The beneficial impact of revision of Kasai portoenterostomy for biliary atresia: an institutional study.
ABSTRACT To determine whether portoenterostomy (PE) revision in patients afflicted with biliary atresia (BA) is a viable treatment option and, if so, identify which patients may benefit.
BA, the most common cause of neonatal liver disease, results in biliary tract obstruction and hepatic fibrosis. Kasai PE is the initial surgical intervention performed and, if successful, restores drainage and preserves the native liver. Portoenterostomy failure warrants liver transplantation, but because of complications related to transplantation, treatment strategies to salvage the native liver may be beneficial. Using uniformly applied criteria, we have revised PEs to delay or avoid transplantation.
A retrospective review of medical records of patients diagnosed with BA since 1983 was performed. Patient demographics, symptoms, indications for revision, laboratory values, and outcomes were recorded. A cohort of patients who underwent revision after initial PE was identified. Survival rates were assessed using the Kaplan-Meier method. For patients who required transplantation, operative data from the revised PE cohort were compared with those from the unrevised PE cohort. A Cox proportional hazards model was used to determine covariates predictive of a favorable outcome.
Of 181 children who underwent PE, 24 underwent revision. Adequate biliary drainage, as evidenced by normalized conjugated bilirubin levels, was achieved in 75% of revised patients. Overall survival in patients who underwent revision, regardless of transplantation, was 87%. Among patients who underwent PE revision, 46% have survived with their native liver.
Experience at our center suggests that with appropriate patient selection, PE revision may delay the need for liver transplanation yielding encouraging patient outcomes.
- SourceAvailable from: Masaki Nio[show abstract] [hide abstract]
ABSTRACT: The aim of this study was to elucidate the epidemiology and short- and long-term results of biliary atresia in Japan analyzing the data of the Japanese Biliary Atresia Registry (JBAR). In 1989, the Japanese Biliary Atresia Society started a nationwide registry, JBAR, to investigate all aspects of biliary atresia. A total of 1,381 patients, 863 girls, 507 boys, and 11 unknown, were registered between 1989 and 1999. JBAR includes an initial and follow-up questionnaires. Using these patients' data, the incidence, sex distribution, associated anomalies, the type of obstruction, the type of operation, and the surgical results were evaluated. The 5- and the 10-year results of 735 patients who were registered initially in or before 1994 also were analyzed. The incidence of biliary atresia was 1 in 9,640 live births. One hundred sixty-four patients (11.9%) had type I atresia of the common bile duct, 34 (2.5%) had type II atresia of the hepatic ducts, and 1,162 (84.1%) had type III atresia at the porta hepatis. Congenital associated anomalies were found in 19.6% of the patients including 33 cases associated with polysplenia. Impact of the age at operation on bile flow was not clear until 90 days of age, and after 90 days the bile flow rate worsened. The original Roux-en-Y procedure had been used in more than 50% of the patients since 1995. In 1999, 96% of the patients underwent the original Roux-en-Y procedure or the Roux-en-Y with an intestinal valve, and only 3 patients (3.5%) underwent other modifications. There were no significant differences in either the rate of disappearance of jaundice or the incidence of cholangitis among these 3 procedures. Of the 735 patients registered in or before 1994, 19 patients (2.6%) were lost to follow-up. The 5-year survival rates of patients registered in 1989, 1990, 1991, 1992, 1993, and 1994 were 62%, 64.5%, 61.3%, 59.0%, 58.7%, and 52.7% without liver transplantation (LTx), and 69.4%, 74.2%, 75.2%, 79.5%, 78%, and 78.3% with LTx, respectively. Although the overall 5-year survival rate changed from 69.4% to 78.3%, the difference was not statistically significant. According to the 10-year follow-up results of the 108 patients initially registered in 1989, 72 (66.7%) and 57 (52.8%) survived with and without the aid of LTx, respectively. The overall 5- and 10-year survival rates were 75.3% (553 of 734) and 66.7% (72 of 108), respectively. In spite of the increasing number of survivors after LTx, there was no significant improvement in the 5-year survival rate. It was shown that the JBAR system was functioning well with only 19 patients lost to follow-up among the 743 patients registered from 1989 to 1994.Journal of Pediatric Surgery 08/2003; 38(7):997-1000. · 1.38 Impact Factor
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ABSTRACT: Although liver transplantation has been the standard of care therapy for life-threatening liver diseases for >20 years, data on the long-term impact of liver transplantation in children have been primarily limited to single-center experiences. The objective of this study was to characterize and evaluate the clinical course of children who have survived >or=5 years after pediatric liver transplantation in multiple centers across North America. Patients enrolled in the Studies of Pediatric Liver Transplantation database registry who had undergone liver transplantation at 1 of 45 pediatric centers between 1996 and 2001 and survived >5 years from liver transplantation were identified and their clinical courses retrospectively reviewed. The first graft survival for 461 five-year survivors was 88%, with 55 (12%) and 10 (2%) children undergoing a second and third liver transplantation. At the 5-year anniversary clinic visit, liver function was preserved in the majority with daily use of immunosuppression therapy, including a calcineurin inhibitor and oral prednisone, reported by 97% and 25% of children, respectively. The probability of an episode of acute cellular rejection occurring within 5 years after liver transplantation was 60%. Chronic rejection occurred in 5% patients. Posttransplant lymphoproliferative disease was diagnosed in 6% children. Calculated glomerular filtration rate was <90 mL/minute per 1.73 m2 in 13% of 5-year survivors. Age- and gender-adjusted BMI>95th percentile was noted in 12%, with height below the 10th percentile in 29%. Children who are 5-year survivors of liver transplantation have good graft function, but chronic medical conditions and posttransplantation complications affect extrahepatic organs. A comprehensive approach to the management of these patients' multiple unique needs requires the expertise and commitment of health care providers both beyond and within transplant centers to further optimize long-term outcomes for pediatric liver transplant recipients.PEDIATRICS 01/2009; 122(6):e1128-35. · 4.47 Impact Factor
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ABSTRACT: Kasai operation for biliary atresia (BA) and living-related liver transplantation (LRLT) for failed Kasai cases have been performed by the same team in our hospital. Outcome of the treatment for BA in the pretransplantation and posttransplantation era in our institution were compared. As the "pre" group, 35 children first underwent the Kasai operation between 1982 and 1989. The "post" group consisted of 25 patients who underwent Kasai after introduction of the LRLT program in our hospital (1990). As for the reconstruction procedure after portal dissection during Kasai, jejunal interposition hepatic portoduodenostomy for the pre group was intentionally changed to Roux-en-Y procedure for the post group to decrease the risk of duodenal leakage after LRLT. Patients who needed revision of the Kasai decreased from 57% for the pre group to 28% for the post group. There was only one re-revision in the post group compared with seven in the pre group. In the pre group, 10 patients (28.6%) died at age 6 to 16 (mean, 8.5) months before the transplantation program was initiated. In the pre group, three patients could receive a cadaveric liver transplantation in other countries, and seven had LRLT in our hospital. Ten patients (40%) in the post group had LRLT at 7 to 22 (mean, 9.3) months old, and no deaths occurred in this group. When the patients who had liver dysfunction were excluded, the percentage of the patients who could be expected to survive and accomplish growth without undergoing liver transplantation was similar for the two groups, (31.4% v 36%). The change in the reconstruction procedure and the tendency for fewer revisions of Kasai procedure were effects of the start of the liver transplant program. As a general strategy for BA, Kasai operation should be the initial procedure, followed by liver transplantation for failed Kasai. In the late-diagnosed patients, primary LRLT can be considered if the liver has already been cirrhotic. LRLT in the infantile period is a significant component of this strategy for helping all patients to survive.Journal of Pediatric Surgery 09/1997; 32(8):1201-5. · 1.38 Impact Factor