Reconstruction of skull base defects in sphenoid wing dysplasia associated with neurofibromatosis i with titanium mesh

Mohamed Lotfy, Risheng Xu, Matthew McGirt, Sameh Sakr, Basim Ayoub, Ali Bydon

Research output: Contribution to journalArticle

Abstract

Sphenoid wing dysplasia occurs in 3-7% of patients with neurofibromatosis type 1 (NF1). The typical radiological features are partial or complete absence of the greater wing of the sphenoid. This condition is slowly progressive and may result in temporal lobe herniation into the orbital cavity, producing pulsating exophthalmos and gross facial deformity. Thus, reconstruction of the orbit is important for both cosmetic and functional reasons. Traditional surgical treatment of sphenoid dysplasia involves split bone grafting and repair of the anterior skull base defect. However, several reports have demonstrated complications of graft resorption and recurrence of proptosis and pulsating exopthalmos. In this case series, we present two patients suffering from pulsating exophthalmos due to sphenoid dysplasia. Radiological and MRI studies demonstrated orbital enlargement and complete absence of the greater wing of the sphenoid. Surgical management of these patients involved dural defect repair, and the use of titanium mesh in conjunction with bone graft to act as a barrier between the orbit and the middle cranial fossa. The mesh was fixed by fine screws. Proptosis improved markedly post-operatively and resolved within a few weeks. Ocular pulsation subsided and remained quiescent with at least 1-year follow-up.

Original languageEnglish (US)
Pages (from-to)909-914
Number of pages6
JournalClinical Neurology and Neurosurgery
Volume112
Issue number10
DOIs
StatePublished - Dec 1 2010

Keywords

  • Dysplasia
  • Pulsating exophthalmos
  • Reconstruction
  • Skull base
  • Sphenoid wing
  • Titanium mesh

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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