The Vertebral Manifestations of Sickle Cell Disease: A Literature Review

Abstract

Sickle cell disease (SCD) is the most common monogenic inherited blood disorder, causing extremely high morbidity and mortality rates worldwide. SCD has established manifestations in the musculoskeletal system, particularly the vertebrae. Despite its prevalence, there is a profound scarcity of literature about this important topic. This literature review aims to shed light on the commonest vertebral manifestations in patients with SCD from a pathophysiological and clinical perspective, identify the best diagnostic investigations for every manifestation, discuss the diagnostic difficulties of differentiating between each manifestation, and highlight the current medical and surgical treatments. The results of the literature review demonstrated that patients with SCD are at a much higher risk of developing vertebral manifestations such as vaso-occlusive crises (VOCs) and osteomyelitis acutely, and osteopenia, osteoporosis, and osteonecrosis chronically, with the younger population being the most affected age group. The vertebral column and its manifestations in SCD are primarily affected by the pathophysiologic mechanisms of hemolysis and VOCs. Our findings indicate that the lumbar vertebrae are most affected by manifestations of SCD and should, therefore, be considered the primary site for investigations. There remains a major obstacle in diagnosing and distinguishing between VOCs and osteomyelitis, which complicates and prolongs the treatments and leads to longer hospital stays and poorer outcomes. Finally, we concluded that vertebral manifestations of SCD, while not significantly affecting mortality, cause substantial morbidity and severely impact quality of life. A consistent management strategy is required, focusing on conservative care and a multidisciplinary approach that integrates medical, surgical, and rehabilitative interventions. Continued research to tackle the issue at its genetic source might improve outcomes and quality of life for SCD patients, particularly as we await advances in genetic editing like the CRISPR-Cas9 gene therapy.