Fibrous dysplasia-of-maxilla


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Fibrous dysplasia-of-maxilla

  1. 1. International Journal of Advancements in Research & Technology, Volume 2, Issue 7, July-2013 318 ISSN 2278-7763 Copyright © 2013 SciResPub. IJOART FIBROUS DYSPLASIA OF MAXILLA DR.KRIPAMOY NATH1 DR.AAKANKSHA RATHOR2 1 Resident Surgeon, Department of Otorhinolaryngology, Silchar Medical College And Hospital, Silchar, Assam,India; 2 Post-Graduation trainee,Department of Otorhinolaryngology, Silchar Medical College And Hospital, Silchar, Assam, India. Email: ABSTRACT Fibrous dysplasia is a developmental tumor like condition in which normal bone is replaced by excessive proliferation of cellu- lar fibrous connective tissue intermixed with irregular bony trabeculae. May involve one or several bones and consists of one or more foci of fibro osseous tissue within the matrix of the affected bone. Patient presents with bone pain, deformities & patholog- ic fractures. a case of monostotic fibrous dysplasia of maxillary bone has been reported in a 22 year old female . A review of the literature and management comprising of imaging and sur- gery for the patient are discussed. Keywords : Maxilla, Fibrous dysplasia, Monostotic form. 1 INTRODUCTION FIBROUS DYSPLASIA IS A BENIGN , CHRONIC , SLOWLY PROGRESSIVE BONE DISORDER OF UNKNOWN ETIOLOGY . Histologically it’s a fibro-osseous lesion characterised by the replacement of normal bone with a variable amount of fibrous tissue and woven bone which causes progressive ex- pansion and deformity of bones.It comprises 2.5% of all osse- ous & 7% of all benign bone tumours. In general they affect 1 in 4000 to 10,000 individuals. It has a genetic basis i.e,- GNAS1 gene, fibrous dysplasia is the result of mutation of this gene. 1. Case history: A 22 yrs female presented with swelling of left facial region for the last 5yrs with nasal obstruction for the last 2yrs.Pain left cheek for about a month.Watering of left eye with swelling of lower lid for the same duration. 2.1.Local Examination : 2.1.1Inspection: swelling of left cheek including lower eyelid and lateral side of nose. -redness of left lower eye region - asymmetrical eccentric position of diplopia. 2.1.2Palpation: -smooth , irregular swelling of left maxilla. - mild tenderness of the swelling.-consistency of the swelling was bony hard. 2.1.3Anterior rhinoscopic examination : marked narrowing of the left nasal cavity with gross deviation of septum to right side. 2.1.4Posterior rhinoscopic examination: irregular narrowing of the nasopharynx 3.PROVISIONAL DIAGNOSIS: Ossifying fibro- ma.Paget’s disease.Giant cell tumour.Acute maxillary sinusi- tis with cellulitis.Aneurysmal bone cyst 4.INVESTIGATION : 4.1 Routine blood investigation. 4.2 Biochemical tests : 1. S.alkaline phosphatase level: 280IU/ml. 2. S.calcium level : 8.6 mg/dl. 3. Phosphorus level : 4.2 mg/dl. 4.2Radiological : 4.2.1 Skiagram of PNS shows dense opacity in left maxillary region. 4.2.2CT-SCAN of PNS shows -A large lobulated mass lesion measuring approx. 6.3x 5.4 cm with internal bony & soft tissue densities , is noted expanding the left maxillary antrum. Superiorly pressure erosion of inf. Wall of orbit , inferiorly erosion of alveolar process of maxilla and laterally demineral- ization of posterolateral wall of left maxilla. Post contrast study :- mild enhancement of the soft tissue con- tents of the mass lesion is noted. 4.2.3FNAC of the left maxillary swelling under USG guidance : The smear shows scattered adipocytes , occasional fibroblasts like cells , osteoblasts and RBCs in the background. The above cytomorphological features suggestive of Benign bony lesion 5.SURGERY:Incision : Weber Ferguson incision.Exposure and elevation of flap.Osteotomy done of Zygomatico orbital fissure Supraalveolar region. Frontal process of maxilla In- traoperative findings: -Large mass destroying the floor of orbit , lateral nasal wall, filling the nasopharynx , destroying the sphenoid bone , involving the infratemporal fossa was seen. -Erosion of the alveolar process and posterolateral wall of left maxilla seen. Excision of fibrous dysplastic bone along with reconstruction of floor of orbit by prolene sling was IJOART
  2. 2. International Journal of Advancements in Research & Technology, Volume 2, Issue 7, July-2013 319 ISSN 2278-7763 Copyright © 2013 SciResPub. IJOART done.The mass was resected and sent for HPE. Fig7: postoperative photo after 1 year of follow up 6.HISTOLOGY : showing fibrous dysplasia with the charac- teristics thin, irregular (chinese character like) bony trabeculae and fibrotic marrow space. 7.DISCUSSION : 7.1.Head and neck region constitute about 25% cases of fi- brous dysplasia. Maxilla is the most commonly involved site. Fibrous dysplasia is of 3 types: Monostotic(involve one bone) Polyostotic( involve multiple bone). McCune-Albright syndrome, presents as a combina- tion of polyostotic FD, skin hyperpigmentation and endocrine dysfunction. 7.2.Treatment plan : 1. Depends on the extent of involvement , functional disability , danger to function , neurologic symptoms and asthetic consideration. 2. Differentiation should be made between monostotic and polyostotic form of the lesion. 3. The treatment ranges from observation for minor le- sions to radical resection. 4. The type of surgery performed varies from shaving and contouring of the bone to radical surgery. 5. For large cosmetic deformities recontouring and repo- sitioning of bone may be required. 6. Biopsy : for confirmation and follow up 7. Radition therapy is contraindicated because of report- ed incidence of malignant transformation 8. Orbital involvement with loss of vision is one of the most severe but relatively uncommon complication of fibrous dysplasia. Fibrous dysplasia is a bone disorder of unknown origin char- acterized by slow progressive replacement of medullary bone by abnormal pro- liferative isomorphic fibrous tissue which appears radiolucent on radiographs with classic ground glass appearance [1]. Fibrous dysplasia has 4 different disease pat- terns Monostotic (70%), Polyostotic (30%), Craniofacial form and Cherubism(rare) [3,4]. The range of skeletal involvement varies from an asymptomatic monostotic lesion to polyostotic involvement resulting in progressive functional deficit & aes- thetic problems. The clinical severity depends on time when the mutation of GNAS-1 occurs. Polyostotic Form: It is seen if mutation occurs during 6th week of intrauterine life. Multiple bones may get involved. This form commonly involves the skull & facial bones, pelvis, spine & shoulder girdle. Initial symptoms are bone pain and spon- taneous fracture of the involved bone. Femur shows shephered’s crook deformity [4]. Polyostotic form is again sub- classified into Jaffe’s type & Albright syndrome. Both type consists of variable bony involvement with café-au-lait spot. Albright syndrome has additional feature of endocrine dis- turbances of varying type [4,5]. Polyostotic fibrous dysplasia with soft tissue called mazabraud syndrome. Monostotic Form: If mutation occurs during postnatal life the progeny of that mutated cells are essentially confined to one site resulting in fibrous dysplasia affecting a single bone. About 70% cases are of monostotic form and they involve mainly ribs, femur, tibia & craniofacial bones [1-2, 4]. Any bone may be affected, but monostotic forms are never reported as becoming polyos- totic [4]. The lesion is asymptomatic and usually discovered incidentally. It causes enlargement & distortion of bone. Craniofacial Form: 50%-100% of patient with polyostotic disease & 10% patient with monostotic disease have craniofacial in- volvement [1,5]. Maxilla is more commonly involved than mandible [1]. When maxilla is affected it may involve zygomatic & sphenoid bone. Involvement of frontal, sphenoid, naso-ethmoid & maxillary bone may lead to nasal obstruction, si- nus obstruction & sinusitis [1]. Hypertelorism, cranial asymmetry, facial deformity, visual impairment, exophthalmos and blindness may occur due to involvement of orbital and parietal bone [4]. Ma- lignant changes with fibrous dysplasia include Osteosarcoma, Fibro- sarcoma, Chondrosarcoma, Malignant fibrous histiocytoma & Ewings sarcoma [1]. Association of Amelobastoma[1], Cystic de- generation[2], Angiosarcoma, Frontal sinus mucocele have also re- ported. Treatment is primarily surgery. When the only tooth bearing area is involved conservative treatment is bone shaving. Use of Calcitonin & Pamindronate is also reported for its treatment. Bi- opsy can be taken to rule out the lesion. Fibrous dysplasia usually get stabilized after puberty [1]. 8.Conclusion: Our case is a monostotic type of fibrous dysplasia involving left maxilla, managed by surgery in which the fibrous dysplas- tic bone was removed by osteotomy. Reconstruction of floor of orbit was done by prolene sling.On follow-up after 3 months of surgery: -Facial asymmetry corrected. -There is no diplopia. -no watering of left eye - and relieved of nasal obstruction. HPE revealed benign fibro-osseous lesion. The patient and family felt that the surgical procedure result- ed in significant improvement of her cosmetic appearance But each patient may present with variable symptoms and clinical findings, thus the care of these patients must be customized to their needs and sites of involvement. Fibrous dysplasia may manifests as monostotic or polyostotic form. Diagnosis of polyostotic form is easier due to extra-skeletal involvement. Monos- totic form is common in the jaw. Fibrous dysplasia is a tumor like developmental disorder with minimal chances of malignancies. Aesthetic correction is done by surgeries 9.Recommendations  Aggressively screen for and manage  Fibrous dysplasia is a developmental condition with a genetic basis i.e. point mutation of GNAS1 gene.  Patients mostly females presents as chronic diffuse unilateral enlargement or asymmetry of face usually in the 1st and 2nd decade of life.  Diagnosis is made by its clinical presentation , charac- teristic radiological and histological features. IJOART
  3. 3. International Journal of Advancements in Research & Technology, Volume 2, Issue 7, July-2013 320 ISSN 2278-7763 Copyright © 2013 SciResPub. IJOART  Surgical intervention should be reserved to correct functional impairments or significant cosmetic de- formities.  HPE is necessary to rule out malignancy though its very rare. 10.References [1] Scott-Brown’s Otorhinolaryngology,Head @ Neck Surgery. 7th edition , vol 2 , pg 1522-1523. [2] Stell and Maran’s Textbook Of Head @ Neck Sur- gery And Oncology. 5th edition, pg 250-251. [3]BYRON J. BAILEY @ JONAS T. JOHNSON Head @ Neck Surgery; fourth edition ; 189 – 191. [4] CUMMINGS OTOLARYNGOLOGY Head @ Neck Surgery; fourth edition 2895-2897. [5] SMITH AG’ ZAVALETAA. Osteoma ossifying fi- broma , fibrous dysplasia of facial @ cranial bones. AMA Arch Pathol . 1952 Dec;54 (6): 507-527. IJOART
  4. 4. International Journal of Advancements in Research & Technology, Volume 2, Issue 7, July-2013 321 ISSN 2278-7763 Copyright © 2013 SciResPub. IJOART IJOART