Sickle cell disease (skeletal manifestations)
Citation, DOI, disclosures and article data
At the time the article was created Alexandra Stanislavsky had no recorded disclosures.View Alexandra Stanislavsky's current disclosures
Skeletal manifestations of sickle cell disease result from three interconnected sequelae of sickle cell disease 5:
vaso-occlusive crises resulting in bone infarcts and subperiosteal hemorrhages
chronic anemia resulting in expansion of the medullary spaces
These, in turn, can predispose individuals to other complications, such as growth disturbance and pathological fractures.
For a general discussion of sickle cell disease, please refer to sickle cell disease.
Vaso-occlusive crises are common and usually result in skeletal pain. Osteomyelitis will present with localized pain and systemic features of infection.
The radiographic features will depend on the specific complication and are therefore discussed separately where appropriate.
bone infarcts typically involve medullary cavities and epiphyses
the proximal humeri, proximal femora, and vertebral bodies are often affected
in the humeri, serpiginous sclerosis is characteristic of infarction
vertebral infarcts may result in
subperiosteal and epidural hemorrhage 6
hand-foot syndrome (dactylitis)
imaging findings include patchy areas of lucency with periostitis and soft tissue swelling of metacarpals or metatarsals which are difficult to distinguish from osteomyelitis 4
osteosclerosis: especially in those with the S/beta-thalassemia genotypes 7
- 1. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics. 21 (4): 971-94. Radiographics (full text) - Pubmed citation
- 2. Ejindu VC, Hine AL, Mashayekhi M et-al. Musculoskeletal manifestations of sickle cell disease. Radiographics. 27 (4): 1005-21. doi:10.1148/rg.274065142 - Pubmed citation
- 3. Stoller DW, Tirman PF, Bredella MA. Diagnostic imaging, Orthopaedics. Amirsys Inc. (2004) ISBN:0721629202. Read it at Google Books - Find it at Amazon
- 4. Espinosa GA. Hand-foot roentgen findings in sickle cell anemia. J Natl Med Assoc. 1979;71 (2): 171-3. Free text at pubmed - Pubmed citation
- 5. Andreu-Arasa VC, Chapman MN, Kuno H, Fujita A, Sakai O. Craniofacial Manifestations of Systemic Disorders: CT and MR Imaging Findings and Imaging Approach. (2018) Radiographics : a review publication of the Radiological Society of North America, Inc. 38 (3): 890-911. doi:10.1148/rg.2018170145 - Pubmed
- 6. S C Thust, C Burke, A Siddiqui. Neuroimaging findings in sickle cell disease. (2014) The British Journal of Radiology. 87 (1040): 20130699. doi:10.1259/bjr.20130699
- 7. De Luna G, Ranque B, Courbebaisse M, Ribeil JA, Khimoud D, Dupeux S, Silvera J, Offredo L, Pouchot J, Arlet JB. High bone mineral density in sickle cell disease: Prevalence and characteristics. (2018) Bone. 110: 199-203. doi:10.1016/j.bone.2018.02.003 - Pubmed