Osteonecrosis in Youth with Sickle Cell Disease—A Narrative Review

Abstract

Highlights What are the main findings? Magnetic resonance imaging is the most sensitive tool for early diagnosis and plays a central role in disease staging and risk stratification. Management requires a multidisciplinary approach that includes analgesia, physical therapy, activity modification, optimization of bone health, and treatment of associated endocrine abnormalities. Despite growing recognition of osteonecrosis as a major complication of SCD, important gaps remain in our understanding of disease progression and optimal treatment. What is the implication of the main finding? Earlier identification of high-risk patients and the development of effective joint-preserving strategies remain key priorities for future research. Abstract Osteonecrosis can cause significant and chronic musculoskeletal morbidity in children and adolescents with sickle cell disease (SCD). Although the femoral head is most frequently affected, lesions can also occur in the humeral head, vertebral bodies, knees, and other joints. Progressive joint damage can result in chronic pain, impaired mobility, loss of function, and the need for surgical intervention at a young age. As survival continues to improve in SCD, the long-term burden of osteonecrosis is becoming increasingly important. This narrative review summarizes the current understanding of osteonecrosis in pediatric and adolescent SCD. We review its epidemiology, pathophysiology, clinical presentation, imaging findings, and management. Disease burden increases with age and is closely linked to markers of severe SCD, including frequent vaso-occlusive crises and acute chest syndrome.