Sickle cell disease (skeletal manifestations)

Skeletal manifestations of sickle cell disease result from three interconnected sequelae of sickle cell disease ⁵: 

1. vaso-occlusive crises resulting in bone infarcts and subperiosteal hemorrhages

2. chronic anemia resulting in expansion of the medullary spaces

3. infection

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 where 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 ⁶

  • 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 ⁴

  • growth disturbance

• 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

• infection

  • osteomyelitis

• osteosclerosis: especially in those with the S/beta-thalassemia genotypes ⁷

See also

• sickle cell disease (general article)

  • acute chest syndrome in sickle cell disease

  • chronic lung disease in sickle cell disease

  • abdominal manifestations of sickle cell disease

  • cerebral manifestations of sickle cell disease