Implications of radiologic-pathologic correlation for gallbladder disease in children and young adults with sickle cell disease.

The purpose of this study is to describe gallbladder imaging findings in patients with sickle cell disease, and to determine how they correspond with occurrence of complications, need for cholecystectomy, and surgical pathology. This study is IRB approved and HIPAA compliant. Informed consent requirements were waived. We reviewed records of 77 children with sickle cell disease ages 0-18 years at the time of their first gallbladder imaging study. Demographics, hospital courses, and radiologic and pathologic reports were collected. Two pediatric radiologists independently and retrospectively reviewed the imaging studies. Statistical analysis was performed using kappa statistic, chi-squared test, and ANOVA F-test. Continuous variables were described with mean, median, variance, and range. Patients who underwent cholecystectomy (N = 25) were more likely than the patients who did not undergo cholecystectomy (N = 52) to have gallstones or sludge (100 versus 36.5 %, p = <0.0001) or other gallbladder or biliary abnormality (70.8 versus 1.9 %, p = <0.0001). Patients who did not undergo cholecystectomy more frequently had normal-appearing gallbladders and biliary tracts (63.5 versus 0 %, p = <0.0001). Ninety-two percent of patients with cholecystectomy had chronic cholecystitis on pathology, and 96 % had a complication, including chronic cholecystitis and sequelae of biliary obstruction. Young patients with sickle cell disease, cholelithiasis, and any other biliary imaging abnormality will almost certainly require cholecystectomy, and many will experience complications. The most common surgical pathologic diagnosis in this group is chronic cholecystitis, which has a variable radiologic appearance. Our findings support recommendations to perform elective cholecystectomy for children and young adults with sickle cell disease and cholelithiasis or gallbladder sludge.