Shoulder complications in sickle cell disease: Challenges, management strategies, and future directions

Abstract

Sickle cell disease (SCD) is a genetic disorder characterized by chronic hemolysis and vaso-occlusive crises (VOCs), leading to musculoskeletal complications that significantly affect quality of life. Among these, shoulder complications are a concern, with humeral head avascular necrosis (AVN) being the second most common site of involvement after the femoral head. Other shoulder pathologies, including osteomyelitis and septic arthritis, further contribute to morbidity. However, these conditions remain underdiagnosed and understudied, often due to overlapping symptoms with VOC-related bone infarctions. Imaging, particularly magnetic resonance imaging, is crucial for early diagnosis and accurate differentiation. Management strategies range from conservative pain control to surgical interventions, including core decompression for early-stage AVN and arthroplasty for advanced joint destruction. Surgical outcomes in SCD, however, remain inconsistent due to higher complication rates and a lack of standardized guidelines. Despite advancements in diagnosis and treatment, shoulder pathology in SCD remains an area of limited research. This review highlights the need for larger, long-term studies with a homogeneous etiology to support and refine current treatment strategies and improve patient outcomes.

Keywords: Core decompression; Osteomyelitis; Arthroplasty; Avascular necrosis; Shoulder; Sickle cell disease

Core Tip: This comprehensive narrative review highlights the challenges of managing shoulder complications in sickle cell disease (SCD) which discusses a critical and underexplored part of this disease. The review synthesizes current evidence on the three primary complications avascular necrosis, osteomyelitis, and septic arthritis. SCD leads to chronic hemolysis and vaso-occlousive crisis contribute to ischemic damage, with humeral head avascular necrosis often underdiagnosed due to overlapping symptoms with other conditions. Early diagnosis and differentiation rely on advanced imaging and laboratory tests. Management includes pain control, core decompression for early stages, and arthroplasty for advanced cases. Given the scarcity of research on shoulder pathology in SCD, larger, long-term studies are needed to refine treatment strategies and improve patient outcomes.