A 47-year-old female was seen in the oral surgery clinics with a complaint of vision and hearing disturbances that had developed over the past few months. The patient had a diagnosis of fibrous dysplasia from several years ago. She was offered surgical trimming of the lesion at the time of diagnosis, which she refused.
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Slight asymmetry in a 47-year-old patient with fibrous dysplasia |
Only slight facial asymmetry was noted during the extra-oral exam. A bony hard bulge was palpable in the left buccal vestibule. It was not prominent enough to be photographed. The patient was requested to return with imaging studies.
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CT scan of a patient with fibrous dysplasia
(Courtesy Dr. Asma Pervez) |
On her CT scan, a radio-opaque mass was noted to obliterate the maxillary sinus and encroach the nasal wall and the orbital floor.
The oral surgeons in the clinic offered the patient hemimaxillectomy. They believed that since the disease process was active, and continued to involve more tissue, it was best to remove it completely. The defect would be filled with a specially designed obturator. This would obviously result in suboptimal esthetics, much worse than what the patient was presenting with. However, the patient's sight would be preserved, and future complications, such as involvement of the base of skull, would be prevented.
As extreme as this sounds, this was the best approach in my opinion. However, I did speak to the surgeons about the possibility of a relatively conservative plan, possibly removing the orbital plate and contouring the lesion involving the maxillary sinus. They said leaving any part of this lesion would be asking for recurrence.
The patient was not happy with this treatment plan. She hasn't returned to schedule surgery.
Fibrous Dysplasia:
Fibrous dysplasia is a developmental condition that results in replacement of normal bone with fibro-osseous tissue. It is linked to a mutation in GNAS 1 (guanine nucleotide-binding protein), which plays an important role in development of bone, endocrine system and skin pigmentation. The severity of bone involvement depends on the time when the mutation takes place, mutations during early embryonic development can cause multiple bone involvement.
Fibrous dysplasia is broadly classified into polyostotic (multiple bone involvement) and monostotic (one bone involvement) types.
Monostotic type is more common. The lesions present as a slow-growing, painless swellings. Patients are usually in their teens at the time of diagnosis. Jaws are a frequent site of involvement. The maxilla is more commonly affected than the mandible. For fibrous dysplasia of the maxilla, the use of the term craniofacial fibrous dysplasia is preferred. It is not uncommon for bones in the vicinity to get involved.
Radiographically, the lesions of fibrous dysplasia are described as having a "ground glass" appearance. The margins of the lesion are ill-defined and merge with the adjacent normal bone. Diagnosis is confirmed by biopsy.
In most cases, fibrous dysplasia stabilizes once the skeletal growth ceases. Our case is an obvious exception. Surgical contouring is done to correct minor asymmetries. Rate of recurrence after the procedure vary between 25-50%. Larger lesions may require surgical resection. Bisphosphonate therapy helps to control polyostotic disease.