| Summary: | Neurofibromatosis type 1 disease is characterized by pigmented cutaneous lesions and generalized tumors of neural crest origin. Sphenoid dysplasia is one of the characteristics of this syndrome which occurs in 5-10% of the cases. However, complete sphenoid wing agenesis is rare. Traditional surgical treatment of sphenoid dysplasia involves split bone grafting and repair of anterior skull defect. However, the results of this procedure may not be entirely sustainable owing to bone graft resorption and recurrence of proptosis and pulsating exophthalmos. We report here a rare case of neurofibromatosis type 1 disease with associated absence of sphenoid wing, for which the newer method of reconstruction with titanium mesh was employed by intracranial approach.
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