Long-term results with the Kasai operation for biliary atresia
F. M. Karrer, M. R. Price, D. D. Bensard, R. J. Sokol, M. R. Narkewicz, D. J. Smith and J. R. Lilly
Department of Surgery, University of Colorado School of Medicine, Denver, USA.
OBJECTIVE: To evaluate long-term outcome in a series of children with
biliary results atresia treated by portoenterostomy. DESIGN: Case series of
consecutive infants with biliary atresia with 10-year follow-up. Data were
obtained by retrospective chart review or phone interview. SETTING: A
tertiary academic medical center and regional children's hospital.
PATIENTS: A consecutive series of 104 infants diagnosed with biliary
atresia more than 10 years ago were evaluated. Eighty-nine had totally
obliterated extrahepatic ducts, 4 had proximal hilar cysts (correctable
type), and 11 had patency of the gallbladder and distal common duct.
INTERVENTIONS: Ninety-eight patients underwent biliary reconstruction and 6
had exploration only. Seventy-four infants underwent reconstruction using a
Rouxen-Y with exteriorization. The 11 infants with distal patency underwent
a portocholecystostomy ("gallbladder Kasai"). The remainder had various
modifications of the Kasai operation. MAIN OUTCOME MEASURES: Survival,
liver function, complications, growth, and development. RESULTS: The 6
patients who did not have a portoenterostomy died. Of the 98 who had a
reconstruction, 63 died (mean age at death, 27 months; median, 13.4
months), 10 following liver transplantation. Twelve of the 35 survivors
ultimately required liver transplants. Twenty-three children are alive more
than 10 years after portoenterostomy without the need for transplantation.
Two thirds have experienced some manifestation of portal hypertension (ie,
variceal bleeding, hypersplenism, ascites). Nineteen patients (79%) are
anicteric with normal liver synthetic function and are in an
age-appropriate school grade or working and living independently.
CONCLUSIONS: We found that surgical correction of biliary atresia offers
long-term survival for about one quarter of patients, provides palliation
until liver transplantation becomes necessary, and if surgical correction
is not feasible, biliary atresia is uniformly fatal. The outlook is good
for those children who survived more than 10 years and justifies continued
attempts to establish bile flow in infants with biliary atresia.
