A POLYOSTIC FORM OF FIBROUS DYSPLASIA: CORRECTION OF THE SHORTENED AND DEFORMED RIGHT UPPER LIMB WITH TSF EXTERNAL FIXATOR SYSTEM– CASE REPORT

Abstract

Fibrous dysplasia (FD) is a congenital skeletal disorder characterized by the replacement of the bone marrow with fibrous tissue. FD may occur in isolation or association with endocrinopathies, and in that case, labeled McCune–Albright syndrome (MAS). FD can cause bone deformities or/and limb length discrepancies. The surgical intervention aims to correct limb deformities and length discrepancies while improving regional aesthetics. We report a case of a 14-year-old girl with FD that had affected her upper limbs. More characteristic had been the shortening of the right humerus (10 cm discrepancy from the left humerus) and the gun-stock deformity of the elbow. On the X-ray, a prominent feature was the shortening of the humerus and the valgus deformity of its distal end. The lengthening and correction of the deformity of the right humerus had been performed using the Taylor spatial frame (TSF) system. The software program had provided us with the estimated minimum correction time of 143 days for 7 cm humeral lengthening. A total of 157 days had been needed to lengthen and correct the deformity. The TSF system allows the simultaneous lengthening and correction of all the components of a multiplanar malformation of a limb and minimizes the required time.