Unilateral nasal obstruction since childhood which had aggravated for the last one year, Two episodes of epistaxis two weeks apart since 30 days. There were no other associated complaints of nasal discharge, headache, diplopia or facial deformity. Diagnostic Nasal Endoscopy: showed a bony hard mass possibly arising from the inferior turbinate, occupying the entire right nasal cavity with probable attachments to the middle turbinate and extending up to the posterior choana. CT scan PNS: revealed a well-defined bony lesion with sclerotic borders and ground glass appearance; in the right nasal cavity. Extension was seen into the right ethmoid, frontal and right half of sphenoid sinuses. The mass was endoscopically resected by curetting from its attachments from the inferior and middle turbinate in the nasal cavity up to the anterior skull base

1. INTRODUCTION
Fibrous dysplasia is a genetically based developmental anomaly of the bone-forming
mesenchyme. There is a defect in osteoblastic differentiation and maturation that leads to a
replacement of normal bony tissue by fibrous tissue of variable cellularity and immature woven
bone. Fibrous dysplasia represents about 7% of all benign osseous tumors and may affect any
bone of the skeleton. It may present in the following three forms:
1)Monostotic (in a single bone),
2)Polyostotic (in two or more non-contiguous bones), and
3)Disseminated, also known as McCune-Albright syndrome.
The craniofacial bones are more affected in the polyostotic form (50-100%) than in the
monostotic form (20%).
In craniofacial Fibrous Dysplasia, surgery is intended to relieve symptoms such as visual
impairment due to compression of the optic nerve or to correct aesthetic deformities, but not to
remove the entire lesion.
We present a rare case of polyostotic fibrous dysplasia of the nasal cavity involving the right
inferior turbinate, middle turbinate, ethmoid and part of right sphenoid bone.
CASE REPORT
An 18yr old female patient presented with complaints of:
Unilateral nasal obstruction since childhood which had aggravated for the last one year,
Two episodes of epistaxis two weeks apart since 30 days.
There were no other associated complaints of nasal discharge, headache, diplopia or
facial deformity.

2. Her physical and neurological examination was normal.
On Anterior rhinoscopy: a bony hard mass was seen completely obliterating the right nasal
cavity and pushing the nasal septum to the left.
Diagnostic NasalEndoscopy: showed a bony hard mass possibly arising from the inferior
turbinate, occupying the entire right nasal cavity with probable attachments to the middle
turbinate and extending up to the posterior choana. The mass bled on manipulation. DNS was
seen to the left with mild hypertrophy of the inferior turbinate on the left.
CT scanPNS: revealed a well-defined bony lesion with sclerotic borders and ground glass
appearance; in the right nasal cavity causing deviation of nasal septum to the opposite side.
Extension was seen into the right ethmoid, frontal and right half of sphenoid sinuses. Overall
suggestive of fibrous dysplasia (fig. 1).
A screening X-ray PBH was done because Fibrous Dysplasia has a predilection for membranous
bones such as femur or tibia (fig. 3). But no obvious lesions were detected.
SurgicalProcedure: The mass was endoscopically resected by curetting from its
attachments from the inferior and middle turbinate in the nasal cavity up to the anterior skull
base (fig. 4A, B &C). The curetted material was sent for histopathological studies which
confirmed the diagnosis of Fibrous Dysplasia. Histopathology showed irregular islands of woven
bone; a collagen matrix stroma with fibroblasts in an entangled manner with osseous trabeculae
similar to the "Chinese writing"(fig. 2A&B).
DISCUSSION:
Fibrous Dysplasia is a sporadic benign skeletal disorder and accounts for approximately
7.5% of all benign bone neoplasms. Approximately 50% of patients with polyostotic
disease and 20-30% with monostotic disease have craniofacial involvement. In our
patient the lesion had attachments to the inferior turbinate, middle turbinate and the basal
lamella, ethmoid and part of right side of sphenoid bone.
The symptoms of craniofacial fibrous dysplasia depend on the location of the lesion and
the compressive effect on the adjacent structures as the tumor progresses slowly in size.
Patients may present with Facial asymmetry and deformity; pathological fractures;
obstruction of the nasal cavity or paranasal sinuses; anosmia; headache; loss of visual
acquity due to compression of the optic nerve; alteration of the ocular movements;
exophthalmia, squint; or conductive hearing loss.
Asymptomatic Fibrous Dysplasia can be managed by regular monitoring and follow-ups.
Symptomatic nasal cavity masses often require surgical management. Imaging modalities
in the form of CT scans appear to be more accurate in delineating the origin of the lesion
in comparison to nasal endoscopy.
In case of smaller intranasal masses, endoscopic endonasal approach can be opted as was
done in our case.
In larger, inaccessible lesions with broad attachment to the surrounding structures, open
surgical approaches, like

3. Midfacial degloving
Lateral rhinotomy,
Transantral approach and
Le Fort I osteotomy
Regular follow-up is essential at least during the growth spurt period to monitor any recurrence
or malignant sarcomatous changes. In our case patient was reviewed on the 7th, 15th, 30th and 60th
post op days with no complications or any signs of recurrence.
CONCLUSION
Even though the prognosis of Fibrous Dysplasia is good and recurrence rate is low, patients with
Polyostotic Fibrous Dysplasia should be carefully followed up and monitored since 1% of these
may show malignant potential and can lead to devastating complications in the craniofacial
region.
3. 1.

4. 2B
4A
2A

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4B
4C

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