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
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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.
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Polyos o ic Fib ous Dysplasia o he Mandible and Bony Nasal Sep um 51