Gallstones in children with sickle cell disease followed up at a Brazilian hematology center

Abstract

BACKGROUND: Sickle cell disease causes chronic and recurrent hemolysis which is a recognized risk factor for cholelithiasis. This complication occurs in 50% of adults with sickle cell disease. Surgery is the consensual therapy for symptomatic patients, but the surgical approach is still controversial in asymptomatic individuals. AIMS: To determine the frequency and to describe and discuss the outcome of children with sickle cell disease complicated with gallstones followed up at a tertiary pediatric hematology center. METHODS: In a retrospective and descriptive study, 225 charts were reviewed and data regarding patient outcome were recorded. RESULTS: The prevalence of cholelithiasis was 45% and half the patients were asymptomatic. The mean age at the time of diagnosis of cholelithiasis and surgical treatment was 12.5 years (standard deviation = 5) and 14 years (standard deviation = 5.4), respectively. The prevalence of cholelithiasis was higher in patients with SS homozygous and Sb heterozygous thalassemia when compared to patients with sickle cell disease. In 50% of symptomatic patients, recurrent abdominal pain was the single or predominant symptom. Thirty-nine of 44 patients submitted to surgery reported symptom relief after the surgical procedure. Asymptomatic individuals who did not undergo surgical treatment were followed up for 7 years (standard deviation = 4.8), and none of them presented complications related to cholelithiasis during this period. CONCLUSIONS: The frequency of cholelithiasis in the study population was 45%. One-third of the patients were diagnosed before 10 years of age. Patients with the SS homozygous or Sb heterozygous phenotype were at a higher risk for the development of cholelithiasis than patients with sickle cell disease. About 50% of patients with gallstones were asymptomatic, the most of them did not undergo surgery and did not present complications during a 7-year follow-up period. Cholecystectomy must be considered in symptomatic patients. In asymptomatic patients, conservative management seems to be the better choice.