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Long-term results with the Kasai operation for biliary atresia
F M Karrer1, M R Price, D D Bensard
1Department of Surgery, University of Colorado School of Medicine, Denver, USA.
Insights
Surgical correction of biliary atresia (BA) offers long-term survival for approximately 25% of children. Continued attempts to restore bile flow are justified for infants with BA, as the outlook is positive for long-term survivors.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia (BA) is a severe neonatal liver disease characterized by bile duct obstruction.
- Early diagnosis and surgical intervention are critical for improving outcomes in infants with BA.
Purpose of the Study:
- To evaluate the long-term outcomes of children with biliary atresia who underwent portoenterostomy.
- To assess survival rates, liver function, complications, and overall development in patients with BA post-surgery.
Main Methods:
- A case series of 104 infants diagnosed with biliary atresia over 10 years ago.
- Data collected via retrospective chart review and phone interviews, focusing on portoenterostomy outcomes.
- Key outcome measures included survival, liver function, complications, growth, and development.
Main Results:
- Of 98 patients who underwent reconstruction, 63 died; 10 required liver transplantation. Twelve of 35 survivors ultimately needed transplants.
- Twenty-three children survived over 10 years post-portoenterostomy without needing a transplant.
- Nineteen of these long-term survivors (79%) are anicteric with normal liver function, attending school or living independently.
Conclusions:
- Surgical correction of biliary atresia provides long-term survival for about a quarter of patients.
- Portoenterostomy can palliate symptoms, potentially delaying or avoiding liver transplantation.
- The positive outlook for long-term survivors justifies continued efforts to establish bile flow in infants with biliary atresia.
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.
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