Radiologic Manifestations of Bone Disease in Sickle Cell Anemia
Transcript of Radiologic Manifestations of Bone Disease in Sickle Cell Anemia
Oni Blackstock, HMS IIIGillian Lieberman, MD
Radiologic Manifestations of Bone Disease in Sickle Cell Anemia
Oni J. Blackstock, HMS IIIGillian Lieberman, MD
March 2003
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Presentation Outline
• Meet patient #1• MSK imaging modalities• Brief review of Sickle cell anemia and its
clinical manifestations • Radiologic imaging of the bone
complications of SCA
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Meet Patient #1• 44-year old African-American male with hx
of SCA and associated chronic pain syndrome
• Presents with complaint of left knee pain• Reports increased pain while walking and
occasionally at rest• Has had to “dip” into MSContin for
additional analgesia
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Bone Imaging modalities
• Plain radiograph:– initial radiologic evaluation of the majority
of suspected musculoskeletal abnormalities
– Relatively specific in differentiating potential etiologies of lesion b/c of proven ability to characterize features of the lesion
– May not detect certain processes until they are far progressed
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Bone imaging modalities• CT
– Can detect lesions not seen on routine radiographs or when plain film evaluation is difficult due to nature of lesion or anatomic site
– best technique in assessment of matrix mineralization, cortical detail, and detection of the cystic and fatty lesions
– Can identify subtle bony abnormalities (occult fractures) and their relationship to adjacent organs
– May fail to demonstrate early vascular and marrow abnormalities
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Bone imaging modalities• MRI
– Method of choice for imaging the soft tissues of MSK system
– Sensitive to changes in bone marrow (i.e. subtle marrow edema, marrow infarcts)
– Can detect and stage primary bone tumors and occult bony metastases
– Difficulty visualizing fine bone detail or small calcifications
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Bone imaging modalities• Bone radionuclide scan
– Sensitive, but nonspecific for measuring bone formation and perfusion
– Detects lesions not otherwise seen on radiograph– Effective for imaging bone marrow and
identifying areas of red marrow replacement– Effective screen for detecting multiple lesions– Lacks sufficient spatial resolution to detail
extent and anatomic involvement of disease
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Bone imaging modalities
• Ultrasound– High frequency transducers allow markedly
improved image resolution– Can detect joint effusions, subperiosteal fluid,
soft tissue masses, congenital hip dysplasias, etc.
– Ineffective in visualizing bone detail
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pt #1: AP films of left knee
BIDMC PACS
Ill-defined scleroticdensities
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pt #1: Lateral oblique of knee
BIDMC PACS
Ill-defined sclerotic densities
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Differential dx for multiple sclerotic densities
• Bone infarct• Chronic osteomyelitis• Osteoblastic metastases• Enchondromatosis• Bone islands
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pt #1: Left knee MR – T2 sagittal
heterogeneous bone marrow
BIDMC PACS
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pt #1: Left tibia MR – T2 axial
diffusely heterogeneousbone marrow
BIDMC PACS
sclerotic bone
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pt #1: Left knee MR - coronal
BIDMC PACS
heterogeneous bone marrow
scleroticbone
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Differential dx of bone infarct*
• Idiopathic• Occlusive vascular disease • Sickle cell anemia • Fat embolism• Infection, osteomyelitis
*diff dx for diametaphyseal ischemia
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Quick Review of SCA
• Most common hemoglobin variant in the world (HbS)
• Autosomal recessive• ~8% of African Americans are carriers• High frequency of HbS gene attributed to
“balanced polymorphism”• Clinical severity can vary among those afflicted
with SCA
http://peptide.ncsa.uiuc.edu/tutorials_current/Sickle_Cell_Anemia/SC2001/intro.html
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pathogenesis of SCA
• Mutation endows deoxy HbS new ability to polymerize distorting the RBC contours
= HbS
NucleationGrowth
Alignment
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pathophysiology of SCA
• In addition to polymerization,– Alteration in RBC membrane and cytoskeleton– Increased RBC adherence to vascular
endothelium– Disordered RBC volume control– The above factors act in concert to contribute to
the vasooclusive episodes and hemolysis seen in SCA
http://carnegieinstitution.org/first_light_case/horn/lessons/sickle.html
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Clinical manifestations of SCA• Symptoms typically begin after 6 mo. of age• Heterogeneity in clinical and hematolgic severity• Hematologic:
– chronic anemia– acute severe anemia (splenic sequestration, aplastic
crisis, hyperhemolytic crisis)– acute vassoclusive episodes
• Gastrointestinal: pigmented gallstones• Neurologic:
– Transient ischemic attacks– infarctive stroke– intracerebral hemorrhage– retinopathy
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Clinical Manifestations of SCA• Cardiac: acute myocardial infarction and high
output failure• Pulmonary: acute chest syndrome (incl. pneumonia
and infarction due to in situ thrombosis)• Renal:
– papillary necrosis– focal glomerulosclerosis
• Infectious: bacteremia, osteomyelitis, meningitis
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Bone complications in SCA
• Among the most common manifestations of SCA
• Due to one or both of the following factors:– Bone marrow hyperplasia secondary to chronic
hemolysis– Vaso-occlusion leading to ischemia and
infarction
www.ex.ac.uk/shipss/medhist/
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Marrow hyperplasia
BIDMC PACS
• widespread expansion ofhematopoeitic bone marrow throughout the body
• Commonly seen in chronichemolytic anemias
fatty marrow
hematopoeitic red marrow
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Skull – marrow hyperplasia
Lonergan GJ, et. al. Sickle Cell Anemia. AFIP Archives 2001; 21:971-994
Reynolds, J.The Skull and spine. Seminars in Roentgenology July 1987; 22:168-175
widened diploic spacered bone marrow
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Skull – marrow hyperplasia
Lonergan GJ, et. al. Sickle Cell Anemia. AFIP Archives 2001; 21:971-994
Hair-on-endappearance
• Occurs secondary to thinning of the outer table• Not frequently seen in SCA, but more common in β-thalassemia
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Spinal complications of SCA
• Vertebral sclerosis due to many small infarcts beginning early in life
• Central cupping of the end-plate (aka H- shaped vertebrae)
• Vertebral collapse
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Oni Blackstock, HMS IIIGillian Lieberman, MD
H-shaped vertebrae
BIDMC PACSACR
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Vertebral blood supply
Reynolds, J.The Skull and spine. Seminars in Roentgenology July 1987; 22:168-175
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Hands and Feet
Gaston, M. Sickle cell disease: An overview. Seminars in Roentgenology July 1987; 22:150-159
• Dactylitis also known as hand-foot syndrome
• Most often occurs between 6 and 18 months of age
• Usually the first ischemic manifestation of SCA
• May involve one bone or multiple bones
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Dactylitis
soft tissue swellingof index fingerperiostitis along 3rd
through 5th metacarpals
Diff dx includes:•Pyogenic osteomyelitis (esp. Salmonella)•Tuberculosis
Lonergan GJ, et. al. Sickle Cell Anemia. AFIP Archives 2001; 21:971-994
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Generalized Osteosclerosis• secondary to multiple widespread tiny infarcts• differential dx includes osteoblastic mets
BIDMC PACS
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Long bone circulation
• The nutrient artery supplies themedullary cavity and inner cortex
• Multiple periosteal vessels supplythe outer cortex
• The metaphyseal and epiphysealregions are well-perfused by their own network of vessels
Bohrer SP. Bone ischemia and infarction in sickle cell disease. St. Louis: Warren H. Green Inc., 1981
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Shoulder: bone infarct
BIDMC PACS
metadiaphysealmedullary infarct
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Lower extremity infarcts-bone scan
Increased uptake
• Targets bone with Tc-99m methylene diphosphonate
• Immediately after infarction,scan demonstrates decreased uptake
•With reactive bone formation,there is increased uptake
•May be difficult to distinguishinfarct from osteomyelitis
BrighamRADS website
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Pt #1 revisited: Plain film of hips - AP
BIDMC PACS
sclerosis offemoral head
sclerosis of femoral head
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Companion pt: coronal Hip MR
flattenedfemoral head
BIDMC PACS
low signalintensity infemoral head
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Femoral head anatomy
• vascular supply to femoralhead is an end-organ systemwith poor development of collaterals
• femoral head more vulnerableto episodes of vaso-occlusion
http://home.pacific.net.au/~rossjones/avn.htm
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Complications of bone infarction• Fractures
– Resulting from full thickness cortical necrosis• Osteomyelitis
– Necrotic bone marrow fertile site for 2º infection– Most common offender is Salmonella
• Growth disturbances– Usually secondary to deficiency in the nutrient
artery circulation to the central region of the metaphyseal side of the growth plate
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Radiographic progression of osteomyelitis
Initial radiograph
7 days later
5 weeks later
periostitis
centrallucency
Lonergan GJ, et. al. Sickle Cell Anemia. AFIP Archives 2001; 21:971-994
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Summary• Bone abnormalities in SCA result secondary to
marrow hyperplasia and episodes of ischemia leading to infarction
• Multiple modalities can be used in conjunction with one another to image these bony changes
• Notably, MRI is most sensitive to the detection of bone marrow changes which are commonly seen in SCA
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Oni Blackstock, HMS IIIGillian Lieberman, MD
References• Reynolds, J.The Skull and spine. Seminars in Roenthenology July 1987; 22:168-175• Lonergan GJ, et. al. Sickle Cell Anemia. AFIP Archives 2001;21:971-994• Gaston, M. Sickle cell disease: An overview. Seminars in RoentgenologyJuly 1987; 22:150-159• Bohrer SP. Bone ischemia and infarction in sickle cell disease. St. Louis:Warren H. Green Inc., 1981• Alvavi A, et al. Scinitgraphic Examination of Bone and Marrow Infarcts inSickle cell Disorders. Seminars in Roentgenology. July 1987;22:213-223• Bohrer, S. Bone changes in the Extremities in Sickle Cell Anemia. Seminars in Roentgenology.July 1987;22:176-185
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Oni Blackstock, HMS IIIGillian Lieberman, MD
Acknowledgments
• Gillian Lieberman, MD• Pamela Lepkowski• Ferris Hall, MD• Kemi Babagbemi, MD• Daniel Saurborn, MD• Joe Makris, MD• The patients!!• Larry Barbaras • Cara Lyn D’amour