Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience

Abstract

IntroductionFibrous dysplasia (FD) of bone can be present with pain, deformity and pathological fractures. Management is both medical and surgical. Little literature exists on the surgical management of both monostotic and polyostotic FD. We present our experience of limb reconstruction surgery in this pathological group of bone disease.Materials and methodsA retrospective cohort of children who underwent limb reconstruction surgery at a single high-volume paediatric centre was identified from a prospective database. Case notes and radiographs were reviewed. Surgical techniques, outcomes and difficulties were explored.ResultsTwenty-one patients were identified aged between 7 and 13 at presentation to the limb reconstruction unit. Eleven were female, nine had McCune-Albright syndrome, seven had polyostotic FD and five had monostotic. Proximal femoral varus procurvatum deformity was the most common site requiring surgical intervention. The distal femur, tibia, humerus and forearm were also treated.Methods include deformity correction with intramedullary fixation including endo-exo-endo techniques, elastic nailing, guided growth, circular fixator technique and fixator-assisted plating. Correction of deformity and leg length discrepancies was common.The osteotomies went on to heal with no nonunions or delayed healing. We encountered secondary deformity at distal end of nails as the children grew as expected. These were managed with revision nailing techniques and in some cases external fixation. There was one implant failure, which did not require revision surgery.ConclusionThe surgical management of pathological bone disease is challenging. Corrective osteotomies with intramedullary fixation can be very successful if appropriate limb reconstruction principles are adhered to. Deformity correction, guided growth and lengthening can all be successfully achieved in bone affected by FD. Polyostotic FD can be present with secondary deformities, and these can be difficult to manage.How to cite this articleHampton MJ, Weston-Simmons S, Giles SN, et al. Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience. Strategies Trauma Limb Reconstr 2021;16(1):41–45.