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High rates of recurrent biliary tract obstruction in children with sickle cell disease

Martha O Amoako1, James F Casella, John J Strouse

  • 1Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Pediatric Blood & Cancer
|December 21, 2012
PubMed

Insights

Recurrent biliary tract disease (BTD) is common in children with sickle cell disease (SCD) after gallbladder removal. About 20% of patients experience BTD recurrence, often due to common bile duct stones, even after cholecystectomy.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Hematology

Background:

  • Sickle cell disease (SCD) patients have a high risk of gallstones (cholelithiasis) composed of bilirubin.
  • Symptomatic biliary tract disease (BTD) encompasses conditions like cholecystitis, common bile duct (CBD) obstruction, cholangitis, and gallstone pancreatitis.
  • Cholecystectomy is the primary treatment for symptomatic BTD, but data on recurrence rates is limited.

Purpose of the Study:

  • To evaluate the prevalence of recurrent symptomatic biliary tract disease (BTD) after cholecystectomy in pediatric patients with sickle cell disease (SCD).
  • To identify potential risk factors associated with BTD recurrence in this population.

Main Methods:

  • A retrospective cohort study was conducted.
  • Patients under 22 years old with symptomatic BTD at Johns Hopkins Children Center between July 1993 and June 2008 were identified.

Main Results:

  • The study identified 56 patients with 76 episodes of symptomatic BTD, with a median age of 15.9 years at the first event.
  • 19.6% (11 out of 56) of patients experienced at least one episode of recurrent symptomatic BTD during a median follow-up of 5.3 years.
  • No significant differences in baseline characteristics were observed between patients with single versus recurrent BTD episodes.

Conclusions:

  • Recurrent symptomatic biliary tract disease (BTD) is a frequent complication in pediatric patients with sickle cell disease (SCD), occurring in approximately 20% by age 4.
  • Recurrence often manifests as common bile duct (CBD) obstruction by stones, even after cholecystectomy.
  • Factors such as age at first episode, baseline total bilirubin, gender, and SCD genotype were not associated with recurrence in this cohort.
Abstract

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