Orthopedic manifestations in children with sickle cell disease : A case series

Abstract

Background: Sickle cell disease (SCD) is a disorder with an autosomal recessive inheritance. It is one of the most common single-gene conditions affecting 90,000 to 100,000 persons in the united states and 1 in 500 African Americans. Sickle cell disease has an impact over millions of people and is very common in Saudi Arabia. Methods: This was a retrospective chart review that reports orthopedic related manifestations in children with sickle cell disease encountered in King Abdullah Specialized Children’s Hospital in Riyadh between January 2016 and December 2019. Descriptive analysis was used in this study. Results: A total of 21 cases were identified, 6 (28.6%) were females while 15 (71.4%) were males. The patients aged between 2 and 16 years with a median age of 12. Six patients (28.6%) were diagnosed with avascular necrosis (AVN), 6 patients (28.6%) were diagnosed with osteomyelitis, 4 patients (19%) were diagnosed with septic arthritis, 3 patients (14.3%) were diagnosed with bone infarction, 1 patient (4.8%) was diagnosed with cellulitis, and 1 patient (4.8%) was diagnosed with bursitis. The primary imaging modality was MRI. Three treatment modalities were used; antibiotics (61.9%), analgesics (23.8%), or analgesics plus physiotherapy (14.3%). A significant correlation was found between the diagnosis and the length of hospital stay (P < 0.001). Conclusion: Sickle-cell disease is a multi-system disorder. Musculoskeletal involvement accounts for much of the morbidity suffered by these patients. One should, however, be aware of these complications especially non orthopedic physicians.

Keywords

• musculoskeletal complications; orthopedic manifestations; osteonecrosis; osteomyelitis and septic arthritis; sickle cell disease.