Sickle cell disease (skeletal manifestations)
Citation, DOI & article data
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 were appropriate.
- vaso-occlusive crises
- 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 hemorrhage6
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
- growth disturbance
- chronic anemia
- 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