J Korean Neurosurg Soc.  1977 Jun;6(1):163-168.

A Case of Pulsating Exophthalmos due to Defective Development of the Sphenoid Bone with Plexiform Neurofibroma

Affiliations
  • 1Department of Neurosurgery, Yonsei University, College of Medicine, Seoul, Korea.

Abstract

A case of unilateral pulsating exophthalmos due to defective development of the sphenoid bone with plexiform neurofibroma was reported. There were missing of orbital plate of the fronral bone, lesser and great wing of the sphenoid, and lacrimal plate of the ethmoid bone. Middle fossa became deepening accompaning with thining of temporal bone, and the tip of the temporal lobe was covered by tremendously dilated subarachnoid spaces with thick membranes. There were diffuse neurofibromatosis and plexiform neurofibroma resulted in elephantiasis of the lids. The skull bone defect was covered with Resin after removal of the plexiform neurofibroma. The patient was improved.


MeSH Terms

Elephantiasis
Ethmoid Bone
Exophthalmos*
Humans
Membranes
Neurofibroma, Plexiform*
Neurofibromatoses
Orbit
Skull
Sphenoid Bone*
Subarachnoid Space
Temporal Bone
Temporal Lobe
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