Abstract
Introduction: Sickle Cell Disease (SCD) is the most prevalent genetic disease in the world predominantly in the African population with Sickle Cell Anaemia (SCA) being its dominant form. One of the most frequent complications of SCD is osteomyelitis. SCA is due to a point mutation in the beta globin chain of haemoglobin. This is responsible for the sickled shape of RBCs under low oxygen tension conditions leading to obstruction in the microcirculation. This leads to vaso-occlusive crises (VOC) which has a similar clinical presentation to that of osteomyelitis, another complication of SCD. Case Presentation: We present the case of a three-year-old girl with SCA who presented with an inability to bear weight in a febrile context. A diagnosis of VOC was initially made, which was later on changed to both a left chronic tibial and right distal femoral osteomyelitis following a series of biological, and imaging investigations. Surgical debridement and drainage were performed, resulting 9 weeks later in the involution of fever and leg pain. Conclusion: Osteomyelitis when associated with SCD is a dreadful and deathly disease in low income countries as it also presents like VOC therefore higher suspicion index is recommended. It is therefore important to take this into consideration at an early stage in patients with homozygous sickle cell disease so as to rapidly initiate multidisciplinary care. Appropriate investigations, appropriate antibiotic therapy, and timely surgical intervention would help to greatly reduce morbidity and mortality.
Highlights
Sickle Cell Disease (SCD) is the most prevalent genetic disease in the world predominantly in the African population with Sickle Cell Anaemia (SCA) being its dominant form
This leads to vaso-occlusive crises (VOC) which has a similar clinical presentation to that of osteomyelitis, another complication of SCD
Osteomyelitis when associated with SCD is a dreadful and deathly disease in low income countries as it presents like VOC higher suspicion index is recommended
Summary
Sickle Cell Disease (SCD) is the most prevalent genetic disease in the world with a higher predominance in populations of African descent. RBCs that contain an abnormal form of Haemoglobin (Hb) become deformed (or sickle-shaped) and rigid. Medullary bone infarction and necrosis create an apt condition for bacterial growth and spreading. In these patients, osteomyelitis is commonly seen in the tibia, diaphysis of femur and humerus, along with the infection of the vertebrae. Patients will suffer pain, swelling, fever and increased flow of inflammatory markers (CRP/ESR) in blood serum. This shows parallel characteristics with that of painful bone crises, which makes differentiation with bone infarction a difficult task. We report the case of a three-year-old female sickle cell patient with chronic bone pain, which was later diagnosed as chronic osteomyelitis and cared for
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