1 CRANIOFACIAL FIBROUS DYSPLASIA PRESENTING WITH VISUAL IMPAIRMENTAUTHOR: DR PRADOSH KUMAR SARANGI ,JUNIOR RESIDENT CO-AUTHORS: DR SASMITA PARIDA,ASSOCIATE PROF DR JAYASHREE MOHANTY,PROF &HOD DR BASANTA MANJARI SWAIN,ASSOCIATE PROF DR PRATISRUTI HUI ,JUNIOR RESIDENT DR DINESH HARVEY RAJ,JUNIOR RESIDENT DR DINESH KISKU,JUNIOR RESIDENT DEPT OF RADIODIAGNOSIS, SCB MEDICAL COLLEGE & HOSPITAL CUTTACK,ODISHA

2 CLINICAL HISTORY A 24 yr male patient presented with gradual painless proptosis and diminution of vision in left eye and nasal obstruction for past 9 yrs. To start with he developed swelling on forehead which was insidious in onset, painless and gradually progressive for which he got operated at a local hospital. He was apparently alright for one and half year after surgery .Then he complained of pain and scanty mucopurulent discharge from the scar of previous surgery. He noticed fullness of nose, eye ,lower aspect of forehead & upper aspect of cheek on the left side ,gradually growing to present size and leading to outward and downward proptosis with diminution of vision in left eye. He also complained diificulty in breathing through nose with mucopurulent , foul smelling nasal discharge . No h/o trauma, hearing loss, epistaxis, loosening of teeth, trismus, neurological deficit, difficulty in swallowing and mastication. He had no skin lesions or any other swellings in body. He had no history of fever ,loss of weight and appetite. He had no significant medical history.

3 EXAMINATION General physical and systemic examination revealed no findings . On local examination, a bony hard swelling noted with a ulcer on left side of nose with mild serosanguinous discharge Opthalmological examination - visual acuity 6/6 in right eye, counting fingers at 1 mt distance in left eye, left opthalmoplegia Fundoscopy - pale optic disc, macular edema, tortuous vessels noted in LE.RE fundoscopy is normal Anterior rhinoscopy - a firm mass noted in left nasal cavity from which biopsy was taken His thyroid function tests, testosterone, prolactin, insulin-like growth factor, and FSH/LH levels showed no signs of an endocrinopathy. Routine blood and urine examination were normal A skeletal survey was carried out which did not reveal involvement of any other bone. Histopathological examination of nasal cavity mass showed- aggregate of moderately dense fibrous connective tissue containing bony trabeculae in haphazard distribution ,proliferating capillaries without any evidence of malignancy A NCCT brain and orbit was done.

5 Small white dots are due to digital radiography artefacts ( )FRONTAL LATERAL frontal and lateral radiograph of skull showing dense sclerotic lesion involving left maxilla,zygoma,orbit,frontal bone causing leontiasis ossea appearance Small white dots are due to digital radiography artefacts ( )

6 Alveolar process of maxillaMaxillary sinus Hard palate (1) (3) (2)

7 (6) (5) (7) (9) (8) Inferior orbital fissure (4) Optic nerveF .ovale & spinosum superior orbital fissure Optic canal (6) Intracranial extension (5) (4) Optic nerve Rt frontal bone (7) (9) (8)

8 DESCRIPTION OF FINDINGSNCCT revealed expansile sclerotic lesions (HU ) predominantly with few areas of mixed/pagetoid pattern (sclerotic and lucent) & ground glass opacity involving b/l frontal,ethmoid bone,left maxilla,maxillary sinus,orbit Fig(1) involvement of alveolar process of maxilla Fig(2) involvement of hard palate and maxilla Fig(3) obliteration of left maxillary sinus & nasal cavity,collection in (rt) maxillary sinus Fig(4) F.ovale & spinosum,inferior orbital fissure are essentially symmetrical with contralateral side Fig(5) lesion encroaching etmoid, (lt) orbit, abutting optic nerve,medial rectus .Superior orbital fissures are not involved , ground glaas opacity noted in (lt) mastoid Fig(6) involvement of roof of left orbit, b/l optic canals are normal Fig(7) pagetoid lesion in rt frontal bone Fig(8) brain window showing lt orbit involvement with optic nerve abuttment Fig(9) intracranial extension to left frontal lobe DIAGNOSIS :CRANIOFACIAL FIBROUS DYSPLASIA

9 DISCUSSION Fibrous dysplasia (FD) is a benign developmental anomaly of the bone forming mesenchyme where the medullary bone is replaced by fibrous tissue, woven bone and spindle cells.[1], usually presents in late childhood or early adolescence [2], sarcomatous transformation occures in 0.4-4% cases .[3] It occurs in two distinct forms -polyostotic (30%)which involves several bones and the monostotic (70%) which involves a single bone. [4] Craniofacial involvement in FD occurs in nearly 100% of polyostotic and 30% of monostotic forms [2]. The bones commonly involved are mandible (12%) and maxilla (12%), involvement of the ethmoid, sphenoid, frontal and temporal bones are infrequent .[5] These lesions cause expansion,thickening and sclerosis of the involved bones with resultant visual complications, hearing disturbances,facial asymmetry and tooth displacement depending on the bone involved.[6] Radiographic features of FD vary depending on the amount of bony and fibrous matrix within the lesion and have been sub-classified into three different patterns : pagetoid type 56%, sclerotic type 23% and the radiolucent type 21% ; sclerotic type is most common in facial bone and base of skull.[2,7 ,8] CT accurately establishes the diagnosis and extent of bone involvement. Involvement of optic canals, orbital fissures, frontonasal ducts and ostiomeatal complex can be best evaluated by CT scanning .[7] On MRI, It exhibits homogenous, moderately low signal intensity on T1 weighted images ,On T2 weighted images. the tissue very high signal intensity. With moderate to significant central contrast enhancement with some rimenhancement. [2,3] Radionuclide scanning in FD shows areas of intensely increased uptake .[2] Based on clinical and neuroimaging studies DDs include Paget's , hyperostotic meningioma, ossifying fibroma, osteoblastoma and other fibrous lesions (benign: non-ossifying fibroma or fibroxanthoma, cortical desmoid tumor, desmoplastic fibroma; and malignant: fibrosarcoma, malignant fibrous histiocytosis). [3 ,8] Treatment is surgical with unroofing of opticcanal or cosmetic remodelling of the orbit to provide adequate room for the intraorbital content .[2 ,8]

10 REFERENCES 1. Weissleder R, Rieumont MJ, Wittenberg J. Introduction to diagnostic imaging. 2nd ed. Rio de Janeiro, RJ:publisher Revinter 2004. 2. Wg Cdr A Alam, Gp Capt BN Chander.Craniofacial Fibrous Dysplasia presenting with Visual Impairment. ,MJAFI 2003; 59 : 3. DA Lisle, PAJ Monsour and CD Maskiell, Imaging of craniofacial fibrous dysplasia,Journal of Medical Imaging and Radiation Oncology (2008) 52, 325–332 4.Grabias SL, Campbell CJ. Fibrous dysplasia. Orthop Clin NorthAm 1997;8: 5. Hudson TM, Stiles RG, Monson DK. Fibrous lesions of bone.Radiol Clin North Am 1993;31(2):279-97 6. Araghi HM, Haery C. Fibro-osseous lesions of craniofacialbones. The role of imaging. Radiol Clin North Am1993;31(1): 7. Greenspan A. Orthopaedic Radiology : A practical approach.3rd ed. Lippincot William and Wilkins, 2000;602-9. 8.Sharma R R, Mahapatra A K,pawar S J et al. Symptomatic cranial fibrous dysplasias:clinico-radiological analysis in a series ofeight operative cases with follow-up results Journal of Clinical Neuroscience (2002) 9(4),