Polyostotic fibrous dysplasia

Case contributed by George Harisis
Diagnosis almost certain

Presentation

Fall with headstrike. No past medical history stated.

Patient Data

Age: 50 years
Gender: Male

CT Brain (bone windows):

Incidental asymmetrical right sided thickening of the frontal bone and extending to involve the floor of the right anterior cranial fossa and right clinoid process.  This demonstrates predominantly internal ground glass density with multiple irregular lucencies interspersed with a few sclerotic foci.  No periosteal reaction or pathological fracture identified.

Cervical spine:

There are multiple, expansile lucent lesions with endosteal scalloping involving the right 2nd and 4th and left 5th ribs, the right aspect of the T1 vertebral body and neural arch and the T2 and T4 vertebral bodies.  

Normal appearance of the perivertebral soft tissues. No acute fracture is identified.  Paraseptal emphysematous changes noted at the imaged lung apices.

Comment:

Incidental finding of asymmetrically thickened right frontal bone along with multiple expansile lucent lesions in several bilateral ribs and vertebral bodies.  These findings are typical of polyostotic fibrous dysplasia.

Case Discussion

Upon further questioning, the patient confirmed a known history of fibrous dysplasia.

Although a benign condition caused by replacement of bone marrow with proliferating fibrous stroma, when symptoms occur, they tend to relate to mass effect and compression of adjacent structures, particularly in the craniofacial form of the disorder1. In this case, the patient was asymptomatic. 

Fibrous dysplasia was first described by the German Pathologist Friedrich Daniel von Recklinghausen, of neurofibromatosis type 1 fame 2.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.