Sickle cell anemia: a review for the pediatric dentist.

Sickle cell anemia is an inherited defect that affects the structure and synthesis of hemoglobin. In sickle cell trait, the affected individuals carry one gene for the abnormal hemoglobin (HbS). Sickle cell disease, however, is the homozygous state in which the abnormal hemoglobin is predominant in red blood cells, leading to devastating multisystem problems. Complications of the disease in children include: painful crises, stroke, pulmonary disease, delayed growth, osteomyelitis, organ damage and psychosocial dysfunction. Oral and dental manifestations include: orofacial pain, paresthesia of the mental nerve, stepladder appearance of the alveolar bone on radiographs, pulpal necrosis and enamel hypomineralization. The purpose of this manuscript was to review: (1) the pathophysiology of the disease; (2) its manifestations in the craniofacial complex; (3) contemporary medical therapy; and (4) recommendations for dental care.