Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography.
- Author:
Omer ONBAS
1
;
Cihangir ALIAGAOGLU
;
Cagatay CALIKOGLU
;
Mecit KANTARCI
;
Mustafa ATASOY
;
Fatih ALPER
Author Information
- Publication Type:Case Report
- Keywords: Neurofibromatosis; Computed tomography
- MeSH: *Tomography, X-Ray Computed; Sphenoid Bone/*abnormalities/radiography; Neurofibromatosis 1/*radiography; Male; Imaging, Three-Dimensional; Humans; Adult
- From:Korean Journal of Radiology 2006;7(1):70-72
- CountryRepublic of Korea
- Language:English
- Abstract: 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.
