Korean J Radiol.  2006 Mar;7(1):70-72. 10.3348/kjr.2006.7.1.70.

Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography

• Onbas O
• Aliagaoglu C
• Calikoglu
• Kantarci M
• Atasoy M
• Alper F

• KMID: 753918
• DOI: http://doi.org/10.3348/kjr.2006.7.1.70

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.

Keyword

Neurofibromatosis; Computed tomography

MeSH Terms

*Tomography, X-Ray Computed
Sphenoid Bone/*abnormalities/radiography
Neurofibromatosis 1/*radiography
Male
Imaging, Three-Dimensional
Humans
Adult

Figure

• 

  Fig. 1 Physical examination revealed a normotensive man with cafe-au-lait spots on his chest and abdomen.

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  Fig. 2 A. Anterior volume rendered 3D CT image illustrates details of the bony defect in the right orbital region. B. Thick multiplanar axial reconstructions illustrates the absence of the right sphenoid wing and the intracranial and orbital contents separated only by dura mater. C. Transverse bone window multidetertor CT scan, performed at the level of the orbit, illustrates the absence of the right sphenoid wing, right eye microphthalmos, increased fat tissue in the retrobulbar space and enlarged middle cranial fossa. The left globe is larger than the right.

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