Orthotopic liver transplantation in a patient with acute liver failure resulting from aortic dissection

We report a case of acute liver ischemia resulting in liver failure that occurred due to celiac trunk occlusion by an aortic dissection. A 53-year-old patient presented with clinical signs of acute abdomen. He underwent an urgent laparotomy, which revealed a splenic infarction and thrombosis of the celiac artery all the way to the porta hepatis. Imaging showed that an acute thoracoabdominal aortic dissection was present, resulting in compression of the celiac trunk. The aorta was repaired with an intervascular graft; however, an acute insufficiency of the liver ensued, and the patient was urgently transplanted. To the best of our knowledge, this is the first report of vascular compromise of the liver due to acute aortic dissection that would require liver transplantation.     

Liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis

BACKGROUND: Sickle cell intrahepatic cholestasis is a potentially catastrophic complication of sickle cell anemia Once acute liver failure develops, transplantation is the only option. We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplantation. METHODS: Data were obtained from the chart. Serial hemoglobin S levels were monitored, and measures were taken to maintain hemoglobin S <20% to prevent sickle cell crisis. RESULTS: Although the allograft functioned well initially, the patient developed veno-occlusive disease and required repeat transplantation at 5 months after transplant. Histologic examination of the explant revealed occlusion of the terminal hepatic venules due to fibrosis and packed red cells. Repeat transplant was complicated by thrombosis of the intrahepatic portion of the hepatic artery, and sepsis. The patient died of sepsis after a third transplant. CONCLUSION: Liver transplantation for sickle cell disease involving the liver may carry a high risk of graft loss due to vascular problems. Repeat transplantation may not be feasible if disease recurs.     

Bridging hyperacute liver failure by ABO-incompatible auxiliary partial orthotopic liver transplantation

Uncontrollable intracranial pressure elevation in hyperacute liver failure often proves fatal if no suitable liver for transplantation is found in due time. Both ABO-compatible and auxiliary partial orthotopic liver transplantation have been described to control such scenario. However, each method is associated with downsides in terms of immunobiology, organ availability and effects on the overall waiting list.     

Liver transplantation for acute liver failure in a SARS-CoV-2 PCR-positive patient

Current guidelines recommend deferring liver transplantation (LT) in patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection until clinical improvement occurs and two PCR tests collected at least 24 hours apart are negative. We report a case of an 18-year-old, previously healthy African-American woman diagnosed with COVID-19, who presents with acute liver failure (ALF) requiring urgent LT in the context of SARS-CoV-2 polymerase chain reaction (PCR) positivity. The patient was thought to have acute Wilsonian crisis on the basis of hemolytic anemia, alkaline phosphatase:bilirubin ratio <4, AST:ALT ratio >2.2, elevated serum copper, and low uric acid, although an unusual presentation of COVID-19 causing ALF could not be excluded. After meeting criteria for status 1a listing, the patient underwent successful LT, despite ongoing SARS-CoV-2 PCR positivity. Remdesivir was given immediately posttransplant, and mycophenolate mofetil was withheld initially and the SARS-CoV-2 PCR test eventually became negative. Three months following transplantation, the patient has made a near-complete recovery. This case highlights that COVID-19 with SARS-CoV-2 PCR positivity may not be an absolute contraindication for transplantation in ALF. Criteria for patient selection and timing of LT amid the COVID-19 pandemic need to be validated in future studies.     

Successful orthotopic liver transplantation in a patient with a positive SARS-CoV2 test and acute liver failure secondary to acetaminophen overdose

SARS-CoV2, first described in December 2019, was declared a pandemic by the World Health Organization in March 2020. Various surgical and medical societies promptly published guidelines, based on expert opinion, on managing patients with COVID-19, with a consensus to postpone elective surgeries and procedures. We describe the case of an orthotopic liver transplantation (OLT) in a young female who presented with acute liver failure secondary to acetaminophen toxicity to manage abdominal pain and in the setting of a positive SARS-CoV2 test. Despite a positive test, she had no respiratory symptoms at time of presentation. The positive test was thought to be residual viral load. The patient had a very favorable outcome, likely related to multiple factors including her young age, lack of respiratory COVID-19 manifestations and plasma exchange peri-operatively. We recommend a full work-up for OLT in COVID-19 patients with uncomplicated disease according to standard of care, with careful interpretation of COVID-19 testing in patients presenting with conditions requiring urgent or emergent surgery as well as repeat testing even a few days after initial testing, as this could alter management.     

人工肝联合肝移植治疗急性肝功能衰竭

急性肝功能衰竭（acute liver failure，ALF）疾病凶险，自然死亡率高达80％以上。20世纪80年代中期肝移植开始应用于治疗急性肝功能衰竭。现今，结合肝移植治疗急性肝功能衰竭病人存活率大大提高，英国伯明翰伊丽莎白医院一组110例ALF结合肝移植病人1年存活率达到81％，5年存活率高达73%。笔者一组86例ALF结合肝移植资料表明病人的1年存活率为88％，5年存活率达71％。这些数据表明现代外科干预处理ALF可获得满意疗效。     

Pediatric liver transplantation for acute liver failure

The only proven therapy for patients unlikely to recover from acute liver failure (ALF) is liver transplantation. Correct diagnosis of these individuals and rapid referral to a transplant center are crucial. We evaluated 12 pediatric patients with ALF who underwent liver transplantation (LT) at our institution during a 3-year period. The reasons for transplantation were hepatitis A (3 patients); non-A, non-E hepatitis (3); autoimmune hepatitis (1); fulminant Wilson's disease (3); Amanita phalloides (mushroom) poisoning (1); and hepatitis B and toxic hepatitis with leflunomide treatment (1). Seven of the participants were female and five were male (mean age, 9.1 +/- 4.2 years). Three received right liver-lobe grafts, one received a whole liver graft, and the remainder received left or left-lateral liver lobe grafts. All patients recovered from hepatic coma the second postoperative day. Two patients died at postoperative days 57 and 71 due to adult respiratory distress syndrome and sepsis with multiorgan failure, respectively. One patient required retransplantation because of chronic rejection 7 months after the initial transplantation. That patient died 10 days after retransplantation because of sepsis. Nine patients were healthy at follow-up (range, 2-46 months). LT is the only treatment option for ALF in patients in countries with low organ-donation rates. In this scenario, donor preparation in a limited time frame is difficult. We have been able to decrease the duration of donor preparation to approximately 4 hours (including biopsy of the donated liver tissue).     

Brain preservation during orthotopic liver transplantation in a patient with acute liver failure and severe elevation of intracranial pressure

CONCLUSION We presented a patient for OLT with ALF and progressive deterioration in mental functioning due to severe encephalopathy and a critical rise in ICP. We used a Camino cranial bolt for ICP measurement and vasopressors to elevate the MAP and CPP while ICP was controlled with mild hypothermia, hyperventilation, mannitol, and furosemide. As a result, wemaintained a viableCPPand prevented irreversible brain damage during OLT. Presented at the Fifth Biennial Meeting of the American Hepato-Pancreato-Biliary Association, Fort Lauderdale, Florida, April 14–17, 2005.     

Live donor liver transplantation for acute liver failure

BACKGROUND: Acute liver failure (ALF) carries a high mortality unless urgent orthotopic liver transplantation (OLT) is performed on time. Live donors are utilized to treat this irreversible condition first in pediatric cases and then in adults. Herein, we aimed to report our experience with live donors for ALF in a country of a deceased donor organ donation rate is only 1.5 per million people. METHODS: Among the 245 live donor liver transplantations (LDLT) performed from June 1999 to December 2005, 14 of them (6%) were performed for ALF in 8 pediatric and 6 adult cases. Right lobes were harvested for the adult cases whereas left lateral segments were harvested for pediatric cases, except one child transplanted with a right lobe graft. The etiology of the disease was; acute hepatitis B in four cases, hepatitis A in three cases, Wilson disease two cases, autoimmune hepatitis in two cases, and was unknown in three cases. RESULTS: Three-year graft and patient survival is 79% for these series. Five of the six adult patients and six of the eight pediatric cases survived after transplantation. There was not any donor mortality or major morbidity. CONCLUSIONS: LDLT offers a safe and effective modality of treatment for ALF for both pediatric and adult patients to overcome the problem of organ shortage especially in countries where the chance of receiving an organ from a deceased donor is low.     

Temporary liver transplantation in acute liver failure.

The ability of a heterotopic graft to prolong life in animals dying in hepatic coma due to liver necrosis has never been definitely established. Acute hepatic failure was produced in 15 dogs by an hour of total interruption of the hepatic blood supply. Nine dogs received an intrathoracic hepatic homograft concurrently. Nontransplanted dogs died within 21 hours in hepatic coma, while transplanted dogs survived significantly longer (P less than .001). In all transplanted dogs, biological signs of hepatic failure were corrected in 24 hours. In four animals, the graft was removed on the fifth postoperative day. Two of those survived for 10 and 15 days respectively with normal hepatic function. These results demonstrate that a temporary heterotopic liver transplant is able to support life during the acute, normally lethal phase in dogs with massive liver necrosis.     

分子吸附循环系统联合肝移植治疗急性肝功能衰竭

目的　探讨应用分子吸附循环系统结合肝移植治疗急性肝功能衰竭的效果。方法　 8例急性肝功能衰竭患者 (7例原发病为乙型肝炎后肝硬化 ,1例为Wilson病 )在等待肝移植期间经分子吸附循环系统治疗 3～ 7次 ,所有患者均接受同种异体改良背驮式肝移植术。结果　患者等待供肝时间 8～ 30d ,平均 13d ,所有患者均顺利渡过围手术期 ,痊愈出院。应用分子吸附循环系统治疗后 ,患者的血清丙氨酸转氨酶、天冬氨酸转氨酶及总胆红素水平明显下降 (P <0 .0 1) ,但凝血功能的改善不明显。结论　分子吸附循环系统可暂时替代肝脏的部分功能 ,帮助急性肝功能衰竭患者顺利渡过肝移植等待期。     

Auxiliary heterotopic partial liver transplantation in pigs with acute liver failure

Fulminant hepatic failure is usually fatal without liver transplantation; however, orthotopic liver transplantation is often difficult to perform due to the high risk of coagulopathy and the development of multiple organ failure. Auxiliary heterotopic partial liver transplantation (APLT), however, has the potential to provide an effective hepatic support system considering that the host liver is left in situ and the surgical procedure is less invasive. In this report, we describe the beneficial effects of performing 60% APLT on the hepatic function and survival of pigs with acute hepatic failure induced by hepatic artery ligation. The pigs were divided into a control group of nine animals (group 1) that had portal vein and hepatic artery ligation with a side-to-side portacaval shunt, and an APLT group of seven animals (group 2) that had portal vein and hepatic artery ligation with APLT. The two left lateral lobes of the donor liver were resected, reducing the liver weight to about 60%, and the graft was placed in the right subhepatic space. No deaths occurred intraoperatively. In group 1, eight pigs died of massive liver necrosis within 48 h and one died between 48 and 72 h (median surivival 23 h). In group 2, two pigs died within 72 h due to preservation or anesthetic problems, but five survived for more than 3 days (median survival 13.4 days), with a significant difference between the two groups (P<0.05). One animal was killed 30 days after APLT and excellent graft function was demonstrated by the synthesis of clotting factors, ammonia detoxification, and glucohomeostasis. Moreover, evidence of hepatic regeneration was found in the transplanted livers. These results indicate that APLT provides metabolic support and improves survival in animals with induced acute liver failure.     

急性肝功能衰竭患者肝移植术后急性肾功能衰竭的病因分析及综合治疗

目的 分析急性肝功能衰竭(acute liver failure,ALF)患者肝移植术后肾功能衰竭的原因,评价以持续肾脏替代治疗(continuous renal replacement therapy,CRRT)为基础的综合疗法的疗效.方法 回顾性分析2001年1月至2006年6月在我院施行的412例肝移植资料,根据UNOS肝功能分级标准筛选出54例ALF患者(UNOS1和2A),其中17例移植术后出现急性肾功能衰竭(acute renal failure,ARF).在CRRT治疗基础上,进行抗排斥、抗感染、营养支持等治疗,并对患者围手术期情况、术后并发症、死亡原因及随访结果进行了分析.结果 CRRT治疗过程中无并发症发生.无ARF组围手术期死亡率为5.4%,术后并发症发生率为35.1%,1、3年生存率分别为89.2%和81.1%.ARF组围手术期死亡率为58.8%,术后并发症发生率为100%,1、3年生存率分别为41.2%和41.2%.结论 肝移植效果主要取决于肝外器官功能和术前肝功能状态.ALF患者围手术期死亡率较高,其中术前血肌酐高术后出现ARF率高,死亡率更高.以CRRT为基础的综合疗法能有效治疗ARF患者.     

Results of liver transplantation in acute liver failure caused by viral hepatitis

Abstract Fulminant liver failure due to acute viral hepatitis is the most common emergency indication for liver transplantation. The postoperative course is highly correlated with the type and duration of infection. The complication rate is lowest in fulminant hepatitis B patients and highest in subacute hepatitis C/NANB patients.     

Liver transplantation in a patient with hemophilia A and end stage liver failure

We report the case of a 50 year-old man factor VIII deficient haemophiliac and hepatitis C cirrhosis. The patient underwent orthotopic liver transplantation because of episodes of variceal bleeding and encephalopathy. He received factor VIII replacement therapy perioperatively. Factor VIII returned to normal within 24 hours postoperatively and factor VIII replacement was stopped. Liver transplantation can be considered as definitive therapy for haemophilia.