A Successful Surgical Management of Paediatric Ossifying Fibroma in

Peripheral Limited Setting: A Rare Case Report

Nur Setiyo NOV., Chandra S., Indrasari SR.

Department of Otorhinolaryngology-Head and Neck Surgery
Faculty of Medicine Universitas Gadjah Mada/Dr. Sardjito Hospital
Yogyakarta

ABSTRACT

Background: Ossifying fibroma (OF) is a rare non-malignant fibro-osseous lesion of the craniofacial
area with the characteristic of slow-growing and locally aggressive. The incidence rate in paediatric
patients is still not clear. The management is varied from observation and serial radiologic imaging to
radical resection of the tumour. Purpose: To provide knowledge and information about diagnosing
and managing a rare case of craniofacial ossifying fibroma. Case: A-5-year-old boy diagnosed with
orbito-inferonasal region ossifying fibroma underwent extirpation of the mass with the lateral
rhinotomy - Webber Ferguson approach, anterior orbitotomy by the ophthalmologist, and obturator
placement by the prosthodontist. Method: A literature search was done on PubMed, The Cochrane
Library, ScienceDirect and Google Scholar using keywords ‘ossifying fibroma’ AND ‘craniofacial’ AND
‘surgery’. Result: Ten articles were found from 4 search engines in the last ten years. Based on
inclusion and exclusion criteria, there were two articles relevant to the scope of our case. Conclusion:
Definitive excision is the treatment of choice for OF. Successful surgical outcome was due to a
multidisciplinary team and surgical incision technique. Vision and facial nerve function were preserved
as well as good facial symmetry, aesthetics, and recurrence prevention. Regular follow-up is necessary
for young OF patients.

Keywords: ossifying fibroma, craniofacial, surgery

INTRODUCTION Patients with ossifying fibroma are often

asymptomatic. Most cases are found
Ossifying fibroma (OF) is a rare non-malignant
incidentally when the patient is subjected to
fibro-osseous lesion of the craniofacial area
imaging examination for other indications.
with slow-growing and locally aggressive
When a patient complains of mass effect
characteristics. OF is made of fibrous cellular
manifestation, swelling of the face is the most
stroma with foci of mineralization or
common sign found.1 Symptoms are often due
ossification, which replaces the normal bone. It
to local tumour expansion. Visual complaints
is more common in the mandible followed by
include proptosis, eyeball displacement,
the maxilla and rarely in the paranasal sinuses,
lagophthalmos, restricted eye movement, and
orbits and skull bones. The aetiology of OF is
decreased visual acuity. Lesion invasion to the
still unclear. However, it is known that
sinus shows symptoms of recurrent sinusitis or
developmental abnormalities of odontogenic
nasal obstruction.3
or traumatic origin are predicted to be the
underlying causes.1 OF is classified based on Imaging examination is required to discover
histopathologic examination, including tumour location, extension, and involvement
juvenile psammomatous, juvenile trabecular of nearby structures. Computed tomography
and cemento-ossifying fibromas.2 (CT) scan is the preferred imaging, with OF
manifesting as radiopaque uninoculated or
multiloculated lesion with well-defined
corticated borders, without soft tissue
involvement. We can find isointense
calcification rim on T1-weighted images and
hypointense on T2- weighted images on MRI
examination. MRI with contrast will show bony
shell enhancement, suggestive of tumour
tissue rather than reactive hyperostosis.3

A multidisciplinary team is needed for

managing the patient. Conservative
management or watchful waiting is less Figure 2: CT scan showed a 5.1x5.5x5.0 cm iso-
favourable in young adults because the hypodensity lesion
probability of a slow-growing tumour risks Computed Tomography (CT) result showed a
compressive symptoms.2 Total excision of the 5.1x5.5x5.0cm iso-hypodensity lesion in right
lesion tends to be the definitive management ethmoidal bone with regular, well-defined
of craniofacial ossifying fibroma. The surgical margin, ground-glass opacity expanding to left
approach to tumour resection is determined ethmoidal sinus, right maxillary sinus, bilateral
by anatomical location and tumour size. sphenoidal sinus, right extraconal orbital,
Radiation therapy has not been recommended which caused proptosis of the right eye.
in the direction of ossifying fibroma.1

CASE REPORT

A 5-year-old boy was referred to our hospital

in June 2021 with a lesion in the right eye,
which caused incomplete right eye closure.
a
The lesion was progressively enlarging in the
past year. He also complained of a lesion on
the right nose with nasal obstruction and a
non-odorous clear secret. He had no complaint
of vision changes and hearing impairment.
History of nosebleed and decrease of appetite b
was denied. History of malignancy in the family Figure 3: Histopathological examination
was also denied. Biopsy was done in Sardjito result 100x magnification (a) and 400x
General Hospital in October 2020. We found a magnification (b)
fixed mass with hard consistency on the right
nose anterior rhinoscopy from palpation.
Histopathological examination revealed an
immature bone fragment (woven bone)
without osteoblastic rimming with fibroblast
proliferation. The tumour cell was relatively
monomorphic with a round, oval, spindle fine
chromatin nucleus and no sign of malignancy.
EMA, B catenin, and S100 were negative,
Vimentin and Ki67 was positive.
Figure 1: Clinical presentation of the patient
Based on the light of the current clinical, on the craniofacial, 3) Surgical management,
radiological, and histopathological findings, and 4) Full text availability. The exclusion
the patient was diagnosed with orbito- criteria was endoscopic management. A critical
inferonasal region tumour (ossifying fibroma). analysis was done on selected journals.
The patient management plan involved an
extirpation of the mass with the lateral
rhinotomy - Webber Ferguson approach,
anterior orbitotomy by the ophthalmologist,
and obturator placement by the
prosthodontist.

The procedure was performed with lidocaine

infiltration at the incision site. Lateral
rhinotomy Weber Ferguson incision was done
layer by layer until the anterior maxilla bone
was completely visualized. The
ophthalmologist did anterior orbitotomy to Figure 4: Complete surgical excision of
expand the operation area up to orbit. Anterior the specimen
maxilla bone was drilled starting from the
lateral border of piriformis aperture up to the
lateral border of anterior maxilla sinus, inferior
border of piriformis aperture as triangle form,
until anterior maxilla bone wall can be
removed. Palpation and complete extirpation
of the mass were done. Bleeding control was
done using tampons and cauterization. Hernial
mesh was placed behind the right oculi for eye
support to prevent enophthalmos and Figure 5: Hernial mesh and radiologic film
radiologic film to separate orbit space from were placed behind the right oculi space
nasal and maxilla spaces to ensure that the
right and left eye positions are symmetrical.
Then sutured was done layer by layer neatly.
Histopathology examination was performed to
determine the definitive diagnosis, and it was
confirmed as an ossifying fibroma.
Postoperative complications such as epistaxis,
blocked nose, headache or aesthetic problems
of the nose were not found.

METHOD

A literature search was done on PubMed, The

Cochrane Library, ScienceDirect and Google
Scholar using keywords ‘ossifying fibroma’
AND ‘craniofacial’ AND ‘surgery’. We found
eight articles on PubMed, two on
ScienceDirect, and 0 on The Cochrane Library Figure 6: Postoperative clinical photograph
and Google Scholar. Inclusion criteria are 1)
Case report of ossifying fibroma, 2). Location
Literature Result Keywords
PubMed 8 ‘ossifying fibroma’ AND
Science Direct 2 ‘craniofacial’ AND ‘surgery’
Cochrane 0
Google Scholar 0
Table 1: Search strategy

Figure 7: Literature search flow

RESULT reconstruction. Weber-Ferguson incision with
left sub-ciliary extension and lynch extension
From four search engines, we found ten
was done to access the tumour. A 4-year
articles published in the last ten years. Based
follow-up showed no recurrence of JOF. The
on inclusion and exclusion criterias, there were
second case presented a 15-year-old girl with
two articles relevant to the scope of our case.
progressively enlarging right maxillary mass for
Turin4 reported a case of an 11-year-old girl
six years. At 10, she had previous partial
with left maxilla juvenile psamommatoid
resection and tumour debulking through the
ossifying fibroma (JPOF). JPOF commonly
lateral rhinotomy approach. JOF recurred in
presents in the paranasal sinuses (70%),
the right maxillary sinus, causing laterally
maxilla (20%), and mandible (10%). Two years
orbital displacement with diplopia on upward
prior, a CT scan examination revealed a large
and downward gaze. MRI revealed an
expansile osseous lesion involving the left
extended tumour to the frontal sinus and
maxilla with predominantly hyperdense
anterior cranial fossa to the sphenoid sinus.
ground-glass appearing component, with
Reconstruction and osteotomies were
associated dense bone and cystic change
performed via the trans-nasal and right lateral
without periosteal reaction or significant soft
rhinotomy approach. There was no sign of
tissue component, which was suggestive for
recurrence at four-year follow-ups.
monostotic fibrous dysplasia. At the 2-year
follow-up visit, the lesion showed extreme DISCUSSION
growth with a complaint of mass effect which
Ossifying fibroma (OF) is a rare non-malignant
caused the patient to not be able to see below
fibro-osseous lesion of the craniofacial area
the lesion with an extreme downward gaze of
with a characteristic of slow-growing and
the left eye. The goal management was a
locally aggressive.1 WHO (2017) classifies OF as
surgical intervention to debulk the lesion for
odontogenic origin (cemento OF) and juvenile
aesthetic and visual field concerns. Operative
ossifying fibroma (JOF), which is further
access was obtained through a Weber-
divided into Trabecular Juvenile Ossifying
Ferguson incision. A glossy, yellow, gelatinous
fibroma (TrJOF) and Psammomatoid Juvenile
consistency lesion covered by a thin cortex of
Ossifying fibroma (PsJOF). Based on several
bone was found and predicted to be immature
studies, the most common prevalence is
active FD. However, histopathological
cemento OF (71.4%), followed by JOF (25.4%).
examination confirmed fibro-osseous lesion
For the JOF subtype, the prevalence of TrJOF
with cellular fibroblastic stroma and uniformly
(10.3%) was higher than that of PsJOF (8.9%).6
distributed ossicles appropriate for JPOF. A 4-
month follow-up after the definitive excision, Generally, OF affects patients in the second to
good improvement was visible in facial contour third decades of age. The incidence rate in
accompanied by a full range of visual gaze and paediatric patients is still unclear; however,
no obstruction of the visual field. about 10% of cases are reported in patients
under 18 years of age.7 Several studies
Hachach-Haram, et al.,5 presented 2 cases of
reported OF is more common in women than
large juvenile orbital OF. The first case was an
men, 55.4% vs 44.6%. The dominant age group
8-year-old boy with recurrent left maxillary
is under 18 years (51.6%), and over 18 years is
antrum and left frontal sinus JOF. He came with
40.8%.6
progressively enlarging left maxillary mass with
diplopia on the upward and downward gaze of Of all craniofacial bones, OF is more common
the left eye. He had two previous debulking in the mandible, followed by the maxilla. It has
surgeries and developed a recurrence. MRI been reported to rarely occur in the ethmoid
revealed high signal mass and planned for sinus, orbit, and skull base.1 Yet, when it affects
tumour excision and left midface paranasal sinus, OF is most common in the
ethmoid sinus and nasal cavity, followed by most recurrence occurring in conservative
sphenoid, maxillary, and frontal sinuses, as we surgery. The recurrence rate after conservative
found in the patient.7 surgery (19.7%) was higher compared to
radical surgery (10.6%).6 It is hypothesized that
OF tends to be asymptomatic. Symptoms are
the infiltrative nature of the tumour leads to
often due to local tumour expansion to
incomplete excision and cause a recurrence.4
surrounding tissues or organs because of the
Therefore, total excision of the lesion is
tumour locally aggressive behaviour, such as
considered the definitive management of
facial asymmetry, pain, and local
5,8
craniofacial ossifying fibroma.4,10 In our case,
destruction. Visual complaints include
the patient management involved an
proptosis, eyeball displacement,
extirpation of the mass with the lateral
lagophthalmos, restricted eye movement, and
rhinotomy - Webber Ferguson approach,
decreased visual acuity. Lesion invasion to the
anterior orbitotomy by the ophthalmologist,
sinus shows symptoms of recurrent sinusitis or
and obturator placement by the
nasal obstruction.3 In this case, the patient
prosthodontist. Hernial mesh was placed
complained of a lesion in the right eye, which
behind the right oculi for eye support to
caused uncomplete right eye closure. The
prevent enophthalmos and radiologic film to
lesion was progressively enlarging in the past
separate orbit space from nasal and maxilla
year. He also complained of a lesion on the
spaces to ensure that the right and left eyes’
right nose with nasal obstruction and a non-
positions are symmetrical. Postoperative
odorous clear secret. He had no vision changes,
complications such as epistaxis, blocked nose,
hearing impairment, or history of nosebleeds.
headache or aesthetic problems of the nose
CT scan is the preferred imaging, presented as were not found.
a unilocular or multilocular radiolucency,
The surgical approach to tumour resection is
radiolucent-radiopaque, or radiopaque lesion.9
determined by anatomical location and
The pathognomonic sign of OF is a well-defined
tumour size.10 Surgical management with
margin (62.9%), followed by an ill-defined
lateral rhinotomy, bilateral craniotomy, trans-
margin (12.2%) and a moderate-defined
facial, craniofacial or trans-maxillary
margin (0.9%).6 CT scan result in this patient
approaches has been applied to paranasal
showed a 5.1x5.5x5.0cm iso-hypodensity
sinuses and skull base OF resection. However,
lesion in the right ethmoidal bone with regular,
this approach has drawbacks, namely the
well-defined margin, ground-glass opacity,
morbidity in the form of facial scars.10
which expanding to left ethmoidal sinus, right
Management of blood loss during surgery is
maxillary sinus, bilateral sphenoidal sinus, right
very important in paediatric patients because
extraconal orbital, which causes proptosis of
massive bleeding can cause several
the right eye.
perioperative complications. Preoperative
Based on OF aggressiveness, the management embolization to reduce bleeding is an effective
is varied from observation and serial radiologic way for haemorrhagic neoplasms, including
imaging, which is preferable for asymptomatic hemangiomas and angiofibroma.7
patients, to radical resection of the tumour.10
We conclude that multidisciplinary teamwork
Some studies reported that enucleation or
is crucial for the resection of these difficult
curettage is the initial treatment of choice for
tumours. Complete surgical excision is
OF. However, many studies showed that
beneficial, accompanied by long-term follow-
radical surgery, including local, marginal, and
up to monitor function and craniofacial
total resection, is superior to conservative
skeleton development.5 There is a possibility of
surgery.8 It is supported by data that the
tumour growth in young patients during the
recurrence rate after surgery was 14.4%, with
follow-up period. Growth hormone plays a role
 in tumour growth. Excess growth hormone although its benign characteristic. Definitive
becomes a risk factor for the occurrence of excision is the treatment of choice for OF.
compressive optic neuropathy. Therefore, Successful surgical outcome was due to a
careful and regular follow ups are necessary in multidisciplinary team and surgical incision
young OF patients. The surgeon must be aware technique. We found that lateral rhinotomy
of tumour regrowth, complications, or Weber Ferguson incision showed excellent
malignancy transformation in teenage exposure. Vision and facial nerve function
patients.11 were preserved as well as good facial
symmetry and aesthetics and recurrence
CONCLUSION
prevention. Regular follow-up is necessary for
OF remains a rare disease, particularly in young OF patients.
paediatric patients, which is locally aggressive

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