Sickle cell disease (skeletal manifestations)
Skeletal manifestations of sickle cell disease result from three interconnected sequelae of sickle cell disease :
These, in turn, can predispose individuals to other complications, such as growth disturbance and pathological fractures.
For a general discussion of sickle cell disease, please refer to sickle cell disease.
Clinical presentation
Vaso-occlusive crises are common and usually result in skeletal pain. Osteomyelitis will present with localized pain and systemic features of infection.
Radiographic features
The radiographic features will depend on the specific complication and are therefore discussed separately were appropriate.
- vaso-occlusive crises
- 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
- H-shaped vertebrae
- tower vertebra
- vanishing vertebra
- anterior vertebral vascular notches
- subperiosteal and epidural hemorrhage
- hand-foot syndrome (dactylitis)
- imaging findings include patchy areas of lucency with periostitis and soft tissue swelling of metacarpals or metatarsals which are difficult to distinguish from osteomyelitis
- growth disturbance
- osteonecrosis
- chronic anemia
- 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
- osteopenia and pathological fractures
- extramedullary hematopoiesis
- red marrow reconversion
- marrow hyperplasia
- infection
See also
Siehe auch:
- Aseptische Knochennekrose
- Osteomyelitis
- extramedulläre Hämatopoese
- Sichelzellenanämie
- Bürstenschädel
- H-förmige Wirbel
- Acute chest syndrome bei Sichelzellanämie
- Sichelzellenanämie (abdominelle Manifestationen)
- Sichelzellenanämie (zerebrale Manifestationen)
- Sichelzellenanämie (chronische Lungenerkrankung)
und weiter:
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