DOI: 10.1148/rg.274065142
Musculoskeletal Manifestations of Sickle Cell Disease1
Vivian C. Ejindu, MRCP,
Andrew L. Hine, FRCR,
Mohammad Mashayekhi, FRCR,
Philip J. Shorvon, FRCR, and
Rakesh R. Misra, FRCR
1 From the Department of Radiology, Central Middlesex Hospital, North West London Hospitals NHS Trust, Acton Lane, Park Royal, London NW10 7NS, England (V.C.E., A.L.H., P.J.S.); Department of Radiology, Barnet and Chase Farm Hospitals NHS Trust, London, England (M.M.); and Department of Radiology, Buckinghamshire Hospitals NHS Trust, Wycombe, England (R.R.M.). Presented as an education exhibit at the 2005 RSNA Annual Meeting. Received July 26, 2006; revision requested October 19 and received February 2, 2007; accepted February 16. All authors have no financial relationships to disclose.
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Figure 1. Persistent red marrow in a 29-year-old woman. Sagittal T1-weighted MR image of the spine shows low signal intensity indicative of cellular (red) marrow. Vertebral endplate concavity at multiple levels is due to bone softening.
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Figure 2. Effects of intramedullary hyperplasia. Posteroanterior chest radiograph shows a thickened trabecular pattern and loss of corti-comedullary differentiation in the ribs, changes caused by hematopoietic bone marrow. Stage II avascular necrosis in the left humeral head and striation of the intramedullary cavity in the proximal left humerus also are visible.
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Figure 3. Bone marrow expansion within the skull vault. Posteroanterior radiograph obtained in a 12-year-old boy with heterozygous sickle cell disease (Hb SC) demonstrates widening of the medullary cavity with thinning of the inner and outer tables (arrows).
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Figure 5. Fish-mouth vertebral deformities. Lateral radiograph of the lumbar spine in a young man shows the effects of bone softening and resultant compression of vertebrae by adjacent intervertebral disks (arrows).
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Figure 6a. Extramedullary hematopoiesis. T2-weighted coronal (a) and sagittal (b) MR images of the thoracolumbar spine in a 47-year-old woman show a right-sided paravertebral soft-tissue mass (arrow). The mass had intermediate signal intensity on both T1- and T2-weighted images, similar to the signal intensity of normal intramedullary hematopoietic tissue. Vertebral endplate depression due to central infarction also is depicted.
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Figure 6b. Extramedullary hematopoiesis. T2-weighted coronal (a) and sagittal (b) MR images of the thoracolumbar spine in a 47-year-old woman show a right-sided paravertebral soft-tissue mass (arrow). The mass had intermediate signal intensity on both T1- and T2-weighted images, similar to the signal intensity of normal intramedullary hematopoietic tissue. Vertebral endplate depression due to central infarction also is depicted.
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Figure 7. Dactylitis in the hands of an infant. Radiograph shows periosteal new bone formation along the diaphyses of the first three metacarpals of the right hand (arrows) and early destructive lesions in the base of the second metacarpal of both hands (arrowheads).
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Figure 8. Dactylitis in the feet of a 1-year-old child. Radiograph shows periosteal new bone formation along the shafts of the metatarsals in the right foot (arrows) and marked destructive changes that may lead to permanent deformity of the fourth metatarsal in the left foot.
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Figure 9a. Bone infarction of the tibial diaphyses in a 5-year-old child. (a) Initial lateral radiograph shows periosteal new bone formation along the tibial diaphyses (arrows), particularly in the upper third of the right tibia, with normal bone texture. (b) Lateral radiograph obtained 6 weeks later shows distortion of the bone texture, with incorporation of the region of periosteal reaction into cortical bone and resultant cortical thickening in both tibiae, as well as linear serpiginous areas of sclerosis in the left tibial shaft. These are typical radiographic features of chronic infarction.
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Figure 9b. Bone infarction of the tibial diaphyses in a 5-year-old child. (a) Initial lateral radiograph shows periosteal new bone formation along the tibial diaphyses (arrows), particularly in the upper third of the right tibia, with normal bone texture. (b) Lateral radiograph obtained 6 weeks later shows distortion of the bone texture, with incorporation of the region of periosteal reaction into cortical bone and resultant cortical thickening in both tibiae, as well as linear serpiginous areas of sclerosis in the left tibial shaft. These are typical radiographic features of chronic infarction.
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Figure 10. Infarction in a 12-year-old boy with homozygous sickle cell disease, left tibial pain, and a low-grade fever. Medial (left) and lateral (right) views of the left (L) tibia from late static phase 99mTc MDP scintigraphy show an area of decreased tracer uptake within the proximal shaft (arrowheads), a finding suggestive of infarction. A bone aspirate culture was negative, and the patient underwent conservative treatment. (Images courtesy of Muriel Buxton-Thomas, Nuclear Medicine Department, King’s College Hospital NHS Trust.)
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Figure 11. Increased bone density in a 38-year-old man. Anteroposterior radiograph shows patchy sclerosis of the pelvic bone and vertebrae, caused by medullary infarction and dystrophic calcification.
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Figure 12. Vertebral infarction in a 29-year-old woman with back pain. Sagittal T2-weighted MR image of the lumbar spine shows abnormal heterogeneous signal intensity in the L1 and L2 vertebrae.
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Figure 14a. (a) Anteroposterior radiograph obtained in a 44-year-old man shows stage IV avascular necrosis in the left hip and a normal right hip. (b, c) Coronal T2-weighted short inversion time inversion recovery MR images show stage I avascular necrosis in the right hip (arrow in b) as well as flattening of the left femoral head (c), a feature that helped confirm the diagnosis of stage IV avascular necrosis.
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Figure 14b. (a) Anteroposterior radiograph obtained in a 44-year-old man shows stage IV avascular necrosis in the left hip and a normal right hip. (b, c) Coronal T2-weighted short inversion time inversion recovery MR images show stage I avascular necrosis in the right hip (arrow in b) as well as flattening of the left femoral head (c), a feature that helped confirm the diagnosis of stage IV avascular necrosis.
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Figure 14c. (a) Anteroposterior radiograph obtained in a 44-year-old man shows stage IV avascular necrosis in the left hip and a normal right hip. (b, c) Coronal T2-weighted short inversion time inversion recovery MR images show stage I avascular necrosis in the right hip (arrow in b) as well as flattening of the left femoral head (c), a feature that helped confirm the diagnosis of stage IV avascular necrosis.
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Figure 16. Growth disturbance in the distal radius in a 12-year-old girl. Anteroposterior radiograph of the left wrist shows epiphyseal shortening and a cup deformity of the adjacent metaphysis.
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Figure 17. Growth effects. Anteroposterior radiograph of the ankle shows a tibiotalar slant deformity—an angled deformity of the ankle mortise—caused by premature closure of the lateral tibial epiphysis, a condition secondary to ischemia.
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Figure 18a. H-shaped vertebral deformity. Lateral (a) and anteroposterior (b) radiographs of the thoracic spine show vertebral endplate depressions. The sharp depression due to bone infarction in the central endplate contrasts with the smooth indentation seen in the presence of bone softening.
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Figure 18b. H-shaped vertebral deformity. Lateral (a) and anteroposterior (b) radiographs of the thoracic spine show vertebral endplate depressions. The sharp depression due to bone infarction in the central endplate contrasts with the smooth indentation seen in the presence of bone softening.
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Figure 19a. Chronic osteomyelitis. Anteroposterior (a) and lateral (b) radiographs of the left tibia and fibula in an infant show an involucrum that surrounds the shaft of the fibula.
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Figure 19b. Chronic osteomyelitis. Anteroposterior (a) and lateral (b) radiographs of the left tibia and fibula in an infant show an involucrum that surrounds the shaft of the fibula.
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Figure 20a. Osteomyelitis in a 19-year-old woman with left distal tibial and ankle pain. Anterior views of the lower legs, obtained with 99mTc MDP scintigraphy in the dynamic phase (a), blood pool phase (b), and late static phase (c), show increased tracer uptake in the region of the left ankle (arrowhead), a finding suggestive of infection. In c, a slight uptake asymmetry between the upper tibiae also is visible. This appearance represents infarction of the left tibia (arrow) with remodeling. (Images courtesy of Muriel Buxton-Thomas, Nuclear Medicine Department, King’s College Hospital NHS Trust.)
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Figure 20b. Osteomyelitis in a 19-year-old woman with left distal tibial and ankle pain. Anterior views of the lower legs, obtained with 99mTc MDP scintigraphy in the dynamic phase (a), blood pool phase (b), and late static phase (c), show increased tracer uptake in the region of the left ankle (arrowhead), a finding suggestive of infection. In c, a slight uptake asymmetry between the upper tibiae also is visible. This appearance represents infarction of the left tibia (arrow) with remodeling. (Images courtesy of Muriel Buxton-Thomas, Nuclear Medicine Department, King’s College Hospital NHS Trust.)
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Figure 20c. Osteomyelitis in a 19-year-old woman with left distal tibial and ankle pain. Anterior views of the lower legs, obtained with 99mTc MDP scintigraphy in the dynamic phase (a), blood pool phase (b), and late static phase (c), show increased tracer uptake in the region of the left ankle (arrowhead), a finding suggestive of infection. In c, a slight uptake asymmetry between the upper tibiae also is visible. This appearance represents infarction of the left tibia (arrow) with remodeling. (Images courtesy of Muriel Buxton-Thomas, Nuclear Medicine Department, King’s College Hospital NHS Trust.)
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Figure 21a. Anterior views of both tibiae (a) and both ankles (b) from 99mTc hexamethylpropyleneamine oxime–labeled leukocyte imaging (same patient as Fig 20). The appearance of increased radiotracer uptake localized to the region of the left (L) ankle (arrowhead in b) supports a diagnosis of infection. The incidentally observed absence of activity in the upper shaft of the left tibia (arrow in a) is indicative of bone infarction. (Images courtesy of Muriel Buxton-Thomas, Nuclear Medicine Department, King’s College Hospital NHS Trust.)
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Figure 21b. Anterior views of both tibiae (a) and both ankles (b) from 99mTc hexamethylpropyleneamine oxime–labeled leukocyte imaging (same patient as Fig 20). The appearance of increased radiotracer uptake localized to the region of the left (L) ankle (arrowhead in b) supports a diagnosis of infection. The incidentally observed absence of activity in the upper shaft of the left tibia (arrow in a) is indicative of bone infarction. (Images courtesy of Muriel Buxton-Thomas, Nuclear Medicine Department, King’s College Hospital NHS Trust.)
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Figure 22. Osteomyelitis of the left femur in a 24-year-old patient with sickle cell disease. Axial gadolinium-enhanced T1-weighted fat-suppressed MR image shows heterogeneous marrow enhancement, a rounded low-signal-intensity fluid collection adjacent to the shaft, and enhancement of the soft tissues around the shaft and of the adjacent musculature. The areas of enhancement are likely to be infected. (Reprinted, with permission, from reference 22.)
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Figure 23. Chronic osteomyelitis in a 19-year-old woman with known homozygous sickle cell disease. Coronal T2-weighted inversion-recovery MR image obtained at a follow-up examination shows cortical thickening and medullary high signal intensity in the left femur as well as multiple soft-tissue fluid collections (arrows) that were later found to be abscesses.
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Figure 24a. Acute bone infarction. Axial T2-weighted MR images of the femur in a 13-year-old boy with leg pain. (a) Initial image shows regions of both high and low signal intensity within the medullary cavity and high signal intensity in the vastus intermedius muscle, features that may represent either infarction or infection. Infection was not suspected clinically. (b) Follow-up image obtained after 4 weeks of standard management shows resolution of the area of low signal intensity within the medullary cavity and the area of high signal intensity in the adjacent muscle, a finding indicative of infarction.
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Figure 24b. Acute bone infarction. Axial T2-weighted MR images of the femur in a 13-year-old boy with leg pain. (a) Initial image shows regions of both high and low signal intensity within the medullary cavity and high signal intensity in the vastus intermedius muscle, features that may represent either infarction or infection. Infection was not suspected clinically. (b) Follow-up image obtained after 4 weeks of standard management shows resolution of the area of low signal intensity within the medullary cavity and the area of high signal intensity in the adjacent muscle, a finding indicative of infarction.
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Figure 25a. Septic arthritis in a 19-year-old woman with left-sided hip pain and fever. (a, b) Axial T2-weighted inversion recovery MR images show a left hip effusion (arrow in a) that extends into a multilocular fluid collection adjacent to the anterior margin of the hip (b). Diffuse edema also is visible within the superficial soft tissues lateral to both hips.
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Figure 25b. Septic arthritis in a 19-year-old woman with left-sided hip pain and fever. (a, b) Axial T2-weighted inversion recovery MR images show a left hip effusion (arrow in a) that extends into a multilocular fluid collection adjacent to the anterior margin of the hip (b). Diffuse edema also is visible within the superficial soft tissues lateral to both hips.
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Figure 26a. Soft-tissue infection in a 52-year-old man with homozygous sickle cell disease and chronic ankle ulceration. (a) Longitudinal high-frequency (12-MHz) US image of the left ankle shows a hypoechoic collection (arrow) with some internal echoes and layering, located approximately 1 cm deep to the Achilles tendon. Thick pus was aspirated from this area with US guidance. (b) Sagittal T1-weighted MR image shows a low-signal-intensity fluid collection within the pre-Achilles fat space (arrow). Areas of intermediate signal intensity due to the persistence of appendicular red marrow in the medullary cavities of the distal tibia and calcaneum (arrowhead) make the assessment of any changes due to bone involvement difficult; therefore, this sequence cannot be used to exclude osteomyelitis. (c) Sagittal T2-weighted inversion recovery MR image shows the high-signal-intensity fluid collection in the pre-Achilles space (arrow) and a small ankle joint effusion. Bone involvement may be excluded on the basis of this image.
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Figure 26b. Soft-tissue infection in a 52-year-old man with homozygous sickle cell disease and chronic ankle ulceration. (a) Longitudinal high-frequency (12-MHz) US image of the left ankle shows a hypoechoic collection (arrow) with some internal echoes and layering, located approximately 1 cm deep to the Achilles tendon. Thick pus was aspirated from this area with US guidance. (b) Sagittal T1-weighted MR image shows a low-signal-intensity fluid collection within the pre-Achilles fat space (arrow). Areas of intermediate signal intensity due to the persistence of appendicular red marrow in the medullary cavities of the distal tibia and calcaneum (arrowhead) make the assessment of any changes due to bone involvement difficult; therefore, this sequence cannot be used to exclude osteomyelitis. (c) Sagittal T2-weighted inversion recovery MR image shows the high-signal-intensity fluid collection in the pre-Achilles space (arrow) and a small ankle joint effusion. Bone involvement may be excluded on the basis of this image.
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Figure 26c. Soft-tissue infection in a 52-year-old man with homozygous sickle cell disease and chronic ankle ulceration. (a) Longitudinal high-frequency (12-MHz) US image of the left ankle shows a hypoechoic collection (arrow) with some internal echoes and layering, located approximately 1 cm deep to the Achilles tendon. Thick pus was aspirated from this area with US guidance. (b) Sagittal T1-weighted MR image shows a low-signal-intensity fluid collection within the pre-Achilles fat space (arrow). Areas of intermediate signal intensity due to the persistence of appendicular red marrow in the medullary cavities of the distal tibia and calcaneum (arrowhead) make the assessment of any changes due to bone involvement difficult; therefore, this sequence cannot be used to exclude osteomyelitis. (c) Sagittal T2-weighted inversion recovery MR image shows the high-signal-intensity fluid collection in the pre-Achilles space (arrow) and a small ankle joint effusion. Bone involvement may be excluded on the basis of this image.
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Figure 27. Photograph shows extensive ulceration of the lower leg of an adult patient with sickle cell disease. (Image courtesy of Christine Lawrence, MD, FACP, Albert Einstein College of Medicine Gallery of Hematology Images, Yeshiva University.)
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Copyright © 2007 by the Radiological Society of North America.
