Outcome of living donor liver transplantation for post-Kasai biliary atresia in adults.

Previous reports described the effectiveness of living donor liver transplantation (LDLT) for post-Kasai biliary atresia (BA) in the pediatric population. Information on the outcome of LDLT in patients that have reached adulthood after the Kasai procedure, however, is limited. A recent report postulated a poorer long-term outcome of LDLT in these adults. We reviewed our experience to evaluate the validity of this hypothesis. Between January 1996 and October 2006, 385 LDLTs were performed at our institution. There were 80 post-Kasai BA cases in the series; 60 (75%) were pediatric, and 20 (25%) were adults. There were no ABO blood type-incompatible cases. None were complicated with severe hepatopulmonary syndrome, portopulmonary hypertension, or hepatocellular carcinoma. The 5-year overall survival rates were 90% for the adults and 90% for the children (P > 0.99). The median follow-up period was 7 years in the adults and 11 years in the children. There was no donor mortality. The outcome of LDLT in adult post-Kasai BA patients in the present series was satisfactory; that is, adult and pediatric patient survival rates were not different. This finding suggests that for post-Kasai BA patients without serious comorbidity at the time of transplantation, LDLT can be performed safely in all age groups.     

Liver transplantation in patients with situs inversus

Situs inversus has been considered an absolute contraindication to liver transplantation due to technical difficulties. Associated vascular malformation and distorted anatomy may make the procedure even more complicated or impossible. Only three cases of patients with abdominal situs inversus who underwent successful liver transplantation have been reported in the English literature. We describe two additional patients with situs inversus who suffered from biliary atresia and underwent successful liver transplantation. The preoperative evaluation and the operative procedure are presented, and technical difficulties are discussed. Since biliary atresia is associated with polysplenia syndrome, including vascular malformation and visceral malposition, we suggest that each case be extensively evaluated preoperatively to determine the size requirement for the donor liver and the feasibility of reconstruction.     

Left lobe living donor liver transplantation in an adult patient with situs inversus: technical considerations.

Situs inversus (SI) is a rare congenital disorder involving a complete mirror image of the thoracic and abdominal organs. Living donor liver transplantation (LDLT) in SI cases poses particular challenges on account of its technical complexity, and only a few cases have been reported. Here, we present an adult with SI who was managed successfully by LDLT using a left lobe graft. Some technical modifications, including triangulated anastomosis of the hepatic vein, were required but no vascular graft was necessary. Graft function and vascular integrity were excellent throughout the postoperative course, although sepsis secondary to wound infection with methicillin-resistant Staphylococcus aureus developed. In conclusion, LDLT using a left lobe graft is a feasible procedure for patients with SI, even for adults. Therefore, this condition, while rare, should not be a contraindication for LDLT. Meticulous preoperative simulation and planning of the vascular reconstruction are important steps in LDLT for this rare anomaly.     

Toward 300 liver transplants a year

The technical success of cadaveric whole-size liver transplantation and better immunosuppressive drugs has extended the application of this life-saving procedure to include patients with irreversible acute and chronic liver diseases. However, because of the scarcity of cadaveric liver grafts, living-donor liver transplantation (LDLT) has emerged as an alternative to cadaveric-donor liver transplantation (CDLT), especially in Asia. In Korea, 8% of the population are hepatitis B virus (HBV) carriers, and the resultant HBV cirrhosis, with or without hepatocellular carcinoma (HCC), is common in the 40- to 60-year-old generation. Accordingly, many patients require orthotopic liver transplantation (OLT). In 1992, we started performing CDLTs in the Asan Medical Center. In 1994, the first successful pediatric LDLT was performed in Korea, on a 9-monthold infant with biliary atresia. In 1997, the first successful adult LDLT was performed in our department, using a left lobe, on a 37-year-old patient with HBV cirrhosis associated with HCC. Even after the first successful right-lobe LDLT, we faced the obstacle of anterior segment congestion of a right-lobe graft, and initiated reconstruction of the middle hepatic venous tributaries of a right-lobe graft in 1998. In 1999, we performed more than 100 OLTs a year. Insufficient graft size has hindered the expansion of adult LDLT, when the remaining left-lobe of potential donors is too small to assure donor safety. Dual two-left-lobe graft LDLT (transplanting from two donors into one recipient) was developed in 2000 to solve graft-size insufficiency and minimize donor risk. More than 200 OLTs a year have been performed since 2004, while broadening the indications for adult LDLT to near complete obstruction of the portal vein, with the application of intraoperative portography (IOP) and portal vein stenting. In 2007, 320 LTs were performed, including 276 adult LDLTs, 10 pediatric LDLTs, and 34 CDLTs (including 7 adult and 1 pediatric split-liver transplant). There has been no donor mortality in LDLT. With technical refinement and advanced perioperative care, the in-hospital mortality of recipients has dropped to 4%: attributed to the dedication of our liver transplantation team members.     

Living‐donor liver transplantation for congenital biliary atresia with porto‐pulmonary hypertension and moderate or severe pulmonary arterial hypertension: Kyoto University experience

Porto‐pulmonary hypertension with moderate or severe pulmonary arterial hypertension (PAH) is viewed as a contraindication to liver transplantation (LT) because of associated poor outcomes; however, patients with biliary atresia (BA) are generally good candidates for LT. Ten patients with moderate/severe PAH underwent living‐donor liver transplantation (LDLT) at our institution; eight of these patients had BA and were the focus of this study. Preoperative therapies, including prostaglandin (PG)I₂, were introduced. When mean pulmonary arterial pressure (mPAP) after treatment was <40 mmHg or initial mPAP without therapy was <35 mmHg, we performed an acute volume challenge test to evaluate right ventricular function. LDLT was performed when mPAP after anesthetic induction was confirmed at ≤35 mmHg. Six patients had favorable responses to preoperative treatment and catheter testing, but two patients showed poor responses. The two patients with poor responses had poor clinical courses with unstable mPAP after LDLT. The other six patients had successful courses with well‐controlled mPAP, and PGI₂ was withdrawn or weaned following LDLT. Survival did not significantly differ between the eight BA recipients with moderate/severe PAH and 77 age‐matched BA recipients without PAH from the same time period. LDLT has major benefits for BA patients with well‐controlled PAH.     

Biliary atresia and polysplenia syndrome: outcome and growth post-transplantation

Biliary atresia (BA) is the most common indication for liver transplantation in children. Approximately 7-10% of these patients have the associated polysplenia syndrome (PS). The prognosis of patients with BA and PS has been reported to be poorer than that in patients with BA without PS. All patients who underwent liver transplantation for BA and who still attend periodic controls at the outpatient clinic were considered. A retrospective study of outcome and growth in children with BA was made, and compared with a subgroup of patients with BA and PS. There were no significant differences on complications, liver and renal function tests, lipids and growth data. We concluded that BA and PS do not preclude successful liver transplantation.     

Progress in living donor liver transplantation for biliary atresia and challenges faced: A thirty-year single institutional experience

BackgroundBiliary atresia (BA) is the most common indication for liver transplantation in the pediatric population, and living donor liver transplantation (LDLT) and deceased donor liver transplantation (DDLT) have been established as a radical treatment for BA .The aim of this study was to clarify the long term outcomes and risk factors affecting the LDLT outcomes, as well as the challenges faced.MethodsBetween 1990 and 2019, 666 BA patients underwent LDLT in our institution and were enrolled in this study. Data regarding the recipient's age, anatomic findings of the biliary tree at Kasai's portoenterostomy, basic characteristics at transplantation, transplant profiles, donor characteristics, and outcomes of LDLT were analyzed.ResultsThe 1-, 5-, 10-, 15-, 20-, and 25-year graft survival rates of BA patients who underwent LDLT were 88.1%, 85.4%, 81.5%, 78.9%, 76.6%, and 75.5%, respectively. The transplant era, age at transplantation, ABO incompatible transplant, and presence of pulmonary vascular complications were identified as significant risk factors for overall graft survival. When the study period was divided into the first (1990–1999) and second (2000–2019) phases and re analyzed, the outcomes of ABO-incompatible transplants and LDLT for adult BA patients remained inferior to others in the second phase. The 20-year graft survival rate in patients who underwent re transplantation in the second phase was 54.2%.ConclusionsThe outcomes of LDLT in children are generally good, but the immunosuppression procedures need to be further improved for ABO-incompatible cases in the future. Further improvements in LDLT results for adult patients and re transplantation remain challenges to be addressed in this field, and future attempts, including revision to the organ allocation system of deceased donors, are necessary.Level of evidenceLevel III (case control study).     

Situs inversus of donor or recipient in liver transplantation

Situs inversus is a rare anatomical abnormality that is often associated with multiple, complex malformations. In the past, patients with situs inversus were considered unsuitable candidates for transplantation or organ donation because associated visceral, and especially vascular, anomalies pose special technical difficulties. Recently, several cases of successful liver transplantation in recipients with situs inversus have been published using modified surgical techniques. This report reviews the literature and describes our own experience, including two liver graft recipients with complete and incomplete situs inversus, and one patient who underwent successful transplantation using a liver from a donor with situs inversus. Received: 10 October 1997 Received after revision: 22 December 1997 Accepted: 9 January 1998     

Long-Term Outcome of Adult-to-Adult Living Donor Liver Transplantation for Post-Kasai Biliary Atresia

Our objective was to analyze problems in the perioperative management and long-term outcome of living donor liver transplantation (LDLT) for biliary atresia (BA). Many reports have described the effectiveness of liver transplantation (LT) for BA, particularly in pediatric cases, but little information is available regarding LT in adults (> or =16 years old). Between June 1990 and December 2004, 464 patients with BA underwent LDLT at Kyoto University Hospital, of whom 47 (10.1%) were older than 16 years. In this study, we compared the outcomes between adult (> or =16 years old) and pediatric (<16 years old) patients. The incidence of post-transplant intestinal perforation, intra-abdominal bleeding necessitating repeat laparotomy and biliary leakage was significantly higher (p < 0.0001, <0.001 and <0.001, respectively) in adults. Overall cumulative 1-, 5- and 10-year survival rates in pediatric patients were significantly higher (p < 0.005) than in adults. Two independent prognostic determinants of survival were identified: a MELD score over 20 and post-transplant complications requiring repeat laparotomy. Outcome of LDLT in adult BA patients was poorer than in pediatric patients. It seems likely that LT will be the radical treatment of choice for BA and that LDLT should be considered proactively at the earliest possible stage.     

Is there an advantage of living over deceased donation in liver transplantation?

Living donor liver transplantation (LDLT) is a well‐established strategy to decrease the mortality in the waiting list and recent studies have demonstrated its value even in patients with low MELD score. However, LDLT is still under a high level of scrutiny because of its technical complexity and ethical challenges as demonstrated by a decline in the number of procedures performed in the last decade in Western Countries. Many aspects make LDLT different from deceased donor liver transplantation, including timing of transplantation, procedure‐related complications as well as immunological factors that may affect graft outcomes. Our review suggests that in selected cases, LDLT offers significant advantages over deceased donor liver transplantation and should be used more liberally.     

A New Strategy of Liver Transplantation for Locally Advanced Unresectable Perihilar Cholangiocarcinoma Using Living Grafts With Simultaneous Resection of Recipients’ Hepatic Artery and Portal Vein Without Neoadjuvant Radiation: A Case Report

BackgroundPerihilar cholangiocarcinoma (pCCA) is often unresectable, because it includes crucial blood vessels in portal area. The prognosis of locally advanced unresectable cholangiocarcinomas is extremely poor. Recently, there have been several reports of the prognosis improving drastically with transplantation and combined chemoradiation therapy. However, liver transplantation for pCCA has 2 big problems. The first is that pCCA is located at a lethal position and its progress is sometimes rapid; therefore, the optimal timing of transplantation is sometimes lost. The second is vascular complications associated with neoadjuvant radiation, especially in living donor liver transplantation (LDLT).To overcome these problems, we performed conversion surgery using LDLT with simultaneous resection of the hepatic artery and portal vein, instead of neoadjuvant radiation. Herein, we report our experience of interposition reconstruction.MethodsA 31-year-old man with primary sclerosing cholangitis (PSC) was diagnosed with locally advanced unresectable pCCA. The patient underwent radical chemotherapy (gemcitabine/cisplatin/S-1) and avoided radiation because of PSC. After 6 months, positron emission tomography–computed tomography revealed no lymph node metastasis. There was no time to wait. We immediately performed LDLT with simultaneous resection of hepatic artery and portal vein, and microsurgical reconstruction using auto-vessel grafts.ResultsThe recipient recovered and was discharged 31 days posttransplant. His liver function improved, and he has had no recurrence after LDLT.ConclusionLDLT with neoadjuvant radiation is associated with high risk of vascular complications. In some cases, conversion surgery after radical chemotherapy using good timing LDLT without radiation may increase chances of transplantation for locally advanced pCCA.     

Living donor liver transplantation for Budd-Chiari syndrome with inferior vena cava obstruction and associated antiphospholipid antibody syndrome

BACKGROUND: Recently, the antiphospholipid antibody syndrome (APLS) has been recognized as the cause of the Budd-Chiari syndrome (BCS) in adults. However, APLS-induced BCS has been seen rarely in children. The surgical strategy for BCS depends on the patency of the inferior vena cava (IVC) and on the primary disease. METHODS: A 10-year-old boy with a diagnosis of BCS complicated with IVC obstruction caused by APLS received medical treatment and radiologic intervention for 2 years. In spite of these treatments, no relief of the symptoms could be achieved. Finally, the patient underwent living donor liver transplantation (LDLT), which required cavoplasty. He has had an uneventful course since the LDLT. CONCLUSIONS: IVC-obstructed BCS associated with APLS seems to be a good indication for LDLT with cavoplasty. Because liver transplantation (LT) itself is not a cure for APLS, the risk of thrombosis cannot be eliminated entirely by LT. Although immunosuppression may influence antiphospholipid antibody production, long-term observation and life-long anticoagulant treatment is needed even after LT. J Pediatr Surg 36:659-662.     

Monosegmental living donor liver transplantation

BACKGROUND: Living donor liver transplant (LDLT) program has been started from 1990 in Japan, and is still major form of liver transplantation because of the scarcity of cadaveric donor organs. In small infants, implantation of left lateral segment grafts can be a problem because of a large-for-size graft. Until November 2002, we performed 867 transplants for 828 patients (561 children and 306 adults), and 14 cases received monosegment grafts from living donors. METHODS: Fifteen patients, median age 211 days, median weights 5.95 kg, received monosegmental LDLT. The indication for using this technique was infants with an estimated graft-to-recipient weight ratio of over 4.0%. RESULTS: Graft and patient survival is 85.7%. There were no differences in donor operation time and blood loss between monosegmentectomy and left lateral segmentectomy. Segment III grafts were indicated in 13 cases. Two vascular complications were observed (one hepatic artery thrombosis and one portal vein thrombosis). CONCLUSIONS: Monosegental living donor liver transplantation is a feasible option with satisfactory graft survival in small babies with liver failure.     

Living donor liver transplantation for biliary atresia: An analysis of 2085 cases in the registry of the Japanese Liver Transplantation Society

Biliary atresia (BA) is the most common indication for liver transplantation (LT) in pediatric population. This study analyzed the comprehensive factors that might influence the outcomes of patients with BA who undergo living donor LT by evaluating the largest cohort with the longest follow‐up in the world. Between November 1989 and December 2015, 2,085 BA patients underwent LDLT in Japan. There were 763 male and 1,322 female recipients with a mean age of 5.9 years and body weight of 18.6 kg. The 1‐, 5‐, 10‐, 15‐, and 20‐year graft survival rates for the BA patients undergoing LDLT were 90.5%, 90.4%, 84.6%, 82.0%, and 79.9%, respectively. The donor body mass index, ABO incompatibility, graft type, recipient age, center experience, and transplant era were found to be significant predictors of the overall graft survival. Adolescent age (12 to <18 years) was associated with a significantly worse long‐term graft survival rate than younger or older ages. We conclude that LDLT for BA is a safe and effective treatment modality that does not compromise living donors. The optimum timing for LT is crucial for a successful outcome, and early referral to transplantation center can improve the short‐term outcomes of LT for BA. Further investigation of the major cause of death in liver transplanted recipients with BA in the long‐term is essential, especially among adolescents     

Sequential Pancreaticoduodenectomy after Living Donor Liver Transplantation for Cholagiocacinoma

Liver transplantation (LT) for patients with primary sclerosing cholangitis (PSC) is often contraindicated due to concomitant occurrence of cholangiocarcinoma (CC). Cases of simultaneous pancreaticoduodenectomy (PD) with LT have been sporadically reported; however, the applicability of such an invasive procedure to patients with CC has not been validated. We report here a case of sequential PD performed 44 days after a successful living donor liver transplantation (LDLT) using a left lobe graft. Although a clear pancreatic juice leakage through the drain persisted for days after surgery, the patient recovered from the complication and was discharged 32 days after the procedure. Currently, 1 year after LDLT, the patient is doing well with no evidence of recurrence. In conclusion, a sequential PD following LDLT is a safe and feasible option to treat CC complicating PSC. Long‐term follow‐up and accumulation of cases are necessary to evaluate the effectiveness of this procedure for this complicated disease.     

Live donor liver transplantation in adults

Live donor liver transplantation (LDLT) was initiated in 1988 for children recipients. Its application to adult recipients was limited by graft size until the first right liver LDLT was performed in Hong Kong in 1996. Since then, right liver graft has become the major graft type. Despite rapid adoption of LDLT by many centers, many controversies on donor selection, indications, techniques, and ethics exist. With the recent known 11 donor deaths around the world, transplant surgeons are even more cautious than the past in the evaluation and selection of donors. The need for routine liver biopsy in donor evaluation is arguable but more and more centers opt for a policy of liberal liver biopsy. Donation of the middle hepatic vein (MHV) in the right liver graft was considered unsafe but now data indicate that the outcome of donors with or without MHV donation is about equal. Right liver LDLT has been shown to improve the overall survival rate of patients with chronic liver disease, acute or acute-on-chronic liver failure and hepatocellular carcinoma waiting for liver transplantation. The outcome of LDLT is equivalent to deceased donor liver transplantation despite a smaller graft size and higher technical complexity.     

Indications for hepatic vein reconstruction in living donor liver transplantation of right liver grafts

BACKGROUND: To overcome problems arising from a graft of insufficient size, right liver grafts have been used extensively for adult-to-adult living donor liver transplantation (LDLT). However, there are reports of severe congestion in the anterior segment of the graft after transplantation. CASE REPORTS: Right liver transplantation without the middle hepatic vein was performed in six cases. In the second and third cases, the inferior right hepatic vein was reconstructed because it was thick (whereas the middle hepatic vein was not). Abdominal CT revealed congestive infarction of the anterior segment in the second case and of the posterior segment in the third. It was suspected that the former resulted from the lack of an middle hepatic vein, and the latter from obstruction of the reconstructed inferior right hepatic vein. Both patients died without improvement in liver function. Accordingly, in the fifth case, the middle hepatic vein was reconstructed. The postoperative course of this case was uneventful. Doppler ultrasonography showed profuse blood flow in the interposition graft. In the sixth case, the middle hepatic vein was not reconstructed because of technical difficulties. Although abdominal CT showed a congestive area in the anterior segment, the patient recovered uneventfully, probably because the volume of functional graft was sufficient even without the congestive area. CONCLUSION: When the color becomes dark in more than half of the surface of the anterior segment following clamping of middle hepatic vein tributaries and the hepatic artery, the middle hepatic vein should be reconstructed. When the diameter of the inferior right hepatic vein is more than 5 mm, its reconstruction is also recommended.     

Living donor liver transplantation for Budd–Chiari syndrome with hepatic inferior vena cava obstruction after open pericardial procedures

Living donor liver transplantation (LDLT) for Budd–Chiari syndrome (BCS) presents a unique challenge as it does not involve replacement of the hepatic inferior vena cava (IVC). We report a case of successful LDLT in a patient with BCS associated with occlusion of the hepatic veins as well as the IVC. A 34-year-old woman with a history of two open pericardial procedures had decompensated liver failure and portal hypertension. Venography showed complete obstruction of the hepatic IVC and well-developed collateral vessels. We performed LDLT via sternotomy and laparotomy, with an end-to-end anastomosis between the left hepatic vein of the donor and the patient’s suprahepatic vena cava in the pericardium. The patient recovered uneventfully and has been doing well for 5 years. LDLT without caval replacement for BCS in a patient with hepatic IVC obstruction is feasible if the patient has good functional collaterals before liver transplantation.     

亲属活体供肝移植治疗儿童终末期肝病六例

目的 探讨亲属活体供肝移植(LDLT)治疗儿童终末期肝病的效果,并总结治疗经验.方法 2005年9月至2007年1月对6例终末期肝病患儿进行了LDLT.6例患者中,原发病为肝内外胆管弥漫性囊性扩张症Ⅳ型伴肝硬化1例、肝豆状核变性2例、门静脉海绵样变性3例;供者为患儿的母亲3例、父亲2例及舅父1例,分别切取供者的右半肝1例和左半肝5例作为供肝;在切除受者全部病肝和保留下腔静脉后,对受者施行部分供肝的原位肝移植.术后依据供、受者的临床表现、血液学指标和影像学检查,对活体供肝移植的治疗效果进行评价.结果 术后对供、受者随访了6～21个月.6例供者均健康存活,未发生并发症.6例受者中,1例于术后第4天死于门静脉血栓形成,1例于术后5个月时死于肝内静脉血栓形成,其余4例均长期健康存活.结论 亲属活体供肝移植是治疗儿童终末期肝病的有效方法.术前进行仔细的供、受者选择和完善的影像学检查,术中应用精确的手术技术,术后给予严格的管理是儿童LDLT成功的关键.     

Living donor liver transplantation for fulminant hepatic failure

BACKGROUND: Living donor liver transplantation (LDLT) was originally indicated only for elective cases of pediatric patients with end-stage liver disease. In Japan, however, where liver transplantation from brain-dead donor is performed very rarely, this indication has been expanded to emergency cases such as fulminant hepatic failure (FHF). METHODS: Thirty-eight patients with FHF were treated between May 1992 and April 1999. Causes of acute liver failure were non-A, non-B hepatitis in 27 patients, hepatitis B virus in seven, and hepatitis A virus, Epstein-Barr virus, herpes simplex virus, and chrome poisoning in one each. RESULTS: Four patients did not undergo LDLT because of severe brain damage or combined multiple organ failure. The remaining 34 patients underwent a total of 36 LDLTs, including two retransplantations; 16 children received transplants of 17 lateral segments, three children and eight adults transplants of 11 left lobes, and seven adults transplants of eight right lobes. A total of 15 recipients died, four of primary graft dysfunction, three of refractory acute rejection, two of pneumonia, and one each of ductopenic rejection, sepsis, aplastic anemis, recurrence of Epstein-Barr virus hepatitis, multiple organ failure by chrome poisoning, and unknown hepatic failure. Primary graft dysfunction developed in adult recipients with small-for-size graft transplants, whereas refractory acute rejection and ductopenic rejection occurred in six grafts each of children with non-A, non-B FHF. CONCLUSIONS: LDLT can be safely expanded to cases of FHF in adult patients. Primary graft dysfunction in adult recipients with small-for-size left lobe grafts can be overcome by using right lobes. However, refractory acute rejection and ductopenic rejection in children remain a major problem.