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DRJ Dent Res J Dental Research Journal Dental Research Journal 1735-3327 2008-0255 Medknow Publications Pvt Ltd India DRJ-11-518 10.4103/1735-3327.139431 Case Report Craniofacial polyostotic fibrous dysplasia: A rare case Owlia Fatemeh Karbassi Mohammad-Hassan A Department of Oral Medicine, Yazd Shahid Sadoughi University of Medical Sciences, Yazd, Iran Department of Oral Medicine, Yazd Shahid Sadoughi University of Medical Sciences, Yazd, Iran Address for correspondence:Fatemeh Owlia, Daha Fajr Ave, Yazd Dental School of Shahid Sadoughi University of Medical Science, Yazd, Iran dr.olia@ssu.ac.ir Jul–Aug 2014 11 4 518 521 Copyright: © Dental Research Journal 2014

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Craniofacial fibrous dysplasia (FD) is one of three types of FD which can affect the craniofacial complex. It is the proliferation of cellular fibrous connective tissue intermixed with irregular bony trabecules. It is a developmental tumor-like condition that is characterized by replacement of normal bone. The purpose of this report is to present a rare case of craniofacial polyostotic FD that led to vision loss in the same affected side.

 Craniofacial non syndromic polyostotic fibrous dysplasia </sec> <sec> <title>Introduction

Craniofacial fibrous dysplasia (FD) is a bone development anomaly characterized by hamartoma proliferation of fibrous tissue within the medullary bone, with secondary bony metaplasia, producing immature, newly formed and weakly calcified bone without maturation of osteoblast which appears on radiographs as a radiolucent, mixed, or radiopaque lesion. 1

FD is a benign and slow-growing lesion. The most common presenting symptom is painless enlargement. In most cases, a specific diagnosis cannot be made on a histological basis alone, nor can it be made without appropriate radiographs of high quality. 2 , 3 , 4

Involvement of the face and skull by FD could be mentioned, which is known as "leontiasisossea". 5 It tends to expand during childhood and stabilize after puberty. 6 , 7 There is no sex inclination. It may be seen as monostatic or polyostotic type. Sometimes, it is associated with abnormal endocrine hyper function and pigmented skin lesions, which is known as "Maccune-Albright" syndrome. 8

Cranial bones are common places for involvement with 10 to 27% in monostatic and 50% in polyostotic cases. 9 , 10 Temporal bone involvement is less common. 11 , 12 Depending on the type and location of FD, the signs and symptoms vary and include facial deformity and asymmetry, vision changes, hearing impairment, nasal congestion and/or obstruction, pain, paresthesia, and malocclusion. 13

In this paper, a case of craniofacial polyostotic FD in a 10-year-old boy is described. Prominent properties of this case could be mentioned as: Extensive involvement of left hemicranium, progressive course of disease, especially proptosis in left eye and frontal bossing due to the large growth of diseased bone and occurrence in temporal bone.

 Case Report

A 10-year-old boy was referred to our department with complaint of painless swelling in the left side of the skull and face since he was 3 years old. Swelling was small at first and gradually reached to present extension Figure 1.

Clinical view of patient

Figure 1

In extra oral examination, diffused expansion was seen on left side of his face. The lesion displaced his left eye and led to exophthalmos. Frontal bossing was seen on the left side. Surface temperature of skin was higher in left side compared to right side. A non-tender mass was palpated on his left temporal bone. Consistency was bony hard Figure 2, Figure 3and Figure 4.

Frontal bossing

Figure 2

Occipital involvement of cranial

Figure 3

Swelling of left side

Figure 4

In intra oral examination, a bony expansion was found on buccal surface of maxilla from distal of lateral incisor to distal of first molar. Horizontal dimension was approximately 4 cm. Palatal aspect wasn′t expanded. Overlying mucosa was intact and non-tender Figure 5.

Intraoral view of lesion was extended from distal of lateral to distal of first molar

Figure 5

There were no signs of precocious puberty. Cutaneous pigmentation or any endocrinological disorder was not detected in the physical and laboratory examinations. His vision in left eye was lost 2 years before in a firecracker incident.

Radiological findings

In an initial panoramic image, the vertical height of the body of the mandible in this area seemed to be larger in comparison with the right side. There was a generalized ground glass appearance in left maxilla and mandible quadrant Figure 6.

Panoramic view shows widening of the body in left side

Figure 6

Cranial and facial bones were evaluated with computerized tomography (CT) scan in axial and coronal plans. Cranial CT scan showed extensive involvement of most cranial bones in left side Figure 7.

CT scan in coronal plans. Cranial CT scan showed extensive involvement of most cranial bones in left side

Figure 7

Laboratory findings

Because of high temperature of head and face in affected side compared to other side, a list of laboratory tests was requested. Ca level was 10.5, ALP was 1424, and phosphorus was 4.2. These findings affirmed increase bone metabolic activity.

Pathological findings

Poorly oriented trabeculae of bone were separated by cellular fibrous connective tissue. The bone appeared woven rather than lamellar Figure 8. Chinese alphabet pattern was seen that confirmed FD Figure 8.

Histopathologic view of lesion in incisional biopsy

Figure 8

Discussion

FD is one of the several fibro-osseous lesions that affect craniofacial region. This lesion-like cherubism and focal cement osseous dysplasia can occur anywhere in the skull. But occurrence in maxillofacial region is more usual. 14 Ground glass and orange peel appearance are the main radiographic pattern of FD. 15

This lesion have wide spectrum of radiographic patterns from completely radiolucent, mixed to completely radiopaque. 16

Hence, other lesions like cemento ossifying fibroma (COF) can imitate this radiographic pattern. 17 It is critical for the clinician to consider it among differential diagnosis. 18

In most cases, the radiographic characteristics of polyostotic FD and clinical information are sufficient to render definite diagnosis without biopsy. Usually, diagnosis of polyostotic type opposed to monostatic type is based on clinical behavior. 15

Because cherubism is a bilateral lesion growing without any neurological damage or deformity in the face, it wasn′t a reasonable diagnosis. 17 Among the other diagnosis was focal cemento osseous dysplasia (FCOD). Although FCOD is a maturable lesion, it usually affects mandible in single form and affects more quadrants in both sides. But, in this case, the lesion affected left side of both the jaws. Also, FCOD usually appears in females in fourth decade of life and not in children. 18

COF is a fibro osseous lesion which can show a ground glass pattern, but it usually occurs in females and it doesn′t have skull involvement. For definite diagnosis, an incisional biopsy was achieved from buccal surface of expanding lesion on left maxillary quadrant.

 Conclusion

FD may manifest as monostotic or polyostotic forms. In polyostotic form, it is important to monitor growth of lesion for possible first step surgery to prevent neurological defect or face deformity.

 Cholakova R Kanasirska P Kanasirski N Chenchev Iv Dinkova A Fibrous dysplasia in the maxillomandibular region - Case report.J IMAB - Annu Proc (Scientific Papers) vol , book , 0 Fibrous dysplasia in the maxillomandibular region - Case report J IMAB - Annu Proc (Scientific Papers) vol 16, book 4, 2010 YuÈceer N Kutluhan A Bekerecioqlu M Arslan H Akman E Polyostotic fibrous dysplasia with craniofacial localization presenting with frontal lobe compression in a 14-year-old girl Acta Neurochir (Wien) 1999 141 203 7 Diah E Morris DE Lo LJ Chen YR Cyst degeneration in craniofacial fibrous dysplasia: Clinical presentation and management J Neurosurg 2007 107 504 8 Suarez EP Santiagoa VS Gracia Calvoa PS Penas JJ, Loópez Pino YM.Cefaleaprogresiva Clinica de displasiafibrosa craniofacial Penas JJ, Loópez Pino YM Cefaleaprogresiva Clinica de displasiafibrosa craniofacial Progressive migraine Craniofacial fibrous dysplasia Ann Pediatr 2011;75:136-7 Esping Family Foundation The Chatlos foundation, Inc.A guide to understanding fibrous dysplasia Children′s Craniofacial Association, Dallas, Tx, 5 The Chatlos foundation, Inc A guide to understanding fibrous dysplasia Children's Craniofacial Association, Dallas, Tx, 2005 Collins MT International meeting on fibrous dysplasia/McCune-Albright syndrome and cherubism Orphanet J Rare Dis ;; 1 International meeting on fibrous dysplasia/McCune-Albright syndrome and cherubism Orphanet J Rare Dis 2010;7;1 Tanaka Y Tajima S Maejima S Umebayashi M Craniofacial brous dysplasia showing marked involution postoperatively Ann Plast Surg 1993 30 71 6 Albin J Wu R Abnormal hypothalamic pituitary function in polyostoticbrous dysplasia Clin Endocrinol 1981 14 435 43 Harris WH Dudley HR Jr Barry RJ The natural history of brous dysplasia: An orthopedic pathological and roentgenographic study J Bone Joint Surg 1962 44 207 33 Leeds N Seaman WB Fibrous dysplasia of the skull and its di¨erential diagnosis Radiology 1962 78 570 82 Brown EW Megerian CA McKenna MJ Weber A Fibrous dysplasia of the temporal bone AJR Am J Roentgenol 1995 164 679 82 Derome PJ Les tumeursspheno-ethmoidales Neurochirurgie 1972 181 139 45 Lee JS Fitz Gibbon EJ Chen YR Kim HJ Lustig LR Akintoye SO Clinical guidelines for the management of craniofacial fibrous dysplasia Orphanet J Rare Dis 2012 7 1 Chen YR Wong FH Hsueh C Lo LJ Computed Tomography characteristics of non-syndromic craniofacial fibrous dysplasia Chang Gung Med J 2002 25 1 8 Abdelkarim A Green R Startzell J Preece J Craniofacial polyostotic fibrous dysplasia: A case report and review of the literature Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008 106 e49 55 Gupta S Umesh K Warad NM Ahmed S Fibrous dysplasia of maxillary bone: A case report Al Ameen J Med Sci 2011 4 92 7 Wood NK Goaz PW Differential diagnosis of oral and maxillofacial lesions Mosby Louis 1997 363 4 Lello GE Sparrow OC Craniofacial polyostotic fibrous dysplasia J Maxillofac Surg 1985 13 267 72