What is the role of radiography 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

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 (see the images below).

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. Advanced dactylitis. Skeletal sickle cell anemia. Advanced dactylitis. Lytic processes are present at the first and fifth metacarpals, along with periostitis, which is most prominent in the third metacarpal.
Skeletal sickle cell anemia. Osteonecrosis. Image Skeletal sickle cell anemia. Osteonecrosis. Image shows flattening of the femoral heads with a mixture of sclerosis and lucency characteristic of osteonecrosis.
Skeletal sickle cell anemia. Bone infarct. Image s Skeletal sickle cell anemia. Bone infarct. Image shows patchy sclerosis of the humeral head and shaft representing multiple prior bone infarcts.
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 can affect the articular portions of the long bones. Collapse of the bone surfaces that are affected by osteonecrosis usually result in degenerative joint destruction. Radiographs of the affected joint may show patchy sclerosis initially, followed by flattening of bone (see the images below).

Skeletal sickle cell anemia. Osteonecrosis. Detail Skeletal sickle cell anemia. Osteonecrosis. Detail of the right hip.
Skeletal sickle cell anemia. Osteonecrosis. Detail Skeletal sickle cell anemia. Osteonecrosis. Detail of the left hip.

Widened medullary cavities may be noted. In the calvaria, the major trabecular spicules in the widened diploic space are aligned perpendicular to the inner table, giving the skull the characteristic hair-on-end appearance (see the images below).

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.
Skeletal sickle cell anemia. Detailed view of the Skeletal sickle cell anemia. Detailed view of the expanded medullary cavity in the same patient as in the previous image.

Thickened bone can be observed. In the extremities, the medullary cavities are widened and the cortices are thinned, with loss of the normal modeling of the bone due to marrow hyperplasia.

Infarction of the central portion of the vertebral endplates results in the characteristic H deformity (angular depression) of vertebral bodies. When present in multiple vertebrae, this finding is virtually pathognomonic for sickle cell disease (see the image below).

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.

Shortening of bone may be depicted. Epiphyseal infarction may produce cone-shaped epiphysis or premature fusion of the epiphysis resulting in abnormal shortening of the involved bone (see the image below).

Skeletal sickle cell anemia. Bone deformity. Image Skeletal sickle cell anemia. Bone deformity. Image shows shortening of the second and third metacarpals and phalanges with partial or complete early fusion of the growth plates due to osteonecrosis in infancy. Osteomyelitis is now superimposed the third metacarpal.

After multiple infarctions of the long bones, sclerosis may assume the appearance of a bone within a bone, reflecting the old cortex within the new cortex (see the image below).

Skeletal sickle cell anemia. Bone-within-bone appe Skeletal sickle cell anemia. Bone-within-bone appearance. Following multiple infarctions of the long bones, sclerosis may assume the appearance of a bone within a bone, reflecting the old cortex within the new cortex.

Regarding myelosclerosis, the cumulative effects from repeated chronic small episodes of infarction result in a mottled, strandlike increased opacity in the medullary region (see the image below).

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.

Although radiography is not as sensitive as other studies for osteomyelitis in the first 1-2 weeks, plain images subsequently show cortical destruction, periosteal new bone, and (with time) sinus tracts and sequestra (see the images below).

Skeletal sickle cell anemia. Bone deformity. Image Skeletal sickle cell anemia. Bone deformity. Image shows shortening of the second and third metacarpals and phalanges with partial or complete early fusion of the growth plates due to osteonecrosis in infancy. Osteomyelitis is now superimposed the third metacarpal.
Skeletal sickle cell anemia. Radiograph of osteomy Skeletal sickle cell anemia. Radiograph of osteomyelitis shows a lytic process with periostitis and marked soft tissue swelling that is best seen on the lateral view.

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