Sickle Cell Spine¶

Overview¶

Sickle cell disease produces characteristic spinal findings from vaso-occlusive crises and chronic hemolysis. The hallmark is the H-shaped vertebra (Lincoln log vertebra) — central endplate depression from subchondral bone infarction creating a step-like deformity.

Imaging Findings¶

H-shaped (Lincoln log) vertebra — Central endplate depression on lateral radiograph/sagittal CT, pathognomonic of sickle cell disease
Diffuse marrow changes — Low T1 signal throughout vertebral bodies from red marrow reconversion (chronic hemolysis demands increased erythropoiesis)
Bone infarctions — Patchy marrow signal changes from vaso-occlusive crises
Compression fractures — From weakened, infarcted bone
Osteomyelitis — Increased risk; Salmonella is a characteristic organism in sickle cell (unlike S. aureus in the general population)
Extramedullary hematopoiesis — Paravertebral soft tissue masses from compensatory red marrow expansion

Clinical Pearl

The H-shaped vertebra is virtually pathognomonic of sickle cell disease. It results from infarction of the central subchondral bone (supplied by the most distal branches of the vertebral body vasculature), while the peripheral endplate remains intact. This creates the characteristic step-off. The differential includes Gaucher disease, which can produce a similar appearance.

Key Points¶

H-shaped (Lincoln log) vertebra is pathognomonic
Diffuse low T1 marrow signal from red marrow reconversion
Salmonella osteomyelitis is characteristic (though S. aureus is still most common overall)
Extramedullary hematopoiesis can produce paravertebral masses
Vaso-occlusive bone infarctions cause patchy marrow signal changes

References¶

Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. RadioGraphics. 2001;21(4):971-94. https://pubmed.ncbi.nlm.nih.gov/11452073/
Ejindu VC, Hine AL, Mashayekhi M, Shorvon PJ, Misra RR. Musculoskeletal manifestations of sickle cell disease. RadioGraphics. 2007;27(4):1005-21. https://pubmed.ncbi.nlm.nih.gov/17620464/
Kosaraju V, Harwani A, Partovi S, et al. Imaging of musculoskeletal manifestations in sickle cell disease patients. Br J Radiol. 2017;90(1073):20160130. https://pmc.ncbi.nlm.nih.gov/articles/PMC5605094/
Sedrak A, Kondamudi NP. Sickle Cell Disease. In: StatPearls. Treasure Island (FL): StatPearls Publishing; updated 2023 Aug 28. https://www.ncbi.nlm.nih.gov/books/NBK482384/
Expert Panel on Pediatric Imaging, Shet NS, Iyer RS, Chan SS, et al. ACR Appropriateness Criteria® Osteomyelitis or Septic Arthritis-Child (Excluding Axial Skeleton). J Am Coll Radiol. 2022;19(5S):S121-S136. https://pubmed.ncbi.nlm.nih.gov/35550797/
Sickle cell disease (skeletal manifestations). Radiopaedia.org. https://radiopaedia.org/articles/sickle-cell-disease-skeletal-manifestations-1