肝移植术后肝动脉狭窄的介入治疗和再移植时机

目的探讨肝移植术后肝动脉狭窄(HAS)的介入治疗效果和再次肝移植的时机。方法回顾性分析2 0例原位肝移植术后HAS患者的临床资料。所有病例经数字减影血管造影(DSA)确诊,均行血管内介入治疗。其中6例接受了再移植。介入治疗方法包括经皮腔内血管成形术(PTA)和支架植入术。结果早期HAS(移植后4周内)8例,介入治疗后肝功能好转6例,1例肝功能恶化及时再移植存活,另1例因肝衰竭死亡。1 2例晚期HAS中1例介入后于术后3 8 d死于严重感染和多器官衰竭;5例因肝功能反复异常、胆道缺血型狭窄和反复胆道感染而接受再移植,再移植围手术期死亡1例。2 0例平均随访13个月,2例分别因原发性肝癌和严重胆道感染死亡;3例出现肝动脉再狭窄并再次动脉内介入治疗成功。共出现缺血型胆道病变8例(4 0.0%)并行胆道介入治疗,5例肝功能改善。早期HAS和晚期HAS的1年和2年累计生存率分别为8 7.5%,4 3.8%和8 1.5%,5 4.3%,两组无统计学差异(P=0.9 7 6)。结论肝移植术后HAS未引起严重肝功能损害时,首选介入治疗,但缺血型胆道病变发生率较高。再次肝移植是治疗肝移植术后HAS导致不可逆性肝功能损害时的惟一有效手段     

胆道闭锁的早期诊断与治疗

胆道闭锁 (Biliary Atresea BA)是新生儿期阻塞性黄疸的常见病因之一 ,其发病率约占出生存活小儿的 1 /1 4万～ 1 /8千。因此在我国并非少见。由于病因不明 ,就诊时晚期病例多 ,治疗效果不满意 ,一直都是小儿外科关注的重点。1　早期诊断1 .1　早期诊断的重要性由于肝内或肝内外胆道闭锁、胆汁淤积、胆汁性肝硬化出现早 ,病情呈进行性发展。日龄超过 3个月 ,肝脏病变不可逆转。有报告 :手术 (Kasai手术 )日龄在 6 0 d以内者 ,82 %～ 90 %术后可获胆汁引流 ,黄疸消退率达 5 5 %～ 6 6 %。随着手术日龄增加 ,手术成功率下降 ,超过 1 2 0 d尚…     

香港小儿肝移植的并发症和预后

在 1 993年～ 2 0 0 1年共有 2 9位患儿行了 31例次肝移植手术 ,平均年龄 1 6个月 ,病因分别为胆道闭锁 (1 9例 )、暴发性肝衰 (7例 )、肝脏代谢性疾病 (2例 )和 Allagille综合征 (1例 )。活体肝移植是最主要的移植术式 ,占 71 % ,减体积肝移植占 1 6 % ,其余 3%为尸体肝移植。     

劈离式肝移植治疗儿童胆道闭锁的临床效果研究

目的比较劈离式肝移植(SLT)和活体肝移植(LDLT)治疗胆道闭锁患儿的临床效果。方法回顾性分析2017年6月至2022年5月在中山大学附属第三医院肝脏外科暨肝移植中心行SLT的64例胆道闭锁患儿及同期实施LDLT的44例胆道闭锁患儿的临床资料。其中接受SLT的患儿中, 男40例, 女24例, 移植时年龄为4～168个月, 中位年龄为8个月;接受LDLT的患儿中, 男24例, 女20例。移植时年龄为4～24个月, 中位年龄为7个月。将64例胆道闭锁患儿根据接受SLT手术时间的先后分为两组:SLT早期组32例(2017年6月至2019年1月)和SLT技术成熟期组32例(2019年2月至2022年5月)。LDLT组和SLT组及SLT早期组和SLT技术成熟期组中胆道闭锁患儿移植肝功能恢复情况的比较采用秩和检验或t检验。术后并发症发生率的比较采用χ2检验或Fisher 确切概率法。生存分析采用 Kaplan-Meier 法和Log-rank 检验。结果 LDLT组冷缺血时间[M(IQR)][218(65)min]、术中出血量[175(100)ml]及移植物与受者体重比(3.0±0.7)低于SL...     

原位肝移植术后胆道并发症的预防与诊治

目的　探讨肝移植术后胆道并发症的预防、诊断和治疗。　方法　对 1993年 4月～2 0 0 1年 11月我科实施的 12 3例肝移植患者临床资料进行回顾性分析。　结果　 12 3例肝移植患者中11例通过胆道造影确诊为肝移植术后胆道并发症 ,9例治愈 ,1例好转 ,死亡 1例。胆道并发症发生率为 8 9% ( 11/ 12 3) ,与胆道并发症相关的死亡率为 0 8% ( 1/ 12 3) ,与T管相关的胆道并发症发生率为4 2 % ( 5 / 119) ,与肝动脉供血相关的胆道并发症发生率为 1 6 % ( 2 / 12 3)。热缺血时间 >3min、冷缺血时间 >8h组胆道并发症发生率明显升高 (P <0 0 5 )。　结论　保存性损伤和缺血性损伤是肝移植术后胆道并发症的重要原因。修肝时应维护肝外胆管的血供和警惕变异胆管的存在。改进T管置管方法可显著降低与T管相关的胆道并发症发生率。术后早期胆道造影有助于及时诊断胆道并发症。介入技术是胆道并发症的主要治疗手段。     

胆道闭锁肝内胆管Bcl-2与Bax基因表达的变化及其意义

本研究拟对1 4例胆道闭锁患儿肝内Bcl 2、Bax基因表达变化进行研究,并对这些患儿的临床治疗效果进行评估,以了解这些基因的表达变化与近期疗效之间的关系。资料与方法1 .一般资料:病变组:取自我科2 0 0 0年9月至2 0 0 2年1月的1 4例术中确诊为胆道闭锁的肝脏组织标本。1 4例患儿就诊年龄为1 9～1 74d ,平均(75±4 2 )d ,其中男8例,女6例。术中在肝右叶切取1cm×0 5cm×0 5cm大小的肝组织,置1 0 %中性福尔马林液体中固定2h。对照组:取自同期的1 2例新生儿手术标本,这些患儿均排除肝胆疾患。标本的采取及固定方式同上。2 .方法:Bcl 2第1抗…     

儿童肝移植20例诊治分析

目的 总结儿童肝移植的临床疗效,探讨儿童肝移植在适应证、手术方式及术后处理等方面的特点.方法 2000年8月至2007年7月对20例儿童(＜14岁)施行肝移植手术23次,患儿年龄6个月～13岁,原发疾病主要包括先天性胆道闭锁、Wilson病、肝糖原累积症和尿素循环障碍.手术方式采用亲体部分肝移植4次,多米诺肝移植1次,劈离式肝移植5次,减体积肝移植10次和尸体全肝移植3次.除1例患儿应用环孢霉素外,其余19例患儿均应用他克莫司,联合激素及霉酚酸酯三联免疫抑制方案.结果 患儿围手术期内死亡3例(15.0%),死亡原因分别为移植肝原发无功、心功能衰竭和腹腔感染.9例患儿术后出现不同的并发症,包括肝动脉血栓形成2例、门静脉血栓形成1例、急性排斥反应1例、胆漏3例、胆道狭窄2例、肠瘘2例、腹腔感染3例、肺感染1例和心功能衰竭1例.患儿术后6个月、1年和2年累积生存率分别为80.0%、73.9%和73.9%.结论 肝移植是治疗儿童终末期肝病的有效方法,手术方式可根据患儿的年龄、体重进行选择.     

胆道闭锁患儿肝移植术后门静脉狭窄的血管腔内治疗

目的探讨血管腔内治疗在胆道闭锁患儿肝移植术后门静脉狭窄(PVS)治疗中的应用价值。方法收集因原发病为胆道闭锁接受肝移植、术后后发生PVS的患儿14例,均经门静脉造影证实,并接受经皮血管成形术和(或)经皮血管内支架成形术治疗。分析14例患儿血管腔内介入治疗的效果。结果 14例患儿共进行23次血管内腔内介入治疗,技术成功率82.61%(19/23)。10例患儿经1~2次球囊扩张治疗后治愈,4例患儿球囊扩张治疗后,行血管腔内支架成形术,支架植入后未发生狭窄。14例患儿均未出现治疗相关并发症。结论胆道闭锁患儿肝移植术后PVS的血管腔内介入治疗安全、有效。     

原位肝移植治疗先天性胆道闭锁(文献综述)

1959年前,先天性胆道闭锁为不治之症,一般于出生后12～24个月由于胆管炎、肝功能衰竭或出血而死亡。1959年,Kasai创用了肝门空肠Roux-en-Y吻合术治疗本病。后来又有人采用皮肤空肠造口或双桶空肠造口对Kasai氏术进行改良,使术后胆管炎发病率降低。但Kasai氏术的成功率仅为20～50%,一般为30%。而Kasai氏术失败者,90%患儿将在5岁内死亡,从而推动了开展肝移植治疗本病。手术适应证及手术时机在小儿肝移植中,胆道闭锁是最主要的手术适应证。Brolsch等报告8例中,3例系胆道闭锁。Asc-her等报告30例中,8例为肝外胆道闭锁,男性2例,女6例,年龄为8～19个月,体重5.4～10公斤。8例移植前均施行过Kasai氏手术,其中1次手术者     

新生儿小肠闭锁8例诊治体会

目的 探讨新生儿小肠闭锁的诊断及手术治疗方法.方法 总结分析收治的8例新生儿小肠闭锁相关的诊断及手术方式资料.结果 8例其中十二指肠降段闭锁3例,空肠闭锁5例.均行手术治疗,其中3例术后5～6 d 2次手术.8例患儿术后恢复良好,随诊2～5 a效果良好,发育均正常.结论 新生儿小肠闭锁是新生儿常见的消化道畸形,早期诊断...     

The efficacy of hepatoportoenterostomy in biliary atresia

This report describes the treatment and outcome of 66 infants with biliary atresia. Mean age was 79.8 +/- 33.2 days. Diagnosis was achieved by 99mTc DISIDA scanning. Hepatoportoenterostomy (HPE) was performed in 48 cases and hepatoportocholecystostomy in four, with microscopic ducts at the porta hepatis. Fourteen infants without microscopic ducts did not undergo HPE. Patients were staged according to the postoperative result. HPE was successful in 25% of patients (group A), resulted in improvement in 19% (group B), failed in 43% (group C), and was short-term in 13% (group D). In patients less than 90 days of age, the HPE success rate was 31%; 23% improved, and 33% showed no improvement. Age (less than 90 days) and bile clearance were prognostic determinants of success. Reoperation was useful only in patients with a previously successful HPE. Ten of 20 patients referred for liver transplantation survived (50%) (7/11) survived after liver transplantation and 3/9 on the waiting list). Fourteen of 15 patients in group A remain anicteric and well without liver transplantation. Patients in group B have had extended survival (greater than 3 years) but eventually required transplantation. Patients in group C and children more than 90 days old at diagnosis require early liver transplantation. HPE is a useful procedure when performed in infants less than 90 days of age who have biliary atresia.     

Outcome of surgery for biliary atresia

Forty-seven infants (26 male, 21 female) with biliary atresia under- went hepatic portoenterostomy during the 16-year period 1971-87. Twenty-six patients (55%) are alive 1-17 years after surgery, with 21 (45%) being jaundice-free. For children who became jaundice-free, the mean age at surgery was 78 days (range: 34-125 days), compared with 97 days (range: 48-224 days) for those who did not. Of 39 patients operated on at less than 120 days of age, 24 (60%) are alive. All four patients operated on after 125 days of life died. Of 31 patients operated on more than 5 years ago, 12 (39%) have survived, the oldest being 17 years. Ten (32%) have normal serum bilirubin concentrations, have non-active cirrhosis on liver biopsy, have had normal growth and development, and lead normal lives. The oldest two patients suffered variceal haemorrhage in their teenage years. In our recent experience, 11 of 16 patients (69%) have had complete clearing of jaundice, lead normal lives and do not currently require assessment for liver transplantation. It is believed that early referral of children with biliary atresia to experienced surgical units for portoenterostomy will lead to long-term survival, without the need for liver transplantation in a majority of cases. Liver transplantation should be offered in infancy only after failed portoenterostomy, except for patients presenting after 120 days in whom transplantation may be considered primary therapy.     

胆道闭锁的外科诊治进展

胆道闭锁是一种病因不明、以胆道梗阻为特点的儿童先天性罕见疾病,主要表现为肝内外胆道系统的进行性炎症以及纤维性梗阻,进而发展为胆汁淤积性肝硬化、肝衰竭。胆道闭锁虽然罕见,但它是婴幼儿胆汁淤积性疾病的最常见原因,如不经手术治疗（如Kasai手术、肝移植手术）,患儿通常可在短期内死亡。Kasai手术可改善胆道引流,但不能改变胆道闭锁患儿的结局,大多数仍会因为胆汁淤积而遭受持续的肝损伤,最终需要进行肝移植。而目前关于肝移植术前是否行Kasai手术尚未达成一致意见。本文就胆道闭锁的诊断、外科治疗现状等进行综述,以期为临床胆道闭锁的诊断和治疗提供参考,改善胆道闭锁患儿的生存。     

Orthotopic liver transplantation for biliary atresia: the U.S. experience.

Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in the pediatric population. The outcomes of liver transplantation for biliary atresia, however, have not been formally examined on a national scale. The objective of this study was to identify pretransplant variables that predict patient survival after primary liver transplantation for biliary atresia. A cohort of 1,976 pediatric patients undergoing primary liver transplantation for biliary atresia between 1/1988 to 12/2003 was enrolled from the United Network for Organ Sharing database after excluding patients with a history of multiorgan transplant or previous liver transplant. Follow-up data up to 16 years post-OLT was available. The 5- and 10-year actuarial survival rates of patients that underwent liver transplantation for biliary atresia in the United States are 87.2% and 85.8%, respectively, and the 5- and 10-year graft actuarial survival rates are 76.2% and 72.7%, respectively. Early deaths (< or =90 days post-OLT) were more often caused by graft failure (P = 0.01), whereas late deaths (>90 days post-OLT) were more often due to malignancy (P < 0.01). An analysis of outcomes over time demonstrated a decrease in post-OLT survival and an increase in the number of OLTs done for biliary atresia at an increasing number of centers. A multivariate analysis revealed that cadaveric partial/reduced liver grafts, a history of life support at the time of OLT, and decreased age were independent predictors of increased post-OLT mortality. In conclusion, OLT is an effective treatment for biliary atresia. Certain pretransplant variables may help predict patient survival following liver transplantation for biliary atresia.     

Surgical experience in children with biliary atresia treated with portoenterostomy

OBJECTIVE: Biliary atresia is the result of a fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Without surgical intervention, mortality reaches 100%. The 5-year survival rate after portoenterostomy ranges from 13% to 60%, with approximately 60% of patients requiring liver transplantation at a later stage because of insufficient bile flow. METHODS: This retrospective analysis includes 30 consecutive patients undergoing portoenterostomy for biliary atresia at our hospital. RESULTS: The 5-year actuarial survival of the 30 patients was 68%. Thirteen patients (43.3%) died 3 days to 7 years after portoenterostomy. Four patients (13.3%) underwent liver transplantation 3 to 24 months after the Kasai procedure with a 100% survival. In 65% of patients without presence of cirrhosis, the portoenterostomy was successful, compared with 35% of cases with liver cirrhosis (p = 0.0148). Liver cirrhosis with extrahepatic biliary atresia alone was present in 5 of 17 patients (29%) as compared with 8 of 12 patients (66%) with intrahepatic biliary hypoplasia in addition to extrahepatic biliary atresia and cirrhosis. CONCLUSIONS: Portoenterostomy remains the treatment of choice for patients with extrahepatic biliary atresia. However, the presence of cirrhosis portends a poorer prognosis and may be an indication for early transplantation. Cirrhosis is more commonly present in the setting of intrahepatic biliary hypoplasia and may account for the lower success rates of portoenterostomy in this group of patients. Five-year survival of the female patients was 88% as compared with 55% of the male patients.     

PS06LONG‐TERM OUTCOME OF BILIARY ATRESIA IN NEW ZEALAND

Objective This is a multicenter study to assess the long‐term outcomes of children with biliary atresia (BA) who have undergone Kasai operation in New Zealand. Material and Methods This retrospective analysis includes 97 patients who underwent a Kasai operation in the tertiary paediatric surgical centres between 1989 and 2006 in New Zealand. The demographic data, age at diagnosis and surgery, success of surgical procedure, need for liver transplantation and long‐term outcomes were studied. Results BA was diagnosed in 58 Mäori and Pacific People, 31 Caucasian and 8 Asian children. The average age and time at presentation and surgery was 50.37(±37.3) and 61.6 (±25.7) days respectively. Eighty‐six (89%) children had recoloration of the stool and 71 (68%) became jaundice free (bilirubin <20 µmol/L) 1 year after the surgery. Ascending cholangitis was the commonest complication and occurred in 41% of children. Five and 10‐year survival rates with native liver were 47% and 23% respectively. Liver transplantation was performed in 50 patients and 38 (76%) survived. The overall actuarial 5 and 10‐year survival rates for BA patients were 45.4% and 27.8% respectively. Conclusion BA was more common in Mäori and Pacific People children. There was a trend of less favourable results in children who had surgery late (>90 d). Long‐term prognosis was not directly related to age at diagnosis and surgery. The survival of patients with and without liver transplantation was similar to that of other series.     

Past and future of biliary atresia

BACKGROUND: With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients? METHODS: From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys. RESULTS: Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P < .001). CONCLUSIONS: Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome.     

The Kasai portoenterostomy: when is it too late?

BACKGROUND/PURPOSE: Kasai portoenterostomy is recommended as the primary initial therapy for extrahepatic biliary atresia if the procedure can be performed within 10 to 12 weeks of life. The optimal management for infants with delayed presentation of biliary atresia remains controversial. The purpose of this study was to determine the success rate and outcome for patients who underwent a "late" Kasai portoenterostomy. METHODS: The authors conducted a retrospective review of the medical records of all patients with biliary atresia who underwent a Kasai portoenterostomy at their institution from 1986 to 1999 (n = 31). The authors analyzed success rates compared with age at the time of the Kasai procedure and the association with patient demographics. Surgical success was defined as achievement of a total serum bilirubin < or = 2 mg/dL. Long-term follow-up assessments included the need for liver transplantation and patient survival rate. RESULTS: The demographics of this study cohort showed a predominance of African-Americans, 19 of 31 (61%), and girls, 23 of 31 (74%). Assessment of success compared with subject age at the time of the initial portoenterostomy showed that 52% (13 of 25) had successful Kasai procedure at 0 to 75 days, compared with 83% success rate (5 of 6) at age 76 days or older (P = .359). Liver transplantation was performed in 16 of 31 patients (45%). Overall survival rate for the entire cohort is 23 of 31 (74%), whereas 12 of 31 (39%) are currently alive without a liver transplant. CONCLUSION: These data suggest that there is no contraindication to performing a Kasai portoenterostomy for biliary atresia in children over 75 days of age.     

Primary vs. salvage liver transplantation for biliary atresia: A retrospective cohort study

IntroductionKasai hepatoportoenterostomy is the standard of care for children with biliary atresia, but a majority of patients progress to end-stage liver disease and require a salvage liver transplant. Given the high failure rates of the hepatoportoenterostomy operation, some have advocated for primary liver transplantation as a superior treatment approach. The aim of this study was to compare outcomes of pediatric candidates with biliary atresia listed for primary vs. salvage liver transplantation.MethodsThe SRTR/OPTN database was retrospectively reviewed for all children with biliary atresia listed for liver transplant between March 2002 and February 2021. Candidates were categorized as primary liver transplant if they had not undergone previous abdominal surgery prior to listing and salvage liver transplant if they had. Salvage transplants were further categorized as early failure if listed within the first year of life or late failure if listed at an older age.Results3438 children with biliary atresia were listed for transplant during the study period, with 15% of them listed for a primary transplant, 17% for salvage transplant after early failure, and 67% after late failure. Recipients of salvage liver transplant with late failure had lower bilirubin levels and were less critically ill as demonstrated by MELD/PELD scores and hospitalization status. Correspondingly, these recipients had higher waiting list and graft survival, though this did not remain statistically significant after adjustment in multivariable models. There were no differences in waiting list, recipient, or graft survival with primary vs. salvage liver transplant after early failure.ConclusionKasai hepatoportoenterostomy should remain the standard of care in biliary atresia as it may delay need for transplant beyond the first year of life in a subset of recipients and does not jeopardize subsequent transplant outcomes, even with early failure.Levels of evidenceRetrospective cohort study (Level III)