Pediatric endocrinology

Lesion Expansion in Gnathic Fibrous Dysplasia: Natural History, Indicators of Progression, and Response to Bisphosphonates

J Bone Miner ResPublished Longitudinal cohort study

Authors' conclusion

Gnathic FD lesion expansion is most rapid in younger children and declines as patients approach adulthood.

Study context

Population
Patients with gnathic fibrous dysplasia (FD), with or without McCune-Albright syndrome, and serial CT imaging; median age at initial scan 8.8 years (range 3.4–18.8)
Participants
52 patients104 lesions
Outcome
FD lesion volume (volumetric CT analysis)
Methodological detailsserial CT imaging, volumetric analysis, generalized mixed model, Median follow-up imaging: median 4 scans per subject (range 2-14); median age at final scan 16.8 years (range 6.9-33.4 years)
  • Serial CT imaging
  • Volumetric analysis
  • Generalized mixed model
  • Median follow-up imaging: median 4 scans per subject (range 2-14); median age at final scan 16.8 years (range 6.9-33.4 years)

Main finding

As reported in the abstract

FD lesion volume

Increase in lesion volume with age, cm³/yr

2.50

95% CI 1.95–3.04p < 0.001

Change in lesion expansion rate over time, cm³/yr

−0.05

95% CI −0.07 to 0.04p < 0.001

Observational data: associations, not effects.

Further findings

Risk factors for lesion expansion

Mandibular lesions tended to expand at a greater rate than maxillary lesions
P < 0.001
GH excess associated with accelerated expansion rate
P = 0.002
Other McCune-Albright syndrome (MAS)-associated endocrinopathies, pubertal status, sex, weight, lesion density, disease severity and bisphosphonate treatment not associated with lesion volume or expansion

Original publication

Lesion Expansion in Gnathic Fibrous Dysplasia: Natural History, Indicators of Progression, and Response to Bisphosphonates. J Bone Miner Res. Published 31 July 2023. doi:10.1002/jbmr.4886

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