The portoenterostomy procedure for biliary atresia: a five year experience.

The portoenterostomy (Kasai) procedure in infants with biliary atresia has dramatically altered the outlook for this heretofore fatal disease. When performed on infants under three months of age, bile drainage can be achieved in a majority of the patients. Since 1972, 37 infants have been treated with this operation at our institution. Diagnostic operative cholangiography and liver biopsy are recommended if the cause of conjugated hyperbilirubinemia is presumed to be obstructive. When biliary atresia is encountered, identification of the atretic ducts with transection high in the porta hepatis is carried out. Thirty-two infants have had the portojejunostomy, while five, in whom the proximal hepatic ducts were atretic but the gall bladder and distal ducts were patent, underwent portocholecystostomy. Examination of the resected fibrous duct tissue revealed a statistically significant correlation between ductal histology and postoperative outcome. Extended bile drainage has been achieved in 26 of 37 patients. Seventeen exhibit near normal growth and development four months to five years postoperatively. Seven have died with progressive liver disease despite bile drainage. Two additional patients died, jaundice free, from unrelated causes. Despite bile drainage, progressive hepatic fibrosis has been confirmed by serial biopsies in 14 patients. This finding indicates that biliary obstruction is not the sole component in the development of biliary cirrhosis. These data suggest that extrahepatic biliary atresia is a dynamic obliterative process, which can be favorably modified in approximately 50% of the infants by early surgical treatment.     

Catheter decompression of hepatic portocholecystostomy

Temporary catheter decompression of the gallbladder was employed in three infants having hepatic portocholecystostomy for biliary atresia. Postoperative biliary obstructive complications were avoided. A progressive increase in the calibre of the distal bile ducts was observed.     

Magnetic resonance cholangiography for the diagnosis of biliary atresia

Purpose: The aim of this study was to evaluate the usefulness of magnetic resonance cholangiography (MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods: Forty-seven consecutive infants with cholestatic jaundice underwent single-shot MRC. The diagnosis of biliary atresia was made by MRC based on the nonvisualization of extrahepatic bile ducts and excluded on the basis of the complete visualization of extrahepatic bile ducts. The final diagnosis of biliary atresia (BA group, n = 23) or nonbiliary atresia (NBA group, n = 24) was established by operation or clinical follow-up until the jaundice resolved. Results: The extrahepatic bile ducts including the gallbladder, the cystic duct, the common bile duct, and the common hepatic duct were visualized in 23 of the 24 infants of the NBA group. The extrahepatic bile ducts, except the gallbladder, were not depicted in any infant of the BA group. MRC had an accuracy of 98%, sensitivity of 100% and specificity of 96%, for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions: MRC is a very reliable noninvasive imaging modality for the diagnosis of biliary atresia. In infants with cholestatic jaundice and considered for exploratory laparotomy, MRC is recommended to avoid unnecessary surgery.     

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.     

Neonatal jaundice: the role of laparoscopy

BACKGROUND: When managing neonatal jaundice, despite continual improvement of diagnostic tests and increasing knowledge regarding its pathogenesis, there is no single test or imaging modality that can reliably define biliary atresia. Early diagnosis is essential for a better surgical outcome. In many situations, mini-laparotomy and operative cholangiography may be needed to settle the definitive diagnosis, with the risk of having negative exploration in those high-risk patients with medical etiology. The use of laparoscopy may help in avoiding unnecessary exploration for such group of patients. METHODS: Thirty-three patients aged between 1 and 4 months with conjugated hyperbilirubinemia were the subject for this study. All had a HIDA scan result suggestive of biliary atresia. They underwent a diagnostic laparoscopy before surgical exploration. When the gallbladder was not visualized we proceeded to laparotomy. In patients with a good size gallbladder visualized at laparoscopy, a laparoscopic-guided cholangiogram was then performed, and laparoscopic liver biopsy done for those who had patent biliary tree. RESULTS: Two groups of patients were identified: the first group (21 patients) showed small atretic gallbladder; 18 patients had biliary atresia with complete intra- and extrahepatic atresia; these patients underwent a Kasai hepatic-portoenterostomy. Two patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a portocholecystostomy. The last patient showed left-sided gallbladder arising from the left lobe of the liver that was missed during laparoscopy, and operative cholangiogram showed hypoplastic biliary ducts. The second group included 12 patients with good-sized gallbladder, and laparoscopic-guided percutaneous cholangiogram showed normal communicating patent biliary system, hypoplastic in 2, and they underwent laparoscopic liver biopsy. No mortality related to the laparoscopic procedure was encountered in this series, and one patient with hypoplastic gallbladder had adhesive intestinal obstruction on the fifth day after laparoscopy necessitating exploration. CONCLUSION: Laparoscopy with laparoscopic-guided cholangiography may be a very useful tool used in accurately diagnosing infants with conjugated hyperbilirubinemia, and in avoiding unnecessary laparotomies performed on these critical babies.     

Agenesis of the extrahepatic bile ducts: report of five cases

Five patients were encountered in whom agenesis either of the proximal extrahepatic biliary ducts (four patients) or total absence of the extrahepatic bile ducts and gall bladder (one patient) were evaluated. Jaundice was diagnosed from at birth to 3 weeks of age (average, 1.2 weeks of age) in these five patients. The patients' ages ranged from 2 to 8 weeks at the time of surgical exploration. Findings at surgery showed either absence of the entire extrahepatic biliary ducts or proximal bile duct remnants, no evidence of an inflammatory process, and no fibrous mass present at the portahepatis. Liver biopsy specimens showed histological evidence of cholestasis, minimal bile duct proliferation and fibrosis, and nearly complete absence of inflammation. In three patients in whom a portocholecystostomy was performed, no bile flow was obtained. Two patients underwent surgical exploration and liver biopsy only. One patient died as a result of severe congenital heart disease at 3 months of age. Four patients have undergone successful hepatic transplantation. These patients are now 10 months to 6 years of age. In our review of the literature, we were unable to find any reports of bile duct agenesis despite the fact that it appears to be a known phenomenon. We conclude that patients with biliary agenesis have early onset of jaundice when compared with patients with biliary atresia, absence of inflammation at the portahepatis at the time of surgical exploration, as well as on biopsy of the liver. Portoenterostomy or portocholecystostomy are likely to fail. We believe that liver transplantation is the treatment of choice for this rare entity.     

The surgery of biliary atresia

One hundred thirty-one consecutive infants with biliary atresia were operated on during the 15-year period between 1973 and 1988. Six patients did not have biliary reconstruction because of advanced cirrhosis or transplant preference. The other 125 infants had excision of all nonpatent extrahepatic bile ducts; biliary drainage was provided by a gallbladder-common bile duct conduit in 14 patients and by a Roux-en-Y portoenterostomy in 111 infants (including the seven patients with correctable biliary atresia). The bilioenteric conduit was temporarily exteriorized and, for the past 2 years, a conduit intussusception valve was incorporated. Immediate postsurgical bile drainage was achieved in 103 infants (82%). Reoperation during the first 6 postoperative weeks restored bile flow in 14 of 18 infants who had shut down. Seventy-two patients (57%) had sustained (more than 1 year) relief of biliary obstruction. Postoperative morbidity was substantial. The six children not having corrective surgery died within 19 months. Three patients were lost to follow-up. Sixty-eight patients having Kasai's operation died, 55 from complications of liver disease, 1 from a coexisting malformation, and 12 after liver transplantation. Fifty-seven patients are alive, 13 by virtue of liver replacement, 9 with mild-to-moderate hepatic sequelae, and 35 (28%) with normal to near-normal liver function. Although none is considered "cured," the 35 children are anicteric, have normal growth and development, and participate in full school activities (including contact sports). Average follow-up is 85.8 months (range 1 to 15 years).     

Surgery of coexisting biliary malformations in choledochal cyst

Five of 13 infants and children with a choledochal cyst had coexisting biliary malformations. The specific lesions were main hepatic duct cyst (2), common hepatic duct stenosis (1), left hepatic duct stenosis (1), and Caroli's disease (1). The malformations were corrected or partially corrected at the time of operative resection of the cyst. The lateral portion of either or both of the major hepatic ducts was incised to the liver hilus thereby dividing areas of stenosis or laying open cystic malformations. Biliary reconstruction was by Roux-en-Y jejunostomy to the fileted common hepatic bile duct. The opportunity to correct coexisting biliary malformations is another advantage of total excision of choledochal cysts.     

腹腔镜在新生儿及幼婴胆道疾病诊治中的应用

目的探讨腹腔镜在新生儿及幼婴胆道疾病诊治中的价值.方法回顾性分析2003年1月～2004年8月我院9例新生儿及幼婴胆道疾病腹腔诊断和治疗的临床资料.结果9例患儿行腹腔镜探查,其中胆总管囊肿2例,胆道闭锁5例,胆汁黏稠1例,胆道发育不良1例.8例胆道造影成功.2例胆总管囊肿行腹腔镜下囊肿切除、肝管空肠Roux-Y吻合术.5例胆道闭锁中3例行开腹扩大肝门、肝肠吻合术;1例行腹腔镜下扩大肝门、肝肠吻合术;1例拒绝根治,放弃手术.1例胆道发育不良行开腹扩大肝门、肝肠吻合术.1例胆汁黏稠行胆道冲洗术.结论腹腔镜是诊断新生儿及幼婴胆道疾病简单、可靠的方法.腹腔镜治疗胆道疾病具有微创、美观、出血少、恢复快优特点,临床效果可靠.     

In biliary atresia duct histology correlates with bile flow

Three basic types of microscopic biliary structures at the portahepatis were distinguishable in infants with biliary atresia: bile ducts, collecting ductules of biliary glands, and biliary glands. Correlation between the type of biliary structure and the quantity and quality of post-operative bile flow was possible in 23 instances. At 2 weeks after operation, the 11 patients in whom a bile duct was identified had a daily bile flow of 68.0 +/- 11.5 mL. Bilirubin concentration in the bile was 13.6 +/- 3.3 mg/dL and total daily bilirubin excretion was 8.77 +/- 2.74 mg. In contrast, bile flow in 12 patients having only collecting ductules and/or biliary glands in the porta hepatis was 19.1 +/- 3.9 mL and bilirubin concentration in bile was 1.7 +/- 0.3 mg/dL. Thus, total daily bilirubin excretion was 0.34 +/- 0.08 mg (P less than 0.001). Postoperative cholangitis occurred only in patients with ducts. It is concluded that only bile ducts communicate with the intrahepatic biliary system and drain bile after hepatic portoenterostomy.     

成人右半肝活体肝移植胆道重建探讨

目的探讨成人右半肝活体肝移植胆道重建的技术问题.方法回顾性分析我院2007年4月至2009年5月完成的21例成人右半肝活体肝移植资料.供肝右肝管与受者肝总管单个吻合10例;供肝两支胆管开口分别与受者两支胆管吻合5例;供肝胆管整形成一个开口与受者胆管吻合5例,其中采用T管支撑2例,Y型管支撑1例;右肝管空肠Roux-en-Y吻合1例.结果4例受者术后1个月内死亡,1例因术后急性肝坏死行再次肝移植.其余受者存活至今,1年存活率为77.65%.受者术后发生胆道并发症7例,其中胆漏5例,胆道狭窄2例,均经外科手术处理痊愈.胆管与胆管单个吻合口组、胆管整形成一个开口与受者胆管吻合组和两支胆管开口分别与受者胆管吻合组比较,胆道并发症发生率差异无统计学意义(x2=0.659,P=0.719).结论根据供受者胆管情况,可以灵活采用单根胆管吻合、胆管整形、分别吻合和肝管空肠吻合等不同重建方式.后壁连续、前壁间断以及显微外科技术的采用可能有助于降低胆道并发症的发生率.     

肝移植术变异胆管胆漏的预防与处理

目的 探讨肝移植术中变异肝管胆漏的预防及治疗.方法 回顾性分析我院3例肝移植术后发生变异肝管胆漏的诊断及预防、治疗方法.3例供肝切取均采用肝肾联合切取的方法,胆管重建方式为胆总管端端吻合.结果 1例右后叶副肝管汇入胆囊管患者在胆管吻合后发现肝门处胆囊管残端胆汁漏出,立即拆除原胆管吻合口,成型后一期吻合,术后痊愈.1例Luschka胆管漏患者术后胆漏经过充分引流漏口自行闭合痊愈,但最后终因肝内外感染而于术后7个月再次肝移植.另一例右后叶副肝管汇入胆总管患者,术中遗漏断端导致术后胆漏.该患者因严重并发症行二次肝移植.结论 了解肝内外胆管的解剖和常见变异形式,供肝修整时仔细辨认肝门组织,提高对存在副肝管及迷走胆管变异的警惕性,对预防肝移植术后胆管断端胆漏的发生非常重要.     

Bile duct patterns in the hilar region of the liver in two cases of biliary atresia

Bile duct patterns in the hilar region of the liver were demonstrated in two autopsy cases of biliary atresia by three-dimensional graphic reconstructions. In one case, jaundice had completely disappeared after successful hepatic portojejunostomy. However, at the age of 4 months, this patient died of cardiac failure caused by endocardial cushion defect. The histological reconstruction of this case showed that there were large patent bile ducts, 150 microns to 400 microns in diameter, and plenty of bile ductules surrounding the large ducts to form networks. The other patient died of hepatic failure at the age of 9 months. In this case, only a few large ducts ended in bile lakes that did not connect with the portojejunostomy, and only a few ductules were found around the large ducts. These results indicate that the existence of networks of bile ductules and the patency of large bile ducts at the porta hepatis contribute to good postoperative bile excretion.     

Small Bile Duct Reconstruction of the Caudate Lobe in Living-Related Liver Transplantation

OBJECTIVE: To justify the technique of biliary reconstruction without mucosa-to-mucosa alignment for reconstructing the caudate lobe bile duct. SUMMARY BACKGROUND DATA: The use of a left hepatic lobe graft with the caudate lobe (LHGC) has been introduced to resolve the problem of small-for-size grafts in living-related liver transplantation. The authors have found that the LHGCs occasionally have independent openings of the bile duct of the caudate lobe. METHODS: The graft bile ducts were anastomosed to Roux-en-Y jejunal loops. The main left hepatic ducts were reconstructed in a standard manner. Small bile ducts of the caudate lobe were anastomosed to the intestine without mucosa-to-mucosa alignment, with an external biliary drainage tube, positioned transanastomotically. RESULTS: In 8 of the 19 patients who received LHGCs, nine bile ducts of the caudate lobe (median 1 mm) were reconstructed. After surgery there was no bile leakage. In five of the eight patients, the tubes were removed a median of 92 days after transplantation. Bile duct dilatation had not been observed at a median of 363 days after surgery. CONCLUSIONS: The authors consider their technique of biliary reconstruction without mucosa-to-mucosa alignment useful for the safe reconstruction of small bile ducts of the caudate lobe.     

Clinico-pathological studies on a transitional type between extrahepatic biliary atresia and paucity of the interlobular bile ducts

Among the neonatal and infantile cases of obstructive jaundice seen at Niigata and Yamagata University Hospitals between 1976 and 1990, extrahepatic bile ducts were visualized in 19 cases by either preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiography. Neonatal hepatitis was diagnosed in 3 of these cases by clear images of the bile duct system extending from the common bile duct to the intrahepatic bile duct. In 7 cases, the common bile duct was able to be seen, while the common hepatic duct was only slightly visualized. Four of these 7 cases were consistent with paucity of the interlobular bile ducts (PILBD) based on hepatic histology, while the remaining 3 showed fibrosis, bile ductular proliferation, and many bile plugs in the bile ductuli of the portal areas, concurrent with histological changes in extrahepatic biliary atresia (EHBA), not PILBD. In 9 cases, only the common bile duct was visualized while the common hepatic duct was not seen, 7 of these 9 cases being consistent with type III-al EHBA. In 2 cases, neither fibrosis nor proliferation of the bile ductuli was observed in the portal areas, and portal areas without any bile ductuli were also seen, in accordance with findings for PILBD. Three cases which showed similar hepatic histological findings to EHBA despite the presence of patent extrahepatic bile ducts, and 2 cases which had obstructed extrahepatic bile ducts and hepatic histological findings similar to PILBD, were thought to be of a transitional type between EHBA and PILBD. Thus, it is postulated that the entire bile duct system covering hepatocytes to extrahepatic bile ducts is affected by a certain factor, but the expression of individual disease types may be dependent upon the location of the site most seriously affected.     

A histopathological study of the remnant of extrahepatic bile duct in so-called uncorrectable biliary atresia

Histopathological study of the remnant of extrahepatic bile ducts in 40 cases of so-called uncorrectable biliary atresia, upon which we operated the last three years, has been performed. The histological findings of the remnant were classified into three types.Only two cases were found to have type 1a ducts in the porta hepatis area, from which we can expect better prognosis postoperatively. We also found that as the patients become older, the size of the duct in the remnant becomes smaller and the hepatic fibrosis becomes more remarkable. Therefore the operation should be performed in the infant with this lesion as young as possible.As for the evaluation of operative results of hepatic portoenterostomy for this lesion, a proper evaluation can be made only in those cases in which a microscopic examination of the remnant of extrahepatic bile duct at the porta hepatis area has been adequately performed.Concerning the pathogenesis of biliary atresia, we presume that congenital abnormalities of bile ducts are a basic factor, and additional nonspecific inflammation and bile stasis complete its pathological condition.     

Identification of the jaundiced infant who is likely to recover without surgical intervention.

A series of 32 infants with persistant jaundice in whom an unequivocal differentiation between intrahepatic cholestasis and biliary atresia could not be made is reviewed. A protocol including Lipoprotein-X (LP-X) determinations before and after a short course of cholestyramine (CSM) was carried out in all. A fall in serum LP-X after CSM indicates the presence of patent extrahepatic bile ducts (even microscopic) which will function without benefit of hepatic portoenterostomy. A rise in LP-X levels after CSM means an atretic biliary system. The LP-X, CSM protocol was not able to differentiate between the anatomical variants of biliary atresia that may respond to hepatic portoenterostomy and those that will not. Patent bile ducts (even microscopic) in the porta hepatis and/or proximal hepatoduodenal ligament, which are in continuity with intrahepatic ducts, must be present if hepatic portoenterostomy is to be successful. None of our 12 infants undergoing hepatic portoenterostomy showed evidence of bile excretion after the procedure. Microscopic study of serial sections taken through the excised hepatoduodenal ligament tissues of these 12 infants revealed that none had anatomical findings conducive to the success of the operation.     

Massive bile pertonitis in infancy due to spontaneous bile duct perforation with portal vein occlusion.

A case of massive bile peritonitis due to spontaneous slough of the junction of the cystic, common, and common hepatic ducts in a 5-wk-old male infant is reported. This was managed successfully by a Roux-en-Y jejunal anastomosis to the remnant of common hepatic duct. Subsequent ascites (4 mo postoperatively) was relieved by peritoneovenous drainage by a Holter valve from the peritoneal cavity to the right atrium. Comments are made relative to an etiologic commonality between spontaneous perforation of the bile ducts in infancy, “congenital” choledochal cyst, and biliary atresia and a possible role for birth-acquired viral infection, particularly with a herpes type of virus, in these disorders.     

医源性胆道损伤52例报告

目的:总结医源性胆道损伤的经验教训。方法:对过去33年间5 2例医源性胆道损伤进行回顾性分析。结果:肝外胆道手术所致4 8例,胃大部切除术及肝脏手术所致各2例。损伤部位在肝总管与胆总管交界处34例,肝总管6例,胆总管6例,左右肝管汇合部4例,左、右肝管各1例。胆管完全性损伤30例,部分性损伤2 2例。结论:要警惕医源性胆道损伤的发生,及早诊断并修复胆道的连续性是提高疗效的关键