Early outcomes of laparoscopic surgery for biliary atresia

Aim

The aim of the study was to present early outcomes of the laparoscopic technique for biliary atresia with some technical modifications.

Materials and Methods

We reviewed charts of all patients with biliary atresia who underwent laparoscopic portoenterostomy from July to December 2008. There were 11 patients with biliary atresia, including 5 boys and 6 girls. The operation was carried out using 4 trocars. The liver was elevated by 2 transcutaneous stay sutures: one on the round ligament and the other on the gallbladder remnant. The left and right hepatic arteries and portal veins were dissected and retracted laterally by 2 transcutaneous sutures to expose the liver hilum. A stay suture was placed on fibrotic tissue at the liver hilum to facilitate its maximal removal. A jejunal end-to-side anastomosis was constructed extracorporeally. Portoenterostomy was carried out laparoscopically.

Results

Mean operative time was 245 ± 31 minutes. No patient required conversion. There were no operative deaths. Blood loss during operation was minimal. One patient died on day 65 after operation because of intractable hepatic liver. Follow-up after discharge from 10 to 16 months revealed that 6 patients still survived and 4 patients died. One patient died because of milk aspiration at 12 months of age. Three patients died because of repeated cholangitis and liver failure at 10, 10, and 14 months, respectively.

Conclusion

With a modified laparoscopic technique, good early outcomes of laparoscopic surgery for biliary atresia were achieved.     

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。     

Determinants of survival after Kasai's operation for biliary atresia using actuarial analysis.

Survival after Kasai's operation for "noncorrectable" biliary atresia is influenced by (1) age, (2) large bile ducts, and (3) concentration of the bile bilirubin. Critical values are: age less than 10.5 wk, any evidence of a large bile duct, and a bile bilirubin concentration greater than 8.8 mg/100 ml. Using these 3 factors, a predictive model is able to identify a favorable group with an 89% expected 4 yr survival.     

Cystic biliary atresia: an etiologic and prognostic subgroup

Introduction

Cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA) in which prognosis may be relatively favorable but liable to misdiagnosis as choledochal cyst, and potentially offers insights into the etiology of BA. Because some cases can be detected antenatally, CBA in general may have its origins in utero life. We assessed our experience with CBA.

Methods

Single-center retrospective review of infants with CBA over a 13-year period (January 1994 to December 2006) was done. Data are given as medians (range).

Results

Of 270 infants with BA, 29 (9 male) were identified as CBA. Antenatal ultrasonography had detected an abnormality in 12 (41%) infants at a median of 22 weeks (17-34 weeks) of gestation. All infants underwent postnatal excision and Kasai portoenterostomy (KP). Those with antenatally detected CBA came to surgery younger (36 [14-67] vs 48 days [35-147 days], P = .004). Twenty cysts (69%) had a fibroinflammatory wall with no biliary epithelial lining and 6 (26%) contained bile. Age at KP was significantly and positively correlated (r = 0.46, P = .01) with liver fibrosis, as assessed in liver biopsy materials obtained at KP, but not with grade of “hepatocyte disarray” (P = .74). Twenty infants (69%) cleared their jaundice (bilirubin <20 μmol/L) within 6 months after KP. Age at KP markedly affected outcome.

Conclusion

Cystic BA is a clinically distinct variant of BA. Despite onset in prenatal life, earlier than presumed for isolated BA, it has a better prognosis, particularly with early surgery.     

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。     

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.     

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.     

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.     

Successful revision of portoenterostomy in an infant with biliary atresia

We present a case report of a boy with biliary atresia who, after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abruptly developed acholic stools. Nuclear medicine imaging study showed no excretion. Broad-spectrum antibiotics and corticosteroids were administered but did not lead to clinical improvement; a surgical revision of the original anastomosis was undertaken at 4 months of age. At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation.     

胆道闭锁诊治体会

目的　分析胆道闭锁诊断、治疗及预后情况 ,探讨胆道闭锁的早期诊断和治疗策略。方法　回顾性分析我院近 5年收治的 2 8例胆道闭锁患儿诊断手段、手术年龄、手术方式及术后生存率。结果　胆道闭锁早期诊断率为 1 4 % (4 / 2 8) ,手术时年龄 1 3～ 2 70d ,6 0d以内 4例 (1 4 % ) ;6 1～ 90d 1 3例 (4 6 % ) ;91～ 1 2 0d 8例 (2 9% ) ;1 2 0d以上 3例 (1 1 % )。没有患儿接受肝移植 ,1～ 5年生存率为35 % (9/ 2 5 )。结论　密切观察临床表现、掌握胆道闭锁的影像学特点和及时采用腹腔镜探查可提高胆道闭锁的早期诊断率。完善手术方式、让更多的患儿接受肝移植、开辟新的治疗方法如抑制肝胆管细胞凋亡、抗体及基因治疗可提高胆道闭锁的长期治愈率     

Comparison of drainage techniques for biliary atresia

Purpose

Traditional Kasai portoenterostomy and porto-appendiceal duodenostomy have been utilized for biliary atresia. Differences in outcome between patients who underwent either Kasai portoenterostomy or porto-appendiceal duodenostomy were compared.

Methods

A review of all children who underwent a drainage procedure for biliary atresia from 1986 to 2000 (n = 30) was performed. Age at drainage procedure, subsequent liver transplantation, and outcomes were evaluated. Outcome variables included success rates (total bilirubin < 2.0 mg/dL) and survival rate. Statistical analysis was done with χ² and Student’s t test.

Results

Long-term follow-up was available on 28 of 30 patients. Age at biliary drainage was insignificant. Success rates between porto-appendiceal duodenostomy (31%) and Kasai portoenterostomy (82%) were statistically significant. Survival rate for patients who underwent a Kasai portoenterostomy was 10 of 11 patients. Survival rate for patients who underwent porto-appendiceal duodenostomy was 14 of 16 patients. Overall survival rate was comparable between porto-appendiceal duodenostomy (88%) and Kasai portoenterostomy (91%).

Conclusions

Although overall survival rate was comparable, patients who underwent porto-appendiceal duodenostomy were less successful in alleviating hyperbilirubinemia compared with Kasai portoenterostomy. This is shown further by the greater incidence of subsequent liver transplantation in infants with prior porto-appendiceal duodenostomy. Although the appendix may serve as an alternative biliary conduit, traditional Kasai portoenterostomy appears to achieve better biliary drainage.     

The surgical management of the unusual forms of biliary atresia

Of 50 infants undergoing laparotomy for biliary atresia during the last 9 yr at the Kobe Children's Hospital, 9 had variant forms, including (A) a gallbladder with a demonstrable connection with the intrahepatic biliary system, (B) a gallbladder with a connection with the extrahepatic biliary system and duodenum, and (C) a grossly visible subhepatic cyst. Three patients with form A underwent cholecystoduodenostomy. All these patients died of cholangitis and hepatic failure within 2 mo after operation. Two patients with form B were successfully treated by hepatic portocholecystostomy without any episodes of cholangitis. Two of the patients with form C, occurring in 1970, underwent an anastomosis between the subhepatic cyst and the duodenum. There was no bile excretion in these patients. The other two patients were treated subsequently by hepatic portoenterostomy with complete excision of the subhepatic cyst and excellent bile excretion was obtained. Infants with forms A and C should be treated by hepatic portoenterostomy instead of a direct anastomosis between the dilated portion of the extrahepatic biliary system and the alimentary tract. For patients with form B, hepatic portocholecystostomy seems to be the most suitable procedure relative to the prevention of postoperative cholangitis, which is the most serious complications in infants obtaining successful bile excretion.     

腹腔镜肝门肠吻合术治疗胆道闭锁的探讨(12例报告)

目的:探讨经腹腔镜辅助行肝门解剖和肝门肠吻合术治疗先天性胆道闭锁的临床效果。方法:先天性胆道闭锁患儿12例,29~87d11例,5.5个月1例。2例为肝总管闭锁(Ⅱ型),肝门部有直径1.2~2.5cm囊肿与肝内胆管相通;10例为肝门部胆管闭锁(Ⅲ型)。于脐部纵切口置入10mmTrocar,然后分别于右上腹、右中腹和左上腹置入3个5mmTrocar。术中胆道造影,确诊为胆道闭锁,暴露肝门;切除胆囊,游离切除肝门纤维块,空肠行Roux-en-Y吻合术保留肝支30~35cm,然后将肠管送回腹腔,将空肠肝支经结肠后拉至肝门下;用5-0可吸收缝线将空肠与肝门端侧连续吻合。结果:本组12例患儿中1例因肝门渗血中转开腹手术;另11例均在腹腔镜辅助下完成手术,手术时间平均为3.5h(3.1~4.6h),出血量约10ml,没有需在术中和术后输血者,全组患儿无手术后肠粘连梗阻和腹腔感染。结论:经腹腔镜行肝门肠吻合术治疗先天性胆道闭锁安全可靠,具有暴露肝门清晰、肝门纤维块分离和切除准确、对患儿打击小等优点。     

Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy

Purpose

This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA.

Methods

Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained.

Results

Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher's Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without (P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86% (18/21) in patients with steroids and 82% (18/22) in those without. Transplant survival (74%) was comparable to previously reported historical controls (82%).

Conclusions

The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE.     

Stimulation of bile output by gastrointestinal hormones following portoenterostomy for biliary atresia

For biliary atresia portoenterostomy with externally draining conduit provides a model for quantitation of hepatic excretory function and for assessment of the physiologic response of the intrahepatic biliary system to gastrointestinal hormones in a human hepatopathologic condition. Four patients with biliary atresia were serially evaluated from 2 weeks to 43 months following total bile diverting portoenterostomy. A fifth patient with no bile flow provided a control for these studies. The pattern of Rose Bengal excretion for three patients with a satisfactory clinical course was different from that of a fourth patient with highly variable flow and persistent cholestasis. Marked volume and bicarbonate concentration increases in bile were noted 30 to 45 minutes after secretin infusion but only in the four patients with bile flow. The volume response to glucagon was more diffuse. Bilirubin and bile acid concentrations decreased in the stimulated bile flow periods and hourly outputs of these cholephils were not increased above basal. During two intervals of low bile output, secretin markedly increased bile flow in the patient with persistent cholestasis establishing the patency of the hepatoenteric anastomosis (functional obstruction) in contrast to the lack of secretin response in the control (structural obstruction).     

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。     

Effect of previous abdominal surgery and gallbladder appearance on biliary atresia outcomes

Background

Biliary atresia (BA) is typically treated by Kasai portoenterostomy (KPE), and there is a relationship between age at surgery and outcome. We hypothesize that previous abdominal surgery (PAS) for associated congenital intestinal conditions could be used to identify BA earlier, perhaps improving prognosis.

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。