Coxa Vara Deformity in Fibrous Dysplasia/ McCune‐Albright Syndrome: Prevalence, Natural History and Risk Factors: A Two‐Center Study
Authors' conclusion
In conclusion, in tertiary care centers, the prevalence of FD/MAS coxa vara deformity was 36%. Risk factors included presence of MAS, high percentage of femur affected, calcar destruction, radiolucency, NSA <120 degrees and age < 15 years.
Study context
- Population
- Patients <30 years with any subtype of FD/MAS, FD involving the proximal femur and ≥1 X-ray; National Institutes of Health and Leiden University Medical Center274 FD femurs
- Participants
- 180 patients
- Outcome
- Prevalence of coxa vara deformity (NSA <110 degrees or >10 degrees below age-specific values)
Methodological detailslinear mixed effects model
- Linear mixed effects model
Main finding
As reported in the abstract
Prevalence of varus deformity
Femurs with varus deformity
99
36%
Observational data: associations, not effects.
Further findings
Risk factors for deformity, nested case-control analysis
- Presence of MAS
- P < 0.001
- Hyperthyroidism
- P < 0.001
- Hypophosphatemia
- P < 0.001
- High percentage of femur affected
- P < 0.001
- Calcar destruction
- P < 0.001
Risk factors for temporal neck-shaft angle (NSA) decrease, linear mixed effects model
- GH excess
- β = 7.2, p = 0.013
- Hyperthyroidism
- β = 11.3, p < 0.001
- >25% of the femur affected
- β = 13.2, p = 0.046
- Calcar destruction
- β = 8.3, p = 0.004
- Radiolucency
- β = 3.9, p = 0.009
- Bilateral involvement
- β = 9.8, p = 0.010
- Most progression of deformity if NSA <120 degrees with age < 15 years (visual inspection)
- 114 femurs (non-operated, ≥2 X-rays)
Original publication
Coxa Vara Deformity in Fibrous Dysplasia/ McCune‐Albright Syndrome: Prevalence, Natural History and Risk Factors: A Two‐Center Study. J Bone Miner Res. Published 21 May 2023. doi:10. 1002/ jbmr. 4818
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