RESUMO
Fibrous dysplasia is a rare benign intramedullary fibro-osseous lesion characterized by fibro-osseous proliferation with intervening areas of normal or immature bone. It can either be a monostotic or a polyostotic presentation. The etiology of fibrous dysplasia has been linked with a missense mutation in the GNAS1 gene on chromosome 20. The most common fibrous dysplasia is first diagnosed in children or young adults. There is no gender predilection. Overall, fibrous dysplasia constitutes 5% of all benign bone lesions.The monostotic form is the most frequent, accounting for 75% to 80% of fibrous dysplasia cases. We report a case of unilateral monostotic fibrous dysplasia in a 30-year-old male in the proximal femur with Shepherd's crook deformity. The patient underwent a single-stage procedure of curettage of lesion and valgus osteotomy with dynamic hip screw (DHS) fixation and fibular strut graft. The lesion resulted in alteration of hip joint anatomy with a decrease in the neck-shaft angle to 114 degrees and leading to coxa vara. After surgical correction, the neck-shaft angle was restored to 130 degrees. The patient was followed up in the outpatient department (OPD), x-rays were taken, and signs of radiological healing were seen at three months. Partial weight-bearing was allowed at three months postoperatively and full weight-bearing at six months with no restriction in the activity. After six months, the patient was able to perform all activities without any difficulty, and shortening of 1.5 cm was compensated with footwear modification. No evidence of recurrence was noted in the follow-up x-ray. Fibrous dysplasia of proximal femur treated with curettage and bone grafting and supported with an osteotomy to correct mechanical alignment provides excellent results. DHS, though old hardware, provides a versatile option to support osteotomy and helps in maintaining the correction. To support the neck femur after curettage, the fibula strut graft provides an excellent option. When the procedure is done in a single stage, it gives good functional and radiological outcomes along with early rehabilitation.
