Past and future of biliary atresia.
BACKGROUND
With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients?


METHODS
From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys.


RESULTS
Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P < .001).


CONCLUSIONS
Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome.
Carceller
A
A
Division of Pediatrics, General Surgery, and Gastroenterology, Ste-Justine Hospital, Montreal, Quebec, Canada.
Blanchard
H
H
Alvarez
F
F
St-Vil
D
D
Bensoussan
A L
AL
Di Lorenzo
M
M
D001656:Biliary Atresia / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation / Q000379:methods / Q000401:mortality / Q000639:trends*; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000379:methods / Q000401:mortality / Q000639:trends*; D011336:Probability; D011792:Quebec; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D016896:Treatment Outcome