Sickle cell dactylitis
Radiographic findings :
Frontal and oblique radiographs of both the hands reveal periosteal thickening with medullary expansion of the first and the fifth metacarpals on left , first and the fourth metacarpal on the right in in immature skeleton. There is no appreciable soft tissue swelling. Rest of the bones and joints are normal.
Sickle cell disease is a genetically transmitted autosomal recessive disorder, resulting from an amino acid substitution of valine for glutamic acid at the sixth position on the beta chain of the hemoglobin molecule in red blood cells. This substitution creates instability of the hemoglobin molecule in the deoxygenated state, during which HbS polymerizes and causes red blood cells to change from the usual biconcave disc shape to an irregular sickled shape.The abnormal shape of these red blood cells and their propensity to adhere to the walls of blood vessels can occlude the vessels leading to osteonecrosis.
Infarction of bone and bone marrow in patients with sickle cell disease can lead to the following changes: osteolysis (in acute infarction), osteonecrosis (avascular necrosis/aseptic necrosis), articular disintegration, myelosclerosis, periosteal reaction (unusual in the adult), H vertebrae (steplike endplate depression also known as the Reynold sign or codfish vertebrae), dystrophic medullary calcification , bone-within-bone appearance.
Sickle cell dactylitis (acute hand/foot swelling):
- affects 20%-50% of children with sickle cell disease;
- usually occurs between ages of 6 months and 2 years;
- presents w/ swelling of hands and feet, limitation of motion of extremities, & elevated temperature,
thought to be result of vascular occlusion;
- radiologic findings:
- small osteolytic lesions in metacarpals, metatarsals;
- phalanges show periostitis;
- dactylitis is self limiting after a few days or weeks
- affects 20%-50% of children with sickle cell disease;
- usually occurs between ages of 6 months and 2 years;
- presents w/ swelling of hands and feet, limitation of motion of extremities, & elevated temperature,
thought to be result of vascular occlusion;
- radiologic findings:
- small osteolytic lesions in metacarpals, metatarsals;
- phalanges show periostitis;
- dactylitis is self limiting after a few days or weeks
The early plain radiographic findings of dactylitis consist of soft tissue swelling. Periosteal new-bone formation can be seen on radiographs 7-10 days later. Additionally, medullary expansion, cortical thinning, trabecular resorption, and resultant focal lucency may be seen 2-3 weeks after the onset of symptoms, but these findings usually resolve within weeks.