Craniofacial fibrous dysplasia

Case contributed by Dr Mostafa El-Feky

Presentation

Long standing craniofacial deformity and right proptosis.

Patient Data

Age: 30 years
Gender: Male
MRI

MRI of the brain

All the involved bones show different degrees of expansion, heterogeneous sclerosis and cortical interruptions. The following bones are involved:

  •  left hemimandible, from just beyond the symphysis to the condylar process
  • right maxilla, involving the alveolar process, obliterating and expanding the whole maxillary antrum, and elevating the right orbital floor
  • left middle and inferior turbinates
  • both sphenoid bones, more on the right, involving the pterygoid processes, greater and lesser wings
  • squamous and petrous parts of the right temporal bones
  • both frontal bones
  • clivus

Previous surgical resections with a deficient sphenoid roof, as well as plate reconstruction at the junction of roof and lateral walls of the right orbit. Wide right frontal craniotomy.  Left frontal encephalomalacia. A meningoencephalocele is noted at the junction of roof and left lateral wall of the sphenoid sinus, harboring gliotic gyri representing the site of an osteo-dural defect with underlying the CSF leak. 

CT

Postoperative 3D CT facial bones

Postoperative follow up CT shows the craniofacial features of fibrous dysplasia and reduction of the right proptosis.

Case Discussion

Craniofacial fibrous dysplasia is a benign developmental tumor-like bone lesion. It is one of four types of fibrous dysplasia and is characterized by involvement of the skull and facial bones. 

It is a chronic, slowly progressive process that produces craniofacial deformities and complications that usually arise from compression upon skull foramina and their neurovascular contents, and other intracranial structures. Most common manifestations are neurological symptoms such as hearing loss, visual loss, headache, and proptosis.

The suggestive features are the facial deformity, bony expansion with a heterogeneous appearance of hypointensity at T2WI that is due to the fibro-osseous ground-glass matrix. It typically shows heterogeneous contrast enhancement.

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Case information

rID: 42871
Published: 26th Feb 2016
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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