Musculoskeletal complications of sickle cell disease in Enugu, Nigeria.

Background: Sickle cell disease (SCD) is one of the most important haemoglobinopathies. It is an autosomal recessive genetic condition in which a defective form of haemoglobin, haemoglobin S (HbS) results from a single amino acid substitution. The amino acid valine replaces glutamic acid at position 6 in the beta globin gene. Musculoskeletal complications are often observed in the evolution of this disease and are common causes of morbidity and disability in these patients. The objective of this study is to describe the pattern and presentation of musculoskeletal complications in sickle cell disease at the National Orthopaedic Hospital Enugu Nigeria.

Method: a retrospective review of the patients with musculoskeletal complications who are genotype-confirmed sicklers who were treated in our hospital from January 1993 to December 2007 was carried out. The data collected included age, sex, complications, anatomic site, grade of disease, treatment, outcome of management and follow up. Patients with incomplete data were excluded from the study.

Results: Twenty seven patients with musculoskeletal complications of SCD were treated within the study period. Two patients were excluded from the study because of incomplete data. Twenty-five patients with 44 complications were analyzed. The age range was between 7 years to 30 years with a mean age of 19.2 years. Fifty six percent of patients were males. Malleolar ulcers were the commonest complications. This was followed by avascular necrosis (AVN) of the femoral head and osteomyelitis. Septic arthritis and osteomyelitis were most common in children less than 10 years while avascular necrosis and malleolar ulcers occurred more commonly in patients more than 15 years. Majority of the malleolar ulcers were treated by split skin grafting. Seventy five percent of the femoral head avascular necrosis was treated conservatively

Conclusion: Musculoskeletal complications are common causes of morbidity and disability in sickle cell disease. Malleolar ulcers were the commonest musculoskeletal complications. The predominant presentation in children below the age of 10 years is osteomyelitis and septic arthritis while AVN and malleolar ulcers occurred mostly in adolescents.