Management of Osteomyelitis in Sickle Cell Disease: Review Article

Abstract

Sickle cell disease (SCD) is an autosomal recessive disorder that is characterized by abnormal "sickle-shaped" erythrocytes. Because of their shape, these erythrocytes are more likely to become trapped in small slow-flowing vessels, leading to vaso-occlusion. Because this commonly happens in the bones, patients with SCD are at an increased risk for orthopaedic manifestations such as osteomyelitis, septic joint, or osteonecrosis. Osteomyelitis is a serious and potentially disabling condition but can be difficult to differentiate from benign conditions of SCD, such as vaso-occlusive crisis. Diagnosis of osteomyelitis requires careful evaluation of the clinical presentation, laboratory testing, and imaging. Treatment of osteomyelitis in patients with SCD may be medical or surgical, but considerations in antibiotic selection and management preoperatively and postoperatively must be taken to ensure optimal outcomes.

Figure 1

Illustration showing the pathophysiology of sickle cell disease from the level of the chromosome to the morphology of the erythrocyte.

Figure 2

Illustration showing that sickle cell disease (SCD) may present with various clinical manifestations because the disease affects many organ systems.

Figure 3

Ultrasonography of the lateral soft-tissue of the hip showing a subcutaneous collection measuring 8.4 cm by 2.4 cm. This collection was used as an indirect indication of the presence of OM because it is likely to have formed secondary to the infection.

Figure 4

Coronal T1 (A), coronal T1 postcontrast (B), and coronal short tau inversion-recovery (C) images of the femur demonstrating extensive marrow edema with associated serpiginous and tubular marrow enhancement in the distal femoral metaphysis/diaphysis (solid arrows) with extensive associated periosteal reaction (arrowhead), adjacent soft-tissue swelling, and edema (dashed arrows). These findings are characteristic of osteomyelitis. The figure reproduced with permission from Kosaraju et al.

Figure 5

Radiograph of LeukoScan showing increased uptake at right thigh (arrow), which may indicate infective pathology.

Figure 6

A, Preoperative AP radiograph of posttraumatic femoral infection demonstrating notable bone loss secondary to débridement in a 30-year-old female patient. B and C, Segmental resection of infected bone (aggressive surgical débridement) according to intraoperative fluoroscopy location. D, Excision of infected bone. E, First stage postoperative AP radiograph demonstrated that bone defect was filled with antibiotic bone cement spacer, and locking compression plate was used to limb stabilization after débridement. F, Second stage postoperative AP radiograph demonstrating placement of iliac crest autograft after removal of bone cement and replacement of locking compression plate with intramedullary nail to limb stabilization. G and H, AP and lateral radiographs showing bony consolidation 15 months after bone grafting. The figure reproduced with permission from Han et al.

Figure 7

Preferred algorithm by authors for management of OM in sickle cell disease (SCD).