Sickle cell disease (skeletal manifestations)
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Skeletal manifestations of sickle cell disease result from a combination of bone marrow hyperplasia in the setting of chronic anaemia, and bone infarction as well as their complications.
For a general discussion of sickle cell disease, please refer to sickle cell disease.
Clinical presentation
Bone pain is a frequent complaint, although vaso-occlusive events may also be silent.
Radiographic features
-
marrow hyperplasia
- long bones: widening of medullary spaces and thinning of cortical bone
- skull: widening of diploic space, thinning of the outer table, “hair-on-end” appearance
- osteopaenia and pathological fractures
- extramedullary haematopoiesis
- red marrow reconversion
-
osteonecrosis
- bone infarcts typically involve medullary cavities and epiphyses
- the proximal humeri, proximal femora, and vertebral bodies are often affected
- in the humeri, serpiginous sclerosis is characteristic of infarction
- vertebral infarcts may result in
-
dactylitis also known as hand-foot syndrome
- early manifestation of sickle cell disease in first two years of life which results from bone infarcts in the diaphysis of small long bones
- imaging findings include patchy areas of lucency with periostitis and soft tissue swelling of metacarpals or metatarsals which could be difficult to distinguish from osteomyelitis 4
- growth disturbance
- osteomyelitis
- osteosclerosis
See also
-<strong><a href="/articles/avascular-necrosis">osteonecrosis</a></strong><ul>- +<a href="/articles/avascular-necrosis">osteonecrosis</a><ul>
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