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| CASE REPORT |
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| Year : 2021 | Volume
: 6
| Issue : 2 | Page : 209-212 |
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Monostotic fibrous dysplasia in a 10-year-old patient
Gayatri Nayanar, Mamatha G S. Reddy
Department of Oral Pathology and Microbiology, Dr. D. Y. Patil Vidyapeeth's, Dr. D. Y. Patil Dental College and Hospital, Pune, Maharashtra, India
| Date of Submission | 21-Aug-2020 |
| Date of Decision | 11-Oct-2021 |
| Date of Acceptance | 17-Oct-2021 |
| Date of Web Publication | 11-Aug-2021 |
Correspondence Address:
Dr. Mamatha G S. Reddy
Department of Oral Pathology and Microbiology, Dr. D.Y. Patil Vidyapeeth's, Dr. D. Y. Patil Dental College and Hospital, Pimpri, Pune, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/bjhs.bjhs_81_20
Fibrous dysplasia is a rare congenital bone disorder which manifests as a localized defect in osteoblastic differentiation and maturation with the replacement of normal bone with an abnormal scar-like fibrous connective tissue. It can be classified into monostotic and polyostotic variety with the former seen mostly in the adults and the latter common in children. Craniofacial involvement in case of fibrous dysplasia is very common, affecting maxilla more commonly than the mandible. This report, however, describes a case of fibrous dysplasia in a 10-year-old female patient who presented with a swelling of the left mandible.
Keywords: Fibro-osseous lesions, fibrous dysplasia, monoostotic, woven bone
How to cite this article:
Nayanar G, S. Reddy MG. Monostotic fibrous dysplasia in a 10-year-old patient. BLDE Univ J Health Sci 2021;6:209-12 |
Fibrous dysplasia is a noncancerous skeletal developmental anomaly which is characterized by the substitution of the regular architecture of bone by fibrous connective tissue.[1],[2] The WHO defines it as “A benign lesion, presumably developmental in nature, characterized by the presence of fibrous connective tissue with a characteristic whorled pattern and containing trabeculae of immature bone.” It constitutes about 2.5% of all bone lesions and 7% of all benign bone neoplasms with an incidence of 1:4000–1:10,000.[3],[4] The etiology of fibrous dysplasia is mostly idiopathic but is also believed to have a genetic predisposition.[2],[3] It has been associated with GNAS gene mutation occurring after somatic cell fertilization and is located on the q arm of chromosome 20 at position 13.3. The condition is believed to occur as a consequence of the failure of developmental bone remodeling leading to the formation of immature irregular bony trabeculae embedded in a densely fibrous stroma.[5] The condition is mostly localized to either a single bone termed as monostotic fibrous dysplasia or multiple bones may be involved which is termed as polyostotic fibrous dysplasia. Clinically, the condition is usually asymptomatic with the only concern among the patients being esthetics caused by facial asymmetry.[6]
Monostotic fibrous dysplasia is commonly seen in the second and third decades of life, whereas polyostotic fibrous dysplasia is common in children, especially younger than 10 years with no specific gender predilection; although some studies suggest a slight female predilection. Involvement of maxilla is more common as compared to mandible.[3],[6] This case report shows findings contrary to the usual with a 10-year-old female presenting a monostotic type of fibrous dysplasia affecting the left side of the mandible.
| Case Report | |  |
A female aged 10 years, reported to the Department of Oral Medicine and Radiology with a swelling in her lower left side of the jaw, which was apparent for the past 4 years. The swelling initially was not very appreciable but progressed to produce a considerable facial asymmetry. History of presenting illness revealed no incidence of trauma, paraesthesia, or any difficulty in chewing food. There was no relevant history of any systemic conditions or drug allergies.
On extra-oral examination, a diffuse swelling was seen on the left side of the face extending from the corner of the mouth to the angle of the mandible and inferiorly below the lower border of the mandible [Figure 1]. On palpation, the consistency was bony hard and nontender. The overlying mucosa appeared normal and firm. Intraoral examination revealed the extension of the swelling from the distal aspect of the left mandibular deciduous canine up to the retromolar area with a buccal and lingual cortical expansion in the region of 73–36 [Figure 2]. | Figure 2: Intraorally a well noticeable buccal and lingual cortical expansion is seen
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Radiological investigations included an IOPA, an occlusal radiograph, a panoramic radiograph and a computed tomography (CT) scan. The sparse trabecular pattern in the bone, giving a characteristic “ground glass” pattern was evident on an IOPA. The cortical outlines were thinned with buccal and lingual cortical expansion as revealed on an occlusal radiograph. The panoramic radiography showed a fusiform enlargement of the left side of mandible extending from the region of 73–36 with characteristic ground-glass appearance. A typical bowing of mandible was also evident. The CT scan showed a considerable buccolingual expansion of the cortical plates [Figure 3]. Radiographic and clinical findings suggested a benign fibro-osseous lesion. However, surgical intervention was planned, and an incisional biopsy was performed under general anesthesia to decide further treatment on the basis of a histopathological report. | Figure 3: Computed tomography scan reveals a considerable buccolingual cortical expansion
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On histopathological evaluation, the hematoxylin and eosin-stained section revealed numerous irregularly shaped islands of woven bone devoid of osteoblastic rimming in the background of mature collagenous stroma with few cellular elements. Overall section showed a typical “Chinese letter” appearance suggesting a diagnosis of fibrous dysplasia [Figure 4] and [Figure 5]. A second surgery was planned by the department of oral and maxillofacial surgery considering the requirement of the patient's parents concern with the patient's facial asymmetry. A surgical recontouring of the affected left side of the mandible under general anesthesia was planned and executed. The curetted tissue was then sent for histopathological evaluation. The report revealed features consistent with fibrous dysplasia. On the basis of clinical, radiological and histopathological findings, a confirmatory diagnosis of monostotic fibrous dysplasia was made. | Figure 4: Section shows irregularly arranged trabeculae of woven bone in the background of fibrous connective tissue with the trabeculae devoid of osteoblastic rimming (H and E, ×100)
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 | Figure 5: Tissue section shows highly cellular plump proliferating fibroblasts with no inflammatory changes. Few areas show altered woven bone deposition (H and E, ×400)
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| Discussion | | |
Fibrous dysplasia is a benign fibro-osseous disorder, in which the intramedullary bone gets replaced with fibrous tissue. Fibrous dysplasia shows no specific gender predilection except for Mc Cune Albright Syndrome which has shown female predilection in few of the reported cases.[6] However, in this case, the female patient did not show any features of the syndrome or any other systemic abnormalities. Monostotic fibrous dysplasia is a lot more common among young adults and seldom seen in children <10 years of age as in this case. Clinically it leads to slow progressive enlargement of the affected jaws presenting an asymptomatic unilateral swelling of the face which supports the current report. Cortical plate expansion is usually more on the buccal aspect than lingual, which is also a similar finding in this case. Overlying mucosa appears normal and remains intact as reported.[1]
Fibrous dysplasia can present three types of radiographic patterns, Type 1: Small unilocular/larger multilocular radiolucency with well-defined borders and a fine network of trabeculae. Type 2: the lesion acquires a mottled appearance and appears opaque on account of an increased trabeculation. Type 3: Considerable opacity and many thin trabeculae together gives a “ground-glass” appearance to the lesion.[7] Hence, radiographically, the lesion does not reveal a homogenous appearance each time. The differential diagnosis with similar radiographic appearances such as ameloblastoma, ameloblastic fibroma, ameloblastic odontoma, ameloblastic fibro-odontoma, central giant cell granuloma, odontogenic cyst, ossifying fibroma, osseous dysplasia, chronic sclerosing osteomyelitis, and oteosarcoma should be considered.[8] In the present case, a sparse trabecular pattern of bone with mixed radiopaque and radiolucent areas with thinned cortical plates was evident. On the basis of these findings and so to rule out the differential diagnosis, a biopsy was carried out to arrive at a definitive diagnosis.
Microscopically, fibrous dysplasia presents an arrangement of the woven bone devoid of osteoblastic rimming lying irregularly in a fibrocellular stroma depicting a “Chinese script writing” which was in support with the current case.[9],[10] The bony changes in fibrous dysplasia usually occur during the period of active skeletal growth and it ceases after puberty during the adult life with a spontaneous bone remodeling. Any surgical intervention can be, therefore, deferred in young patients till the complete skeletal maturity is achieved. However, surgical recontouring may be needed in larger lesions for cosmetic or functional reasons. In the current case, the patient was a female aged 10 years and so considering her age the treatment could have been delayed. However, for cosmetic reasons, surgical recontouring on the left side of the mandible was considered as the treatment option. There are no universally accepted treatment guidelines for fibrous dysplasia. Fibrous dysplastic lesions that are asymptomatic should be followed up for a long term with serial radiographs at decided time intervals.[11],[12] The contribution of medical therapy in the management of fibrous dysplasia has not acquired significant role to date. Surgical management in these lesions mainly aims at restoration of facial deformity and functional rehabilitation.[3]
| Conclusion | | |
Fibrous dysplasia may result in many dysfunctions and deformities as it has the tendency to affect not only the jawbones but also the facial and cranial bones, thus making it important for the dentists. The cells of fibrous dysplasia are devoted osteogenic cells with impaired ability to form normal bone. The mutated protein also affects melanocytes and endocrine cells along with osteoblasts leading to cafe-au-lait spots and several endocrinopathies. Malignant transformation occurs rarely with pronounced frequencies ranging from 0.4% to 4%. Monostotic fibrous dysplasia in case of young aged patients should be followed up at regular intervals, and a minor bone osteoplasty or recontouring at the affected site would suffice for better esthetics and functional purpose.
Acknowledgment
We would like to thank Dr. Nilesh Khandelwal, Postgraduate student, Department of Oral and Maxillofacial Surgery and Dr. Tanvi Datir, Postgraduate student, Department of Oral Medicine and Radiology, Dr. D. Y. Patil Dental College and Hospital, Pimpri, Pune for providing us with the clinical data.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Singla S, Verma A, Shetty A, Kini R. Fibrous dysplasia a report of two cases with emphasis on radiographic features. Clin Cancer Investig J 2016;5:184-7.  [Full text] |
| 2. | Anitha N, Sankari SL, Malathi L, Karthick R. Fibrous dysplasia-recent concepts. J Pharm Bioallied Sci 2015;7:S171-2. |
| 3. | Menon S, Venkatswamy S, Ramu V, Banu K, Ehtaih S, Kashyap VM. Craniofacial fibrous dysplasia: Surgery and literature review. Ann Maxillofac Surg 2013;3:66-71. [ PUBMED] [Full text] |
| 4. | Assaf AT, Benecke AW, Riecke B, Zustin J, Fuhrmann AW, Heiland M, et al. Craniofacial fibrous dysplasia (CFD) of the maxilla in an 11-year old boy: A case report. J Craniomaxillofac Surg 2012;40:788-92. |
| 5. | DiCaprio MR, Enneking WF. Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-64. |
| 6. | Canıtezer G, Gunduz K, Ozden B, Kose HI. Monostotic fibrous dysplasia: A case report. Dentistry 2013;3:1-4. |
| 7. | Sivapathasundaram B, Madhavan Nirmal, Rajendran R. 2012. Bone and Joint Diseases. Shafer's textbook of oral pathology. 7 th Elsevier India; 713. |
| 8. | O'Connell KJ. Bony enlargement of the left maxilla. J Am Dent Assoc 1981;102:340-2. |
| 9. | Neville BW, Damm DD, Allan CM, Bouquot, JE.2002.Bone Pathology. In oral and maxillofacial pathology 2 nd ed.. WB Saunder Company, 635-640. |
| 10. | Sakamoto A, Oda Y, Iwamoto Y, Tsuneyoshi M. A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to Gsalpha mutation at the Arg201 codon: Polymerase chain reaction-restriction fragment length polymorphism analysis of paraffin-embedded tissues. J Mol Diagn 2000;2:67-72. |
| 11. | Feller L, Wood NH, Khammissa RA, Lemmer J, Raubenheimer EJ. The nature of fibrous dysplasia. Head Face Med 2009;5:22. |
| 12. | Chandar VV, Priya; Amita. Bilateral fibrous dysplasia of the mandible in a 7-year-old male patient--a rare case. J Indian Soc Pedod Prev Dent 2010;28:126-9. [ PUBMED] [Full text] |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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