小儿亲体部分肝移植的手术要点探讨

目的 探讨小儿亲体部分肝移植的手术技术要点。方法 对2例肝功能衰竭患儿进行亲体部分肝移植术，供体分别为患儿的母亲和父亲。手术分别切取供体的右半肝和左外侧叶。受体行保留下腔静脉的全肝切除术，然后将供肝进行原位移植。结果 两例患儿分别移植肝重565克和329克，供体和受体术后已经分别随访5．5个月和6．5个月，均无并发症健康生活。受体未发生肝静脉、门静脉、肝动脉和胆管吻合口的并发症。结论 亲体肝移植是治疗小儿终末期肝病的有效方法，术前准确的血管影像学检查，严格的围手术期管理特别是精确的手术技术是小儿亲体肝移植成功的关键。     

亲体肝移植术后并发噬血细胞综合征的治疗

目的 总结儿童重型肝炎行儿童亲体肝移植术后并发噬血细胞综合征的治疗经验.方法 1例6岁女孩,因不明原因急性进行性黄疸、腹水及全身出血等住院,经化验、彩色多普勒、螺旋CT等确诊为亚急性重型肝炎,于2008年2月26日施行亲体肝移植术,供肝为患儿父亲的左外叶.术后3 d开始出现全血象进行性下降,13 d降至最低,血清铁蛋白升高,EB病毒DNA阳性,骨髓细胞学检查发现噬血细胞,确诊为儿童亲体肝移植术后EB病毒相关性噬血细胞综合征,用环孢素A、地塞米松和静脉注射丙种球蛋白等治疗,并进入层流病房,加强抗感染治疗.结果 术后患儿肝功能恢复顺利,各种酶学指标术后3d各种开始下降,7d接近正常,14d完全正常.术后20 d全血象开始逐渐上升,40 d升至正常水平,准予出院随访,至今无复发,情况良好.结论 儿童亲体肝移植手术后出现不明原因的全血象下降时应想到并发噬血细胞综合征的可能,早期诊断,及时正确的治疗能使患儿顺利康复.     

儿童亲体肝移植围手术期并发症分析

目的探讨儿童亲体肝移植围手术期并发症的原因及处理措施,为提高儿童亲体肝移植的临床疗效提供依据。方法回顾性分析2006年6月至2009年3月本院25例亲体肝移植患儿围手术期临床资料。其中胆道闭锁合并胆汁性肝硬化15例,肝豆状核变性（Wilson’s病）3例,肝糖原累积症2例,门静脉海绵样变性3例,急性药物性肝功能衰竭1例,原发性肝硬化1例。对其并发症的种类及原因进行分析,总结治疗措施。结果25例手术均获成功,围手术期死亡3例,其中1例于术后3d死于门静脉血栓;1例亲体肝移植术后7d出现肝动脉血栓形成,再次行尸体肝劈离式肝移植,术后1个月因消化道出血而死亡;1例术后感染曲霉菌死亡;其余22例痊愈出院。结论正确有效的围手术期处理及术后治疗,是儿童亲体肝移植成功的关键。     

亲体部分肝移植治疗小儿肝豆状核变性病

目的：通过亲体部分肝移植的方法治疗小儿肝豆状核变性病（Wilson‘s病），探讨Wilson‘s病的根治方法。方法：对术前均明确诊断为Wilson‘s病的患儿（男1例，女4例）施行亲体部分肝移植，患儿平均年龄9岁，本组供肝者均为患儿母亲，Child分级：B组4例，C级1例，除1例患儿血型为B型，供体血型为O型外，其余均为供受体血型相同，结合临床资料对本组所有Wilson‘s病患儿进行回顾性分析。结果：所有供，受体手术顺利，术后恢复良好，5例Wilson‘s病患儿术后3周患儿进行回顾性.分析结果：所有供，受体手术顺利，术后恢复良好，5例Wilson‘s病患儿术后3周肝功能恢复正常，复查铜氧化酶均正常，K－F环均不同程度变浅，神经系统症状有所好转，术后并发症，1例出现肝动脉栓塞（术后第6d再次行减体积肝移植），术后分别随访12．5个月，11个月，10个月，9个月和7．5个月，现均已康复出院。结论：亲体部分肝移植是根治Wilson‘s病等代谢性疾病的有效方法。     

活体肝移植治疗肝豆状核变性5例

目的 探讨活体肝移植治疗小儿肝豆状核变性的临床疗效。方法 采用亲体部分肝移植术及术后监测临床生命体征、血生化指标,抗感染、免疫抑制、营养支持等综合方法,治疗肝豆状核变性5例。结果 供体术后顺利康复;患儿术后健康存活,至今分别为3(3例)、2、1年。肝脏功能、血铜蓝蛋白已恢复正常,正常饮食,均无复发。结论 活体肝移植可纠正小儿肝豆状核变性的肝脏病变及原有的代谢缺陷,提高患儿生活质量,挽救其生命,是治疗小儿肝豆状核变性终末期肝病的一种有效方法。     

婴幼儿亲体部分肝移植

目的回顾性分析我院2002年10月-2005年10月6例婴幼儿亲体部分肝移植临床疗效。方法婴幼儿亲体部分肝移植6例，年龄9个月～3岁，男3例，女3例；手术时体重为7～16kg。胆道闭锁Kasai术后肝硬化腹水4例，先天性肝内胆汁淤积症、肝硬化腹水1例，药物性肝炎1例。随访时间为6～36个月。结果供肝提供者分别为母2例，父4例，提供左外叶肝，平均住院7d，无并发症。5例生存，最长36个月。1例因凝血功能障碍，术后24h肾功能衰竭、肺出血死亡。术后免疫治疗药物包括甲基强的松龙、FK506和赛尼派。术后并发症为2例胆瘘，术后出血、再次进腹止血1例，肺部感染1例。结论婴儿期进行部分活体肝移植，扩大了供体来源，临床疗效较好，是婴儿终末期肝病有效的治疗方法。     

犬部分肝移植模型的实验研究

目的建立犬部分肝移植的动物模型。方法杂交犬124只随机分两组：供体组62只和受体组62只；供体组又随机分为活体供肝组12只和尸体供肝组50只；活体供肝组行标准左半肝切取术。尸体供肝组行原位肝脏灌注后，切取肝脏，然后去除右叶、方叶和尾状叶，保留门静脉、肝动脉和胆管于左叶待用。受体组行“H”型分流术和背驼式左半肝原位植入术。结果活体供肝组12例：术中死亡2例，术后存活10例。供肝的热缺血时间几乎为零，冷缺血时间平均29．5min。尸体供肝组50例：供肝的冷缺血时间平均47．5min。受体组犬62例：受体犬手术时间平均5．6h，无肝期时间平均45min。术中死亡5例，其中创面出血3例，下腔静脉吻合口出血2例。术后存活57例。57例肝脏血管重建后即可有胆汁从肝管中渗出。结论犬是理想的部分肝移植动物模型，“H”型分流可有效维持受体无肝期血循环稳定，免除了门腔转流的操作。为临床开展亲体和小儿部分肝移植提供了技术训练和经验的参考。     

小儿尸体肝移植探讨

目的　本研究探讨尸体肝移植治疗小儿终末期肝病的方法和疗效。方法　我院分别于2 0 0 2年 4月～ 2 0 0 2年 12月对 5例患儿 (先天性胆道闭锁 4例 ,肝脏血管内皮肉瘤 1例 )进行肝移植术 ,患儿年龄 6个月～ 5岁。减体积肝移植 3例 ,取左半肝和左外侧叶为移植物。割离式肝移植 2例 ,分别取左外后叶和右后叶为移植物。受体行保留下腔静脉的全肝切除术 ,然后将供肝进行原位移植。结果　 5例患儿移植肝重 2 6 0～ 5 6 5 g ,占受体体重1.94 %～ 5 .4 6 % ,4例术后已经健康存活 7～ 15个月 ,1例于术后第 5d死于心功能衰竭。结论　尸体肝移植是治疗小儿终末期肝病的有效方法 ,严格的围手术期管理特别是精确的手术技术是小儿肝移植成功的关键。     

儿童肝移植20例诊治分析

目的回顾性分析20例肝移植患儿的临床资料,旨在总结儿童肝移植的临床诊治经验。方法回顾性分析2017年5月至2019年6月湖南省儿童医院联合中南大学湘雅附二医院对20例儿童实施肝脏移植手术的临床资料,原发疾病主要包括胆道闭锁、Alagille综合征、先天性肝内门静脉海绵样变性。手术方式采用亲体部分肝移植手术(n=16)和儿童心脏死亡器官捐献(donation after cardiac death,DCD)肝脏移植手术(n=4)。20例均应用他克莫司联合激素免疫抑制方案,其中3例增加了吗替麦考酚酯联合抗排斥反应。术后患儿给予抗感染、抗排斥等对症支持治疗,密切监测肝血管吻合处血流情况及肝功能变化,观察术后并发症及预后。结果 20例患儿均移植成功,肝移植供体均康复出院,无并发症发生。受体术后早期主要并发症为感染,以细菌感染为主,感染部位为肺部和腹腔。术后1例出现肝动脉栓塞,3例出现乳糜漏,2例出现胆道狭窄,3例出现早期排斥反应,1例出现消化道大出血,经对症治疗均痊愈出院,检测肝功能及血药浓度均在正常范围。结论儿童终末期肝病可通过活体肝移植或DCD肝移植手术取得理想的效果,手术方式可根据患儿的年龄、体重进行选择。     

亲体肝移植成功治疗婴儿先天性胆道闭锁一例

胆道闭锁(biliary atresia,BA)是婴幼儿肝移植的主要人群,但对小年龄、低体重婴儿进行肝移植手术难度很大.2009年3月12日我院成功地为1例出生仅8()d、体重3.98 kg的患BA男婴进行了亲属供肝活体肝部分移植术(living related liver transplahtation,LRLT),术后供受者恢复顺利,目前已出院并健康生活至今.     

Hepatoblastoma in a child with progressive familial intrahepatic cholestasis

End-stage liver cirrhosis because of metabolic or infectious diseases predisposes to hepatic malignancies like hepatocellular carcinoma. We report the first case of hepatoblastoma incidentally detected in the explanted liver of a 2-yr-old child undergoing liver transplantation for cirrhosis because of progressive familial intrahepatic cholestasis (PFIC). The diagnosis was difficult to obtain. The hepatoblastoma was not seen on ultrasound examination of the cirrhotic liver. As we could confirm retrospectively, alpha fetoprotein (AFP) was found elevated prior to transplantation. Two years after successful transplantation, there are no signs of malignancy detectable by clinical and radiological methods. We conclude from this case that PFIC may induce hepatoblastoma and that children with liver cirrhosis should undergo routine screening of serum AFP concentration.     

The use of everolimus in pediatric liver transplant recipients: first experience in a single center

The role of mTOR inhibitors, such as EVL, has not been established for pediatric liver transplant recipients up to now, although data from adult solid organ graft transplantation are very promising. Major complications following pediatric liver transplantation in the long-term course include chronic graft rejection and CNI-derived nephrotoxicity. The purpose of our study was to report first results using EVL as a rescue therapy in pediatric liver transplant recipients for the following indications: chronic graft dysfunction n=12, suspected CNI toxicity n=3, hepatoblastoma n=2, and recurrence of primary sclerosing cholangitis post-Ltx n=1. Four patients with chronic graft dysfunction developed completely normal liver function tests using EVL, six patients showed partial improvement, and two patients did not respond at all. One patient with CNI-induced nephropathy showed a slightly improved GFR. Both patients with hepatoblastoma did not develop any metastasis post-Ltx. First experience with EVL in pediatric liver transplant recipients shows promising results in patients with chronic graft failure when standard immunosuppression has failed. The future role of EVL in immunosuppressive protocols for children post-Ltx has to be proven by controlled clinical trials.     

Successful autologous peripheral blood stem cell transplantation with a double-conditioning regimen for recurrent hepatoblastoma after liver transplantation

Abstract:  A four-yr-old boy developed a solitary metastasis nine months after living-related liver transplantation for unresectable hepatoblastoma. After resection of the metastatic lesion, he received an auto-PBSCT with a double-conditioning regimen consisting of melphalan and thiotepa. Auto-PBSCT could be safely performed without any serious regimen-related toxicity or infection. However, transient cessation of tacrolimus during myelosuppression resulted in graft rejection of the liver just after hematological engraftment, but rejection was resolved by tacrolimus and methylprednisolone. The patient is alive and free from disease two yr after auto-PBSCT without any signs of graft rejection. High-dose chemotherapy using this conditioning regimen may be feasible for recurrent hepatoblastoma after liver transplantation in terms of safety and anti-tumor activity.     

Risk of hearing impairment in pediatric liver transplant recipients: a single center study

As survival rates following liver transplantation have increased, health care providers must assess the impact of transplantation on dimensions other than traditional medical measures. Hearing impairment may adversely impact social, emotional, cognitive, academic, and speech and language development. We hypothesized that children who undergo liver transplantation are at risk for hearing impairment due to exposure to ototoxic drugs. We conducted a review of 74 children who had undergone liver transplantation between December 1996 and September 2000 at Cincinnati Children's Hospital Medical Center. Hearing was assessed at discharge by an audiologist using age and developmentally appropriate techniques. The principal outcome measure was sensorineural hearing impairment. Independent variables were age at transplantation, United Network for Organ Sharing (UNOS) status at transplantation, primary diagnosis, post-transplant length of hospital stay, days of treatment with aminoglycosides, and days of treatment with loop diuretics. Eleven of 74 children (15%) had sensorineural hearing loss, of whom four had severe to profound hearing loss. Multivariate analyses showed that the adjusted relative risk for hearing loss in patients with hepatoblastoma was 66 and that there was a 5% increase risk for hearing loss for each additional day of hospitalization. Age at transplantation, UNOS status, and days of treatment with loop diuretics or aminoglycosides did not achieve significance in the model. Sensorineural hearing impairment occurs in a subset of pediatric patients following liver transplantation. Patients with hepatoblastoma or those who experience prolonged hospitalization after transplantation are at increased risk. Our observations are of particular importance for pediatric liver transplant recipients since the median age at transplantation is 12-18 months, a critical period for language acquisition.     

Chemoresistant hepatoblastoma in a patient with mosaic trisomy 18 treated with orthotopic liver transplantation

We present a 9-month-old male with mosaic trisomy 18 with a right hepatic lobe mass. The tumor was completely resected and identified as pure fetal histology hepatoblastoma but contained increased mitotic activity. Adjuvant chemotherapy consisted of cisplatin, vincristine, and 5-fluorouracil. After the first and fourth cycles of chemotherapy, recurrent tumor developed. The patient underwent rescue orthotopic liver transplantation, and is currently alive without evidence of hepatoblastoma 28 months after transplantation. This report demonstrates the use of orthotopic liver transplantation in a child with mosaic trisomy 18 and hepatoblastoma.     

Surgical treatment of hepatoblastoma in children

Hepatoblastoma is the most common liver malignancy in children. With rare exceptions, complete tumour resection is required to cure the patient. Radical tumour resection can be obtained either with standard partial hepatectomy or orthotopic liver transplantation. At present, the surgical approach to hepatoblastoma differs significantly between treatment groups in different parts of the world. Our aim was to review current surgical policy in hepatoblastoma. All aspects of surgery in hepatoblastoma are discussed, including biopsy, tumour resection principles, modern achievements in the field of liver surgery, and the indications and potential contraindications for liver transplantation. Every effort should be made to resect hepatoblastoma completely either by standard partial hepatectomy or by the use of liver transplantation in difficult or clearly unresectable cases.     

Use of a donor iliac vein graft for reconstruction of the inferior vena cava in liver transplantation for hepatoblastoma with caval extension

Complete microscopic tumor resection is critical for successful treatment of hepatoblastoma, and this may include when liver transplantation is required. For tumors involving the IVC or PV, complete resection should include the involved IVC or PV to ensure full tumor clearance. When this is required, the venous reconstruction at transplant or post‐excision can be challenging. We present the management of an 18‐month‐old girl with PRETEXT Stage IV (P, V, F) hepatoblastoma and IVC involvement, where native caval resection and reconstruction was required. The preoperative staging following neoadjuvant chemotherapy was POSTTEXT Stage IV (P, V, F). An orthotopic liver transplantation was performed using a left lateral segment graft from a deceased adult donor. With native hepatectomy, retrohepatic IVC resection from just above the hepatic venous confluence to just above the entry of the right adrenal vein was performed. For caval reconstruction, a venous graft from a deceased donor was used. The graft included the lower IVC with the right common iliac vein and a short stump of the left common iliac vein. The common iliac was a perfect size match for the IVC, and the three natural ostia matched the upper cava, lower cava, and the outflow from the donor left hepatic vein. The patient had an uneventful postoperative course and remains well and disease‐free 2 years after transplant with continued patency of the reconstructed cava. When indicated, a donor iliac vein graft with its natural ostia should be considered in caval reconstruction for pediatric liver transplantation.     

The efficacy of liver transplantation in malignant liver tumors associated with tyrosinemia: clinical and laboratory findings of five cases

To evaluate clinical and laboratory findings of these patients and the efficacy of liver transplantation in children with hepatocellular carcinoma (HCC) and hepatoblastoma (HB) associated with tyrosinemia. Among 113 children with liver tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular clinic visits for tyrosinemia, elevated alpha-fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given chemotherapy including cisplatin and adriamycin. In three patients, living-related liver transplantation was performed. They had no treatment after transplantation. All of them are disease free. One patient was treated with chemotherapy and right hepatectomy. She had no suitable donor for living-related liver transplantation. Three months after completing chemotherapy, she had recurrent tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living-related liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for tyrosinemia. In these patients liver transplantation is curative both for liver tumor and tyrosinemia.     

Transmission of clear cell tumor in a graft liver from cadaveric donor: Case report

Backes AN, Tannuri ACA, de Mello ES, Gibelli NEM, de Castro Andrade W, Tannuri U. Transmission of clear cell tumor in a graft liver from cadaveric donor: Case report. Abstract: Neoplasms in children after organ transplantation are related to the type and intensity of immunosuppression and the donor–recipient serostatus, especially in relation to the Epstein–Barr virus. The patient was a two‐yr‐old female child with biliary atresia who underwent a liver transplantation from a female cadaver donor. Two adults received kidney transplants from the same donor. Nine months after transplantation, one of the adult recipients developed an urothelial tumor in the kidney graft. Imaging tests were repeated monthly in the liver‐transplanted child and revealed no abnormalities. However, one yr and two months after the transplantation, the patient developed episodes of fever. At that time, imaging and liver biopsy showed a clear cell tumor of urothelial origin in the graft and the disease was limited to the liver. The patient underwent liver retransplantation, and she is currently free of tumor recurrence. Although rare, the occurrence of tumors in the post‐transplant period from cadaver donors, without previously diagnosed tumors, is one of the many problems encountered in the complex world of organ transplantation.     

Auxiliary partial orthotopic liver transplantation for acute liver failure in two children

Acute liver failure in children and adults is associated with a high mortality rate. At present the treatment of choice is orthotopic whole-liver transplantation. However, allogeneic liver transplantation necessitates lifelong immunosuppressive therapy, which is associated with substantial risks to the patient. Temporary auxiliary partial orthotopic liver transplantation has been developed recently as an alternative, enabling the native liver to regenerate while avoiding the risks of long-term immunosuppressive treatment. Here we describe two cases of partial orthotopic liver transplantation in children. Auxiliary partial orthotopic liver transplantation was performed in two boys (5 and 6 years old) suffering from acute liver failure of unknown origin. The native left lateral liver lobes (segment II and II) were removed and replaced by left lateral liver grafts from young blood-group-compatible adults. In the first child the native liver, which was 80% necrotic at time of transplantation, showed regeneration within two weeks and the partially necrotic graft could be surgically removed on day 15 after auxiliary transplantation. Four years after transplantation, the child is in excellent condition with normal liver function and does not require any treatment. In the second case the native liver (90% necrotic at time of transplantation) regenerated within 6 weeks of transplantation, at which time the transplanted liver was removed. The patient developed aplastic anemia and died 2 months after transplantation from candida sepsis. The conclusion was that auxiliary partial liver transplantation in childhood provides a valuable option to maintain liver function in acute liver failure until functional recovery of the native liver. The main advantage over whole-liver transplantation is the chance to avoid lifelong immunosuppression. However, there is a higher surgical risk. Therefore, auxiliary transplantation should be considered carefully in every case of acute liver failure in children.