Which imaging modalities are used in the workup of sickle cell disease (SCD)?

Updated: Jul 24, 2019
  • Author: Ivan Ramirez, MD; Chief Editor: Felix S Chew, MD, MBA, MEd  more...
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Answer

Answer

MRI is the best method for detecting early signs of changes in bone marrow due to acute and chronic bone marrow infarction, marrow hyperplasia, osteomyelitis, and osteonecrosis. [5, 17, 18, 19, 20, 11]  Differentiation of bone infarct from osteomyelitis is one of the most challenging issues in the evaluation of acute bone pain in patients with SCD. Atypical findings on diffusion-weighted images have been identified in the early phase of presentation and can help differentiate bone infarct from osteomyelitis. [11]

(See the SCD images below.)

 

Skeletal sickle cell anemia. Osteonecrosis. Corona Skeletal sickle cell anemia. Osteonecrosis. Coronal T1-weighted MRI shows a slightly flattened femoral head with a serpentine margin of low signal intensity around an area of ischemic marrow with signal intensity similar to that of fat.
Skeletal sickle cell anemia. Osteonecrosis in the Skeletal sickle cell anemia. Osteonecrosis in the same patient as in the previous image. Coronal T2-weighted MRI shows a serpentine area of low signal intensity and additional focal areas of abnormal low signal intensity in the femoral head; these findings reflect collapse of bone and sclerosis.

Nuclear imaging can also be used to detect early osteonecrosis (see the image below). This modality also plays a role in detecting osteomyelitis. Likewise, indium leukocyte scanning has an important role in diagnosing osteomyelitis.

Skeletal sickle cell anemia. Bone scan of bone inf Skeletal sickle cell anemia. Bone scan of bone infarct shows an area of increased uptake in the distal femoral metaphysis corresponding to the infarct demonstrated on the previous plain radiograph.

Plain radiography of the extremities is useful in evaluating subacute and chronic infarction and in assessing the number and severity of prior episodes of infarction (see the images below). Plain radiographs are also excellent for evaluating deformities and other complications of bone infarction.

 

Skeletal sickle cell anemia. Chronic infarcts and Skeletal sickle cell anemia. Chronic infarcts and secondary osteoarthritis. Image shows advanced changes of irregular sclerosis and lucency on both sides of the knee joint reflecting numerous prior infarcts. The joint surfaces are irregular and the cartilages are narrowed due to secondary osteoarthritis.
Skeletal sickle cell anemia. Medullary sclerosis. Skeletal sickle cell anemia. Medullary sclerosis. Image shows patchy sclerosis of the proximal tibia due to old infarctions. In other cases, sclerosis may be diffuse rather than patchy.

Osteonecrosis is visible on plain images only in the later stages after the affected bone is substantially damaged.

 

Skeletal sickle cell anemia. H vertebrae. Lateral Skeletal sickle cell anemia. H vertebrae. Lateral view of the spine shows angular depression of the central portion of each upper and lower endplate.

 

Skeletal sickle cell anemia. Hand-foot syndrome. S Skeletal sickle cell anemia. Hand-foot syndrome. Soft tissue swelling with periosteal new-bone formation and a moth-eaten lytic process at the proximal aspect of the fourth phalanx.

 

Skeletal sickle cell anemia. Expanded medullary ca Skeletal sickle cell anemia. Expanded medullary cavity. The diploic space is markedly widened due to marrow hyperplasia. Trabeculae are oriented perpendicular to the inner table, giving a hair-on-end appearance.

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