OBJECTIVE: Given the high frequency of cholelithiasis in children with sickle cell disease and the controversies about its management, the aim of this study was to critically review the literature about this issue. DATA SOURCES: Published studies in English and Portuguese from 1980 to 2007 about cholelithiasis and sickle cell anemia in children were retrieved from Medline and Lilacs databases. DATA SYNTHESIS: Cholelithiasis is the most common digestive complication of sickle cell disease patients and its frequency increases with age. Physiopathology of gallstones and diagnostic methods are well established. Cholecystectomy is the recommended therapy for symptomatic cholelithiasis, preferentially by laparoscopic approach. In asymptomatic patients, most studies favors the surgical management of the disease in order to avoid complications and to reduce difficulties with the differential diagnosis of future abdominal pain. However, the clinical management of this condition is also possible and there is no consensus in the literature regarding the best approach. CONCLUSIONS: Sickle cell disease presents great variations in clinical presentation, natural history and severity of hemolysis. Some populations may present lower frequency of cholelithiasis and its symptoms. Variability in the frequency of this complication may be attributed to genetic variation in haplotipes, environmental factors, clinical course and management of the sickle cell disease. Scientific evidence regarding the best time to indicate cholecystectomy in asymptomatic patient is not available. Therefore, decision regarding the need of clinical or surgical approach of the cholelithiasis depends on individual and population factors.
anemia; sickle cell; cholelithiasis; cholecystectomy; child
