腹腔镜肝门空肠吻合术治疗小儿胆道闭锁的临床观察

目的:探讨腹腔镜肝门空肠吻合术治疗小儿胆道闭锁的临床疗效。方法:回顾分析2011年1月至2012年12月收治的12例Ⅰ、Ⅱ型胆道闭锁症患儿的临床资料,分析其术中情况、术后并发症、术后胆汁引流情况及住院时间。结果:12例患儿均顺利完成腹腔镜手术,手术时间平均(3.5±1.2)h,出血量平均(11.2±1.6)ml。无术中、术后(腹腔感染、肠漏等)并发症发生。术后患儿黄疸症状明显减轻,血生化各项指标较术前明显降低,差异有统计学意义(P<0.05)。结论:腹腔镜肝门空肠吻合术治疗小儿胆道闭锁安全、有效,术野暴露更充分,可更清晰地显示肝门处的细微结构,如肝门纤维条索、门静脉及肝动脉等。术中不游离肝脏,对肝脏血供、下腔静脉回流影响小,对患儿打击小,术后肠蠕动恢复快,避免了术后肠梗阻的发生,间接降低了术后胆管炎的发生几率。     

腹腔镜与开腹行肝门肠吻合术治疗小儿先天性胆道闭锁效果的对比研究

目的探讨腹腔镜下行肝门空肠吻合术治疗先天性胆道闭锁的效果。方法2003年8月~2005年9月,对26例胆道闭锁(Ⅲ型)患儿在腹腔镜下行肝门空肠吻合术,充分显露肝门,游离并于肝门纤维块处切除胆囊,提起距Tre itz韧带20 cm处空肠,经脐将空肠提出腹壁外并逐渐拉出远端40 cm范围,距Tre itz韧带20 cm横断空肠并封闭远端,将近端与远侧30~35 cm处空肠行端侧吻合,把肠管送回腹腔。将肝支空肠襻经结肠后隧道上提至肝下,肝门空肠端侧吻合。与同期同年龄段34例胆道闭锁(Ⅲ型)行开腹肝门空肠吻合术的患儿进行对照,比较两组手术时间、术中出血量、手术前后肝功能指标、平均住院天数和住院费用及术后转归情况。结果腹腔镜组手术出血量少于开腹组[(15.4±5.0)m l vs(33.8±19.4)m l,t=-4.709,P=0.000],住院费用高于开腹组[(19 153.9±619.5)元vs(15 116.7±898.4)元,t=19.607,P=0.000];两组手术时间、住院天数无显著差异。术后腹腔镜组血清总胆红素、直接胆红素、ALT和AST比开腹组下降明显。腹腔镜组术后切口疝1例;开腹组急性肝衰竭、伤口裂开各1例,伤口感染2例,两组早期并发症发生率无显著性差异(2χ=0.395,P=0.530)。术后4个月时随访,腹腔镜组13例(占50%)患儿退黄;开腹组18例(53%)患儿退黄。结论腹腔镜肝门空肠吻合术治疗小儿先天性胆道闭锁微创效果明显,方法安全、有效。     

肝门胆管切开整形、肝门空肠吻合术治疗Ⅰ型胆道闭锁

目的 了解肝门胆管切开整形、肝门空肠吻合术治疗Ⅰ型胆道闭锁的疗效。方法 1992-2001年本院对4例Ⅰ型胆道闭锁患儿行肝门胆管切开整形、肝门空肠吻合术,对临床资料（病史、体征、肝脏功能、B超、术中胆道造影、组织学检查等）进行回顾性分析,并对患儿的症状、体征、肝脏功能及肝胆系统B超进行长期随访。结果 4例患儿的临床表现均为阻塞性黄疸,B超示胆囊及胆管囊肿均小于同龄胆总管囊肿组,术中胆道造影确立诊断同并发现肝门部肝管和左右肝管狭窄,肝脏组织学改变与Ⅲ型胆道闭锁者相似,术后患儿全部成活,无近期并发症发生,黄疸于术后30-74d,完全消退,术后远期随访患儿生长发育正常,无黄疸,肝功能恢复正常。结论 Ⅰ型胆道闭锁常伴有肝管及肝内胆管异常,肝门胆管切开整形,肝门空肠吻合术治疗该畸形可取得满意的疗效。     

微小切口肝门空肠Roux-en-Y吻合术治疗Ⅲ型胆道闭锁

目的探讨微小切口肝门空肠Roux-en-Y吻合术的可行性和疗效。方法 2008年3月～2010年6月腹腔镜下胆道造影或探查术确诊Ⅲ型胆道闭锁82例,行微小切口肝门空肠Roux-en-Y吻合术。上腹横切口,4把S拉钩分别在上下左右对拉,暴露肝门,游离切除胆囊,彻底游离切除肝门纤维块,行肝门空肠吻合。结果 82例均完成微小切口肝门空肠Roux-en-Y吻合手术。手术切口长度平均4.2 cm（4～4.5 cm）;开腹手术操作时间平均71.8 min（55～90 min）;平均出血量8.3 ml（5～20 ml）,无术中输血者。均未放置腹腔引流。术后24～48 h拔除胃管,48～72 h进食。无围手术期死亡病例。术后第2天消化道出血1例,术后切口裂开2例。82例随访6～18个月,平均10.5月,术后4周获得胆汁引流率（直接胆红素降至正常水平或较术前下降超过60%）占69.5%（57/82）,术后胆管炎发作3次以上占29.3%（24/82）。结论微小切口肝门空肠Roux-en-Y吻合术治疗胆道闭锁,可以不将肝脏拖出腹腔外进行吻合,避免对患儿循环系统的严重影响,同时直视下肝门空肠吻合可以保证吻合确切在门静脉后方,手术安全可靠。     

腹腔镜辅助与开腹肝门空肠吻合手术治疗Ⅲ型胆道闭锁的疗效分析

目的 比较腹腔镜辅助与开腹肝门空肠吻合术治疗Ⅲ型胆道闭锁的临床效果.方法 2003年5月～2005年9月,手术治疗Ⅲ型胆道闭锁71例.腹腔镜辅助肝门空肠吻合术26例,排除3例因肝门部渗血而中转开腹病例;同期开腹肝门空肠吻合术42例.比较2组间的近期胆汁引流效果、远期胆管炎发生率和大于18个月生存率. 结果 与开腹组相比,腹腔镜组手术时间长[(189.0±36.2)min vs(157.0±30.4)min,t=3.919,P=0.000)];出血量少[(15.4±5.0)ml vs(33.8±19.4)ml,t=-4.727,P=0.000)].术后1～4周胆汁引流率(术后4周直接胆红素降至正常水平或较术前下降超过60%)2组相近[65.4%(17/26)vs 69.0%(29/42),χ2=0.098,P=0.754];随访期间胆管炎发作>3次发生率相近[15.4%(4/26)vs 33.3%(14/42),χ2=2.658,P=0.103];>18个月生存率相近[38.5%(10/26)vs 52.4%(22/42),χ2=1.249,P=0.264].腹腔镜组1例术后脐部切口疝.2组均无术中和术后输血者,无粘连性肠梗阻和腹腔感染. 结论 传统开腹肝门空肠吻合术治疗Ⅲ型胆道闭锁远期疗效与腹腔镜辅助肝门空肠吻合术差异无显著性.     

肝移植治疗儿童胆道闭锁合并肝脏恶性肿瘤4例报告

目的 探讨肝移植治疗儿童胆道闭锁(biliary atresia,BA)合并肝脏恶性肿瘤的临床疗效及预后.方法 2015年1月1日—2019年12月31日期间天津市第一中心医院儿童器官移植科的儿童肝移植患者中,有4例原发疾病为胆道闭锁合并肝恶性肿瘤,回顾性分析这4例患者的临床资料.结果 4例患者中BA合并肝母细胞瘤(h...     

腹腔镜及胆道镜联合治疗胆道结石(附50例报告)

我院于1999年3月至2006年2月开展了腹腔镜及胆道镜联合治疗胆道结石50例,临床效果满意,现报告如下。     

机器人腹腔镜辅助Kasai手术治疗囊肿型胆道闭锁:国内首例报告

本文报道2020年9月采用da Vinci Si机器人手术系统治疗出生52天囊肿型胆道闭锁1例,普通腹腔镜并稍扩大脐部切口牵出肠管,完成离断空肠和侧侧吻合,对接机器人完成Kasai手术.总手术时间210 min,无术中并发症发生,术后第2天排墨绿色胆汁样便,术后12天出院.口服利胆、抗炎及护肝药物治疗,随访6个月,黄疸...     

活体肝移植治疗儿童胆道闭锁44例

目的 观察活体肝移植治疗儿童胆道闭锁的效果,总结其临床经验.方法 2006年10月至2010年12月间共有44例胆道闭锁患儿接受了活体肝移植,其中男性26例,女性18例,年龄(12.1±9.0)个月,中位数为9个月(6～60个月);44名供者全部为患者直系亲属,年龄(32.7±8.0)岁,中位数为31岁(20～54岁).供、受者ABO血型相容,供肝均为供者肝脏的左外叶.对供、受者术前评估过程、手术方法、术后管理以及预后等临床资料进行总结.结果 术后对全部供者进行系统随访,随访时间(17.5±13.3)个月,无供者发生严重并发症和死亡,所有供者均恢复健康.44例受者中,死亡9例,死因分别为门静脉栓塞3例、肝动脉栓塞1例、胆道并发症2例、切口感染1例、腹腔出血1例及肺部感染1例,其余35例健康存活.术后1年和2年累积存活率分别为81.2%和76.1%,无一例受者接受再次肝移植.术后主要并发症有门静脉血栓、肝动脉血栓、胆汁漏及逆行性胆管炎、肺部感染、切口感染及急性排斥反应等.结论 活体肝移植是治疗儿童胆道闭锁的有效方法,预后良好.完善缜密的术前评估,熟练精细的手术操作以及精心的术后管理是改善受者预后的关键因素.

Abstract：

Objective To observe the outcomes of living donor liver transplantation (LDLT) for children with biliary atresia (BA) and to summarize the clinical experiences. Methods Forty-four BA patients (26 boys and 18 girls) underwent LDLT between October 2006 and December 2010. Mean (SD) and median (range) age at operation was (12.1 ± 9.0) months and 9 (6-60) months,respectively. The 44 donors were lineal relatives to the consorted recipients. Their mean (SD) and median (range) age at operation was (32. 7 ± 8. 0) months and 31 (20～54) years, respectively. All donor graft types were the left lateral segments with compatible ABO blood groups. Clinical data,including pre-operative evaluations, surgical technique, postoperative management and outcomes in all donors and recipients were retrospectively analyzed. Results All donors were followed up for (17. 5 ± 13. 3) months. No donor mortality was encountered, with a minimal morbidity and no long-term sequelae. Nine out of 44 recipients died. Three patients died of portal vein thrombosis (PVT), one of hepatic artery thrombosis (HAT), two of biliary complications, one of surgical site infections, one of abdominal bleeding and one of pulmonary infection. The overall 1-year and 2-year cumulative survival rate in recipients was 81. 2% and 76. 1 %, respectively. No re-transplantation was done. Postoperative complications included PVT, HAT, biliary leakage and refluxing cholangitis, pulmonary infections,surgical site infections and acute rejection. Conclusion LDLT has been the effective treatment for pediatric recipients with BA and provides favorable prognosis. To improve prognosis of recipients, the key points are pre-operative evaluations, surgical technique, and postoperative management     

腹腔镜治疗新生儿高位肠闭锁12例

随着腹腔镜技术的不断发展及腹腔镜器械的精细,越来越多的小儿外科疾病应用腹腔镜治疗,但对新生儿的手术应用不多,国内大多数治疗新生儿高位肠闭锁仍是传统的直接开腹手术,手术切口大,术中损伤大,操作复杂,出血多,术中、术后并发症多,恢复慢,住院时间长。回顾性分析2010年2月至2013年1月新疆维吾尔自治区人民医院收治的新生儿高位肠闭锁12例,术后取得良好效果,现报道如下。     

Revision of porto-enterostomy in congenital biliary atresia

For the patients with insufficient bile flow following porto-enterostomy for congenital biliary atresia, removal or resection of granulation or scar tissue at the porta hepatis has been performed. Of 11 reoperations, constant bile excretion was obtained in four. Sufficient resection of scar tissue at the porta hepatis was most important for revision of porto-enterostomy. For resection of scar tissue, a special scissors devised by us was used.     

Mother-to-daughter occurrence of biliary atresia: a case report

We herein report a case of mother-to-daughter occurrence of biliary atresia (BA). The mother underwent a Kasai hepatic portoenterostomy at the age of 64 days with a diagnosis of type III BA. Her postoperative course was uneventful, with a good bile discharge, and she became pregnant at the age of 20 years. At 21 weeks of gestational age, fetal ultrasonographic examination of the baby revealed a cystic mass at the hepatic hilum. The baby was born via vaginal delivery at 41 weeks of gestation with a birth weight of 3269 g. Because of an increasing direct bilirubin level and the absence of bile excretion, a laparotomy was performed at the age of 29 days. Intraoperative cholangiography revealed the diagnosis of BA I-cyst, and a hepaticojejunostomy was performed with a good bile discharge. To our knowledge, these patients are the first occurrence of BA in a transmission pattern of mother to daughter.     

Hepatolithiasis after hepatic portoenterostomy for biliary atresia

Background

Hepatolithiasis after hepatic portoenterostomy for biliary atresia has been paid little attention, with only 22 reported cases.

Patients and Methods

Fifteen patients underwent living-related liver transplantation for biliary atresia after hepatic portoenterostomy in our hospital between 1998 and 2004. The resected livers were examined for the existence and location of hepatolithiasis, composition of the calculi, and bacterial infection of bile. The relation between a history of cholangitis and the presence of hepatolithiasis was analyzed.

Results

Intrahepatic calculi were found in 8 (53%) of 15 patients. The calculi consisted of almost 100% calcium bilirubinate. Calculi were found in bile lakes in 8 patients. Bacteria were present in the bile in 8 (53%) of the 15 patients. Of the 8 patients, 7 (88%) had a history of ascending cholangitis.

Conclusions

Hepatolithiasis occurs after hepatic portoenterostomy for biliary atresia more frequently than previously thought. Bile stasis and possibly bile infection are the main causes of calculi formation.     

Hepatic portocholecystostomy for biliary atresia.

Four infants with biliary atresia had gross obliteration of the common hepatic duct but residual patency of the gallbladder, cystic duct and common bile duct. The patients were treated by hepatic portocholecystostomy utilizing the extant bile ducts for biliary reconstruction. Bile drainage was achieved in all four infants. There was a conspicuous absence of postoperative cholangitis. Subsequent obstruction of the distal ducts in two patients necessitated reoperation and construction of a standard biliointestinal conduit. The other two children are surviving, jaundice-free, 5 1/2 and 5 years after operation with minimal sequelae of biliary atresia. Hepatic portocholecystostomy is a feasible surgical alternative to intestinal reconstruction in patients with biliary atresia in whom the disease is limited to the proximal extrahepatic bile ducts.     

Congenital biliary atresia: liver injury begins at birth

Background

The timing of onset of liver injury in biliary atresia (BA) is not known, although in approximately 10% of cases, biliary pathologic condition associated with the biliary atresia splenic malformation syndrome must begin well before birth.

Methods

The study involved retrospective case-note review for infants with definite BA who underwent laparotomy within first week of life.

Results

Three infants were identified who had occlusive BA evident on the first day of life. In all cases, their liver was grossly normal, and histologic changes were trivial.

Conclusion

This suggests that the detrimental cholestatic liver injury, later characteristic of BA, only begins from the time of birth despite a prenatal occlusive biliary pathology. It may be that tissue injury only occurs with the onset of the perinatal bile surge initiating periductal bile leakage and the triggering of an inflammatory and ultimately fibrotic response.     

Corticosteroid therapy in biliary atresia

Sixteen patients with biliary atresia had 44 steroid courses for treatment of cholangitis or diminution of bile flow following Kasai hepatic portoenterostomy operations. A "blast" type (high dose/short duration) steroid administration was employed to potentiate the choleretic and anti-inflammatory effect. There was a significant augmentation of bile flow and a reduction in maximum temperature, serum bilirubin, and alkaline phosphatase.