Sphenoid wing dysplasia

Sphenoid wing dysplasia is a characteristic but not pathognomonic feature of neurofibromatosis type 1 (NF1), it can also occur in isolated cases.

Sphenoid wing dysplasia is seen in 5-10% of cases of NF1 and is one of the diagnostic criteria of NF1 5,6.

Its exact aetiology is not clear. It can be seen isolated or associated with underlying plexiform neurofibroma.

  • hypoplastic/absent sphenoid wing resulting in widening of the superior orbital fissure, elevation of the lesser sphenoid wing and ipsilateral orbital enlargement 3
  • gapping bony defect in the posterior aspect of the orbit 4
  • 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 2
  • expansion and anteroposterior enlargement of the middle cranial fossa, usually associated with anterior temporal arachnoid cysts 1
  • 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 2
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Article Information

rID: 30095
System: Head & Neck
Section: Pathology
Synonyms or Alternate Spellings:
  • Dysplasia of the sphenoid wing

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Cases and Figures

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    Case 1
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    Case 1: 3D image with gapping bony defect
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    Case 2: with NF1
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    Case 3: with bare orbit sign
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    Plexiform neurofi...
    Case 4: with plexiform neurofibroma
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    Case 5
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