sphenoid wing dysplasia
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This image
is part of a series which can be scrolled interactively with the mousewheel or mouse dragging. This is done by using Template:Imagestack. The series is found in the category Neurofibromatosis type I - orbital dysplasia - MRI - case 001. Orbitadysplasie bei Neurofibromatose Typ 1. MRT - T1w koronar mit Kontrastmittel
![](https://pacs.de/sites/default/files/pictures/thumbs/upload.wikimedia.org/4/8/1/5/5/Orbitadysplasie_bei_Neurofibromatose_Typ_1_-_MRT_-_T2_axial_-_009_thumb.jpg)
This image
is part of a series which can be scrolled interactively with the mousewheel or mouse dragging. This is done by using Template:Imagestack. The series is found in the category Neurofibromatosis type I - orbital dysplasia - MRI - case 001. Orbitadysplasie bei Neurofibromatose Typ 1. MRT - T2w axial
![](https://pacs.de/sites/default/files/pictures/thumbs/upload.wikimedia.org/4/8/1/5/6/Orbitadysplasie_bei_Neurofibromatose_Typ_1_-_MRT_-_T2_axial_kleines_FOV_-_010_thumb.jpg)
This image
is part of a series which can be scrolled interactively with the mousewheel or mouse dragging. This is done by using Template:Imagestack. The series is found in the category Neurofibromatosis type I - orbital dysplasia - MRI - case 001. Orbitadysplasie bei Neurofibromatose Typ 1. MRT - T2w axial kleines FOV
Sphenoid wing dysplasia is a characteristic but not pathognomonic feature of neurofibromatosis type 1 (NF1), it can also occur in isolated cases.
Epidemiology
Sphenoid wing dysplasia is seen in 5-10% of cases of NF1 and is one of the diagnostic criteria of NF1 .
Pathology
Its exact etiology is not clear. It can be seen isolated or associated with underlying plexiform neurofibroma.
Radiographic features
- hypoplastic/absent sphenoid wing resulting in widening of the superior orbital fissure, elevation of the lesser sphenoid wing and ipsilateral orbital enlargement
- gapping bony defect in the posterior aspect of the orbit
- absence of the innominate line (which represents the projection of the greater wing of the sphenoid bone) on plain radiograph and CT scan giving the bare orbit sign
- expansion and anteroposterior enlargement of the middle cranial fossa, usually associated with anterior temporal arachnoid cysts
- herniation of the dura, peritemporal subarachnoid space or the temporal lobe into the posterior aspect of the orbit, causing anterior displacement of the orbital contents
See also
Siehe auch:
und weiter:
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