The future role of hepatic portoenterostomy as treatment of biliary atresia.

According to our recent study, 38 of 93 patients (40.9%) who underwent portoenterostomy at Juntendo University Hospital between 1977 and 1986 survived for more than 5 years. In order to learn the future improvement of the prognosis of biliary atresia patients who undergo portoenterostomy, we investigated the relationship between the prognosis of biliary atresia patients and the age and the histological changes of liver at the time of surgery. Twenty-seven of 30 patients (90%) who survived for more than 5 years with no jaundice and no findings of liver cirrhosis had a mild degree of liver fibrosis (F1 or F2) and a mild degree of degeneration of intrahepatic bile ducts (B1 or B2) at the time of surgery, in spite of the difference of size of intrahepatic bile ducts at the porta hepatis. On the other hand, all 8 patients who showed severe liver fibrosis (F3) and severe degeneration of intrahepatic bile ducts (B3) did not survive for more than 5 years. Accordingly, we can expect promising prognoses in patients who undergo refined portoenterostomy procedures and receive proper postoperative treatment before they have severe histological changes of liver. The portoenterostomy will still play an important role as treatment of biliary atresia in the future.     

Surgical experience in children with biliary atresia treated with portoenterostomy

OBJECTIVE: Biliary atresia is the result of a fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Without surgical intervention, mortality reaches 100%. The 5-year survival rate after portoenterostomy ranges from 13% to 60%, with approximately 60% of patients requiring liver transplantation at a later stage because of insufficient bile flow. METHODS: This retrospective analysis includes 30 consecutive patients undergoing portoenterostomy for biliary atresia at our hospital. RESULTS: The 5-year actuarial survival of the 30 patients was 68%. Thirteen patients (43.3%) died 3 days to 7 years after portoenterostomy. Four patients (13.3%) underwent liver transplantation 3 to 24 months after the Kasai procedure with a 100% survival. In 65% of patients without presence of cirrhosis, the portoenterostomy was successful, compared with 35% of cases with liver cirrhosis (p = 0.0148). Liver cirrhosis with extrahepatic biliary atresia alone was present in 5 of 17 patients (29%) as compared with 8 of 12 patients (66%) with intrahepatic biliary hypoplasia in addition to extrahepatic biliary atresia and cirrhosis. CONCLUSIONS: Portoenterostomy remains the treatment of choice for patients with extrahepatic biliary atresia. However, the presence of cirrhosis portends a poorer prognosis and may be an indication for early transplantation. Cirrhosis is more commonly present in the setting of intrahepatic biliary hypoplasia and may account for the lower success rates of portoenterostomy in this group of patients. Five-year survival of the female patients was 88% as compared with 55% of the male patients.     

Kasai portoenterostomy--new insights from hepatic morphology

Background/Purpose

The aim of this paper was to investigate the mechanism of long-term biliary drainage after Kasai portoenterostomy by clinicopathologic study of hepatic morphology in explanted livers.

Methods

Explanted livers from 13 consecutive children undergoing transplantation for biliary atresia were examined in detail using a standardized protocol. Group 1 (n = 6) had no Kasai procedure before transplantation at a median age of 8 m. Group 2 (n = 4) were transplanted at a median age of 10 m after a failed Kasai portoenterostomy. Group 3 (n = 3) had a successful Kasai but required transplantation for complications of chronic liver disease at 12-14 years. Pathology findings were correlated with hepatic morphology determined by pretransplant magnetic resonance imaging.

Results

Large perihilar regenerative nodules (8-14 cm diameter) were observed in 2 patients after successful Kasai portoenterostomy, less well-defined perihilar nodules in group 2 patients, and no regenerative nodules in group 1. Microscopically, group 1 had diffuse biliary cirrhosis with evidence of progressive ductopenia during infancy. In group 2, perihilar regenerative nodules showed variable portal fibrosis but no cirrhosis and bile ducts were present with 68%-100% of hepatic arteries; in peripheral cirrhotic areas, bile ducts were absent in patients older than 9 m. The perihilar regenerative nodules in group 3 patients had a noncirrhotic architecture with preserved bile ducts, but the peripheral parenchyma was cirrhotic; one patient had diffuse macronodular cirrhosis. These morphologic findings correlated well with magnetic resonance images, highlighting the preservation of relatively normal perihilar liver architecture after successful Kasai portoenterostomy.

Conclusions

Unoperated biliary atresia is associated with progressive intrahepatic ductopenia leading to diffuse biliary cirrhosis. Kasai portoenterostomy can result in the growth of large perihilar regenerative nodules, probably as a consequence of surviving intrahepatic ducts in this region. In some patients, long-term success after Kasai portoenterostomy may depend on hyperplasia of the perihilar liver.     

The surgery of "correctable" biliary atresia

Seven of 114 (6%) patients treated in Denver for biliary atresia had a variant of the disease in which only microscopic bile ducts or ductules were identified at the porta hepatis, hepatic lesions were present, even in the neonatal example, and intrahepatic biliary hypoplasia was uniformly present. The excised bile duct cysts consisted of fibrosis or scar tissue and with little or no epithelial lining. Six patients had corrective surgery. The first patient was treated by choledochoenterostomy and became totally obstructed. Definitive operation in this patient and all subsequent patients consisted of excision of all extrahepatic duct structures and Roux-en-Y portoenterostomy. Two patients died, the one who did not have surgical correction and a second who had correction at age 35 weeks. The other five patients have been followed for 18 to 158 months and are anicteric but have some degree of residual liver damage. We conclude that so-called correctable biliary atresia occurs in less than 10% of cases, that because of coincident biliary hypoplasia, "cure" is not a possible outcome, and that the treatment of choice is complete surgical excision of the extrahepatic bile ducts and Roux-en-Y portoenterostomy.     

A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers.

OBJECTIVE: The authors investigated risk factors for failure after portoenterostomy for biliary atresia using univariate and multivariable methods. SUMMARY BACKGROUND DATA: Kasai's portoenterostomy has gained worldwide acceptance as the initial surgical therapy for infants with biliary atresia. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. METHODS: The authors analyzed risks for failure in 266 patients treated from 1972 to 1996 by the Kaplan-Meier product limit estimate and Cox proportional hazards model. Failure was defined as death or transplant. RESULTS: Age at surgery, surgical decade, and anatomy of atretic bile ducts were identified as independent risk factors. Five-year survival was 49% and median survival was 15 years when bile drainage was achieved. Sixty-five patients had liver transplants. Mean age at transplant was 5.4 years. CONCLUSIONS: The outcome after portoenterostomy for biliary atresia is determined by age at surgery and anatomy of the atretic extrahepatic bile ducts. Liver transplant will salvage patients with failed Kasai with 10-year posttransplant survival of 71%.     

A study of patients with long-term bile flow after hepatic portoenterostomy for biliary atresia

Thirty-five cases of biliary atresia that demonstrated the creation of internal fistula between intrahepatic bile ducts at the portahepatis and intestine and showed long-term bile flow after our hepatic portoenterostomy were investigated from various aspects. These 35 cases were divided into Group A cases, in which jaundice disappeared within three months after surgery, and Group B cases, in which persistent jaundice was seen more than three months after surgery. (1) Severe liver fibrosis and degeneration of intrahepatic bile ducts were more often seen in Group B cases than in Group A cases. (2) The age at the time of surgery and the size of intrahepatic bile ducts at the portahepatis did not have much influence on the operative results in those 35 cases. (3) Measurement of the amount of bile flow and bile acid excretion obtained from Suruga II enterostomy is useful for evaluating the postoperative results of biliary atresia patients. (4) Reoperation that includes curettage and rehepatic portoenterostomy, and serious postoperative ascending cholangitis were each closely related to postoperative persistent jaundice.     

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.     

Incidence of intrahepatic biliary cysts in biliary atresia after hepatic portoenterostomy and associated histopathologic findings in the liver and porta hepatis at diagnosis.

BACKGROUND/PURPOSE: The association of intrahepatic biliary cysts in biliary atresia (BA) after hepatic portoenterostomy has drawn the attention of many pediatric surgeons and has become an important subject in clinical management, but the incidence and causes of this association are not well known. METHODS: During the last 14 years, we operated on 33 BA patients by hepatic portoenterostomy. Five patients could not be followed up over a 1-year period, so the remaining 28 patients had their intrahepatic structure investigated by periodic magnetic resonance imaging (MRI), and were confirmed at autopsy. The authors also reviewed the preoperative conditions of these patients and the histopathologic changes in the liver biopsy and the porta hepatis specimens taken at portoenterostomy. RESULTS: (1) Intrahepatic biliary cysts were found in 6 of the 28 patients studied (21%). Of 16 patients who were operated on after 1989 and are undergoing follow-up by MRI, 4 (25%) had cysts. (2) Five of the 6 patients with cysts had episodes of cholangitis before or at the time of discovery of the cysts, and 4 of them have had their jaundice reappear since the discovery of the cysts. (3) The intralobular spaces (ILS) with fibrosis and the number of bile ducts had increased in the group with cysts compared with those in the group without cysts. (4) The bile duct maximum size in the porta hepatis (PH) was similar in the groups with and without cysts, but a denudation of the mucosal lining cells in the duct, and a fibrosis and inflammation around the duct had increased in the group with cysts compared with those in the group without cysts. (5) Statistically, the fibrosis found in the ILS and the inflammation around the bile duct found in the PH were induced as a correlative factor affecting cyst formation. CONCLUSIONS: The association of intrahepatic biliary cysts in BA after portoenterostomy is frequent. The fibrous change in the ILS and the inflammatory process around the bile ducts might be potential causes of cyst formation.     

Extrahepatic biliary atresia: a review of current management

Macroscopic examination of the extrahepatic bile ducts in infants with biliary atresia reveals fibrous occlusion of variable extent from an inflammatory process of unknown aetiology. Histological studies have shown that bile duct remnants at the porta hepatis frequently contain small epithelium-lined channels which communicate with intrahepatic ducts and through which effective bile drainage may be established by the operation of portoenterostomy. The 4-year survival rate in untreated cases is 2 per cent, but surgical treatment can improve the outlook and recent reports suggest that a 5-year survival rate of over 35 per cent can be achieved with portoenterostomies performed before 10 weeks of age. Complications after surgery include progressive liver disease, ascending bacterial cholangitis and portal hypertension.     

Ultrasonographic findings and management of intrahepatic biliary tract abnormalities after portoenterostomy

Ultrasonographic examinations was made in 24 children who had undergone a portenterostomy to correct extrahepatic biliary atresia. Abnormalities were observed in six patients. These were the result of ongoing inflammatory reactions because all had been suffering from cholangitis after surgery, and the size of the biliary tract structure changed in accordance with the occurrence and subsidence of the cholangitis. Ultrasonographic examination showed dilation of the intrahepatic bile duct in one patient and cystic lesions in five patients. Treatment included percutaneous transhepatic bile drainage for dilated bile ducts, alcohol injections for intrahepatic cysts, and reoperation for cysts in the porta hepatis. Treatment was not required for cysts in controllable cholangitis. The results of these approaches were excellent, indicating that they were of benefit in treatment intrahepatic abnormalities occurring after portoenterostomy.     

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.     

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.     

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 Kasai portoenterostomy: when is it too late?

BACKGROUND/PURPOSE: Kasai portoenterostomy is recommended as the primary initial therapy for extrahepatic biliary atresia if the procedure can be performed within 10 to 12 weeks of life. The optimal management for infants with delayed presentation of biliary atresia remains controversial. The purpose of this study was to determine the success rate and outcome for patients who underwent a "late" Kasai portoenterostomy. METHODS: The authors conducted a retrospective review of the medical records of all patients with biliary atresia who underwent a Kasai portoenterostomy at their institution from 1986 to 1999 (n = 31). The authors analyzed success rates compared with age at the time of the Kasai procedure and the association with patient demographics. Surgical success was defined as achievement of a total serum bilirubin < or = 2 mg/dL. Long-term follow-up assessments included the need for liver transplantation and patient survival rate. RESULTS: The demographics of this study cohort showed a predominance of African-Americans, 19 of 31 (61%), and girls, 23 of 31 (74%). Assessment of success compared with subject age at the time of the initial portoenterostomy showed that 52% (13 of 25) had successful Kasai procedure at 0 to 75 days, compared with 83% success rate (5 of 6) at age 76 days or older (P = .359). Liver transplantation was performed in 16 of 31 patients (45%). Overall survival rate for the entire cohort is 23 of 31 (74%), whereas 12 of 31 (39%) are currently alive without a liver transplant. CONCLUSION: These data suggest that there is no contraindication to performing a Kasai portoenterostomy for biliary atresia in children over 75 days of age.     

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。     

Histologic features of the portal plate in extrahepatic biliary atresia and their impact on prognosis—a Danish study

Background/Purpose

The aims of this study are as follows:

1.

To evaluate the outcome of the patients with extrahepatic biliary atresia (EHBA), operated at the Rigshospitalet, Denmark, during the last 24 years.

2.

To relate the histologic findings of the portal plate to the outcome.

3.

To compare the histologic findings in the portal plate of children affected by EHBA with those found at the porta hepatis in age-matched children dead from other causes.

Method

From 1979 to 2003, 57 children have been operated by the Kasai procedure. Only 40 of these have had their portal plate removed for histologic examination. We divided the patients according to clinical outcome into a successful and a failure group and compared the histologic features of the portal plates in the 2 groups. Afterward, the portal plate histology from EHBA was compared with the porta hepatis area from patients dead from other causes.

Results

A significant difference between the success and the failure group was found with regard to the number of bile ducts, the maximal length measurable in any direction for bile duct structures, and the proliferation, but not for any type of diameter. The normal portal plate was different from the portal plate of children with biliary atresia by always having 2 large biliary structures and the cells being mucinous and columnar in the largest bile ducts. Only 1 of 4 normal portal plates showed signs of proliferation. There was no significant difference between the normal and the success group with respect to the number, maximal length, and proliferation of the bile ducts. A difference in the diameter between the normal group and the entire EHBA group was significant for the maximal internal diameter but not for the other types of diameter measurements.

Conclusion

The present study shows the following:

1.

In portal plates from patients with EHBA, the longest measurable length of the bile duct structures, the number of bile duct structures, and less proliferation are positively correlated to prognosis.

2.

Patients with EHBA have a more favorable prognosis when the histology of the portal plate resembles the normal portal plate regarding the number of bile ducts, proliferation, and maximal measurable length.

     

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.     

Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia.

BACKGROUND: Progressive destruction of intrahepatic bile ducts may determine outcome in extrahepatic biliary atresia (EHBA) despite successful portoenterostomy. The aim of this study was to characterize the inflammatory infiltrate of a large series of cases of biliary atresia and relate these findings to clinical outcome. METHODS: Immunohistochemical analysis was performed on frozen tissue sections of extrahepatic biliary tree and liver biopsies obtained (August 1996 to March 1998) from 28 infants with EHBA and 8 liver biopsy specimens from age-matched controls with other cholestatic liver disorders. A semiquantitative scoring system was designed to evaluate the staining with a panel of antibodies to the CD4, CD8, CD25, CD56, CD68, CD71 antigens and to HLA-DR, ICAM-1, VCAM-1, E-selectin and LFA-1. The infants then underwent followup prospectively and divided into 2 prognostic groups at 12 months postoperatively: those who had cleared their jaundice (graded as a good outcome [n = 19]), and those who required liver transplantation or who had failed to clear their jaundice (defined as > 50 micromol/L; graded as poor outcome [n = 9]). RESULTS: CD4(+) lymphocytes and CD56(+) (NK cells) predominated in the liver of infants with EHBA as compared with controls. The infiltrating cells exhibited marked proliferation (CD71 expression) and activation (particularly LFA-1 but also CD25 expression). A smaller subpopulation of the cells also expressed VCAM and E-selectin. HLA-DR was strongly expressed on Kupffer cells and to a lesser extent on proliferating bile ducts and sinusoidal endothelium. Expression of the majority of markers was lower in the remnant bile duct tissue than in the liver of EHBA (P <.05) with only HLA-DR and LFA-1 (on infiltrating cells) and ICAM (on endothelium) expressed strongly in the remnant bile duct tissue. Although quantitatively less pronounced, all of these immunohistochemical features also were noted in non-EHBA cholestatic liver tissue. A good outcome at 12 months was associated with lower CD68 (macrophage) expression in both the liver (P <.05) and biliary tree (P <.05) and with reduced expression of ICAM-1 (P =.05) on infiltrating cells in the biliary remnant. CONCLUSIONS: Immunohistochemical patterns of immune-mediated liver injury and inflammation were prevalent features at the time of portoenterostomy. They were neither exclusive to nor characteristic of EHBA. A reduction in the expression of the macrophage marker (CD68) within the liver and biliary remnants and reduction of ICAM-1 expression on infiltrating cells in the biliary remnants appear to be associated with a better postoperative prognosis.     

Endoscopic sphincterotomy for common bile duct calculi

A series of 74 patients having endoscopic sphincterotomy for common bile duct calculi is reported. Complete stone extraction was achieved in 53 cases (72%). Seventeen of 21 patients with retained calculi following recent biliary surgery had successful extractions (80%). Of 30 patients having had a cholecystectomy, 21 (70%) were successful, but only 15 of 23 patients with obstructive jaundice and no previous biliary surgery had the ducts cleared of calculi. Failure was due to multiple stones in the duct, or calculi too large to pass through the sphincterotomy. Endoscopic sphincterotomy is advocated in patients with obstructive jaundice due to stones, moving to early surgery should it prove unsuccessful. The results in patients with a T-tube in situ are comparable to extraction of the calculi along the T-tube tract.     

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).