Massive haemoptysis after living donor liver transplantation

A 27 year old man with hereditary haemorrhagic telangiectasia who developed progressive liver dysfunction underwent living related right lobe transplantation. Pulmonary arteriography did not reveal arteriovenous malformation or abnormal intrapulmonary venous channels. The postoperative course was characterised by persistent hypoxaemia and respiratory failure developed. On day 6, a massive haemoptysis developed and the patient died shortly thereafter. The native liver showed a nodular pseudocirrhotic transformation, with highly dilated and irregularly interconnected vein-like or arterial-like structures in the fibrous septa. Pathological examination of both lungs showed irregular thickening of the wall of the arteries, secondary to eccentric and/or concentric myointimal hyperplasia. This case suggests that massive haemoptysis can develop even when arteriovenous malformations are undetectable by pulmonary arteriography, and it questions the role and the appropriateness of living donor liver transplantation in high risk patients.     

活体肝移植肝右静脉的临床解剖研究

目的:为活体肝移植(LDLT)提供有关肝右静脉(RHV)的形态学资料.方法:利用50例无病变成人尸体肝脏进行剥离解剖,对RHV和副肝静脉进行观察,测量RHV主干及相关数据并进行统计分析.结果:RHV 80.0%(40/50)开口于下腔静脉(IVC)肝后段上份10:00处,其肝外段长(5.8±3.5)mm,外科干长(7.0±5.5)mm,主干长(68.3±30.2)mm,汇入IVC处内径为(10.7±2.4)mm;主干在距IVC 1 cm、2 cm、3 cm、4cm及主干汇合起始处上壁距肝膈面的距离分别为(10.8±5.3)mm、(22.7±7.0)mm、(32.8±8.2)mm、(40.1±7.3)mm、(33.5±9.8)mm;RHV与IVC的夹角为(40.5±9.4)°;根据RHV的引流范围及有无直径≥5.0 mm的副肝右静脉将RHV分为两型,各型再分为两个亚型.直径≥3.0mm和≥5.0mm的副肝右静脉出现率分别为60.0%(30/50)、32.0%(16/50).结论:RHV解剖变异较大,其引流范围受副肝右静脉和肝中静脉的影响,LDLT术前应掌握其结构特点,制定合理的手术方案.     

Postoperative liver regeneration and complication in live liver donor after partial hepatectomy for living donor liver transplantation

The safety of donor is the first priority during whole procedure in living donor liver transplantation. We evaluated the short-term results of partial living donor liver transplantation in the view of donor safety. We prospectively evaluated the extent of liver regeneration, the recovery of liver function, and the perioperative complications in 41 live liver donors for partial liver transplantation at our institution. We developed novel personal computer volumetry program for the evaluation of liver regeneration. Serial CAT scan was performed preoperatively, at postoperative day (POD) #7 and POD #30 and liver volume was measure by using volumetry program. The serum level of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and total bilirubin (T.bil.) was serially monitored. There were 34 males and 7 females. The mean preoperative liver volume was 1320.6 cm3. The remained mean liver volume was 687.8 cm3 after harvest, and increased to 954.4 cm3 (144.6%) at POD #7, and 1169.5 cm3 (81.4%) at POD #30, which was 88.5% of preoperative total liver volume. The serum level of ALT/ AST and T.bil. peaked at POD #1 and declined thereafter, and finally returned to preoperative level at POD #30. The regeneration rate was significantly different by age, type and size of graft according to the donors. Six donors experienced postoperative complications and they were four pleural effusions, one wound infection and one case of bile duct stenosis that was treated by endoscopic nasal biliary drainage. All of them were right lobe donors. In conclusion, the donor liver regenerated up to 88.5% of preoperative volume with full recovery of liver function at POD #30. Right lobe donors suffered more complications and need more meticulous operative and postoperative care than left lobe or left lateral segment donors.     

活体肝移植右半肝内肝静脉的应用解剖

目的:为活体肝移植提供右半肝内肝静脉的解剖学资料.方法:采用50例无病变成人尸体肝标本进行解剖剥离,对肝右静脉(RHV)及属支、副肝右静脉(aRHV)和肝中静脉(MHV)右半肝属支等进行观测.结果:RHV分型中A型、B型分别为74.0%和26.0%.RHV主干属支汇合形式可分为3种,其中90.0%为树状,8.0%呈2支型,2.0%呈3支型.RHV主要属支共(5.88±2.26)支,Ⅴ段、Ⅵ段、Ⅶ段、Ⅷ段主要属支直径分别为(4.05±1.06)mm、(3.96±0.86)mm、(4.64±1.57)mm、(4.46±1.30)mm,长度分别为(21.51±11.24)mm、(28.94±15.90)mm、(30.14±14.41)mm和(23.03±11.14)mm;Ⅴ、Ⅷ段静脉血液同时汇入RHV和MHV者分别占66.0%、88.0%,Ⅴ段静脉血仅通过MHV回流者占34.0%.直径≥3.00mm的aRHV的出现率为60.0%.结论:RHV引流范围同时受MHV和aRHV影响,主要属支数目不恒定,其右叶上段属支管径较下段粗,右后叶属支较前叶属支长.右叶部分活体肝移植术前应充分掌握右半肝内肝静脉的解剖学资料,以利于制定合理的手术方案.     

活体肝移植中轻度脂肪变性供肝的应用

背景：在活体肝移植中使用脂肪变性供肝不但影响供者的安全,同时也影响受者的生存。 目的：评价活体肝移植中使用轻度脂肪变性供肝时供者的安全性及受者预后情况。 方法：回顾性分析104例成人间右半肝活体肝移植的资料,根据移植过程中供肝活检病理标本的脂肪变性程度将所有病例分成4个组。比较各组移植供受者移植后2周的肝体积增生率,分析104例成人间活体右半肝肝移植受者移植后死亡情况及原因。 结果与结论：4组病例在供受者移植后肝功能的恢复和受者后移植预后无明显差别,没有肝功能延迟恢复和原发无功发生。轻度大泡性脂肪肝者只要残肝足够可以成为合适的活体肝移植供者。使用轻度大泡性脂肪肝并不增加受者病死率和移植物失功。     

Successful liver transplantation after 21 days of hepatic coma

A 52-year-old women was treated after ingestion of different wild mushrooms. The case demonstrates that successful liver transplantation with full recovery of brain functions is possible even after 3 weeks of persisting severe hepatic encephalopathy.     

Ultrasound of living donor liver transplantation

Liver transplantation is the most effective treatment for various end-stage liver diseases. Living donor liver transplantation (LDLT) was first developed in Asia due to the severe lack of cadaveric graft in this region. The Liver Transplant Service at Queen Mary Hospital (QMH), Hong Kong, has pioneered the application of LDLT to patients using both left lobe and right lobe grafts. The QMH liver transplant programme is the largest of its kind in China and Southeast Asia.Ultrasound (US) is often employed in the initial work-up of potential donor and recipient of LDLT. It is the imaging technique of choice to assess the early and late complications of LDLT, with colour Doppler ultrasound being the most useful in the evaluation of post-LDLT vascular complications. The use of ultrasound contrast agents improves the visualisation of the hepatic vasculature, possibly delaying or removing the need for more invasive investigations. Intra-operative ultrasound facilitates the determination of the resection plane during donor hepactectomy.Computed tomography (CT) or magnetic resonance imaging (MRI) can be used as the single imaging modality in the evaluation of LDLT candidates.Ultrasound is most useful as the initial screening test in detecting hepatic parenchymal abnormalities, while CT or MRI is the modality of choice in the demonstration of vascular and biliary anatomy of the potential liver donor.Biliary complications are more common in LDLT than in cadaveric liver transplantation. The ductal dilatation, resulting from biliary stricture, is clearly demonstrated by ultrasound. Bilomas can be aspirated under ultrasound guidance to confirm the diagnosis and to promote healing. Perihepatic fluid collections and abscesses are also common after LDLT. Intra-hepatic collections may represent seromas, haematomas or infarction. Ultrasound is a sensitive means of detecting these collections and can be employed to guide drainage in suitable patients.Transplant-related malignancies include recurrent neoplasia and post-transplant lymphoproliferative disease (PTLD). Ultrasound can be used to screen for recurrent disease and to detect PTLD in the transplanted liver.     

Clinical anatomy related to the hepatic veins for right lobe living donor liver transplantation

The complexity of liver reconstruction has limited partial right lobe living donor liver transplantation. It is largely due to the difficulty of dealing with the middle hepatic vein. We sought to define the anatomic features of hepatic veins. Forty‐one fresh adult livers, 43 formalin‐fixed adult cadaver livers, and 91 adult liver corrosion casts were used for the study. We determined the number of branches, the maximum diameter, the whole length, the extrahepatic length of the hepatic veins, and the deviation of the middle hepatic vein from the main portal fissure. Nakamura and Tsuzuki's classification of hepatic vein types was used. Type A, B, and C accounted for 59.4, 27.8, and 12.8% of all specimens in this study, respectively. The middle and left hepatic veins formed a common trunk in 60.3% of the specimens, and the length of the common trunk was 1.12 ± 0.62 cm. The degree of deviation to the right of the middle hepatic vein from the main portal fissure was 14.11° ± 12.65°. The frequency of hepatic vein types and the degree of deviation to the right of the middle hepatic vein in this study is markedly different from that reported in other literature. The anatomic features of the hepatic veins in this study suggest that right lobe living donor liver transplantation is more suitable for Chinese. Clin. Anat. 2013. © 2012 Wiley Periodicals, Inc.     

肝中静脉属支解剖及其在活体肝移植中的意义

目的:为临床活体肝移植提供有关肝中静脉属支的形态学资料.方法:采用50例无病变成人尸体肝标本进行解剖剥离,对肝中静脉及属支进行观测,并采集相关数据进行统计分析.结果:肝中静脉主要属支为(5.26±1.16)支,直径大于5.00mm的肝中静脉主要属支的出现率为76.0%;肝Ⅳ段静脉血大多由肝中静脉回流;肝中静脉均参与肝Ⅴ段回流,Ⅴ、Ⅶ段由肝中静脉和肝右静脉共同引流者分别占66.0%和90.0%;肝Ⅲ段静脉单独由肝中静脉引流占10.0%;肝中静脉引流肝Ⅵ段静脉血者占22.0%.结论:肝中静脉主要属支数目不恒定,形态结构多异,临床活体肝移植术前应根据肝中静脉主干的汇合形式、主要属支的分型、管径及引流范围等,进行个性化分析,研究出较理想的手术方案.     

活体肝移植围手术期需要注意的几点病理学问题

活体肝移植是伴随日益突出的供体器官短缺而发展起来的一种肝脏移植手术方式。美国器官移植学会肝移植注册网等待肝移植的患者有近18000名,而每年能够获得的尸肝数量只有4500例。欧洲肝移植注册网每年大约有1100例患者可以得到尸肝移植,有300例以上患者在等待供肝中死亡。该现状促使移植工作者去探索以活体供肝为来源的手术方式。国际上最早的活体肝移植是在成人-儿童供受体间进行,[第一段]     

肝干细胞移植治疗大鼠暴发性肝功能衰竭的实验研究

目的探索肝干细胞移植对暴发性肝功能衰竭大鼠的治疗效果。方法以二乙酰氨基芴(15mg/kgBW)联合2/3肝切除建立雄性大鼠肝干细胞增殖模型,以改进Seglen胶原酶原位灌注及Percoll密度梯度离心方法分离、纯化肝干细胞,经肝脏注射移植(4×106细胞)治疗由D-氨基半乳糖诱导的暴发性肝功能衰竭雌性大鼠,移植前及移植后24、48、72h及1周取血测定肝功能指标如血氨、ALT、TBiL,移植后1、2、4周取受体肝脏组织应用PCR方法进行性别决定因子检测。结果肝干细胞移植可延长暴发性肝功能衰竭大鼠的中位生存时间(P<0.05),移植后肝干细胞移植组大鼠血清ALT、TBiL及血氨水平明显下降,其测定值各时间点组间比较,治疗组明显低于对照组(P<0.01);肝脏病理损伤减轻,肝干细胞移植2周后雌性受体肝脏组织中性别决定因子呈阳性表达,且随时间延长而表达增强。结论肝干细胞移植可延长暴发性肝功能衰竭大鼠中位生存时间,改善肝功能及病理指标。     

Successful domino liver transplantation in maple syrup urine disease using a related living donor

Maple syrup urine disease (MSUD) is an autosomal recessive disease associated withhigh levels of branched-chain amino acids. Children with MSUD can present severeneurological damage, but liver transplantation (LT) allows the patient to resume anormal diet and avoid further neurological damage. The use of living related donorshas been controversial because parents are obligatory heterozygotes. We report a caseof a 2-year-old child with MSUD who underwent a living donor LT. The donor was thepatient''s mother, and his liver was then used as a domino graft. The postoperativecourse was uneventful in all three subjects. DNA analysis performed after thetransplantation (sequencing of the coding regions of BCKDHA,BCKDHB, and DBT genes) showed that the MSUDpatient was heterozygous for a pathogenic mutation in the BCKDHBgene. This mutation was not found in his mother, who is an obligatory carrier forMSUD according to the family history and, as expected, presented both normal clinicalphenotype and levels of branched-chain amino acids. In conclusion, our data suggestthat the use of a related donor in LT for MSUD was effective, and the liver of theMSUD patient was successfully used in domino transplantation. Routine donorgenotyping may not be feasible, because the test is not widely available, and, mostimportantly, the disease is associated with both the presence of allelic and locusheterogeneity. Further studies with this population of patients are required toexpand the use of related donors in MSUD.     

Hepatotoxicity caused by both tacrolimus and cyclosporine after living donor liver transplantation.

We present a case report of a posttransplant patient who had hepatotoxicity due to both tacrolimus and cyclosporine and cholestatic jaundice due to tacrolimus. The patient did not show sustained improvement in enzyme and bilirubin abnormalities after an initial change from tacrolimus to cyclosporine or with a change back to tacrolimus, but he ultimately showed improvement when the blood concentration of tacrolimus was lowered. A 56-year-old man with subacute fulminant hepatitis induced by acarbose was admitted to our hospital for living donor liver transplantation. The liver graft consisted of the left lobe from his ABO-identical son. The early posttransplant course was uneventful. The serum levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and total bilirubin improved initially, but the ALT and AST levels later increased. A liver biopsy suggested a presumptive diagnosis of drug reaction. All drugs were discontinued, the immunosuppressive agent was changed from tacrolimus to cyclosporine. After initial improvement, the ALT and AST levels increased again. Assuming a reaction to cyclosporine, we decreased the concentration of cyclosporine in the blood. The enzyme levels improved temporarily but again began to rise. We changed the immunosuppressive agent to tacrolimus, which resulted in improvements in the ALT and AST levels; however, the total bilirubin level increased. We interpreted this increase as tacrolimus-induced cholestasis; in response, we decreased the blood concentration of tacrolimus to between 3 and 5 ng/dL and added 1,000 mg of mycophenolate mofetil to the drug regimen. The patient recovered without further complications. Repeated liver biopsies throughout the hospital course suggested that the mild mononuclear cell infiltration observed in a few triads had not been caused by acute rejection but had possibly been drug-induced.     

Living donor liver transplantation for congenital hepatic fibrosis in children

Congenital hepatic fibrosis (CHF) often accompanies autosomal recessive polycystic kidney disease (ARPKD), which stems from a PKHD1 gene mutation. The aim of this study was to clarify the prognosis of children with CHF who received living donor liver transplantation (LDLT) from donors who might be heterozygous carriers of a hepatorenal fibrocystic disease. Fourteen children with CHF who underwent LDLT at our center were enrolled. Eight and two patients had ARPKD and nephronophthisis, respectively. Eight of the donors were the recipients’ fathers, and six donors were their mothers. We examined the histological and radiological findings of the donor livers and complications in the recipients following the liver transplantation. Seven of the donor livers presented morphological abnormalities of the bile ducts. Abdominal computed tomography revealed liver cysts in eight donors. One recipient underwent re‐LT for graft failure due to rejection. Three patients presented with rejection, and one presented with sepsis. The overall survival rate was 100% and the original graft survival rate was 93%. In conclusion, the prognosis of recipients who received a LDLT from their parents for CHF was excellent. However, the morphology of half the donor livers was abnormal. Careful follow‐up is needed to ensure long‐term graft survival.     

活体肝移植肝中静脉的临床解剖研究

目的:为活体肝移植(LDLT)提供有关肝中静脉(MHV)的解剖学资料.方法:通过50例成人无病变肝标本剥离解剖,对MHV主干进行形态学观测,采集相关数据并进行统计分析.结果:MHV与肝左静脉(LHV)共干者为80.0%(40/50),单独开口者为20.0%(10/50),其中前者77.5%(31/40),后者90.0%(9/10)开口于IVC肝后段上份1:00点位;MHV依主干支数可分为三型;MHV主干、肝外段、外科干长分别为(68.3±15.4)mm,(6.9±2.8)mm,(8.1±6.2)mm;主干汇入IVC处(近端)、中点(1/2处)及起始端内径分别为(8.7±1.5)mm,(7.3±1.4)mm.(6.4±1.5)mm;MHV与IVC的夹角为(50.0±9.8)°;MHV主干上壁距IVC汇入口1～4 cm处及起始处至肝膈面的深度分别为(14.9±6.4)mm,(26.4±6.9)mm,(35.3±9.2)mm,(38.8±5.0) mm,(34.5±5.2)mm;MHV主干走行与传统定位肝中裂的"标志线"并非完全一致.结论:MHV汇入IVC的形式以MHV与LHV共干者居多;MHV单独汇入IVC者其开口部位较共干者有相对呈向前的趋势;MHV主干在肝膈面的投影多位于"标志线"的右侧.     

Activation of hepatic stellate cells in liver tissue of patients with fulminant liver failure after treatment with bioartificial liver

We studied the explanted livers from 12 patients with fulminant hepatic failure who were treated with a bioartificial liver and subsequently underwent orthotopic liver transplantation and from 18 patients who underwent orthotopic liver transplantation without previous treatment. Ten normal livers were used as controls. In addition to morphologic evaluation, an immunohistochemical analysis was performed with the monoclonal antibodies for alpha-smooth muscle actin and proliferation marker Ki-67. The expression of these markers was graded semiquantitatively from 0 to 3+ in a blinded fashion. The zonal distribution of activated hepatic stellate cells was also evaluated. In all cases, the hepatic stellate cells were activated and expressed alpha-smooth muscle actin. In all patients with submassive or massive liver cell necrosis, the distribution of activated hepatic stellate cells was predominantly in zone 1 of the acinus (periportal area). In contrast, in cases with early nodular regeneration and no significant fibrosis, the activated hepatic stellate cells were distributed throughout the liver parenchyma, involving zones 2 and 3 of the acinus. Expression of the proliferation marker Ki-67 was graded 3+ in all patients treated with the bioartificial liver who had orthotopic liver transplantation and 2+ in patients who underwent orthotopic liver transplantation only.     

Revolution and refinement of surgical techniques for living donor partial liver transplantation

Living donor liver transplantation (LDLT) was first successfully performed on a child in 1990 and the Shinshu group performed the same procedure on an adult for the first time in 1994. Over the past few years adult LDLT has been increasing worldwide because of the severe shortage of cadaveric organs, especially in locations where the transplantation of organs from brain-dead donors is rarely practiced. The surgical procedures for LDLT are more technically challenging than those for cadaveric whole liver transplantation. LDLT requires a full understanding of hepatobiliary anatomy and continuous technical refinement of the procedure. The development of innovative techniques is a key factor for a successful LDLT. Some of the technical highlights include selective vascular occlusion techniques for donor hepatectomy, hepatic arterial reconstruction under the microscope, the introduction of intraoperative ultrasound, graft volume estimation, hepatic venous reconstruction using cryopreserved vascular grafts, and the use of the right lateral sector of the liver. These techniques have improved the success rate of LDLT over the past few years. This review focuses on the surgical techniques for LDLT on the basis of our experience with adult LDLT at the Tokyo University Hospital.