CASE 1641 Published on 11.09.2002

Fibrous dysplasia of the skull

Section

Musculoskeletal system

Case Type

Clinical Cases

Authors

J. Pattison

Patient

26 years, male

Categories
No Area of Interest ; Imaging Technique CT
Clinical History
Bony swelling on the head.
Imaging Findings
The patient presented with a slowly enlarging bony swelling on the head, present for many years. There was no pain or neurological symptoms.
Discussion
Fibrous dysplasia, also known as Jaffe-Lichtenstein disease, is a developmental anomaly of the skeleton of unknown cause. It occurs in monostotic and polyostotic forms, seen in 70-80% and 20-30% of cases respectively. Of these 2-3% are associated with endocrinopathies, typically the McCune-Albright syndrome. The skull and facial bones are affected in 10-25% of monostotic cases and 50% of polyostotic cases. The common sites are the frontal, sphenoid, maxillary and ethmoid bones, with the temporal and occipital being less common. The lesions are usually asymptomatic but may present with deformity or neurological complications, such as optic nerve compression.

On plain radiographs there may be single or multiple lesions, which are predominantly sclerotic but may be lucent. When expansion occurs it tends to be outwards with bulging of the outer skull table, which remains intact. Computed tomography is useful to show the extent of bone involvement, as well as the extracortical involvement. The main differential diagnosis is Paget's disease where the features of facial bone involvement and prominence of the external occipital protruberance are less common and the skull tables may be destroyed. Hypervascularity of fibrous dysplasia of the skull is an unusual association which may present with recurrent haemorrhage.

Differential Diagnosis List
Fibrous dysplasia
Final Diagnosis
Fibrous dysplasia
Case information
URL: https://www.eurorad.org/case/1641
DOI: 10.1594/EURORAD/CASE.1641
ISSN: 1563-4086