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Omer Onbas,Cihangir Aliagaoglu,Cagatay Calikoglu,Mecit Kantarci,Mustafa Atasoy,Fatih Alper 대한영상의학회 2006 Korean Journal of Radiology Vol.7 No.1
Neurofibromatosis type 1 disease is characterized by pigmented cutaneous lesions and generalized tumors of a neural crest origin and it may affect all the systems of the human body. Sphenoid dysplasia is one of the characteristics of this syndrome and it occurs in 5-10% of the cases; further, abnormalities of the sphenoid wings are often considered pathognomonic. However, complete agenesis of a sphenoid wing is very rare. We report here on an unusual case of neurofibromatosis type 1 disease with the associated absence of a sphenoid wing that was diagnosed by using multidetector computed tomography.