[Fibrous dysplasia of the proximal femur in children and teenagers: surgical results in 22 cases].
OBJECTIVE
Fibrous dysplasia is a rare benign bone tumor which occurs preferentially in the proximal femur. In children, there is a risk of repeated fractures and coxa vara deformity, particularly in the polyostotic form. The most common orthopedic problem is fibrous dysplasia of the proximal femur which generally requires surgical treatment. The purpose of this study was to analyze clinical and radiological outcome after surgical treatment.


METHODS
All children who underwent surgical treatment for fibrous dysplasia of the proximal femur between 1979 and 2001 were reviewed retrospectively. The study cohort included 22 children (11 boys and 11 girls). Eight patients had a monostotic form and 14 a polyostotic form of the disease. For the monostotic forms, the type of treatment depended on the size of the tumor and its localization but curettage was used in all cases. For the polyostotic forms, treatment consisted in valgus osteotomy with "humeralization" in the event of associated coxa vara in combination with internal fixation, generally with a centromedullary nail.


RESULTS
In the monostotic forms, the clinical outcome was considered good in all cases. Nearly total involution of the tumor was noted in 75% of patients. In the polyostotic forms, osteotomy with "humeralization" and centromedullary nailing provided stable correction of the deformation. Outcome was less satisfactory because of fractures and deformities.


CONCLUSIONS
In light of our results and those reported in the literature, the prognosis of the monostotic form can be considered good after surgical treatment. For the polyostotic form, preventive fixation is necessary. Osteotomy with "humeralization" appears to correct the deformity and prevent coxa vara in certain cases. For the more severe forms, medical treatment with biphosphonates may be a useful complement to the surgical treatment.
Durand
S
S
Service d'Orthopédie et Traumatologie Pédiatrique, Hôpital des Enfants-Malades, 149, rue de Sèvres, 75015 Paris. sebdurand3@hotmail.com
Hamcha
H
H
Pannier
S
S
Padovani
J-P
JP
Finidori
G
G
Glorion
C
C
D000293:Adolescent; D000328:Adult; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D001858:Bone Nails; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003475:Curettage; D004164:Diphosphonates / Q000627:therapeutic use; D005260:Female; D005269:Femur / Q000601:surgery*; D005357:Fibrous Dysplasia of Bone / Q000601:surgery*; D005358:Fibrous Dysplasia, Monostotic / Q000601:surgery; D005359:Fibrous Dysplasia, Polyostotic / Q000601:surgery; D006801:Humans; D016268:Internal Fixators; D008297:Male; D010027:Osteotomy / Q000295:instrumentation / Q000379:methods; D012189:Retrospective Studies; D016896:Treatment Outcome
D050071:Bone Density Conservation Agents; D004164:Diphosphonates