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The Hip in Fibrous Dysplasia

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The Pediatric and Adolescent Hip

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Abstract

Fibrous dysplasia is a benign condition caused by a known genetic mutation leading to dysplastic bone formation, which may be accompanied by extra-osseous manifestations including cafe-au-lait spots, precocious puberty, and hyperthyroidism. Clinically, these symptoms may present with pain and deformity, and recognition of these lesions is important to prevent pathologic fracture. The diagnosis of FD is made by plain radiograph, and bone scan remains an effective tool for measuring the extent of disease. The managed of FD of the proximal femur aims at preventing pathologic fracture, deformity correction, and pain control. Asymptomatic and small lesions may be managed by observation. Lesions of the proximal femur associated with pain and deformity require surgical intervention. Intramedullary, load sharing, devices are the preferred treatment for prophylactic fixation, and valgus osteotomy procedures are required for the coxa vara (shepherd’s crook) deformity that classically may present. FD is a disease of the growing patient, and once skeletal maturity is reached the disease process tends to stabilize or may normalize in select patients.

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Author information

Authors and Affiliations

  1. Department of Orthopaedic Surgery, Duke University, Durham, NC, USA

    Brian L. Dial & Benjamin A. Alman

Authors
  1. Brian L. Dial
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  2. Benjamin A. Alman
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Corresponding author

Correspondence to Benjamin A. Alman .

Editor information

Editors and Affiliations

  1. Clinical Director of Paediatric Trauma and Orthopaedic Surgery, Royal Manchester Children Hospital, Manchester University NHS Foundation Trust, Manchester, UK

    Sattar Alshryda

  2. Weill Cornell Medicine, Chief of Orthopaedic Surgery Sidra Medicine, Doha, Qatar

    Jason J. Howard

  3. Senior Attending Physician in Pediatric Orthopedic Surgery, Sidra Medicine, Doha, Qatar

    James S. Huntley

  4. Vanderbilt University Medical Center, Jeffrey Mast Chair of Orthopaedic Hip and Trauma Surgery, Nashville, TN, USA

    Jonathan G. Schoenecker

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Dial, B.L., Alman, B.A. (2019). The Hip in Fibrous Dysplasia. In: Alshryda, S., Howard, J., Huntley, J., Schoenecker, J. (eds) The Pediatric and Adolescent Hip. Springer, Cham. https://doi.org/10.1007/978-3-030-12003-0_32

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  • DOI: https://doi.org/10.1007/978-3-030-12003-0_32

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-12002-3

  • Online ISBN: 978-3-030-12003-0

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