoring for hepatotoxicity.

Objectives: Acute liver failure is a rapidly progressive and life-threatening disease in children, whose clinical features differ from those of adults., Materials and Methods: 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., Results: 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., Conclusions: 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.

Reactivation of hepatitis B virus replication is a known complication of immunosuppressive therapy, which can lead to hepatocellular injury, liver failure, and death. In this report, we describe the case of a 44-year-old man with chronic hepatitis B and a dilated cardiomyopathy status after a heart transplant. Reactivation of the patient 's hepatitis B virus occurred 4 months after the heart transplant. Despite prompt administration of antiviral therapy, he developed fulminant hepatitis with hepatic encephalopathy. A successful living-related liver transplant was performed 7 months after the heart transplant. The patient was followed up for 1 year, and during that time was free of hepatitis B virus. We suggest that routine antiviral therapy should be administered to patients with chronic hepatitis B receiving immunosuppressive therapy.

Objectives: Acute liver failure is a life-threatening condition with sudden onset liver injury, decreased liver functions, hepatic encephalopathy, and coagulopathy in patients without preexisting liver disease. In this study, we sought to evaluate the results of liver transplant as a treatment for acute liver failure., Materials and Methods: Between November 1988 and March 2015, we performed 482 liver transplants in 471 patients. We performed 36 liver transplants in 35 patients because of acute liver failure. Only 5 of these were from deceased donors. Thirty of those 34 patients were pediatric (85%) and 5 were adults (15%)., Results: Five patients died (4 in early postoperative period and 1 during the 18th month of living-donor liver transplant). We diagnosed 11 acute rejections (32%); 6 biliary leaks (17%); 6 intraabdominal hemorrhage (17%); 5 hepatic arterial thromboses (15%), and 1 venous complication (3%) during the early postoperative period. We have no morbidity or mortality in living-donor liver transplants., Conclusions: Living-donor liver transplants are an efficient and successful treatment for acute liver failure patients. In our center, we mostly consider and prefer living-donor liver transplants to deceased-donor liver transplant because of the paucity of organ donation, especially for pediatric patients. Considering acceptable postoperative complications, living-donor liver transplant is a lifesaving treatment for acute liver failure.

Objectives: Liver transplant is an established curative therapy for children with chronic end-stage liver disease or acute liver failure. In this study, we aimed to evaluate pediatric liver transplant in terms of outcomes, complications, and long-term followup results., Materials and Methods: Pediatric patients who had liver transplant in our institution were included. We retrospectively evaluated demographic features including body weight, Child-Pugh score, etiology of liver disease, graft source, perioperative outcomes, perioperative complications, postoperative complications, and long-term results. Outcomes of treatment of complications and revision transplant were evaluated., Results: Between September 2001 and December 2013, there were 188 pediatric liver transplants performed in our institution. Most grafts (90.9%) were obtained from living-related donors. There were 13 patients (6.9%) who had an intervention because of a hemorrhage postoperatively. Biliary leakage was observed in 33 patients (17.5%) and biliary stricture during follow-up was observed in 32 patients (17%). Thrombosis rates in the hepatic artery and portal vein were 12.3% and 0.5%. Revision transplant was performed in 11 patients (5.8%); reason for revision transplant was rejection in 50% patients. The remaining children were alive with good graft functioning after treatment of complications and revision transplant. The overall 5- and 10-year survival rates were 82.3% and 78.9%., Conclusions: The overall outcomes of pediatric liver transplant at our center are very promising. With improved care of younger children and the combined efforts of the parents and medical team, the number of the children receiving transplants will increase in the future.

Objectives: We assessed the anesthetic management and short-term morbidity and mortality in pediatrics patients who underwent an orthotopic liver transplant for fulminant hepatic failure or end-stage liver disease in a university hospital., Material and Methods: We retrospectively analyzed the records of children who underwent orthotopic liver transplant from May 2002 to May 2012. Patients were categorized into 2 groups: group fulminant hepatic failure (n=22) and group end-stage liver disease (n=19). Perioperative data related to anesthetic management and intraoperative events were collected along with information related to postoperative course and survival to hospital discharge., Results: Mean age and weight for groups fulminant hepatic failure and end-stage liver disease were 8.6 ± 2.7 years and 10.8 ± 3.8 years (P = .04) and 29.2 ± 11.9 kg and 33.7 ± 16.9 kg (P = .46). There were no differences between the groups regarding length of anhepatic phase (65 ± 21 min vs 73 ± 18 min, P = .13) and operation time (9.1 ± 1.6 h vs 9.5 ± 1.8 h, P = .23). When compared with the patients in group fulminant hepatic failure, those in group end-stage liver disease more commonly had a Glasgow Coma score of 7 or less (32% vs 6%, P = .04). Compared with patients in group fulminant hepatic failure, those in group end-stage liver disease were more frequently extubated in the operating room (31.8% versus 89.5% P < .001). Postoperative duration of mechanical ventilation (2.78 ± 4.02 d vs 2.85 ± 10.21 d, P = .05), and the mortality rates at 1 year after orthotopic liver transplant (7.3% vs 0%, P = .09) were similar between the groups., Conclusions: During pediatric orthotopic liver transplant, those children with fulminant hepatic failure require more intraoperative fluids and more frequent perioperative mechanical ventilation than those with end-stage liver disease.

Objectives: Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We assessed the hepatotoxicity of methotrexate and prednisolone combination therapy in the background of hepatitis B virus infection., Materials and Methods: We report the clinical course of a 55-year-old woman who underwent a deceased-donor liver transplant for fulminant liver failure., Results: The patient's medical history was significant for hepatitis B virus infection and rheumatoid arthritis. Methotrexate and prednisolone combination therapy were started 5 months earlier. The patient was hospitalized because of an elevation in her liver enzymes and total bilirubin. Deterioration of liver functions and encephalopathy were developed 5 weeks after hospital admission. A deceased-donor liver transplant was performed, and pathological examination of recipient liver revealed fibrosing cholestatic hepatitis. The patient was reoperated on for bile leak and discharged 40 days after the deceased-donor liver transplant., Conclusions: The natural course of the current case was similar to previously reported cases with fibrosing cholestatic hepatitis. Clinicians should consider the potential hepatotoxicity of methotrexate and steroid therapy in hepatitis B virus infected patients.

Dissection of the common hepatic artery is a rare complication after orthotopic liver transplant. Subsequent thrombosis and occlusion of the transplant artery can result in graft failure requiring retransplant. We describe a case of hepatic artery dissection, occurring on the basis of primary vasculopathy, extending into the celiac trunk, with subtotal occlusion of the vessel through accompanying thrombosis. An attempt of endovascular rescue led to successful recanalization of the vessel and graft survival.

We report the case of a middle-aged man who died from multiorgan failure 3 weeks after orthotopic liver transplant for fulminant hepatic failure, associated with a rare, often fatal, hematologic condition that usually presents in childhood. We discuss the importance of its diagnosis, treatment, and implications for liver transplant.

Late-onset hepatic failure, the least of the fulminant hepatic failures, has not occurred in patients with hepatitis A virus-related acute liver failure. We report a rare case of hepatitis A virus-related late-onset hepatic failure treated successfully by an emergent liver transplant. A 58-year-old Japanese woman who presented with fever and general malaise was diagnosed as having jaundice and liver dysfunction by a positive serum test for anti-hepatitis A virus IgM, which ultimately led to a diagnosis of acute hepatitis A virus associated hepatitis. Despite intensive treatment, her general condition was poor, and she developed a hepatic coma 79 days from the onset of the disease. Under a diagnosis of hepatitis A virus-related late onset hepatic failure, she was given a living-donor liver transplant 82 days from the start of the disease. The resected native liver revealed submassive necrosis with marked cholestasis, compatible with late-onset hepatic failure. Today, 5 years after the transplant, she is alive and well with no signs of recurrent hepatitis A virus-hepatitis. This case should alert the physician to the clinical management of a patient with hepatitis A virus-related acute liver failure.

Objectives: 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., Materials and Methods: We report a case of successful liver transplant for fulminant hepatic failure in the presence of active tuberculosis infection., Results: 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., Conclusions: Liver transplant can be performed in cirrhotic patients with active infections, such as tuberculosis, as a life-saving procedure.

Objectives: Acute pancreatitis, which can develop after any whole-organ transplant, is often associated with immunosuppression. Pancreatitis that complicates a liver transplant can be a significant problem that results in a high mortality rate., Materials and Methods: We describe the successful use of minimally invasive techniques to treat severe acute pancreatitis. To our knowledge, this is the first reported case in which major laparotomy was precluded by the use of percutaneous necrosectomy to manage necrotizing pancreatitis in a liver transplant recipient. We also briefly review the published literature on severe acute pancreatitis in liver transplant recipients., Results: Our patient, who had a Model for End- Stage Liver Disease score of 39 when transplanted and an Acute Physiology and Chronic Health Evaluation II score of 19 when infected necrosis in his pancreas was diagnosed, recovered completely after 92 days of hospitalization. He underwent 2 percutaneous drainage procedures and 3 percutaneous necrosectomies to treat his pancreatic complication. A review of the literature revealed that severe acute pancreatitis significantly increases morbidity and mortality in liver transplant recipients. Unlike necrotizing pancreatitis, which develops outside the context of liver transplant where there is a distinct shift towards minimally invasive procedures, infected necrosis associated with fulminant liver failure or a liver transplant is usually treated with open necrosectomy., Conclusions: Severe acute pancreatitis in liver transplant recipients should be managed exactly as it is in patients who have not received a liver transplant. Anatomically guided minimally invasive necrosectomy appears to be beneficial, especially when patients are critically unwell, as they are following a liver transplant.

Objectives: Liver transplant in the pediatric population has become an accepted treatment modality for children with end-stage liver disease. In this study, we analyze our experiences with pediatric liver transplant at our center., Materials and Methods: Since September 2001, 8 deceased-donor and 96 living-donor liver transplants have been done in 101 children (mean age, 6.7 +/- 5.5 years; range, 2 months to 17 years). The children's charts were reviewed retrospectively., Results: Indications for liver transplant were cholestatic liver disease (n=17), biliary atresia (n=24), Wilson's disease (n=16), fulminant liver failure (n=18), hepatic tumor (n=13), and other (n=13). The median pediatric end-stage liver disease score was 23.1 +/- 11.1 (range, -8 to 48). The median follow-up was 24.2 +/- 19.4 months (range, 1-77 months). Three children underwent retransplant. The main complications were infections (25.9%) and surgical complications (39.5%) (including biliary complications and vascular problems). The incidence of acute cellular rejection was 42.3%. Sixteen children died during follow-up, and, at the time of this writing, the remaining 85 children (85%) were alive with good graft functioning, showing patient survival rates of 90%, 85%, and 83% at 6, 12, and 36 months, respectively., Conclusions: In conclusion, the overall outcomes of pediatric liver transplantation at our center are quite promising.

Fulminant hepatic failure continues to be a challenge to hepatologists and surgeons. Because of the rapid deterioration in the clinical condition of patients with fulminant hepatic failure and the scarcity of available grafts, an ABO-incompatible liver donor may be the only choice for a patient with life-threatening hepatic insufficiency. Here, we report a patient with fulminant hepatic failure who received an ABO-incompatible liver transplantation who was treated with pre- and posttransplantation double-volume total plasma exchange, splenectomy, and triple immunosuppression (tacrolimus, mycophenolate mofetil, and prednisone) in July 2003. At 26 months' follow-up, the patient's postoperative course has been uneventful. Using protocols aimed at removing existing isohemagglutinins and reducing further antibody production, ABO-incompatible liver transplantation may be performed as a life-saving procedure in patients with fulminant hepatic failure.