Background/Purpose: Kasai portoenterostomy is recommended as the primary in
itial therapy for extrahepatic biliary atresia if the procedure can be perf
ormed within 10 to 12 weeks of life. The optimal management for infants wit
h delayed presentation of biliary atresia remains controversial. The purpos
e 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 portoenterostom
y at their institution from 1986 to 1999 (n = 31). The authors analyzed suc
cess rates compared with age at the time of the Kasai procedure and the ass
ociation with patient demographics. Surgical success was defined as achieve
ment of a total serum bilirubin less than or equal to 2 mg/dL. Long-term fo
llow-up assessments included the need for liver transplantation and patient
survival rate.
Results: The demographics of this study cohort showed a predominance of Afr
ican-Americans. 19 of 31 (61%), and girls, 23 of 31 (74%). Assessment of su
ccess 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 su
rvival 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 perform
ing a Kasai portoenterostomy for biliary atresia in children over 75 days o
f age. J Pediatr Surg 36:97-99. Copyright (C) 2001 by W.B. Saunders Company
.