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.     

Pediatric liver transplantation for biliary atresia: results of primary grafts in 328 recipients

PURPOSE: The purpose of this study was to assess the overall results of recipients undergoing transplantation for biliary atresia (BA), according to age, surgical techniques, and transplant eras, and to identify the prognostic factors affecting outcome. METHODS: Between 1984 and 2000, 328 pediatric recipients with BA who underwent orthotopic liver transplantation (OLT) were reviewed. Median age at OLT was 1.5 years (range, 0.4-14.5 years). Kasai hepatoportoenterostomy (KHPE) had been previously performed in 285 (87%) children. Regarding surgical techniques, 125 (38%) children received a whole-liver graft, 128 (39%) received a reduced-size graft, 16 (5%) received a split-liver graft, and 59 (18%) received a living-related (LR) donor graft. RESULTS: Overall actuarial patient survivals were 87%, 83%, and 81% at 1, 5, and 10 years, respectively. One-year patient survivals in children undergoing transplantation at the different age ranges were 85% (under 1 year), 86% (1-3 years), 83% (3-6 years), 100% (6-10 years), and 100% (beyond 10 years) (not significant). One-year patient survivals for the different transplant eras were 75% (1984-1988), 85% (1989-1992), 93% (1993-1996), and 98% (1997-2000) (P=0.0001). Multivariate analysis demonstrated that pretransplant recipient weight (P=0.004), indication for OLT (P=0.083), and age at OLT (P=0.024) predicted patient survival. The type of baseline calcineurin inhibitor (tacrolimus) and the age at OLT (beyond 6 years) were significantly associated with a better graft survival. CONCLUSIONS: Best results in children undergoing transplantation beyond 6 years indicate the importance of performing a KHPE as the first therapeutic step in BA; innovative surgical techniques, particularly LR donor graft, allowed successful transplantation in infants with early failure of KHPE.     

Liver transplantation for biliary atresia

Biliary atresia is the most common indication for liver transplantation (OLT) in children. We present our experience with OLT as a treatment for end-stage liver disease in children with biliary atresia. We performed a retrospective review of 20 biliary atresia patients (11 male, 9 female patients; mean age, 21.4 months; range, 6 to 84 months) who had undergone OLT. Mean preoperative weight and height were 10.1 +/- 5.8 kg and 72.5 cm, respectively. Thirteen recipients were younger than 1 year of age, and 15 weighed less than 10 kg at the time of OLT. Fourteen recipients had undergone a Kasai operation prior to the OLT. The mean serum total bilirubin level was 22.56 mg/dL before OLT. Eighteen left lateral segment grafts and two whole grafts were transplanted. The mean recipient operative time was 9.25 hours. The mean recipient intraoperative blood loss was 1.81 U. Two hepatic arterial thromboses and one biliary leak occurred soon after surgery. Portal vein stenoses developed in two recipients at 10 and 12 months after OLT; both were treated with balloon dilatation. Two biliary stenoses, which occurred at 10 months and 3.5 years after surgery, were treated with balloon dilatation. Two recipients died at 2 and 12 days after OLT because of respiratory distress syndrome and sepsis, respectively. The remaining 18 (90%) recipients are alive with good graft function. The overall rejection rate was 31.25%. OLT is an effective treatment for children with biliary atresia and a failed Kasai procedure. Living related liver grafts represented an excellent organ supply for these patients.     

A long-term experience with biliary atresia. Reassessment of prognostic factors.

Thirty-four infants with biliary atresia were primarily treated at our institution between 1974 and 1987. The mean age at diagnosis was 8.8 weeks. The Kasai portoenterostomy was used in 11 patients (32%) and the Sawaguchi modification in 23 infants (68%). Overall survival was 47% (16/34), with a mean follow-up of 45 months. Fifteen survivors (94%) are jaundice free, with two having undergone liver transplantation. Survival was not influenced by earlier age at operation, size of ductal remnants, or the use of an external biliary vent. Good bile flow was predictive of a favorable outcome. The incidence of cholangitis was increased in the Kasai (87%) versus the Sawaguchi groups (45%) (p less than 0.05), but 1-year survival rates were similar (55% vs 64%). The authors conclude that portoenterostomy offers a reasonable chance for success and should be the initial procedure for biliary atresia.     

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 influence of portoenterostomy on transplantation for biliary atresia.

After portoenterostomy (PE) for biliary atresia (BA), many patients suffer progressive deterioration of liver function and ultimately require liver transplantation. We retrospectively reviewed a single center's experience with pediatric liver transplantation for BA from 1988 to 2002. Sixty-six patients underwent 69 liver transplants for BA. Forty-two (63%) patients had previously undergone Kasai PE, 11 (17%) biliary appendicoduodenostomy (BAD), and 13 (20%) had no prior biliary drainage (NBD). The BAD procedure offered only short-term biliary drainage--the mean interval between PE and transplant was more than twice that for Kasai patients than for BAD patients (132 versus 49 weeks). The transplants included 11 cadaveric partial, 27 cadaveric whole, and 31 living related transplants. Three patients required retransplant. Prior PE did not increase the incidence of major perioperative complications or unplanned reexploration. After transplant, the 1-, 5-, and 10-year actuarial graft survival rates were 87%, 86%, and 80%, respectively. The 1-, 5-, and 10-year actuarial patient survival rates were 91%, 89%, and 83%. PE remains an important bridge to transplant. In conclusion, transplantation for BA offers excellent long-term graft and patient survival.     

Kasai portoenterostomy: 12-year experience with a novel adjuvant therapy regimen

Aim

The role of adjuvant therapy with corticosteroids and choleretics after Kasai portoenterostomy for biliary atresia (BA) remains uncertain. Experience with a novel postoperative adjuvant therapy regimen is reported.

Methods

Between 1994 and 2006, 71 infants with BA were referred. Four died from uncorrectable congenital heart disease/cardiorespiratory failure without undergoing portoenterostomy, 7 underwent primary liver transplantation (3 referred ≥19 weeks of age), and 60 underwent portoenterostomy at a median of 51 (10-104) days. Of these, 55 (92%) had type 3 BA and 6 had the BA splenic malformation syndrome. Fifty (83%) received the following adjuvant therapy beginning on postoperative day 5: oral dexamethasone 0.3 mg/kg bd for 5 days, 0.2 mg/kg bd for 5 days, and 0.1 mg/kg bd for 5 days together with oral ursodeoxycholic acid 5 mg/kg bd and phenobarbitone 5 mg/kg nocte, both of which were continued for 1 year. All infants received routine perioperative prophylactic antibiotics.

Results

Overall, 42 of 60 (70%) infants cleared their jaundice (bilirubin <20 μmol/L): 38 of 50 (76%) with the dexamethasone/ursodeoxycholic acid regimen compared with 4 of 10 (40%) not receiving this adjuvant treatment. There were 4 late deaths after portoenterostomy: 2 from associated congenital disorders and 2 after liver transplantation. Of the remaining 56 children, 39 (70%) are currently alive with their native liver at a median follow-up of 3.3 years and 17 are alive after liver transplantation. Surgical complications occurred in 3 after portoenterostomy: adhesive bowel obstruction (2) and an anastomotic leak. One infant had gastrointestinal bleeding that may have been related to dexamethasone, but this resolved with ranitidine. There were no perioperative septic complications.

Conclusion

In this series, adjuvant postoperative treatment with a short course of oral dexamethasone and longer-term ursodeoxycholic acid significantly improved the outcome after Kasai portoenterostomy.     

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.     

The therapy of biliary atresia combining the Kasai portoenterostomy with liver transplantation: a single center experience

Survival has improved dramatically for children with extrahepatic biliary atresia (EHBA), but optimal surgical management remains controversial. We have studied 28 infants born between June 1981, and April 1988, who underwent Kasai's portoenterostomy as primary surgical treatment. Those with evidence of subsequent hepatic decompensation were evaluated for liver transplantation (LT). All were cared for by surgeons who perform both the Kasai portoenterostomy and LT. Following the Kasai operation, 16 of 28 patients (57.1%) have achieved total biliary drainage, while 5 of 28 (17.9%) achieved partial drainage, and 7 of 28 (25%) achieved no drainage. Nine of 28 (32.1%) have undergone LT, 4 of whom were transplanted at greater than 2 years of age. To date, 25 of 28 (89.3%) are alive. Twenty three of 25 (92%) living are jaundice-free, 65% of whom have undergone the Kasai operation only. We project that 5 of 28 (17.8%) will come to transplantation, and 12 of 28 (43%) have no current indication that they are likely to come to transplantation. We conclude that combining Kasai's portoenterostomy with LT as needed is an effective therapy for children with EHBA, that the Kasai operation offers significant benefit by delaying LT in many, and that the long-term results of management remain to be determined.     

Biliary atresia--a fifteen-year review of clinical and pathologic factors associated with liver transplantation

Purpose

The aim of this study was to identify clinical and pathologic factors associated with liver transplantation in infants with biliary atresia initially treated with Kasai hepatic portoenterostomy (KHPE).

Methods

Institutional Review Board approval was obtained. Records of patients with biliary atresia diagnosed between January 1986 and December 2000 were reviewed. Patients were divided into those who never required transplantation, those who underwent transplant in the first year after KHPE, and those who required transplantation later in childhood. Analysis of variance (ANOVA) compared multiple factors among the 3 groups. Proportional analysis compared those who required transplantation against those who did not. Statistical significance was considered achieved if P was less than .05.

Results

Forty-five patients were identified. Survival after KHPE was 96% (43 of 45). Sixteen (37%) never required transplantation, 13 (30%) underwent transplant within 1 year after KHPE, and 14 (33%) underwent transplant more than 1 year after KHPE. ANOVA comparison showed that the duration of jaundice before KHPE as a predictor for liver transplantation approached significance (P = .082). Proportional analysis found that a longer initial duration of jaundice before KHPE (P = .016) and failure to establish biliary flow (P = .033) were also significant predictive factors. An initial requirement for phototherapy (P = .057) and ductules less than 200 μm in diameter (P = .060) showed a trend toward predictor of liver transplantation.

Conclusions

A longer duration of jaundice before KHPE, failure to establish bile flow, requirement for phototherapy in the neonatal period, and ductules smaller than 200 μm are associated with liver transplant after KHPE.     

Outcome of surgery for biliary atresia

Forty-seven infants (26 male, 21 female) with biliary atresia under- went hepatic portoenterostomy during the 16-year period 1971-87. Twenty-six patients (55%) are alive 1-17 years after surgery, with 21 (45%) being jaundice-free. For children who became jaundice-free, the mean age at surgery was 78 days (range: 34-125 days), compared with 97 days (range: 48-224 days) for those who did not. Of 39 patients operated on at less than 120 days of age, 24 (60%) are alive. All four patients operated on after 125 days of life died. Of 31 patients operated on more than 5 years ago, 12 (39%) have survived, the oldest being 17 years. Ten (32%) have normal serum bilirubin concentrations, have non-active cirrhosis on liver biopsy, have had normal growth and development, and lead normal lives. The oldest two patients suffered variceal haemorrhage in their teenage years. In our recent experience, 11 of 16 patients (69%) have had complete clearing of jaundice, lead normal lives and do not currently require assessment for liver transplantation. It is believed that early referral of children with biliary atresia to experienced surgical units for portoenterostomy will lead to long-term survival, without the need for liver transplantation in a majority of cases. Liver transplantation should be offered in infancy only after failed portoenterostomy, except for patients presenting after 120 days in whom transplantation may be considered primary therapy.     

Liver Transplantation for Biliary Atresia in Taiwan: A National Study

A national database for orthotopic liver transplantation (OLT) among biliary atresia (BA) cases in Taiwan has not been reported. Using the National Health Insurance (NHI) database to investigate the prognostic features of patients with BA receiving OLT, we studied the prognosis of this procedure for BA. The NHI in Taiwan covers most of the population (>99%). From 1996-2004, 106 BA patients underwent transplantation. A linear time trend analysis was performed to estimate the annual slope for BA patients to receive OLT. The rate of increase per year of 2.6 cases was significant (R² = .649; P = .029). The 5-year overall survival after OLT was 97.1% ± 1.6%, which was not different between those who had or had not previously undergone portoenterostomy (94.9% vs 97.8%; P = .160). It was not different among patients undergoing transplantation during the various seasons (P = .505). Our data confirmed the effectiveness of OLT for the treatment of BA in children with or without KP in Taiwan.     

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.     

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%.     

Bowel perforation after paediatric orthotopic liver transplantation

Bowel perforation is a well-recognized complication following orthotopic liver transplantation. Of 194 paediatric liver transplantations performed in our hospital, 13 patients (6.7 %) developed bowel perforation post-transplantation. Contributory factors included previous operation, steroid therapy and viral infection. The incidence was higher in children who underwent transplantation for biliary atresia after a previous Kasai portoenterostomy. Seven patients (53 % of this group) reperforated. Diagnosis may be difficult and a high index of suspicion is needed. Received: 8 December 1997 Received after revision: 17 February 1998 Accepted: 2 March 1998     

Long-term postsurgical outcome of biliary atresia

BACKGROUND/PURPOSE: A successful Kasai procedure is effective in creating biliary drainage and radically altering the natural history of infants with biliary atresia (BA). Since its introduction in the 1950s, long-term follow-up would appear to show that only 30% to 50% of patients have a good long-term prognosis despite initially good surgical outcome. The authors reviewed their experience in treating BA from 1968 to 1997 to assess long-term outcome. MATERIALS AND METHODS: The records of 163 patients treated surgically for BA from 1968 to 1997 were reviewed. Forty-eight (29%) were alive at the end of 1997, of whom, 14 had received liver transplants (LT). Surviving patients who had not undergone transplantation were divided into two groups according to clinical condition: group A, normal liver function without cholangitis (CG) and portal hypertension (PH) and group B, liver dysfunction with CG or PH. The study period was divided arbitrarily into three periods, 1968 to 1975 (period I, n = 34); 1976 to 1985 (period II, n = 81); 1986 to 1997 (period III, n = 48). RESULTS: Thirty-four patients were alive without LT at the end of 1997. There were eight patients (mean age, 16.3+/-4.8 years) in group A, and 26 patients (mean age, 14.3+/-7.6 years) in group B. Recently, four group A patients (mean age, 19.3+/-1.9 years) shifted to group B because of sudden deterioration in condition involving severe CG with multiple bile lakes (n = 2), uncontrollable intestinal bleeding (n = 1), and liver atrophy (n = 1). Survival deteriorated with length of follow-up. There were three survivors from 34 patients treated in period I, 16 survivors from 81 patients treated in period II (three had LT), and 29 survivors from 48 patients treated in period III (11 had LT). CONCLUSIONS: Although satisfactory bile drainage can be obtained with portoenterostomy, our data suggest that liver function can deteriorate progressively, with a possible turning point in late adolescence, indicating that as the length of follow-up increases, clinical assessment should be regular and comprehensive. The timing of LT in postoperative BA patients with deteriorating liver function is a vital management issue.     

Short-term results of Kasai operation for biliary atresia: experience from one institution

BACKGROUND: The purpose of this study is to review the short-term outcome of patients with biliary atresia (BA) treated by the Kasai operation at our institution. METHODS: Ninety-two BA patients treated by the Kasai operation between January 1996 and December 2002 were reviewed. The diagnosis of BA was confirmed by intraoperative cholangiography. The outcome of treatment was categorized into two groups: jaundice-free (total bilirubin < 2 mg%) and persistent jaundice (>or= 2 mg%). The outcome of Kasai operation was evaluated 1 year after surgery. Data are expressed as mean +/- SD. RESULTS: Average age at the time of surgery was 90.26 +/- 36.44 days. Only 22.8% (21/92) of patients had Kasai operation before 60 days of age. Histologically, 49 patients (54.4%) had liver fibrosis at the time of surgery. Of 92 patients, 17 were not included in outcome evaluation as they were less than 1-year postsurgery. Therefore, 75 patients could be evaluated for the outcome. Thirty-eight patients (50.67%) were jaundice-free 1 year after surgery. Liver histology and age at the time of the Kasai operation did not influence early outcome. The most common complication was ascending cholangitis. CONCLUSION: Half of our BA patients who underwent Kasai operation were jaundice-free 1 year after surgery. The lack of impact of age and liver pathology on outcome is presumably due to the briefness of the follow-up. In general, our patients underwent Kasai procedure too late. It is therefore important for us to conduct a campaign to highlight the plight of these patients and the urgency of referral for neonates with jaundice.     

Kasai's operation for extrahepatic bile duct atresia: a cure or palliative treatment?

The only surgical treatment currently available for patients with extrahepatic biliary atresia is portoenterostomy or portocholecystostomy, the Kasai operation. Seventeen such patients were operated upon at the H?pital Sainte-Justine; 11 had bile excretion. Eight are still alive 12 to 60 months after operation. They have good to fair bile excretion. Twelve of the 17 patients had infectious or hemorrhagic complications and hepatic deficiency is evident in 6 of the 8 living patients. The progressive deterioration of liver function in the majority of the patients still alive raises the question of whether the Kasai procedure should be considered a curative or only a palliative procedure.     

Biliary atresia and the polysplenia syndrome

There is a widely held but unsubstantiated belief that in infants with biliary atresia and coexisting polysplenia syndrome, the Kasai operation fails. An equally poor prognosis has been forecast for patients with this complex treated by liver transplantation. From 1975 to 1989, 16 of 131 infants with biliary atresia (12%) had one or more components of the polysplenia syndrome: polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6), absent inferior vena cava (6), aberrant hepatic artery (4), or abdominal heterotaxia (4). Although technically more demanding, 12 of the 15 polysplenia patients achieved biliary drainage after operation. (One patient had exploration only, because of parental preference). Four children are alive; two are anicteric and well at ages 5 and 8 following Kasai's operation, and two by virtue of liver transplantation done at ages 4 and 7. Three of the five patients who had liver transplantation died. Acturial survival was 44% at 5 years, not significantly different from that of the 115 nonpolysplenia patients (48%). When associated with the constellation of anomalies known as the polysplenia syndrome, biliary atresia is most likely caused by an early (at approximately the fifth week) embryonic insult. The anomalies do not preclude successful biliary reconstruction using the Kasai procedure or successful liver transplantation.     

Kasai portoenterostomy: differences related to race

PURPOSE: The aim of this study was to evaluate the Kasai portoenterostomy in African-American and white children with respect to differences in presentation and outcome. METHODS: A retrospective review of all children with biliary atresia who underwent a portoenterostomy at our institution over the last 15 years (n = 63) was performed. Sex, age at the time of Kasai, preoperative laboratory tests, success rates (defined as postoperative total serum bilirubin < or = 2.0 mg/dL), and survival rate were recorded. Differences between African-American (AA; n = 30) and white (W; n = 33) children were analyzed. Long-term follow-up was available on 59 of 63 patients. RESULTS: Sixty-three percent of all patients (40 of 65) were girls, and 48% were AA (30 of 63). A higher percentage of AA children (73%) were girls than were white children (55%), although this difference did not achieve statistical significance. African-Americans underwent portoenterostomy at a later age, had higher alkaline phosphatase levels, and higher AST. These differences were statistically significant. Preoperative ALT, total bilirubin level, and GGTP levels all were greater in African-Americans, although these differences did not achieve statistical significance. There was a trend toward decreased success and survival rate, although these results also were not statistically significant. CONCLUSIONS: African-Americans underwent primary therapeutic intervention for biliary atresia at an older age than white children with a trend toward less favorable results. These differences related to race may be attributed to greater difficulty in diagnosing jaundice or poorer access to health care in this patient population. Increased effort at identifying biliary atresia in AA children may lead to earlier diagnosis and treatment and improved outcomes.