What is the role of nuclear imaging in the workup of sickle cell disease (SCD)?

Answer

Technetium-99m (^99mTc) bone scanning can be used to detect early stages of osteonecrosis, and it is not as costly as MRI. Osteonecrosis can be detected on bone scans, appearing mostly as focal areas of increased activity. Occasionally, areas of decreased uptake can be seen; this is usually seen in early disease. Technetium-99m bone marrow scans demonstrate areas of decreased activity in marrow infarction.^{[24, 9]}

Skeletal sickle cell anemia. Bone infarction in an infant (see also next image). Image shows a curvilinear area of sclerosis with central lucency in the metaphysis of the femur representing a bone infarct.

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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.

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Indium-111 (¹¹¹In) white blood cell (WBC) scanning is useful to diagnose osteomyelitis, which appears as an area of increased activity within bone. However, areas of marrow proliferation, which are common in patients with sickle cell, would also demonstrate increased activity on ¹¹¹In WBC scans. The combination of a bone scan and a bone marrow scan has been used to differentiate acute osteomyelitis from bone infarcts in patient with sickle cell, since the clinical presentation of these 2 conditions may be very similar. Areas of bone infarction may be identified by decreased activity on the bone marrow scan with corresponding abnormal uptake on the bone scan. Acute osteomyelitis demonstrates increased activity on the bone scan, with normal activity on the bone marrow scan.^{[9, 25]}

Single-photon emission computed tomography (SPECT)/CT has become an important tool to evaluate the different bone lesions in SCD, such as osteopenia, osteoporosis, avascular necrosis, and pathologic fracture.^[15]

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Media Gallery

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. 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. 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. 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 expanded medullary cavity in the same patient as in the previous 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. Osteonecrosis. Detail of the right hip.
Skeletal sickle cell anemia. Osteonecrosis. Detail of the left hip.
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. 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. 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.
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.
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 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.
Skeletal sickle cell anemia. Osteomyelitis. CT scan in a soft tissue window demonstrates a large abscess in the left thigh encircling the femur, with hypoattenuating pus surrounded by a rim of vivid enhancement.
Skeletal sickle cell anemia. Osteomyelitis and bone-within-bone. Bone-window CT scan in the same patient as in the previous image shows a bone-within-bone appearance (concentric rings of cortical bone) in the right femur. On the left, a sinus tract (cloaca) traverses the lateral aspect of the femoral cortex, and a small, shardlike sequestrum is present deep to the sinus tract.
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 osteomyelitis shows a lytic process with periostitis and marked soft tissue swelling that is best seen on the lateral view.
Skeletal sickle cell anemia. Osteomyelitis. Coronal T1-weighted MRI shows marrow edema in the shortened third metacarpal, which appears dark. Note the loss of cortex along the radial aspect of the metacarpal.
Skeletal sickle cell anemia. Osteomyelitis in the same patient as in the previous image. On this T2-weighted MRI, the marrow and surrounding tissues are very bright as a result of inflammatory edema.
Skeletal sickle cell anemia. Osteomyelitis in the same patient as in the previous image. Axial T2-weighted MRI again shows the marrow edema and surrounding subperiosteal pus collection, with a thin, dark rim representing the periosteal membrane. There is diffuse bright signal intensity in the soft tissues; this represents inflammation and edema.
Skeletal sickle cell anemia. Bone infarction in an infant (see also next image). Image shows a curvilinear area of sclerosis with central lucency in the metaphysis of the femur representing a bone infarct.
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.

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Author

Ivan Ramirez, MD Radiologist, CMI Radiology Group

Ivan Ramirez, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Radiology, Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

Welansa Asrat, MD Staff Physician, Departments of Medicine and Pediatrics, Saint Vincent's Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Bernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand

Disclosure: Nothing to disclose.

William R Reinus, MD, MBA, FACR Professor of Radiology, Temple University School of Medicine; Chief of Musculoskeletal and Trauma Radiology, Vice Chair, Department of Radiology, Temple University Hospital

William R Reinus, MD, MBA, FACR is a member of the following medical societies: Alpha Omega Alpha, Sigma Xi, American College of Radiology, American Roentgen Ray Society, Radiological Society of North America

Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, MEd Professor, Department of Radiology, Vice Chairman for Academic Innovation, Section Head of Musculoskeletal Radiology, University of Washington School of Medicine

Felix S Chew, MD, MBA, MEd is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America

Disclosure: Nothing to disclose.

Additional Contributors

Henrique M Lederman, MD, PhD Professor of Radiology and Pediatric Radiology, Chief, Division of Diagnostic Imaging in Pediatrics, Federal University of Sao Paulo, Brazil

Henrique M Lederman, MD, PhD is a member of the following medical societies: Society for Pediatric Radiology

Disclosure: Nothing to disclose.

Acknowledgements

Dvorah Balsam, MD Chief, Division of Pediatric Radiology, Nassau University Medical Center; Professor, Department of Clinical Radiology, State University of New York at Stony Brook

Disclosure: Nothing to disclose.

Mark HJ Choi, MD Fellow in Musculoskeletal Radiology, Department of Radiology, University of Pennsylvania School of Medicine

Mark HJ Choi, MD is a member of the following medical societies: American College of Radiology

Disclosure: Nothing to disclose.

What is the role of nuclear imaging in the workup of sickle cell disease (SCD)?

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