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

Strategies Trauma Limb Reconstr

Department of Paediatric Orthopaedics and Trauma Surgery, Sheffield Children's NHS Foundation Trust, Sheffield, South Yorkshire, United Kingdom.

Published: January 2021

Introduction: Fibrous 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 Methods: A 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.

Results: Twenty-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.

Conclusion: The 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 Article: Hampton MJ, Weston-Simmons S, Giles SN, . Strategies Trauma Limb Reconstr 2021;16(1):41-45.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8311743PMC
http://dx.doi.org/10.5005/jp-journals-10080-1523DOI Listing

Publication Analysis

Top Keywords

limb reconstruction
16
deformity correction
12
fibrous dysplasia
8
surgical management
8
reconstruction surgery
8
intramedullary fixation
8
guided growth
8
deformity
7
surgical
6
limb
6

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!