Extensive Polyostotic Craniofacial Fibrous Dysplasia With Optic Nerve Impingement

J Craniofac Surg. 2021 Jul-Aug;32(5):e435-e437. doi: 10.1097/SCS.0000000000007241.

Abstract

Fibrous dysplasia is a benign overgrowth of metaplastic fibrous material resulting in disorganized deposition of bony matrix. Surgical intervention is the primary treatment modality. Here the authors present the case of a 36-year-old male with extensive and severe fibrous dysplasia of the calvarium, orbit, sphenoid, and facial bones causing significant facial distortion and impingement of his optic nerve. Combined operative treatment with craniofacial plastic surgery and neurosurgery was performed. Repair consisted of extensive intra- and extracranial resection and contouring of involved bones followed by reconstruction of the superior orbital rims, forehead, orbital roof, and calvarium with custom polyetheretherketone (PEEK) implant. The authors discuss the advantages of using computer assisted design/modeling, intraoperative neuronavigation, and custom prosthetic cranioplasty for surgical treatment of extensive fibrous dysplasia; a review of the current surgical literature is provided.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Craniofacial Fibrous Dysplasia*
  • Dental Implants*
  • Facial Bones / diagnostic imaging
  • Facial Bones / surgery
  • Fibrous Dysplasia, Polyostotic* / diagnostic imaging
  • Fibrous Dysplasia, Polyostotic* / surgery
  • Humans
  • Male
  • Optic Nerve
  • Skull

Substances

  • Dental Implants