Craniofacial fibrous dysplasia

Case contributed by Dr Shadi Shalabi

Presentation

Previous history of head trauma following a road traffic accident 20 years earlier, when he underwent craniotomy for skull and orbital fracture repair. Recently, he has been complaining of headache and epiphora.

Patient Data

Age: 34 years
Gender: Male

Diffuse base of skull and facial bone thickening with ground glass appearance involving the frontal, sphenoid and ethmoid bones, causing local compression, and obstruction to sinuses, sella turcica and lacrimal sac. 

Detachment of a piece of the left frontal bone, which is also thickened, which is due to previous surgery following a head trauma 20 years earlier.

Case Discussion

The patient of this case visited the otolaryngology clinic with complaints of headache and epiphora (excessive eye watering). He was aware of the facial deformity, which to him was attributed to the traffic accident he was involved in at age 14.

The patient underwent surgical repair of a fracture in his left frontal bone and orbit. As a sequelae, he had facial deformity and proptosis.

It is not clear to us nor to the patient whether imaging at that time showed that he already had changes of craniofacial fibrous dysplasia or whether they were triggered by the trauma and healing process.

The fact that the patient had no symptoms for many years suggests the lesions have not evolved much since the initial insult, which goes with the diagnosis of fibrous dysplasia is known for not progressing much after puberty.    

The patient is scheduled for functional endoscopic sinus surgery (FESS) to relieve the sinus obstruction and also and endoscopic dacryocystorhinostomy (DCR) for epiphora.  

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