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The spectrum of biliary tract disorders in infants and children. Experience with 300 cases
J L Grosfeld1, F J Rescorla, M A Skinner
1Department of Surgery, Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis.
Insights
Surgical outcomes for pediatric biliary tract diseases show improved survival for biliary atresia with combined hepatic portoenterostomy and liver transplantation. Cholecystectomy is safe and effective for pediatric gallstones.
Area of Science:
- Pediatric surgery
- Gastroenterology
- Hepatobiliary surgery
Background:
- Biliary tract diseases in children require surgical intervention.
- Common conditions include biliary atresia, choledochal cysts, and cholelithiasis.
- Surgical outcomes and morbidity/mortality are key indicators of treatment efficacy.
Purpose of the Study:
- To evaluate the results of surgical treatments for infants and children diagnosed with biliary tract diseases.
- To assess the success rates, complications, and survival associated with various surgical procedures.
Main Methods:
- Retrospective review of 300 pediatric patients treated between 1972 and 1993.
- Analysis of diagnoses (biliary atresia, choledochal cyst, cholelithiasis), patient demographics, clinical presentation, surgical procedures, and outcomes.
- Evaluation of surgical morbidity and mortality rates.
Main Results:
- Hepatic portoenterostomy for biliary atresia had a 32% success rate in infants under 3 months, with overall survival of 71.5% including liver transplantation.
- Choledochal cyst excision and hepatojejunostomy resulted in a 96.4% survival rate.
- Cholecystectomy (open or laparoscopic) for gallstones was successful in all 169 patients with low mortality (0.59%).
Conclusions:
- Combined hepatic portoenterostomy and liver transplantation have improved survival for biliary atresia.
- Hepatic portoenterostomy is the preferred procedure for infants under 3 months with biliary atresia.
- Surgical management of choledochal cysts and cholelithiasis in children yields favorable outcomes.
Objectives:
To assess the results of surgical treatment of infants and children with biliary tract disease.
Design:
The records of children with biliary tract disorders requiring surgical intervention were reviewed retrospectively. Diagnosis, age, sex, clinical presentation, treatment, and outcome were evaluated.
Setting:
A large pediatric referral facility.
Patients:
A total of 300 patients treated from 1972 through 1993 were evaluated, including 102 with biliary atresia, 29 with choledochal cyst, and 169 with cholelithiasis. Hepatic portoenterostomy was performed in 87 patients with biliary atresia, and biopsy alone was performed in 15. Twenty girls and nine boys, 50% of whom were younger than 3 years, had choledochal cyst. Operative management included cyst excision and hepatojejunostomy in 25 patients, cyst jejunostomy in two patients, cystduodenostomy in one patient, and choledochocele excision in one patient. Gallstones were observed in 106 girls and 63 boys; 28 were aged 0 to 5 years, 31 were aged 6 to 10 years, and 110 were aged 11 to 18 years. Open cholecystectomy was performed in 143 patients, and laparoscopic cholecystectomy was performed in 26 patients.
Main Outcome Measures:
Principal outcomes examined were surgical morbidity and mortality.
Results:
Hepatic portoenterostomy was successful in 28 (32%) of 87 patients with biliary atresia (all younger than 3 months), and 23 showed improvement following hepatic portoenterostomy; hepatic portoenterostomy failed in 36 patients. Twenty-eight (82.3%) of 34 patients survived liver transplantation. Overall survival was 71.5%. Survival of patients with choledochal cyst was 96.4% (28/29). Cholecystectomy was successful in all 169 patients. There were eight complications and one death (0.59% mortality).
Conclusions:
Survival in patients with biliary atresia (71%) has improved with hepatic portoenterostomy complemented by liver transplantation. Hepatic portoenterostomy is the procedure of choice in infants younger than 3 months. An improved outlook for patients with choledochal cyst can be expected after cyst excision and hepatojejunostomy. Gallstones are relatively common in children. Both open and laparoscopic cholecystectomy are safe and effective procedures in children.