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Kasai portoenterostomy: 12-year experience with a novel adjuvant therapy regimen
Mark D Stringer1, Suzanne M Davison, Sanjay R Rajwal
1St. James's University Hospital, Leeds LS9 7TF, UK. mdstringer@dial.pipex.com
Insights
Adjuvant therapy with dexamethasone and ursodeoxycholic acid improved outcomes for infants with biliary atresia (BA) after Kasai portoenterostomy. This treatment helped more infants clear jaundice and maintain their native liver function.
Area of Science:
- Pediatric Surgery
- Hepatology
- Neonatal Care
Background:
- Biliary atresia (BA) is a severe neonatal liver disease requiring surgical intervention.
- The efficacy of adjuvant therapies following Kasai portoenterostomy for BA remains debated.
- Corticosteroids and choleretics are commonly considered for postoperative management.
Purpose of the Study:
- To evaluate a novel adjuvant therapy regimen involving dexamethasone and ursodeoxycholic acid post-Kasai portoenterostomy for biliary atresia.
- To assess the impact of this regimen on jaundice clearance and long-term native liver survival.
Main Methods:
- A cohort of 71 infants with BA was analyzed between 1994 and 2006.
- Sixty infants underwent Kasai portoenterostomy, with 50 receiving adjuvant therapy (dexamethasone, ursodeoxycholic acid, phenobarbitone).
- Adjuvant therapy included a tapering course of oral dexamethasone and 1-year of ursodeoxycholic acid and phenobarbitone.
Main Results:
- Jaundice clearance was achieved in 70% of infants overall (76% with adjuvant therapy vs. 40% without).
- At a median follow-up of 3.3 years, 70% of the 56 eligible children were alive with their native liver.
- Surgical complications were infrequent, with one case of potential dexamethasone-related gastrointestinal bleeding.
Conclusions:
- A short course of dexamethasone combined with long-term ursodeoxycholic acid significantly enhanced outcomes after Kasai portoenterostomy for biliary atresia.
- This adjuvant regimen demonstrated improved jaundice clearance and native liver survival rates.
- The findings support the use of this specific adjuvant therapy in the management of biliary atresia.
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 > or = 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 micromol/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.
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