Hepatic portoenterostomy for biliary atresia. A comparative study of histology and prognosis after surgery

Specimens of excised tissue from the porta hepatis in 26 infants with extrahepatic biliary atresia undergoing hepatic portoenterostomy were analysed histologically for the presence and size of biliary ductules. No correlation could be found between the establishment of effective biliary drainage and the number or size of biliary ductules. it is suggested that prognosis after surgery may be related to the intrahepatic lesion and age of the child at operation rather than to the histology of the extrahepatic bile duct remnants.     

Comprehensive assessment of prognosis after laparoscopic portoenterostomy for biliary atresia

Purpose

Total bilirubin (T-bil) is used universally for monitoring post-portoenterostomy (PE) biliary atresia (BA) patients although other biochemical markers [BM; AST/ALT and platelet count (PC)] are also prognostic. We compared open PE (OPE) with laparoscopic PE (LPE) using T-bil, AST/ALT, and PC (3BM) as more comprehensive indicators of postoperative clinical status.

Methods

Subjects were 31 PE cases (LPE: n = 17; OPE: n = 14). BA classification was type III (n = 16), type II (n = 1) in LPE and type III (n = 12), type I (n = 1), type II (n = 1) in OPE.

Results

Mean ages and weights at PE were similar: 65.5 days, 4.4 kg (LPE) versus 69.3 days, 4.1 kg (OPE); and mean follow-up was 2.5 years for both LPE and OPE. Jaundice clearance (T-bil ≤1.2 mg/dL) was achieved in 16/17 (94.1 %) after LPE versus 10/14 (71.4 %) after OPE (p = NS), but 3BM were closer to normal after OPE. At the time of review, 13/17 LPE cases (76.5 %) were alive with native livers and 4/17 had received LTx (23.5 %) and 10/14 OPE cases (71.4 %) were alive with native livers and 4/14 had received LTx (28.6 %).

Conclusions

Although JC was better after LPE, 3BM were better after OPE. Further follow-up will prove the comprehensive prognostic value of 3BM.

     

胆道闭锁Kasai手术后综合管理

胆道闭锁(biliary atresia,BA)是婴儿期肝内外胆管进行性炎症及肝纤维化的疾病,其病因及疾病进展机制尚不清楚.Kasai手术(Kasai portoenterostomy,KPE)是治疗胆道闭锁的首选术式,成功的Kasai手术能够重建胆汁引流,但Kasai手术后长期自体肝生存(native liver s...     

Hepatic calcification in an anicteric patient with biliary atresia after hepatic portoenterostomy

Hepatic calcification was found in an anicteric infant with biliary atresia after hepatic portoenterostomy. When she was 2 years of age, ultrasonography detected an echogenic structure with acoustic shadowing. Computerized tomography located it at the proximal portion of Segment 6 in the right lobe. Although both techniques failed to exhibit dilatation of the peripheral bile ducts, we believe intrahepatic cholelithiasis provides the most likely diagnosis. Although seven cases with biliary atresia were reported to have this complication, all of them were icteric when the diagnosis was confirmed. The patient should be carefully followed up since the succeeding stones may cause jaundice by obstructing the main duct.     

Laparoscopic Kasai portoenterostomy for biliary atresia

Conventional surgery for extrahepatic bile-duct atresia (EHBDA) usually requires a large, painful, muscle-cutting laparotomy, dislodgment of the liver, and wide manipulations, followed by adhesions and possible complications that may disturb the postoperative course and hamper liver transplantation (LT). The main role of laparoscopy in EHBDA has been for diagnostic purposes. Besides all the advantages of minimally-invasive access, it allows excellent visibility and dissection of tiny hilar structures. The authors present the first two cases of successful Roux-en-Y laparoscopic portoenterostomy (LARP) for EHBDA, showing the importance of advanced technical skills and a new approach for extracorporeal enteroanastomosis. Laparoscopic hilar dissection and portoenterostomy was accomplished using four trocars. The umbilical site was used for extracorporeal Roux-en-Y enteroenterostomy, in the first case using a laparoscopic stapler and in the second a hand-sewn suture. Mean operative time was 190 min, and no operative complications were observed. Both girls became anicteric. The first is doing well 15 months after the operation with good hepatic function. The other was anicteric for 6 months, had one episode of cholangitis, developed an umbilical hernia, has shown slow and progressive hepatic failure, and is now being evaluated for possible LT. It is concluded that LARP for EHBDA can be done safely in infants using an extracorporeal transumbilical enteric anastomosis, with several advantages compared with open surgery. The role of LARP in facilitating LT is yet to be defined.     

胆道闭锁Kasai术后糖皮质激素治疗的研究进展

胆道闭锁是累及肝内外胆管的一种进行性疾病,目前广泛采用的治疗策略是行肝门-空肠吻合术(Kasai手术),术后辅以包括糖皮质激素、抗生素等多种药物治疗,然而糖皮质激素在胆道闭锁的治疗中争议较多,其应用方案也多种多样,现将糖皮质激素在胆道闭锁Kasai术后的应用进展进行综述。     

胆道闭锁肝门肠吻合术手术改进的初步探讨

目的 对胆道闭锁(BA)肝门肠吻合术(PE)进行手术改进并评价治疗效果.方法 对2012年3月至2014年2月进行PE的Ⅲ型BA病例的临床资料进行回顾性分析.本研究病例的PE方式分为扩大肝门部病变切除范围PE手术(EPE)、Nakamura的Kasai改进手术(MKPE)和本研究的改进Kasai手术(OMKPE)3种并根据PE方式将病例分组.对病例的术前临床资料、术后黄疸清除率、胆管炎发作率和自体肝生存率按PE方式分组比较,并按不同手术年龄(≤90d和＞90d两组)再进行组间比较.结果 87例获得完整随访,其中EPE 34例,MKPE 19例,OMKPE 34例.各组手术年龄、体重、术前肝功能指标、肝外胆管Ohi亚型和术中肝纤维化Weerasooriya分级比较均无统计学差异(P＞0.05).OMKPE的术后黄疸清除率和自体肝生存率为73.5％和73.5％高于EPE的38.2％和38.2％ (P＜0.05),但MKPE的为57.9％和58.9％,与OMKPE和EPE比较,差异无统计学意义(P＞0.05),其中≤90 d手术年龄组中OMKPE的术后黄疸清除率和自体肝生存率为90.9％和90.9％以及MKPE的为90％和90％,高于EPE的46.2％和38.5％(P＜0.05),但OMKPE与MKPE比较,差异无统计学意义(P＞0.05),＞90 d手术年龄组中OMKPE的术后黄疸清除率和自体肝生存率为65.2％和65.2％,高于MKPE的22.2％和22.2％以及EPE的12.5％和12.5％ (P＜0.05)但MKPE与EPE比较,差异无统计学意义(P＞0.05).胆管炎发作率各组比较差异无统计学意义(P＞0.05).结论 对比EPE和MKPE,OMKPE手术更有利于提高BA术后黄疸清除率和延长自体肝生存,尤其适用于＞90 d大龄患儿.     

Outcome of early hepatic portoenterostomy for biliary atresia

BACKGROUND: The outcome of the hepatic portoenterostomy (Kasai) procedure for biliary atresia is improved when it is performed before 90 days of age. However, it is not known whether intervention before 30 days is better than intervention between 30 and 90 days. METHODS: The authors reviewed the records of all patients seen by the Pediatric Gastroenterology Service at St. Louis Children's Hospital from 1984-1999 to ascertain the outcome of patients who underwent Kasai procedure before or after 30 days of age. RESULTS: Of 92 patients with biliary atresia treated at St. Louis Children's Hospital over 15 years, 9 underwent the Kasai procedure before 30 days of age. Liver transplantation was necessary in 77.8% of these patients at a mean age of 11.0 +/- 4.26 months, as compared with 53.4% at 32.14 +/- 7.14 months for the remainder of the patients who underwent the procedure after 30 days of age. CONCLUSIONS: Although these data suggest that outcomes are worse for patients who undergo the procedure before 30 days of age, they may reflect a difference in the pathogenesis of biliary atresia that brings it to clinical attention earlier and may provide further evidence that biliary atresia is a phenotype for a number of distinct underlying disease processes.     

Intrahepatic biliary cysts after hepatic portoenterostomy in four children with biliary atresia

We report our experience with 4 cases of cystic dilatation of intrahepatic bile ducts following hepatic portoenterostomy for biliary atresia. Two of the cases did not achieve satisfactory bile excretion and all four cases developed recurrent cholangitis after hepatic portoenterostomy. The attacks of cholangitis seemed to be associated with the presence of intrahepatic cysts. Although one case resulted in death from hepatic failure, three other cases are now outpatients. Patients who develop recurrent cholangitis following hepatic portoenterostomy, should be examined to exclude the presence of intrahepatic biliary cysts. Ultrasonography, computed tomography and percutaneous transhepatic cholangiography were all effective in detecting cysts and provided valuable information for planning treatment. Percutaneous transhepatic or surgical drainage of the bile ducts was effective in reducing jaundice, and recurrent cholangitis.     

Histological assessment of bile lake formation after hepatic portoenterostomy for biliary atresia

Bile lakes develop after hepatic portoenterostomy in some patients with biliary atresia, and have been regarded as an indication of poor prognosis. We reported that bile lakes have no epithelium of the bile duct on their wall, and are surrounded by bile ducts; however, the mechanism of bile lake formation is little known. We investigated histologically how bile ducts are formed using whole removed liver, and the characteristics of bile ducts around bile lakes. From April 1980 to July 2006, we encountered 84 patients with biliary atresia. Bile lakes were analyzed histologically in 11 patients who underwent liver transplantation in our hospital. Bile lakes had a fibrotic cyst wall and lacked epithelia. In most cases, bile stasis, calculi formation, damaged bile ducts, and invasion of inflammatory cells were observed around the bile lakes. Bile ducts around bile lakes were not stained by CD56, but bile ducts around liver lobuli were stained by CD56. The present study speculates that bile lakes would arise from original bile ducts, which are damaged, and fuse together after calculi are formed in bile ducts.     

Kasai术后肝内胆管囊性扩张的诊治与预后

目的 总结胆管闭锁术后肝内胆管囊性扩张的诊治经验,分析其对患儿预后的影响.方法 1998年6月至2008年3月,对胆管闭锁行葛西手术的患儿进行追踪随访.通过超声检查,发现8例患儿存在肝内胆管扩张.其中3例再行MRI检查,6例再行CT平扫加增强检杳以明确诊断.此8例患儿术后均有不同程度胆管炎症状.5例行PTCD显示肝内胆管囊性扩张.3例患儿行囊肿与空肠胆支再吻合术,2例患儿仪行PTCD置管引流,2例患儿暂未予任何处理.结果 3例经手术治疗的患儿,术后黄疸消退或减轻.仅行PTCD的患儿肝内胆管扩张长期存在.结论 对反复发作的胆管炎应定期行超声检查,尽早发现肝内囊肿;对肝内胆管囊性扩张,无论其影像学分型如何,应根据其具体临床表现进行相应的积极治疗;PTCD无法长期放置,仅是暂时性的治疗,但对手术时寻找囊肿有指示作用,使手术时囊肿的定位相对容易;囊肿的大小、位置与门静脉的关系,压迫的时间、治疗是否及时有效都可影响患儿的预后.     

Abdominal malignant lymphoma in a child after successful portoenterostomy for biliary atresia

Malignant disorders in long-term survivors of biliary atresia have rarely been reported. We report a case of abdominal malignant lymphoma in a 5-year-old boy who underwent a successful portoenterostomy for biliary atresia at the age of 45 days. Correspondence to: M. Honzumi     

胆道闭锁术后肝内胆管囊性扩张的诊治

目的总结胆道闭锁术后肝内胆管扩张的诊治经验,探讨葛西手术后肝内胆管扩张对患儿远期预后的影响,以及如何早期诊断和治疗。方法2003年4月至2008年3月,对3例因胆道闭锁行葛西手术的患儿进行追踪随访,3例术后均有不同程度胆管炎症状,其中1例合并门脉高压。3例行超声、CT或经皮肝穿刺置管引流（PTCD）,结果显示肝内胆管囊性扩张。2例行胆管扩张与空肠胆支再吻合术,1例仅行PTCD置管引流。结果2例经手术治疗的患儿,术后黄疸消退或减轻。1例仅行PTCD的患儿肝内胆管扩张长期存在。结论肝内胆管扩张使胆管炎反复发作,特别是扩张的囊状胆管压迫门静脉,可使门静脉变窄,血流减少,致受累肝叶萎缩。胆道闭锁患儿葛西手术后应定期行超声检查,及时发现肝内胆管囊性扩张。葛西手术后肝内胆管扩张应早期诊断,早期手术治疗,术前应常规行PTCD,暂时解除胆汁淤积,并为术中定位做好准备。     

A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy

A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group of those who died or received liver transplantation. A score was calculated that assessed nine factors, including laboratory values and complications. The data were assessed at the time complications occurred. The scores were analysed by a trend analysis to see if serial scores predicted the evolution of liver disease. A receiver operating characteristic (ROC) curve was plotted to assess the optimal cut-point for the scoring system. There were 98 patients in the non-transplantation group and 35 in the failure group. The latter group had significantly higher post-operative bilirubin (9.3±7.2 mg/dl versus 3.5±3.1 mg/dl), ALT (136±89 U/l versus 92±88 U/l), prothrombin time, and incidence of cirrhosis, ascites, oesophageal varices, portal hypertension, cholangitis and sepsis than the non-transplantation group ( P <0.05). A score of 8 had a high sensitivity (96.9%) and specificity (89.5%) for predicting the need for liver transplant. Conclusion:based on easily available clinical information, our scoring system can predict which patients with biliary atresia who have already undergone a Kasai procedure should be considered for liver transplantation.Abbreviations EHBA extrahepatic biliary atresia - ROC receiver operating characteristic     

Changes in portal vein hemodynamics after hepatic portoenterostomy in biliary atresia

Portal vein (PV) shrinkage sometimes eliminates the possibility of liver transplantation in biliary atresia patients after hepatic portoenterostomy. To determine the factors leading to PV shrinkage, we performed a serial sonographic study of the portal venous system in 21 children. Cross-sectional PV area and mean portal venous velocity (PVV) were reduced in patients with refractory cholangitis and those with gastroesophageal varices and cholangitis. Although the reduction in cross-sectional PV area was greater in patients with four-time laparotomy than single laparotomy, the mean PVV was not reduced by repeated laparotomy. Patients with varices were lower in age, weight, mean PVV, cross-sectional PV area for age, and had higher serum total bilirubin levels. In conclusion, refractory cholangitis is a significant factor in shrinking the PV. With active bile drainage, varices spontaneously regress, the PV increases in both caliber and total length per unit hepatic volume, and PVV normalizes. It is suggested that pulsed Doppler PV sonography can help to determine the optimal time for liver transplantation referral in biliary atresia patients with progressive cirrhosis.This work was supported by a Grant for Pediatric Research (63-A-05) from the Ministry of Health and Welfare. Offprint requests to: K. Tanaka