A Challenging Surgical Management with Mandible Reshaping of Fibrous

Dysplasia in Adolescent: Case Report

Pebrian Diki Prestya1*, Asri Arumsari2, Indra Hadikrishna3, Sri Suryanti4

1
*Oral and Maxillofacial Sugery Resident, Oral and Maxillofacial Department Faculty of Dentistry
Padjadjaran University and Hasan Sadikin General Hospital, Bandung, Indonesia
2
Oral and Maxillofacial Department Faculty of Dentistry Padjadjaran University and Hasan Sadikin
General Hospital Bandung, Indonesia.
3
Oral and Maxillofacial Department Faculty of Dentistry Padjadjaran University, Bandung, Indonesia
4
Anatomical Pathology Hasan Sadikin General Hospital Bandung, Indonesia.

*Korespondensi: pebrian21001@mail.unpad.ac.id

ABSTRACT
Introduction: Fibrous dysplasia is a benign developmental that affects the craniofacial
bones among mandibular bone, characterized by enlargement because of bone substituted
by abnormal fibrous tissue growth. Two types are monostotic or polyostotic lesions cause a
progressively expanding bone lamp producing cosmetic deformities and functional
impairments. Frequently occurs in the posterior region of the mandible bone and usually
unilateral. Aetiology of Fibrous dysplasia is not clear but usually associated with genetic
factor. Diagnosis of this deformity based on clinical, radiological, and histopathological
examination. Case Report: A 16 years-old-male patient came to Oral and maxillofacial
department Hasan Sadikin General Hospital, Bandung complaining the lump in his left lower
jaw. Patient complained a lump enlarged in the last 7 years ago and caused facial
asymmetry. Extraoral examination showed facial asymmetry of the mandibular bone,
intraoral examination finds a hard consistency of lump, elevated the vestibule and no
ulceration. Computed tomography (CT) imaging showed a solid mass in the left mandibular
area, with endosteal scalloping. We planned to remove the hyperplastic bone of the
mandible with surgical reshaping. Conclusion: Treatment of this case was challenging
because the normal shape of mandible bone anatomically was hard to achieved. In
adolescent, facial asymmetry could occur after surgery because still in the growth stage. In
this case, we were able to obtain a good prognosis for the patient by removing hyperplastic
bone and reconstructing facial deformities

Keywords: Fibrous dysplasia, hyperplastic bone, reshaping

INTRODUCTION
A fibro-osseous bone lesion called fibrous dysplasia is characterized by the

replacement of healthy bone with fibrous tissue and immature, poorly formed woven

bone, the trabeculae are also irregular. The diagnosis of fibrous dysplasia is a

challenge for clinicians because it requires various clinical, radiographic, and

histopathological examinations. Fibrous dysplasia is a non-neoplastic growth bone

disorder, characterized by the replacement of normal bone and bone marrow by

fibrous tissue in a part of the body. Fibrous dysplasia only accounts for roughly 2.5%

of bone tumor instances overall, whereas benign bone tumors make for about 7% of

all cases. Fibrous dysplasia can involve the craniofacial bones including the

mandibular bones. The distinction between monostotic and polyostotic types of

fibrous dysplasia in this instance is based on the number of affected bones.

Fibrous dysplasia is often related to genetic factors. Osteoblasts,

melanocytes, and endocrine cells produce progeny to mutate all cells that will carry

and accelerate the incidence of gene mutations when mutations take place in

undifferentiated stem cells during the embryological phase. The results show clinical

findings in the form of multiple bone lesions, skin pigmentation, and endocrine

disorders. Later stages of embryonic development see migration and differentiation

of skeletal progenitor cells as part of the normal skeletal formation process. If the

mutation develops at later stage, the cells that result from it disseminate and

contribute to the creation of fibrous dysplastic bone lesions. Eventually the mutation

occurs postnatally, then the progeny of these cell mutations is essentially confined to

a single anatomical region, resulting in fibrous dysplasia of the monostotic type. 2,3
 The monostotic type, which has typical clinical symptoms, is the one that

affects craniofacial the most. The most typical clinical symptom of fibrous dysplasia

is a slow, painless enlargement of the involved bone in the craniofacial region of a

single bone. The slowly expanding lesions may result in painless bone enlargement,

leaving just an asymmetrical face appearance. Most fibrous dysplastic lesions are

monostotic, meaning they only affect one bone. Approximately 80–85% of all cases

of fibrous dysplasia are of the monostotic type.

Small and well-localized lesions can be curettage, enucleation, or excision to

restore the anatomical shape without leaving post-surgical defects. If the lesion is

large and the patient has malocclusion and jaw disproportion, then surgery is needed

with reshaping technique using an intraoral approach to correct facial deformities. In

this case report, it was determined that a fibrous dysplasia lesion existed, so it was

decided to reshape the mandibular bone to prevent extraoral sutures from interfering

the aesthetics and to restore facial asymmetry.

Clinically, fibrous dysplasia causes an asymmetrical face as a result of the

enlargement of the fibro-osseous tissue-covered bones, this condition is typically

identified in children and adolescents. This case report demonstrates definitive

surgical treatment for fibrous dysplasia in a teenage patient using the mandibular

reshaping technique with an intraoral approach. Due to aesthetic concerns, it is very

challenging to return the mandible to its normal anatomical shape through surgery

because the lesion boundaries are hard to determine and there is a possibility that

the face will once again become asymmetric because the patient is still in the growth

and development stage.

CASE REPORT
A 16-year-old male patient visited the Hasan Sadikin Hospital's Department of

Oral and Maxillofacial Surgery in Bandung with complained of a lump on his left
lower jaw. The patient has reported a painless marble-sized lump in the lower left

jaw for the past 7 years. The patient feels the tumor is growing, which is why her

face is starting to seem asymmetrical. The patient had no significant medical and

dental history. Extraoral examination revealed a localized and expansive hard mass

on the left mandible with dimensions of 10 x 5 x 2.5 cm in size that causes an

asymmetrical face, this mass was not palpable and did not cause any discomfort

when it was palpated in the lymph nodes. Intraoral examination revealed bony

enlargement from the midline to the angle of the mandible and inferior to the gingival

margin of the mandible. The enlargement of the hard bone tissue causes the left

vestibule's depth to decrease, resulting in a shallow vestibule. The patient was taken

to a biopsy examination, The results provided a histological image in which the bone

lamellae were within normal limits, no osteoblastic rimming was observed,

hyperplastic fibrocystic cells were observed to condense with nuclei within normal

limits, and no symptoms of malignancy were seen.

Picture 1: Pre-operative profile photo, there is left mandible asymmetry

 Picture 2. There is an elevated vestibule in the left mandibular intraorally.

Afterwards, a panoramic radiograph was taken and obtained a ground-glass

appearance. A CT scan of the patient's head was performed, and the results

revealed a solid mass with endosteal scalloping but no swollen lymph nodes. All

clinical, radiographic, and histological examinations led to the diagnosis of fibrous

dysplasia

Picture 3: According to the CT scan, the left mandible has a solid mass that runs from the midline to the left mandibular angle
and has endosteal scalloping, a sign of fibrous dysplasia.
 Picturer 4: Panoramic ground-glass appearance on the left mandible

Picturer 5: The nucleus is within normal limits, the lamellar bone is within normal limits, there is no osteoblastic rimming, the
hyperplastic fibrocytic cells are seen to condense, and there are no symptoms of malignancy. This is the histological image of
fibrous dysplasia.

Following a few examinations, it was determined to reshape the hyperplastic

mandibular bone under general anesthesia. Using an intraoral blade #15, a

trapezoidal incision was made from the midline mandible through the gingival margin

to the retromolars. A reddish-white bone with the dimensions 10 x 5 x 2.5 cm was

discovered after being dissected with a rasparatorium. The bone was divided into

several parts using a chisel and mallet and reshaping with a frasser bur until the

mandibular bone reached a normal and symmetrical anatomical shape when

compared to the right side. To minimize the risk of nerve damage during surgery,

such as paresthesia or neurovascular haemorrhage, the mental nerve must be

preserved. No problems were discovered both during the procedure and afterward.

Following that, the bone's rough surface was refined with a round Frasser bur and a
bone file, palpation is performed to look for any sharp bone edges. Then 0.9% NaCl

was used for irrigation, the flap was returned to its original position and the incision

site was sutured with 4-0 polyglactin suture. There was no substantial bleeding both

during the procedure and after.

Picture 6: methods were used intraoperatively to reshape the mandible to its natural anatomical shape.

Picture 7: the profile image of the patient When compared to the left side, 2 months after surgery, the front, left, and right sides
exhibit no visible lumps, and there are no indications of new tissue growth.

After seven days following surgery, the patient returned for follow-up care.

The patient had complained of intermittent paraesthesia, but they had no significant

impact on the patient's activities, and a clinical examination revealed good suture

healing and good oral hygiene. Histological analysis of the surgical specimen

revealed fibrous dysplasia and no evidence of malignancy. The wound was

completely closed when the stitches were removed on the seventh day, showing no

signs of inflammation. After that, the patient was ordered to return in 2 months to

evaluate the development of the fibrous dysplasia but there was no postoperative

tissue growth based on a clinical examination. The patients are advised to undergo
control procedures every six months to monitor the progress of the lesion until the

patient's growth and development process is complete.

DISCUSSION
Fibrous dysplasia is divided into monostotic and polyostotic types. Monostotic

fibrous dysplasia is the most prevalent type, accounting for 80–85% of all diagnosed

cases and only affecting one bone, whereas polyostotic fibrous dysplasia affects

multiple bones and occurs much less frequently (20–30% of all cases), is more

prevalent in children and women. In addition, there is a type of McCune-Albright

Syndrome which is a polyostotic type of fibrous dysplasia with pigmentation of the

skin (cafe-au-lait spots) and endocrine disorders. Fibrous dysplasia can be related to

genetics and hormones. With or without endocrine disorders and cafe-au-lait spots,

fibrous dysplasia can occur. Diagnosis is based on history, clinical examination,

radiology, and histology. Numerous conditions, including simple bone cysts, non-

ossifying fibromas, osteofibrous dysplasia or ossifying fibromas, adamantinomas,

low grade intramedullary osteosarcomas, and Paget disease, are included in the

differential diagnosis of fibrous dysplasia. In this case, fibrous dysplasia was

identified to be the monostotic type, in which the CT scan revealed thickening of the

fibro-osseous bone only in the left mandible bone.

In this case, a single incisional biopsy was performed to confirm the diagnosis

and rule out the chance of malignancy. Histologically, the picture shows lamellar

bone within normal limits, no osteoblastic rimming, condensed hyperplastic

fibrocystic cells with nuclei within normal limits, and no evidence of malignancy,

leading to the conclusion that fibrous dysplasia is present. According to Karia et al,

histological examination is the gold standard for diagnosing fibrous dysplasia when a

fibro-osseous lesion with blurry margins is present and can be seen on

radiographs. 6,9,10
In pediatric and adolescent patients, a panoramic examination
reveals a ground-glass appearance; as patients become older, the lesion becomes

more heterogeneous and loses radiolucency. In addition, a solid mass with

endosteal scalloping is shown on the CT-scan image.

The aim of the surgery in this case is to restore the patient's facial aesthetics

to normal, so a technique that doesn't compromise facial aesthetics is required.

Additionally, hyperplastic bone in the mandible must be removed to restore the jaw's

natural anatomical shape. Additionally, because the lesion's boundaries are

3

uncertain in this case, it is very challenging to identify them and completely remove

the lesion. As a result, surgical treatment for fibrous dysplasia in this case is

considered adequate when a good anatomical shape is obtained and the remaining

bone tissue does not appear to be woven bone. 4,6,11

Because the patient's growth

period has not ended yet and there is a possibility that there may still be residual

fibrous dysplasia, it is necessary to conduct periodic evaluations every six months to

monitor the growth of the woven radiographically. The patient is also advised to have

an alkaline phosphatase test to check for the possibility of malignancy. In addition,

because the intraoral technique was adopted in this case, the patient was satisfied

with the surgical outcomes from both a functional and aesthetic aspect. 6–8,12

Although it is reported that transformation into malignancy is about 0.4% of

patients who are followed-up without radiotherapy, it must be considered towards

malignancy if there are symptoms of pain around the lump, rapid growth, or other

signs of malignancy. Fibrous dysplasia is not a malignant lesion, so radiotherapy is

contraindicated for the treatment of fibrous dysplasia because it will cause the

transformation of fibrous dysplasia into sarcoma. 3,5,13

Transformation into malignancy

must be a concern; in some situations, osteosarcoma, fibrosarcoma, and

chondrosarcoma can develop. Although there were no indications of a malignant

7
change in this case, the patient was nonetheless followed up on a regular basis to

see how the reconfigured lesion was developing. Alkaline phosphatase examination

is also recommended for patients as a marker for the potential change into

malignancy, as there will be a rise in the level of phosphatase. 9,12,14

CONCLUSION

After recontour the mandible's hyperplastic bone from the midline to the angulus, this

case has a good prognosis. In fact, fibrous dysplasia is commonly associated with

disorders other than the craniofacial bones, necessitating a comprehensive

assessment of the patient. Treatment for fibrous dysplasia must take normal

anatomy and aesthetic function into consideration. It should be noted that age

considerations need to be considered because the mandibular bone is still in the

developmental stage and may occur asymmetrically, necessitating periodic control.

In addition, comprehensive treatment is required to get the best shape of the

mandibular bone after reshaping. fibro-osseous bone may regenerate and develop

into a malignancy, however this is uncommon. For this reason, radiography and

alkaline phosphatase examination are indicated for periodically examination.

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