Surgical treatment of monostotic fibrous dysplasia of the proximal femur in children and adolescents: Observational European Paediatric Orthopaedic Society multicenter study.

Abstract

Purpose: Monostotic fibrous dysplasia is a rare benign fibro-osseous disorder. Proximal femoral monostotic fibrous dysplasia is especially vulnerable to pathological fracture and deformation, requiring specific treatment strategies. Literature on pediatric proximal femoral monostotic fibrous dysplasias is sparse and without consensus. We present the largest observational cohort study on various treatment methods of pediatric proximal femoral monostotic fibrous dysplasia.

Methods: Pediatric patients with proximal femoral monostotic fibrous dysplasia were included, from 10 academic hospitals for oncological orthopedics (2000-2021). Baseline characteristics, treatment strategies, and complications were assessed. Primary outcomes were failure rates, failure-free survival, and risk factors for failure. Failure was defined as fracture, progressive deformity, or surgical (re-)intervention after the start of treatment.

Results: Forty-one pediatric patients with proximal femoral monostotic fibrous dysplasia were included (median age = 11 years (range = 6-16), n = 21 (51%) male). Median follow-up was 5.1 years (range = 0.8-18.6). Index procedure was watchful waiting (n = 9), percutaneous procedure (n = 4), open procedure (n = 15), or internal fixation (n = 13). Failure rates were 11%, 50%, 40%, and 31%, respectively (p = 0.41). Overall, 2- and 5-year failure-free survival was stable at 87.5% (95% confidence interval = 64.6-110.4). Risk factors associated with failure were fracture at diagnosis (hazard ratio = 3.7, 95% confidence interval = 1.2-11.5), calcar involvement (hazard ratio = 2.6, 95% confidence interval = 0.7-9.4), and male sex (hazard ratio = 2.1, 95% confidence interval = 0.6-7.8).

Conclusion: In cases with low fracture and deformity risk, watchful waiting can be a viable management option for proximal femoral monostotic fibrous dysplasia. When intervention is necessary, internal fixation is advised to prevent fractures and deformity. Curettage with grafting or bone substitute injections should be used with hesitance. Currently, there is no clearly superior treatment for pediatric proximal femoral monostotic fibrous dysplasia, leaving treatment choices to be based on individual characteristics.