Orthopedic complications of sickle-cell disease in children

Sickle cell disease is the most common serious genetic disease in the world. It is a systemic disease, characterized by vaso-occlusive phenomena, especially in the bone capillary network. Orthopedic complications are thus the most common, with a strong impact on quality of life. In children, these complications mainly comprise bone infarction, epiphyseal aseptic osteonecrosis, and bone and joint infection: acute or chronic osteomyelitis, osteoarthritis and septic arthritis. Aseptic osteonecrosis mainly involves the proximal extremities of the femur and humerus. It may be completely asymptomatic in childhood, but almost systematically deteriorates during the 3rd decade without early management, while in childhood there are opportunities for revascularization and remodeling. Prognosis is more severe in sickle-cell-related than community-based childhood bone and joint infection, as treatment is often delayed by diagnostic difficulties due to the absence of specific clinical and paraclinical signs differentiating it from an acute vaso-occlusive crisis. The most common pathogens are minor Salmonella and Staphylococcus aureus. Due to high rates of iterative surgery and sequelae, systematic radio-clinical monitoring is required. Any surgery in children with sickle-cell disease requires special precautions, based primarily on preventive measures to avoid dehydration, hypoxia, hypothermia, acute anemia and pain that can trigger a life-threatening vaso-occlusive crisis. All orthopedic complications should be managed in a specialized reference center with experienced multidisciplinary medical-surgical teams. LEVEL OF EVIDENCE: : V, expert opinion.