Polyostotic fibrous dysplasia of the mandible and bony nasal septum with intracranial expansion

CASE REPORT Polyos o ic ib ous dysplasia o he mandible and bony nasal sep um wi h in ac anial expansion Ju a Numminen * , Ma kus Rau iainen Depa men o Ea and O al Diseases, Tampe e Uni e si y Hospi al, Tampe e Uni e si y School o Medicine, P O Box 2000, 33521 Tampe e, Finland Recei ed 28 May 2015; accep ed 4 Augus 2015 KEYWORDS Fib ous dysplasia; Nasal ca i y; Skull base; Endoscopic sinus su ge y Abs ac Fib ous dysplasia is an uncommon benign umou in he nasal ca i y. Due o adjacen i al so issue s uc u es su ge y is o en he ea men o choice. We epo a a e case o polyos- o ic ib ous dysplasia o he mandible and he e hmoid bone including nasal bony sep um wi h in ac anial expansion, and include a e iew o he li e a u e. The umou was success ully esec ed using a combined ansnasal and c anio omy app oach. Fib ous dysplasia is a a e benign umou in he head and neck egion, which o en limi s i s g ow h du ing he ollow-up pe iod. Ca e ul moni o ing o he pa ien is necessa y because in some a e cases he e can be se e e symp oms due o he g ow h o he umou , which a e indica ions o immedia e su ge y in he head and neck egion. Ó2015 Egyp ian Socie y o Ea , Nose, Th oa and Allied Sciences. P oduc ion and hos ing by Else ie B.V. All igh s ese ed. 1. In oduc ion Fib ous dysplasia is a benign uncommon diso de o a bone. I accoun s o abou 2.5% o all bone umou s and 7.5% o benign bone umou s. 1,2 Two o ms o his diso de ha e been ecognised. The monos o ic o m ep esen s 70% o he cases and is de ined as ha ing only one ocus o in ol emen . The mos common si es a ec ed a e he emu and cos ae. Polyos o ic ib ous dysplasia is cha ac e ised by mul iple oci in ol ing se e al bones. The polyos o ic o m is some imes associa ed wi h McCune Alb igh synd ome (MAS) ep esen - ing as an endoc ine diso de and ca e ´-au-lai spo s. His ologically ib ous dysplasia has been desc ibed as an o e g ow h o nondesc ip ib o ic issue wi h a seconda y phenomenon o bony me aplasia. Mo e ecen ly, i has been shown ha he unde lying cause could be a complex de ange- men in he unc ion o he cells in he os eogenic lineage. 3,4 A mu a ion in he subuni o an in acellula G-signalling p o ein (Gs-a) leads o a loss o guanosine iphospha ase ac i i y o he Gs-a. This leads o inc eased in acellula cyclic adenosine monophospha e (cAMP) p oduc ion, inc eased cell p oli e a ion and inapp op ia e cell ma ix p oduc ion. I has also been epo ed ha cul u ed cells om MAS pa ien s sec e e excessi e in e leukine-6 (IL-6) and ha e an inc eased amoun o in acellula cAMP. 5 The na u al cou se o he disease is a iable. Al hough he disease is usually p og essi e be o e he age o 30, some o he lesions con inue g owing well in o adul hood. 6,7 The e a e no uni o mly accep ed guidelines o ea men o his dis- *Co esponding au ho . Tel.: +358 3 31167651. E-mail add ess: [email p o ec ed] (J. Numminen). Pee e iew unde esponsibili y o Egyp ian Socie y o Ea , Nose, Th oa and Allied Sciences. Egyp ian Jou nal o Ea , Nose, Th oa and Allied Sciences (2016) 17,49–51 HOSTED BY Egyp ian Socie y o Ea , Nose, Th oa and Allied Sciences Egyp ian Jou nal o Ea , Nose, Th oa and Allied Sciences www.ejen as.com h p://dx.doi.o g/10.1016/j.ejen a.2015.08.007 2090-0740 Ó2015 Egyp ian Socie y o Ea , Nose, Th oa and Allied Sciences. P oduc ion and hos ing by Else ie B.V. All igh s ese ed. ease. Op ions include moni o ing and medical and su gical ea men . The e a e many epo s o medical ea men wi h bisphosphona es showing a dec ease in pain om he lesions bu no esolu ion o he g ow h i sel . 8,9 Timing o su gical ea men is a iable depending on he loca ion o he lesion and he symp oms i causes. We desc ibe a e y a e case o polyos o ic ib ous dysplasia in ol ing he mandible and he base o he skull as well as he e hmoid bone wi hou any o he signs o MAS. 2. Case epo A 14 yea -old boy was e e ed om ano he hospi al o he Depa men o O o hinola yngology, Tampe e Uni e si y Hospi al o biopsies. The pa ien had a his o y o h ee yea s’ ecu en igh -sided mandibula swelling and se e e pain. O he wise his medical his o y was un ema kable. In he e e - ing uni , he symp oms we e i s mis aken o be o pa o id o igin and an ul asound and sialog aphy we e ob ained wi h no signi ican indings. The pa ien was ea ed wi h epea ed cou ses o an ibio ics wi h no elie in he symp oms. An o opan omog am (OPTG) was ob ained and i showed a lesion suscep ible o ch onic os eomyeli is and he pa ien was ea ed wi h a cou se o o al clindamycin and me onida- zole. Du ing he ea men he symp oms educed somewha . La e on, a magne ic esonance image (MRI) was ob ained. I showed a lesion (size 2 cm in diame e ) in he mandible and inciden ally ano he lesion (size 3.5 2 cm) in he an e io c anial base con inuing in o he nasal bony sep um and e h- moidal cells (Fig. 1). The pa ien was hen asked abou nasal symp oms and a his o y o nasal obs uc ion and discha ge was gi en. A scin ig aphy was also ob ained and i showed no o he lesions. Bo h lesions in he mandible and in he sep- um we e biopsied. Bo h biopsies showed ib ous dysplasia. The pa ien was ea ed wi h a cou se o bisphosphona e (disodium clod ona e – Bone osÒ– 2400 mg a day o 6 mon hs) du ing which he symp oms in he mandibula egion esol ed comple ely. Eigh een mon hs a e he ini ial e e al, howe e , he de eloped blu ed ision, occasional double ision and his nasal conges ion wo sened, and he was e e ed again o consul a ion. Compu ed omog aphy (CT)-scan showed a slow g ow h in umou size in he nasal ca i y. I was measu ed a 5.2 2.2 cm in size. The su ge y was now indica ed because o exace ba ion o he nasal symp- oms and de elopmen o occasional ocula symp oms. This was p esumed o be a possible symp om p esen a ion o he in ac anial g ow h o he umou . Combined app oaches we e chosen as an ope a i e echnique. The neu osu geon pe - o med an an e io c anio omy h ough a co onal incision. Du ing he c anio omy, a neu osu geon comple ed he sub o- al esec ion o he umou using dissec o s and cu e es. The du al de ec was hen epai ed wi h one piece o empo al as- cia, ib in glue and an E hiso b TM Du a Pa ch. The esidual umou in he nasal ca i y was hen esec ed endoscopically h ough he nose by an ea , nose and h oa specialis using endoscopic sinus su ge y ins umen s and angled scopes. The pa ien had no immedia e complica ions and was discha ged a ew days la e . The ollow-up CT nea ly wo yea s a e he su ge y showed an ‘‘egg-shell”o he bony sep um illed wi h e en ion luid bu no esidual umou g ow h. In he con ol isi 10 yea s la e , in 2014, he pa ien was symp om- ee. 3. Discussion Fib ous dysplasia ep esen s a a e benign bony umou ha occu s p edominan ly in young adul s. The mos common loca ion is he ho ax and ex emi ies, and i is e y a ely seen in he head and neck egion. I has been epo ed ha 50–100% o pa ien s wi h a polyos o ic o m o he disease ha e c anio acial in ol emen whe eas in he monos o ic o m only 10% ha e a lesion in he head and neck egion. In c anio acial in ol emen su ge y is indica ed i he lesion com- p omises ision o aes he ics o causes o he signi ican discom- o . 10 A ecen epo o 26 pa ien s showed c anial in ol emen o he ib ous dysplasia, including he on al, pa ie al, sphenoidal, and occipi al bones in 13 (52%) pa ien s. The umou was loca ed in he maxilla y bone in 9 (33%) pa ien s. Tempo al bone in ol emen was ound in 3 (11%) pa ien s. One pa ien (4%) had mandibula in ol emen . 11 E h- Figu e 1 P e-ope a i e CT images. (A) Image wi h co onal sec ion shows a huge ib ous dysplasia in nasal sep um eaching h ough he skull base. (B) Axial sec ion shows he wid h o he umou , which is passing om skull base de ec . 50 J. Numminen, M. Rau iainen moidal in ol emen is a a i y. To ou knowledge, a polyos o ic o m o he disease in ol ing he e hmoid bone and nasal sep- um has no been p e iously epo ed in he li e a u e. We assumed ha ou pa ien ’s isual symp oms we e caused by he op ic ne e being i i a ed by he umou . A e iew o he li e a u e showed ha he pa hogenesis o he isual symp oms is no ully unde s ood ye . I has been p o- posed ha dis u bances in he enous d ainage o he op ic ne e and ul ima ely dec eased e inal pe usion could be he unde lying mechanism. O he heo ies include ac ion on he op ic ne e, spon aneous haemo hage o sinus mucocele o - ma ion. 10 I has gene ally been accep ed ha umou s encasing he op ic ne e should be esec ed and a decomp ession o he op ic ne e should be pe o med. Some au ho s ha e sugges ed ha decomp ession should be done p ophylac ically in cases wi h op ic ne e in ol emen . Howe e , a ecen s udy showed ha in a se ies o 107 op ic ne es ha we e 100% encased, 88% exhibi ed no e idence o op ic neu opa hy. The e was no di e ence in age be ween g oups wi h less han 50%, 50–99%, and 100% encasemen , and pa ien s wi h op ic neu- opa hy we e no olde han hose wi hou op ic neu opa hy. These da a suppo he conclusion ha encasemen does no p og ess wi h age and ha inc easing age does no necessa ily b ing wi h i he likelihood o op ic neu opa hy and blindness. Based on his e idence, he au ho s do no ecommend p o- phylac ic decomp ession on pa ien s wi hou symp oms me ely as an a emp o p e en possible u u e op ic neu opa hy. The au ho s also show ha ision loss is mo e o en associa ed wi h ei he g ow h ho mone excess ela ed o MAS o an aneu ys- mal bone cys . 12 Ou pa ien epo ed in e mi en blu ed ision and double ision al hough CT showed no di ec op ic ne e in ol emen . Pos ope a i ely all he isual symp oms we e esol ed, bu he p ima y eason o he double ision and blu ed ision emained unclea . 4. Conclusion Fib ous dysplasia is a benign bone lesion cha ac e ised by p og essi e eplacemen o no mal bone by ib ous issues. The clinical pic u e is a iable om asymp oma ic pa ien s o pa ien s wi h se e e symp oms p esen ing as pain and pa ho- logical ac u es. Fib ous dysplasia and especially a polyos o ic o m o ib ous dysplasia is a eal a i y ha p esen s diagnos ic and ea men challenges o us. As ou case demons a es, bony lesions in he mandibula egion can some imes mimic so is- sue p oblems. One should always s i e o a his ological o a leas a adiological diagnosis, especially when ea men a emp s ha e ailed. This case also demons a es he need o ca e ul moni o ing o umou s loca ed in a eas whe e he umou g ow h can lead o possibly de as a ing complica ions. Con lic o In e es None. Re e ences 1. Ho man, Jacoway JR, K olls SO. 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Polyos o ic Fib ous Dysplasia o he Mandible and Bony Nasal Sep um 51