Absence of a sphenoid wing in neurofibromatosis type 1 disease: imaging with multidetector computed tomography.
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.
Ataturk University, School of Medicine, Department of Radiology, Erzurum, Turkey. oonbas@hotmail.com
Omer
O
Onbas
Cihangir
C
Aliagaoglu
Cagatay
C
Calikoglu
Mecit
M
Kantarci
Mustafa
M
Atasoy
Fatih
F
Alper
D000328:Adult; D006801:Humans; D021621:Imaging, Three-Dimensional; D008297:Male; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging*; D013100:Sphenoid Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed