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, abnorm...

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Autores principales: Onbas, Omer, Aliagaoglu, Cihangir, Calikoglu, Cagatay, Kantarci, Mecit, Atasoy, Mustafa, Alper, Fatih
Formato: Texto
Lenguaje:English
Publicado: The Korean Radiological Society 2006
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2667581/
https://www.ncbi.nlm.nih.gov/pubmed/16549958
http://dx.doi.org/10.3348/kjr.2006.7.1.70
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author Onbas, Omer
Aliagaoglu, Cihangir
Calikoglu, Cagatay
Kantarci, Mecit
Atasoy, Mustafa
Alper, Fatih
author_facet Onbas, Omer
Aliagaoglu, Cihangir
Calikoglu, Cagatay
Kantarci, Mecit
Atasoy, Mustafa
Alper, Fatih
author_sort Onbas, Omer
collection PubMed
description 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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spelling pubmed-26675812009-04-22 Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography Onbas, Omer Aliagaoglu, Cihangir Calikoglu, Cagatay Kantarci, Mecit Atasoy, Mustafa Alper, Fatih Korean J Radiol Case Report 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. The Korean Radiological Society 2006 2006-03-31 /pmc/articles/PMC2667581/ /pubmed/16549958 http://dx.doi.org/10.3348/kjr.2006.7.1.70 Text en Copyright © 2006 The Korean Radiological Society http://creativecommons.org/licenses/by-nc/3.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Onbas, Omer
Aliagaoglu, Cihangir
Calikoglu, Cagatay
Kantarci, Mecit
Atasoy, Mustafa
Alper, Fatih
Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography
title Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography
title_full Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography
title_fullStr Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography
title_full_unstemmed Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography
title_short Absence of a Sphenoid Wing in Neurofibromatosis Type 1 Disease: Imaging with Multidetector Computed Tomography
title_sort absence of a sphenoid wing in neurofibromatosis type 1 disease: imaging with multidetector computed tomography
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2667581/
https://www.ncbi.nlm.nih.gov/pubmed/16549958
http://dx.doi.org/10.3348/kjr.2006.7.1.70
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