Korean Journal of Radiology

The Korean Society of Radiology (대한영상의학회)
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Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography

  • Onbas, Omer (Ataturk University, School of Medicine, Department of Radiology) ;
  • Aliagaoglu, Cihangir (Erzurum Numune Hospital, Department of Dermatology) ;
  • Calikoglu, Cagatay (Erzurum Numune Hospital, Department of Neurosurgery) ;
  • Kantarci, Mecit (Ataturk University, School of Medicine, Department of Radiology) ;
  • Atasoy, Mustafa (Ataturk University, School of Medicine, Department of Dermatology) ;
  • Alper, Fatih (Ataturk University, School of Medicine, Department of Radiology)
  • Published : 2006.03.31
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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.

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References

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