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Page 1: Reconstruction With Autoclavized Dysplastic Bone Graft in Craniofacial …neurosurgery.dergisi.org/pdf/pdf_JTN_459.pdf · when craniofacial region is involved enlargement of the affected

TlIrkish NellroslIrgen/12: 107 - 112, 2002 YavlI:er: RecolIstructiOlI Witll Alltoc/al'i:eri Dysplastic BOlle Graft 111 Crallinfacial Fil'l'OlI, DI/'pla,itJ

ReconstructionGraft in

With Autoclavized Dysplastic BoneCraniofacial Fibrous Dysplasia:

A Case Report

Kraniofasyal Fibroz DisplazideRekonstriiksiyon:

Otoklavize DisplastikBir Olgu Sunumu

Kemik ile

REHA YAVUZER, YA VUZ BA$TERZl, FiKRET DOGULU, CEM SEZER,M. CEMALETTiN <::ELEBl, KEMALi BA YKANER

Gazi University School of Medicine, Departments of Plastic and Reconstructive Surgery (RY, YB, MC(),Neurosurgery (FD, KB) and Pathology (CS)

Received: 25.4.2002 ¢::> Accepted: 10.7.2002

Abstract: Fibrous dysplasia is a congenital, benign andprogressive disease of bone. It is characterized by thereplacement of normal bone by fibro-osseous tissue andwhen craniofacial region is involved enlargement of theaffected bones, neurovascular compression and facialdeformities can be seen. Complete resection of the affectedbones and immediate reconstruction with unaffected

cranial bone grafts is the best choice for the treatment ofcraniofacial fibrous dysplasia. When the dysplastic areainvolves a significant portion of the craniofacial skeletonas if in here presented case, autoclaved dysplastic bonecan be also used for reconstruction.

Key Words: Autoclavized bone graft, fibrous dysplasia,reconstruction

INTRODUCTION

Fibrous dysplasia is a benign, metabolic,nonhereditary and congenital bone disease that ischaracterized by the replacement of normal bone byfibro-osseous tissue. It usually appears during thefirst two decades of life. Although it is apathologically benign disease, it may show amalignant clinical progress. There are three variants

Ozet: Fibrbz displazi konjenital, benign ve progresif seyirlibir kemik hasta!JgldlT. Normal kemigin yerine fibro­osseoz bir dokunun gee;tigi bll hasta!Jkta kraniofasyalbblgede etkilenen kemiklerde kahnla~ma nbrovaskiileryapllarda basI ve yiizde deformite meydana gelebilir.Etkilenen kemiklerin tam olarak e;lkanlmasl ve aYl1lseansta saghkh kranial kemik greftleri ile onaTll11lenuygun tedavi see;enegidir. Slinulan vakam!Zda oldugugibi, kraniofasyal iskeletin biiyiik bir bbliimii etkilenmi~sedisplastik kemigin otoklavize edilerek yerine iadeedilmesi, diger bir rekonstriiksiyon see;enegidir.

Anahtar Kelimeler: Fibrbz displazi, otoklavize kemikgrefti, rekonstriiksiyon

of the disease: monoostotic form, polyostotic formand McCune Albright syndrome. Contrary to firstdescriptions, the disease is monoostotic in 70% of thepatients and lesions most commonly involve femurand ribs. Polyostotic form usually occurs in thecraniofacial skeleton and sphenoid and frontal bonesare the commonly affected bones (2,8). Skull base andorbital involvement may result in entrapment of theneurovascular structures and sympt0111S related to

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Turkish Neurosurgery 12: 10, - 112, 2002 Yn,Iu:er: Rccollsfrue/ioll Wifh Aufoclal'i:cd Dysplastic BOlle Gmff 111 emlliofarial Filmll/s D1Isl'Iasia

compression can be encountered. Patients usuallypresent with complaints such as deformity of face,atypical facial pain and headache, progressive visualloss, hearing loss and epiphora (8). In this article wepresent a case of polyostotic fibrous dysplasia withextensive craniofacial involvement that was treatedwith radical resection of the affected bones with

intracranial optic nerve decompression andreconstructed with reshaped and autoc!avizeddysplastic bone grafts.

CASE REPORT

31-year-old-man was presented with a swellingon his glabellar region that had been slowly growingover the past two years. On examination a hard massthat extends to the frontal region was detected. PlainX-rays revealed ground glass appearance on thefrontal bone and litic-sc!erotic alterations on the

periorbital bony structures. Computed tomographyscanning showed extensive enlargement of thefrontal, parietal, ethmoid and sphenoid bones (Fig.1). Visual field examination and laboratory findingswere all in normal limits. On the base of thesefindings surgery was performed under the

presumptive diagnosis of fibrous dysplasia. Abicoronal flap was elevated to expose the fronto­parietal, orbital and nasal regions. When the temporalfascia and orbital contents were dissected in thesubperiostal plane it was seen that the frontal,parietal, nasal and sphenoid bones were severelyaffected and significantly enlarged (Fig. 2). With thehelp of frontal and parietal craniotomies cll1dfronto-

Figure 2: Intraoperative view of fronto-orbital region aftercoronal flap was elevated.

Figure 1:Coronal and axial sections of preoperative CT scans, demonstrating the involved parietal, frontal and sphenoidbones and narrowing of the left optic canal.

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Tllrkisll Nellrosllrgery 12: 107 - 112, 2002 YavlI:er: Recollstmetioll With Alltoclavi:crl DI/splastie BOlle Graft III Cnllli"jaeial Fi/>roll' D'/'pitJ.<ia

Figure 3: Fronto-parietal and fronto-orbital bones wereremoved with craniotomies. Dysplasticalterations were significantly enlarged theaffected areas of the bones.

Figure 5 a: Histopathological appearance of the dysplasticbone, showing the fibro-osseous bone formationwith in the fibrous stroma. (HEx40)

zygomatic, fron to-maxillary, fron to-orbi ta Iosteotomies both the fronto-parietal area and thenfronto-orbital bar were removed in two pieces (Fig.3). Affected areas of the nasal and sphenoid boneswere countered down until a margin of no affectedbone was reached by using a high-speed drill.

Figure 4: Appearance of the bones that were countereddown with a high speed pneumatic drill andautoc1aved for 30 min. at 120°C.

b: Histopathological appearance of the dysplasticbone after autoc1avisation, showing destructedcells.(Hex100)

Although, there was no visual field defect,prophylactic optic nerve decompression wasperformed because of the marked narrowing of theoptic canal. Fronto-parietal and fronto-orbital bones,which had been removed, were countered down totheir normal thickness and autoclaved for 30 min. at

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TlIrki,,11 Nellrosllrgery 12: 107 - 112, 2002 Ym'lt=t:r: I~ceollstrllctioll Witll Alltoclavized Dysplastic BOllc Gmft III Cralliofaeial Fi/'roll" D1/"pia"ia

120°C. These autoclaved dysplastic bones were thenreplaced back to their anatomical places forreconstruction and secured by using titanium 2.0mmplate and screw system (Fig. 4). Then the coronal flapwas closed over two 10-mm. silicon suction drains.

Histopathologic examination of the dysplasticbone demonstrated bony trabecules resemblingChinese letters in a rich fibroblastic stroma (Fig. Sa).This typical histopathologic appearance confirmedthe diagnosis of fibrous dysplasia. The dysplasticbone was also examined following autoclavization,which revealed significant deformation of thedysplastic cells (Fig. Sb).

The postoperative period was uneventful andthe patient was discharged from hospital in 8 days.The follow up period extending over two yearsshowed neither recurrence nor severe bone graftresorption (Fig 6).

DISCUSSION

Although, fibrous dysplasia has been described,as a kind of proliferative fibro-osseous tissuesecondary to bony metaplasia, its etiology wasunclear. Recently it has been determined that the

disease is due to a functional disorder of the

osteogenic cells. This functional disorder appears tobe caused by a mutation of the a subunit of Gsignaling protein (Gs-a) in osteoblastic cells 0,11).As a result of this mutation, uncontrolled productionof fibrotic bone matrix and overgrowth occurs at theaffected bones.

Histologically irregularly spreaded bonetrabecules (Chinese letters) and rarely cartilageislands have been detected in a fibrous matrix. Thedisease starts from the medullar cavity and expandsthe bone without disturbing its cortex.

The radiological appearance depends on thelocation of the lesion and the proportions of fibrousand osseous structures. They can appear as sclerotic,litic or mixed type. The bone cortex more and rapidlyexpands in thinner bones such as orbital plate ofmaxillary, ethmoid and frontal bones than in thickercortical bones. On the other hand, expansion is slowerand the lesions have sclerotic appearance in thickerbones. Computerized tomography is the most usefulradiological study to determine the location andextend of the lesion. Where as MR can be especiallyhelpful for the evaluation of neurovascular structurecompressions (2,8). Bone scintigraph,y is also useful

Figure 6: Two years after surgery: there is no recurrence or severe bone graft resorption in follow up cranial CT scans.

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Turkish Nellrosurgery 12: 107 - 112, 2002 YnvlI::er: Recolis/rlIctioll WillI Au/oclnvized Dysplnstic BOlle Crnft III Crnlliofncinl Fi/lrou,; Dy"pinsin

and sensitive tool in evaluating the full extent ofpolyostotic fibrous dysplasia (3).

There is no specific laboratory study to diagnosefibrous dysplasia other than histopathologicconfirmation. However high serum alkalinephosphatase and urinary hydroxyproline levels maybe detected in some of the patients (6,11).

Nonsurgical treatment of the fibrous dysplasiais controversial. Radiation therapy is notrecommended due to both lack of response andpotential of malignant transformation toosteosarcoma or condrosarcoma. Although, medicalagents such as corticosteroids, aluminum acetate,biphosophonates (pamidronate, etidronate etc.) andmithramycin have been used for the treatment of thedisease, their benefits are minimal and temporary.Therefore, radical surgery and immediatereconstruction is advocated by many for thetreatment of fibrous dysplasia. However, there is noconsensus on the timing and extensiveness of surgeryor on the methods of reconstruction. Occasionally,the disease can be self-limiting so some physiciansrecommend that the surgery must be delayed untilthe post puberty. On the other hand, others are infavor of early extensive surgery as incompleteresection is associated with 25% local recurrence(2,6,8,10,11).

There are three alternatives for reconstruction

of defects. The first option is reconstruction withautogenous bone grafts. Iliac, costal and cranial boneauto grafts have been ·used for the purpose ofreconstruction for many years. However their usagecan be troublesome due to donor site morbidity andlimited supplies. Therefore, when extensiveresections are planned autogenous bone grafts failto provide adequate reconstruction material. Anotherproblem with this option is the difficulty of givingthe desired shaped to the bone grafts, especially whencomplex anatomical places are needed to bereconstructed.

The second alternative is reconstruction with

synthetic materials. Some of surgeons prefer largemethyl methacrylate implants for the reconstructionof extensive cranial defects when autogenous bonegrafts are insufficient. Recently hydroxyapatiteimplants are popularised, as unlike methylmethacrylate, they do not cause exothermic reactionand inflammatory response. Donor site morbidity isavoided with the use of these synthetic materials. Onthe other hand, to shape these materials so that they

can fit into the areas that are going to be reconstructedis much more difficult than autogenous bone grafts(2,10).

The last choice is the usage of autoclavizeddysplastic bone grafts. The use of dysplastic boneafter boiling or autoclaving for the reconstruction ofthe large bony defects has been performed inorthopedic surgery since 1937 (4). As for craniofacialsurgery it was first used by Lauritzen et al. (7) in 1985.Reposition of the cranial bone that belongs to thepatient reduces the operation time significantly andalso provides the excellent cosmetic results. This alsoreduces the problem of bone availability, donor sitemorbidity and tissue reaction and provides a perfectfit. However, the delayed bone healing and bone graftresorption can be problematic in some cases makingthis option to be left only for very extensiveinvolvements. When compared with nonautoclavedhealthy bone grafts they poses a longer healingperiod during which they might show a much morepronounced resorption. Although, this may lead usto think that this option is much more secure insmaller defects, one should remember that there are

better alternatives in terms of healing and final resultfor such reconstruction needs. However, for the

larger defects as in here presented patient thereconstruction options are quite limited and thesurgeon might prefer autoclaved bone only becausethe other options can not provide superior resultswhich is not the case in small defects.

Histological studies of the autoclaved boneshowed that autoclavization destroy the entirecellular structures in the bone. On the other hand,

the noncellular frame is protected to some extent.Following implantation of the autoclaved bonerevascularisation, migration of osteoblasts fromadjacent healthy bone, resorption of dead structuresand formation of new bone occur (4,7,9).

Biomechanical properties of bone also get weak afterautoc1avization. Kohler et al. showed the decrease

in strength and stiffness of autoc1aved bone that isclosely related to the autoclaving time andtemperature. The loss of biomechanical properties isminimum for high temperature-short timeapplications. Although, this weakness can be acumbersome for weight-bearing bones like femurand tibia, it is not a problem for skull (5). Severalauthors recorded their concern that devitalized tissue

may increase the risk of infection. However, equalinfection rates were found for autoclaved bone graftswhen compared to the nonautoclaved ones inexperimental studies (9).

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Turkisll Neurosurgery 12: 107 - 112, 2002 Yalluzer: Recollstmctioll Witll Autoclavized Dysplastic BOlle Craft III Cralliofacial Fil,rt'lls Difsplasia

CONCLUSION

Fibrous dysplasia is a local aggressive diseasethat is characterized by the replacement of normalbone by fibro-osseous tissue. Complete resection ofthe affected bones and immediate reconstructionwith unaffected cranial bone grafts is the best choicefor the treatment of craniofacial fibrous dysplasia.When the dysplastic area involves a significantportion of the craniofacial skeleton as if in herepresented case, autoclaved dysplastic bone can bealso used for reconstruction. This technique is not afirst choice and other techniques like bone grafts oralloplastic materials must be preferred inreconstruction of small defects. However if the defect

is large, and difficult to be reconstructed withsynthetic materials or bone grafts, then the use of theautoclaved affected bone segments come forward.

Correspondence: Reha YAVUZERBan~ Sitesi 87. Sokak No:2406530 M.Kemal Mah.ANKARATel: 312 2141000/6418

E-mail: ryavuzer@hotmai!.com

REFERENCES

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2. Edgerton MT, Persing JA, Jane JA: The surgical

treatment of fibrous dysplasia. Ann Surg 202; 459-479,1985

3. Elgazzar HA, Shehab 0, Malki A, Abdulla M:Musculoskeletal system, in Elgazzar HA (ed), Thepathophysiologic basis of nuc1ear medicine, Germany:Springer-Verlag Berlin Heidelberg, 2001: 106

4. Ewers R, Wangerin K: The autoc1aved autogenousreimplant an immediately replaced mineral framE'. J

Maxillofac Surg 14; 138-142, 19865. Kohler P, Kreicbergs A, Stromberg L: Physical

properties of autoc1aved bone. Acta Orthop Scand 57;141-145,1986

6. Lala R, Matarazzo P, Bertelloni S, Buzi F, Rigon P,Sanctis C: Pamidronate treatment of bone fibrous

dysplasia in nine children with McCune Albrightsyndrome. Acta Pediatr 89; 188-193, 2000

7. Lauritzen C, Alberius P, Santanelli F, Vii-llforsB, LiljaJ, Stephensen H: Repositioning of craniofacialtumorous bone after autoc1aving. Scand J Plast ReconstrHand Surg 25; 161-165, 1991

8. Ricalde P, Horswell BB:Craniofacial fibrous dysplasiaof the fronto-orbital region: A case series and literaturereview. J Oral Maxillofac Surg 59; 157-168, 20D1

9. Vanac10cha V, Saiz-Sapena N, Garcia-Casasola C,Alava E: Cranioplasty with autogenous autoclavedcalvarial bone flap in the case of tumoural invasion.Acta Neurochir 139; 970-976, 1997

10. Vories A, Mansfield E: Hydroxyapatite cranioplastyin fibrous dysplasia of skul!' Ear Nose Throat J 80; 29­31,2001

11. Yavuzer R, Bone H, Jackson IT: Franta-orbital fibrousdysplasia. Orbit 19; 119-128, 2000

12. Yavuzer R, Khilnani R, Jackson IT, Audet B: A case ofatypical McCune Albright syndrome requiring opticnerve decompression. Ann Plast Surg 43; 430-435, 1999

Chang Gung Med J 2002 Jan;25(l):1-8

Computed tomography characteristics of non-syndromic craniofacialfibrous dysplasia.

Chen YR, Wong FH, Hsueh C, 10 IJ.

Fibrous dysplasia is a benign £ibro-osseous tumor of bones commonlyinvolving the craniofacial region. Computed tomography (CT) imagingstudy of the disease is useful for evaluation and treatment planningThe average number of bones involved was 3.2 bones per patient.Involvement of more than one craniofacial bone occurred in 70% ofpatients. The maxilla, orbital, and frontal bones were most commonlyinvolved. CT images appeared sclerotic or homogenous in 34%, mixedwhite and dark or heterogenous in 55%, and cystic in 11%.

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