The diagnosis and treatment of focal fibrocartilaginous dysplasia in children

Abstract

Objective To review the clinic presentation, radiographic and MRI appearance of Focal Fibrocartilaginous Dysplasia(FFCD)in children. Methods We retrospectively assessed the radiological and MRI characteristics of FFCD in 4 children. The operative indications and methods were evaluated. Results We reported 4 children with FFCD which have been confirmed by pathology, of which distal femur was involved in one case and distal ulna in one child and proximal tibia in the remaining two infants. The cases involved in distal ulna and distal femur were treated with excision of the lesion, correction osteotomy or femur lengthening with Ilizarov techenique. The two infants with proximal tibia involvement underwent simple excision of the lesion in order to accelerate the correction and to confirm the diagnosis histologically, The. results of the two patients involved distal ulna and distal femur were satisfactory. The angular deformity were completely corrected and normal length was restored at follow-up 66 months and 18 months post-operatively. The proximal tibia vara of FFCD in two infant children improved progressively after 6 and 12 months operatively. Conclusions Focal fibrocartilaginous dysplasia (FFCD) is a rare benign condition inducing bowing deformity of the long bones and the lesions often involved proximal tibia,distal femur and distal ulna. Radiologically, the lesions is characterized by a lucent defect with marginal sclerosis in the medial metepysis of the long bone. The entity represents a bony anchor preventing natural sliding of the periosteum during growth. Treatment indications result from this concept,of which the lesions with a metaphyseal-diaphyseal angle less than 20 degrees,observation is needed for 6 to 12 months; if the deformity improves, the tether likely broke spontaneously, and no treatment is required; if the deformity worsens,, curettage or corrective osteotomy will be needed. Key words: Osteochondrodysplasias; Fibrocartilage; Osteotomy