活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

亲属活体供肝儿童肝移植疗效分析

目的 总结我院10例亲属活体供肝儿童肝移植手术经验,评估其临床疗效.方法 回顾性分析2006年6月至2008年5月期间完成的10例亲属活体供肝儿童肝移植供受体手术经验及临床疗效.本组男5例,女5例.肝移植年龄6个月至14岁,体重7至42 kg.其中,Wilson'S病2例,胆道闭锁3例,严重门静脉高压症3例,重型肝炎1例和肝糖原蓄积症1例.术后8例用他克莫司(FK506)及强的松,2例用环孢素A(CsA)及强的松等抗免疫排斥.结果 10例供体均顺利出院,健康生活,无并发症发生;10例受体中除1例因门静脉血栓患儿围手术期死亡外,其余9例均痊愈出院.随访中除1例于术后5个月死于排异反应、1例术后7个月死于当地意外食物中毒外,其他7例存活至今,最长生存期24个月.术后并发症5例:门静脉血栓1例,胆道狭窄1例,腹腔淋巴漏1例,ICU监护期间严重感染2例等.结论 亲属活体供肝儿童肝移植手术技术要求高,术前仔细的供、受体选择,准确的血管影像学检查、精确的手术技术和严格的围手术期管理是亲属活体供肝儿童肝移植成功的关键.     

腹腔镜与开放Kasai手术治疗Ⅲ型胆道闭锁中期疗效的对比

目的比较腹腔镜与开放Kasai手术治疗先天性胆道闭锁的中期疗效。方法回顾性分析2010年9月至2011年9月在本院行Kasai手术并获得随访的103例Ⅲ型胆道闭锁患儿,根据手术方式分为腹腔镜辅助Kasai手术组(LP组)和开腹Kasai手术组(OP组)。通过术后12~30个月肝生化ALT和TBil中位数的秩和检验,比较两组Kasai术后中期肝功能情况;应用Kaplan-Meier生存分析比较两组中期生存率;比较两组对二期自体肝移植手术及术后生存情况的影响。结果 103例Ⅲ型胆道闭锁患儿中,LP组55例,OP组48例;两组年龄分布、性别比例、术前肝功能(TBil、ALT、AST)中位数等均无统计学差异。术后12个月、18个月、24个月、30个月两组肝功能(TBil,ALT)恢复情况无统计学差异。Kasai术后LP组和OP组的自体肝生存率,1年生存率分别为78.2%和75%,2年生存率分别为66.9%和55.8%,3年生存率分别为66.9%和55.8%。Kasai术后实施肝移植11例(LP组4例,OP组7例),1例移植术后死亡。结论胆道闭锁患儿实施腹腔镜Kasai手术与开放Kasai手术后中期效果无明显差异。     

胆道闭锁患儿Kasai术后远期预后研究--63例带自体肝患者生存20年评估

目的评估胆道闭锁患儿Kasai术后远期预后.方法1968年～1983年,法国巴黎Bicêtre医院儿外科共对271例胆道闭锁患儿行Kasai手术或其变异术式,术后随访20年,对带自体肝生存患者进行综合性评估.结果271例患者中,63例(占23%)带自体肝者至少生存20年.肝功能结果显示21例总胆红素指标正常,其中12例γ-GT和转氨酶指标正常;44例有门脉高压表现;19例仍有晚期胆道感染发作;6例并发胆道结石;61例患者肝穿刺活检提示有不同程度肝硬化表现.20岁之后,2例死于肝功能衰竭;10例已行肝移植;4例正等待肝移植.结论胆道闭锁患儿Kasai术后带自体肝20年生存率仅为23%,大部分患儿转肝移植治疗.即使带自体肝生存患儿随访各项指标也不令人满意.因此密切关注肝脏情况,尽早发现并发症,对决定进一步治疗措施,改善预后,具有积极的意义.     

胆道闭锁手术治疗进展

胆道闭锁是严重威胁生命的小儿肝胆外科疾病,手术是唯一的治疗方式。Kasai手术是治疗胆道闭锁的首选手术方案,术后约60%的患儿会出现并发症,如胆管炎、肠梗阻、吻合口狭窄、胆漏和消化道出血等,2～5年内约有50%的患儿会发展为肝硬化、肝衰竭,需要肝移植。Kasai手术和肝移植序贯结合可明显改善预后。本文总结了国内外对胆道闭锁手术治疗的研究报道,分别从Kasai手术年龄、手术技巧,腹腔内、外Kasai手术,微创与开腹Kasai手术,再次Kasai手术,肝移植等方面对胆道闭锁手术治疗进展进行综述。     

胆道闭锁患儿肝移植术后胆管炎的发生情况分析

目的分析胆道闭锁患儿肝移植术后胆管炎的发生情况。方法回顾性分析2013年1月至2016年7月于本院移植科接受肝移植手术的219例胆道闭锁患儿临床资料。根据肝移植术前是否接受过肝门-空肠吻合术(Kasai手术)、肝移植术前是否发生胆管炎以及肝移植术中肠袢的不同处理方式进行分组,并比较各组间肝移植术后胆管炎的发生率。结果 219例胆道闭锁患儿肝移植术后胆管炎的总体发生率为23.29%。Kasai手术组肝移植术后胆管炎的发生率高于非Kasai手术组(30.70%vs 15.24%),差异有统计学意义(χ~2=7.316,P=0.007)。而Kasai手术组患儿中,术前胆管炎组与术前非胆管炎组肝移植术后胆管炎的发生率组间比较(28.57%vs 32.76%),差异无统计学意义(χ~2=0.235,P=0.628);肠袢保留组与肠袢重建组肝移植术后胆管炎的发生率组间比较(31.43%vs30.38%),差异无统计学意义(χ~2=0.013,P=0.911)。在Kasai术后存在胆管炎的患儿中,原肠袢组与新肠袢组肝移植术后胆管炎发生率组间比较(33.33%vs 24.14%),差异无统计学意义(χ~2=0.579,P=0.447)。结论对于胆道闭锁患儿Kasai术后的顽固性胆管炎,肝移植手术可取得较为满意的结果。胆道闭锁患儿肝移植术后胆管炎较为常见,但基本可以治愈。保证胆肠吻合的输入端肠袢长度足够,可有效控制肝移植术后胆管炎的发生。     

腹腔镜Kasai手术治疗胆道闭锁合并肝右动脉变异北大核心CSCD

目的探讨腹腔镜Kasai手术治疗小儿胆道闭锁合并肝右动脉变异的治疗效果。方法收集2014年1月至2021年8月在淮安妇幼保健院行腹腔镜Kasai手术的8例胆道闭锁合并肝右动脉变异的Ⅲ型胆道闭锁患儿的临床资料,其中男5例,女3例;入院时平均年龄78.1 d;平均体重5.9 kg。手术采用四孔法,在变异肝右动脉和门静脉右支之间游离胆总管,牵拉至肝门。游离并切除肝门纤维块,完成腹腔镜Kasai手术。随访并观察患儿治疗效果。结果所有患儿均经腹腔镜手术,无特殊手术并发症。平均手术时间为235 min,平均出血量为12.8 ml,术后平均随访时间为32.1个月。6例患儿术后4个月内总胆红素降至正常。另外2例患儿胆红素术后显著下降,但由于并发胆管炎再次升高,持续观察中并使用抗生素、熊去氧胆酸和激素治疗。结论对于胆道闭锁合并肝右动脉变异患儿,腹腔镜Kasai手术可安全实施,应注意在变异肝右动脉和右门静脉支之间充分游离胆总管。     

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。     

儿童终末期肝病评分在胆道闭锁患儿肝移植中的应用

儿童终末期肝病(pediatric end-stage liver disease,PELD)评分系统是根据客观的实验室检查数据评估儿童肝脏疾病严重程度的模型,国际上用它来分配日益减少的供肝给儿童肝移植受者.对于Kasai手术效果不佳的胆道闭锁(biliary atresia,BA)患儿来说,肝移植成为唯一的选择.本文通过分析PELD评分系统与经典的临床肝功能Child分级、BA肝移植手术时机的选择、活体肝移植(living donor liver transplantation,LDLT)、肝纤维化、葛西手术(Kasai procedure,KP)以及肝移植手术预后之间可能存在的关联,旨在较全面的了解PELD评分在BA患儿肝移植中所发挥的作用,力求使其应用更加科学、合理.     

Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy?

Kasai portoenterostomy has been the treatment of choice for neonates with biliary atresia since its introduction. With the advance in laparoscopic techniques, a few centers have reported the feasibility of performing laparoscopic Kasai portoenterostomy. However, the outcome of this new technique is not known. Here, we aim to evaluate, as the only referral center for liver transplantation, our experience with patients referred for transplantation after failed Kasai portoenterostomy. A retrospective study was carried out between October 1996 and September 2005. The records of all patients with the diagnosis of biliary atresia were retrieved. The type of procedure and clinical outcome of the patients were noted. Early failure of Kasai enterostomy was defined as the need for liver transplantation within 1-year post-Kasai operation. For the period studied, a total of 72 patients with biliary atresia were identified. Sixty-three of the 72 patients had their Kasai portoenterostomies performed openly while nine patients underwent laparoscopic Kasai portoenterostomy in a center experienced in laparoscopic surgery. Six of these patients were referred for transplantation within 1 year, giving the early failure rate of 66.6%. In comparison, the early failure rate for open Kasai procedure was 38.5%. Regarding post-operative complications, one patient who underwent laparoscopic Kasai procedure also suffered intestinal volvulus after initial surgery and another was found to have internal herniation of the Roux loop. Laparoscopic Kasai portoenterostomy seems to be associated with more post-operative complications and worse early clinical outcome. As a result, we remain guarded about the present-day technique of laparoscopy for biliary atresia.     

Kasai术后生存12年以上患儿的随访

目的 对Kasai术后长期生存者进行随访,了解其生活质量、生理发育和肝功能情况.方法 1989年3月至1997年3月对92例胆道闭锁患儿行Kasai术,对术后存活12年以上并得到随访的23例患儿,进行身高、体重、第二性征等生理发育的评价,以及肝功能、肝脏和门静脉的影像学检查.结果 23例患儿中21例进行门诊复查,2例进行信访和电话复查.21例门诊复查患儿均测量身高和体重,有4例患儿身高低于同年龄同性别参照人群身高的均值,体重则均不低于同性别、同身高参照人群体重的均值-1SD.对21例的性征发育情况进行调查,10例14岁以上男性患儿,阴茎已发育9例,9例中出现遗精4例.女性9例中,5例在15岁以前月经初潮.21例肝功能检查,16例胆红素在正常范围内,白蛋白低于正常值1例,谷丙转氨酶升高7例.血常规检查正常范围的16例,红细胞、白细胞和血小板低于正常值2例.15例患儿超声检查13例有肝硬化改变.结论 ①Kasai手术后虽然仅有小部分(23/92)的患儿存活10年以上,存活者能与正常儿童一样上学读书;②与正常同龄儿相比,患儿远期生长发育还是受一定的影响,男孩的体格生长迟缓比例较高,青春发育延迟.女孩的生长发育和性征发育则无明显延迟;③术后应定期对血清肝功能及肝脏和门静脉包括影像学的检查;④Kasai手术后远期有部分患儿需行肝移植.     

Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?

OBJECTIVES: The prognosis of hepatoportoenterostomy (the Kasai operation) for biliary atresia worsens when the age at surgery increases. This study examines whether the Kasai operation remains justified after 3 months of life. STUDY DESIGN: Records for all patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed, and patients were classified into 3 groups: group 1 (n = 30), no contraindication to the Kasai operation, but orientation to de novo transplantation; group 2 (n = 380), age at Kasai operation <90 days; and group 3 (n = 60), age at Kasai operation > or =90 days. Survival with native liver, survival after liver transplantation, and overall survival (Kaplan-Meier method) were compared by using the log-rank test. RESULTS: Five-year (10-year) survival with native liver was 35% (30%) in group 2 and 25% (22%) in group 3 (P =.03). Five-year overall survival was 57%, 74%, and 55% in groups 1, 2, and 3, respectively (P =.003). Poor results in groups 1 and 3 were mainly due to increased pre-transplantation mortality, but survival after transplantation was not significantly different in the 3 groups. CONCLUSIONS: Performance of the Kasai operation after 3 months of age is justified in selected cases, because it may obviate liver transplantation. Preoperative evaluation should exclude patients with advanced liver disease for whom liver transplantation should not be delayed.     

Biliary atresia: the Croatian experience 1992–2006

The objective of this study was to determine the outcomes of Croatian children with biliary atresia. Health records of infants born in Croatia between January 1, 1992 and December 31, 2006 who were diagnosed with biliary atresia and treated at a single university center were reviewed. Survival rates were calculated with the Kaplan–Meier method. Twenty-nine patients with biliary atresia were identified. Incidence was one in 23,600 live births (95% confidence interval 1/17,400–1/27,200). The median age at Kasai operation was 66 days (range 22–192). Median follow-up was 2.65 years (range 0.2–14.3). Overall survival rates for the patients who underwent Kasai portoenterostomy, including those six (20%) who subsequently underwent liver transplantation, were 75.6%. Five and 10-year native liver survival rates were 51.7% and 38.8%, respectively (median survival time was 7.88 years). Survival rate curves in two groups of patients according to the biliary atresia phenotype (fetal or perinatal form) were divergent. Survival was 87.7% in perinatal form and 43.8% in fetal form (Breslow chi-square 8.082, p < 0.01). Overall survival rates of patients with biliary atresia in Croatia compared unfavorably with current international standards; this could be improved with earlier referral for liver transplantation. Results of Kasai operation (native liver survival rates) compared favorably with results reported elsewhere.     

Long-term prognosis of patients with biliary atresia: a 25 year summary

OBJECTIVE: The purpose of this study was to delineate the long-term prognosis of biliary atresia (BA) in Taiwan. STUDY DESIGN: From 1976 to 2000, 185 children were diagnosed with BA, 22 underwent exploratory laparotomy without Kasai operation, and 163 underwent Kasai operation, of which 141 cases had long-term follow-up and formed the basis of this study. The outcome was analyzed. RESULTS: Among the 141 BA children studied who underwent Kasai operation, 115 (81.6%) had recoloration of stools, and 86 (61.0%) became jaundice-free (bilirubin <34 micromol/L) [corrected]. The resolution of jaundice and the absence of repeated cholangitis contributed to better outcome. Five and 10 year survival rates with native liver were 35% and 31%, respectively. Liver transplantation was performed in 19 patients (all but 2 with a living-related donor), and 15 (79%) survived. Five and 10 year overall survival rates for BA patients were 41.9% and 40.2%, respectively. CONCLUSIONS: The study delineated the long-term outcome of BA in an Asian country other than Japan. Survival with native liver after a Kasai operation in Taiwan was similar to that in the American and European series. Limited donors for liver transplantation in the years of the study accounted for the poor overall prognosis of BA patients in this series.     

Biliary atresia: perspective on transplantation

In North America, long-term, jaundice-free survival following Kasai's operation for biliary atresia is 25%–35%; thus, the majority of patients require liver transplantation for salvage. The timing of organ replacement is crucial. Patients without bile drainage because of either surgical election or operative failure should be referred immediately. Patients with ongoing but inadequate biliary drainage should be referred at a time that coincides with a plateau of the growth curve or if cholangitis is recalcitrant. Portal hypertension itself is not an indication for transplantation irrespective of its manifestations. Ascites, however, when primarily due to hypoalbuminemia, is a solid indication for transplantation. Other tests of liver function are generally unreliable. Factors found to be unimportant or of questionable adverse impact on transplant outcome include previous operation, coexisting infection limited to the liver, nonpatent portal vein, and/or abnormal liver function tests. The only factor that was found to significantly influence outcome was patient size and age. Thus, bile drainage after Kasai portoenterostomy, by permitting growth, improves the chances for a favorable outcome. Transplant 1-year survival is from 60% to 88%. Liver replacement is not without complications, namely technical errors, infection, and rejection. Nonetheless, in a majority of cases hepatic transplantation provides the only opportunity for high-quality long-term survival. Offprint requests to: R. J. Hall     

I型胆管闭锁的外科治疗

目的探讨I型胆管闭锁的外科治疗以及临床意义。方法2003—2011年,作者收治胆道闭锁患儿98例,其中伴有胆总管闭锁的I型胆管闭锁患儿5例,男3例,女2例,年龄62～127d。行胆囊-空肠吻合术2例,肝管-空肠吻合术3例。结果2例胆囊-空肠吻合术患儿退黄时间分别为术后10d和术后17d（退黄标准为总胆红素〈20μmol／L）;3例肝管-空肠吻合术患儿退黄时间分别为术后20d、1个月和2个月（退黄标准同前）。术后随访时间1—5年;2例胆囊-空肠吻合术患儿术后未见黄疸反复。3例肝管-空肠胆道重建手术患儿中,1例术后未见黄疸反复,1例术后5个月出现黄疸,诊断为胆管炎,经抗炎治疗后好转;1例反复发作胆管炎最终选择肝移植。结论术中胆道造影是诊断胆道闭锁的金标准;如果术中造影证实为胆总管闭锁,且胆囊与左、右肝管通畅,主张采取胆囊-空肠吻合术。肝管-空肠吻合容易造成吻合口狭窄;过度解剖肝门对于术后恢复不利。