Journal of Neuro-Ophthalmology, June 1981, Volume 1, Issue 2

Management of primary optic nerve meningiomas. Current status--therapy in controversy.

The diagnosis, pathology, and management of primary optic nerve meningiomas are described from a study of 55 patients collected from the author's own series and the files of the Registry of Ophthalmic Pathology at the Armed Forces Institute of Pathology. Diagnosis may be confusing in childhood, especially in the presence of neurofibromatosis when it must be differentiated from optic nerve glioma. The disease is especially aggressive and life-threatening in the young. Of 15 patients under age 20 years, four died from intracranial extension, five are alive with recurrence, and two without recurrence. In the age group of patients between 20 and 35 years, the disease becomes less aggressive but still life-threatening. Of 14 patients in this group, one died of intracranial extension, four are alive with recurrence and seven without recurrence. In the older groups, the condition becomes more indolent in its growth pattern. In this series, no patients over 35 years of age have died from their disease. Surgical excision of the optic nerve and tumor for diagnosis, followed by craniotomy with total removal by unroofing the orbit and optic canal is advocated in the young. If there is orbital spread, exenteration should be performed. Craniotomy and more radical therapy are only indicated if intracranial extension is demonstrated. Radiotherapy is reserved as an alternative to surgery in the older age group.

}. e/in. N('ur(>.,>phth,llmo/. I: 101-117, 1<IS l. Management of Primary Optic Nerve Meningiomas Current Status-Therapy in Controversy MELVIN G. ALPER, MD., FACS Abstract Tht' di.llgnosis, p.llthology. .lind rn.lln.llgemenl of prim..-y optic nt'TVe meningiom.lls.llre dt'scribt'd from • study of 5-5 p.lltients coll«tt'd from tht' .lIuthor's own st'rit'S .lind the files of the Regislry of OphthOilmic r.llthology.llt lht' Armt'd Forct's Inslitule of rOilhology. Di.llgnosis m.lly bt' confusing in childhood, t'5p«i.llIl~' in lht' preSt'nct' of nt'urofibrom.lltosis when il must be diHt'rentiOitt'd from oplic nervt' gHom.ll. Tht' diseue is especiOilly .lIggressive .lind Iift'-thrutt'ning in Iht' young, Of 15 p.lllit'nis under .lIge 20 yeus, four died from inlr.llcuni.lll ntt'nsion, fivl' .lire .lIlivt' with recUlTence, .lind two wilhout recurrence. In thl' .ge group of p.lltients between 20 .lind 35 ye.llfS, thl' dise.llS(' bt'coml's less .lIggressive but still life-thre.lliening. at J.I pialit'nls in lhis group, one died of inlr.llcr.llni.lll elliension, four .lire .lIlive with r«urrencl' .lind seven without recurrence. In the older .lIge groups, the condilion bt'comes more indoll'nl in its growth p.lltlt'Tn. In this series, no p.lltients over 35 ye.llrs of .lIge h.llve died from Iht'ir disuse, Surginl excision of the optic nerve .lind tumor for di.llgnosis. followed by (uniotomy with tol.lll remov.lli by unroofing the orbit .lind optic unial is .lIdvocated in the young, If there is orbit.lll sprud, exentt'r.lltion should be performed. Cr.llniolomy .lind more r.lldiul ther.llpy .lITe only indiuted if intr.llcr.llnial exlension is demonstr.llted. R.lIdiolher.llpy is reserved aSOIn altt'Tn.lltivl' 10 surgl'TY in the older .lIge group. Introduction The man<lgement of primMy optic nerve men· ingiomas has become quite conlrovE'rsidl in recent years, and is currently under~oin~ d mdjor reo evaluation. I .;' In the past. the tumor W<lS thought to be exceedingly rdre. '·" Conventiondl treiltmt'nl, upon diagnosis, was surgical excision of the optic nerve <lnd tumor in the belief thdt this was neces­sary to S<lve life by preventing intr<lCrdni<l[ exten­sion .... ,~ However, the dppMent indolent growth of Clinic.l rrofe~sor of Ophth.lmo!<>~y.nd f"I'UWI"I(K.1 SUfl(l'ry, The George W.~h,nl(ton UnlVl'r~,ly Ml'd,c.l ~.-h",'I; St'n;<>r Attending Ophth.lmol~isl.Thl' WJ~h'"KI"n H,,~p'I..1Center, W.~h;nKlon,DC June 1951 the tumor when not operated to~ether with the frequency of recurrences <lnd poor prognosis for retention of vision Jher surgery hdve encouf<lged m<lny ophthJ.lmologists to adopt a conservdtive dppro<lch to the m<lnagement of this lesion in the mdjority of patients.t-"· ,,; Recent reports imply that, in some patients, d primdry perioptic meningioma m<lY be successfully removed by <l lateroll dpprodCh utilizing microsurg­ical dissection techniques~·4. ~ with retention of pre­operative vision and/or restoration of visual func­tion which hdS been lost. Indications for this type of surgery, however, hdve not been deline<lted, nor has there been <ldequate follow-up to judge its efficdcy, Since the <ldvent of computed tomography (CT) in 1973, prim<lry intraorbital meningiom<ls have been shown to occur with a much higher incidence th<ln was previously reported.'·"· 10, I" Because this lesion may now be encountered with incre<lsing frequency. it hds become important for the generdt ophth<llmologist to be f<lmiliM with its p<lthology, clinic<ll course, di<lgnostic criteri<l, <lnd tredtment. It is Ihe purpose of this communiC<ltion to report our experience in treilting patients with proven primary optic nervt' meningiomds; to describe the pJ.thology, clinical findings and recurrence dnd surviv.ll r<ltes noted in J. retrospective study of Cdses from the Registry of Ophth.llmic r ....lhology .It thE' Armed Forces Institute of Pathology; and. findlly. to present our current choice of Ire.llment of primary optic nerve meningiomils. Sites of Origin Meningiom.ls found within the orbit molY arise dS prim<lry growths from the meninges of the optic nl,'rve or, most commonly, may invade tht' orbit secundary to sites of origin within the cr,miill CilV­ity. Theoreticillly, dn orbitd! menin~iomd m<lY d[SO drise from ectopic mC'ninge.ll tissue which h<ls been pinched off within the orbit during embryonic tife (Fig. 1). Primary optic nerve meningiomds arise from the Cilp cells of the dt<lchnoid villi of the optic nerve sheJ.th .mywhere <llong its course, but usually in 101 Prim.H)' Optic Nerve MrnlllJo\ium.ls Figure I. $Ill'S of ongln of orblt~l m""ln8IOm..s. II Th.. most common source IS ,nv<IIsion from <II pr,m~ry loc~tion ,n IhO' n~n••1uv,ty (AI or, I"s frO'quO'ntly. the opt'c un.. IIBI. ~) The me",ng" of the optic nerve Within the Orbit Ie) especi.lly neM the opllC for..men ~nd less commonly nUr the globe (Dj ..nd ]) ectop'c memngul1l~5ue '" the orbit Inot show",) lh«>r..tlc~lly m~y give origin to pnm.. ry "'tr..orb,,,,l men"'glom..s. the vicinity of the orbitdl side of the optic fordmen in the dpex of the orbit. These primdry tumors lie within the subdurdl spdce of the optic nerve dnd ,Ire sometimes difficult to differentidte from men~ ingiomds which hdve second<lrily extended from ddjdcent structures into the sheath of Schwdlbe (subdurdl space). Cushing dnd Eisenhdrdt '" stated that meningiomds Mising from the olfJctory ~roove have d propensity to invade the orbit by ~rowth in the subdurdl space of the optic nerve, but in our experience, the sphenoid wing hds been d more common source for these secondary tumors. Course of Primary Optic Nerve Meningiomas Taking onKin from the ("ap c('lIs of the .Hachnoid villi, the mt'nmJo;iumd grows within the subdural "'p.-i\'(' rn.m th('''t,· Slt~ lIf Ilrigin th(' tumor grows "p ,·r ~1"\""11 Ih. dur.11 ~tlt'.lth. invJding the durd • I., I,,,. ,',. :" .•1 1:>1",,.j "urrly. In some instances, the tumor ffidy encircle the optic nerve, compressing it dnd cdusing atrophy with profound ViSUdl loss without invading the nerve itself. In oth('( C<lses, the tumor invades the optic nerve, growing dlong the septde as d course of least re­sistance. It mdy invade the neural tissue itself to present clinicdlly .IS a primary tumor of the optic nerve, or grow into the space around the central drtery dnd vein to occlude these vessels. Some­times, d tumor may dlso spread into the globe including the sclerd, choroid dnd retinol to «IUS(' the clinical picture of d "solid" retinal detachment. Minimal linear exophthdlmos molY exist at first but wh('n the tumor breaks out into the orbit through the durdl sheath to invade the extrdocular muscles <Ind other perineurdl structures, exoph­thalmos increolses. Restriction of gdZe then occurs, most commonly on UpgdZe but usually in the field of dction of the muscle involved. Pain may appear from compression or inVdSion of sensory nerves. Posterior growth molY occur up the nerve sheath into the optic undl where occdsion<llly it causes bony changes. Usually, however. the bone dnd bony optic Cdnal are unchdnged. Intrdcrdnial inVd­sion molY then occur with involvement of the chiasm, interndl cdrotid drlery, dnd contrdlateral optic nerve. It molY invade the sella turcica to present ,IS d pitUltdry tumor. Further intracranial extension may result in dedth by compression of vitdl structures. This dggressive growth is especially true in patients under 20 years of age (Figs. 2.l dnd 2b,3-5). As the tumor expdnds within the durdl sheath, chronic compression of the optic nerve and its blood supply occurs, ledding to the formation of retinocilidry shunt veins. These are cdused by compression of the retrobulbar portion of the cen­trdl vein. BypdSS channels then develop within the eye by dildtion of preexisting vessels that connect the centrdl relindl vein <Ind peripapilldry choroidal veins. (Figs. 6J and 6b, dnd 7). Fluorescein angiog­rdphy defines these abriormdl shunt vessels which fill early in the retindl venous phase. This demon­strates in dyndmic fdshion the fdct thdt incredsed pressure within the edemdtous prelamindr portion of the optic nerve interferes with venous blood flow throu~h the centrdl retindl vein that pdSSes through .In unyielding sclerdl canal. Venous blood, therefore, follows the path of lesser resist<lnce into the cilidry venous system; from there, it It'dves the eye through vortex veins close to the equator of the globe. Eventually the retinociliary shunts be­come Id~er dnd more visible on the surface of the disc (Figs. 8, 9.J and 9b). When the normal circuld­tion is invdded by the tumor or compressed suffi­ciently to occlude either the artery or the vein by invdsion through the optic nerve septde to sur­round these structures, neovasculdrization of the dnterior chdmber angle dnd rubeosis iridis with secondary gl<lucoma molY occur. joumd[ of Cliniul Neuro-ophthdlmology June 1981 Figures 2d dnd 2b. (,n>wth p.,lIen" ,,/ pnm.,ry Illlr.'l'rb,t.,1 m('njng'(l,n.J~ Illu ... tr~ltllln .... tll Jl'mlln ... tr~lh' grllwth l'h.H.lderis­tic" of pnm.Jry intrJllrbitJI Tll('nmgllHn.l" 111 ....lggit.ll (2.)) .uld tr.ln.,dxiJI (2b) view." In thpc..,{' Jr.lwing... , the tumur I'" ... htl\vn to ,Hi.,c in the orbitdl ..lpl'\ ne.H the I,.lplll ftlf.lInen. It In.IV then gn>w down Schwdlbe', 'pdll' (I) intll the gllllw "bl,t"r.,ting th" p'dl blood supply or up Ih" 'p,ll'" (4) Ihn'ugh Ih" "ptll ldn.11Il) "ncd'" th" ch'dsm dnd/'H Ih" ,nlern.,1 cdn>lId .1rt"ry, In llth"r in,tdn(",.•1 m.IY br"dl<. thn>ugh th" Pl'rillpllc ,h".,th, (2) .ntll th" orbit 10 'Ilvdd" th,' ('xlrd,'cuIM mu,c1,', llr .t 111.1y 'pr".,d I11to Ih" llpllC nerv" ,t,,,lf (3), Alper 103 Figu ... J. Drdwinj!; of crr;>SS , ...ct,on of th ... optic n.......... to d...monSlr~te Krowth ~ttem vf d perioplic meninKiom... Ari'inlt m the dTdchno,d ,h"dth, th tumor KTOWS ,n th... subdurdl spdce (Schwdtbe\ SpdC...) to Cl.(umscrib... th ... nl' In som... C"''''', 'I growslh",u!\h 11'1... durdl ,hNth I" mVdde th ... o.b,tdltlS'u"'s 121 o. ,n olhE"s. ,I mdy inVddE' th.. optic nl' ........ ilS... lf Ill· Figur.. 4. P....;optif men;nj!;;t>md '" d ]",·v"'dT."ld 1"'"'.01,' ,ur· '(lund,nj!; th,' "pli, n('........ f",m th,' ;:I,'!>" t<, th,' ,'h,..,nl, Th... .....'hn"id" m... I. dly Ih"I....n...d ,'nd th ..."·,, ,,·v,· .... (ll",P'(',;' ,,,,n .,t,,,phv "I th "pt" ,wrve Mdny r"'.lmm"m" b"di..., "'... f'''''''''' 1"- II A,n".,) I'",,,,, In,,,,,,t<·, "I 1,-"h"I"l'v An·,·~,i"n """I ... I 1'1l'7 A,,,,,,,) I ••".·, 1""",,,.- "11'.111,,,1,,1''1 ",);.,t,,·..., I'. ",I", _""I .'",,,. ·,'.IIn. Figur.. !i.e..", ~....:li,'n 01 sp..dm..n shown in Fig, 4. Th.. optic n........... " ,'''(umscnbed by Ih.. tumor which ...os.. in 11'1.....~ch­,,,,,, I ,h('dth {dusinlt dlrophy. Th.. dU'dl shulh is inudl'd (sing! .. ",,,'wI dnd b.ok..n Ih.ough ldoubl~ ..rrowsl ( duc..d from XIll. A.mE'd ForcE'S Inslitut... of rdlhology Acc ssion number 1.'51014. Ann..d Forcf'5lnslilUlr nr-g..livE' 71-4679. H..m.lloxylin dnd ....lSin s'din, Journal of Clinical Neuro-ophthalmology Alper '--11--0.-.. ......., ... •, \llf----..... -- 4>-t--l-\-+--CAr*W l'IIIlnIIIa' ~ f----..... figur' 6 ... D'J"·m,, "I the """n,,1 "",'uIJll\m .,Irh\' "pI" n('rv<' Jnd ..'tlnJ Figure 6". DrJwinl'of J promJry ml'JdurJI m"n,nj(,"mJ 'Ju~m" ."roni,- ."mpr('~.ion of the Opl'c nel'Ve Jnd ,I. ble>o<l .upplv Th'$ 1....,.1. '" ,-h",,,,,, opl..- di>c ~wdlon" .md rhe i,',mJI,,'" ,~f r('\,noc.hJrv ,hun! "f.~I. th.1I nm'll'<:l th ... «('nlul f<'l,nJI ,",,'In ,,,,th IX'ropJp,lIJ.ry ,h" ...."dJI v(',n. Jnd ult,mJlcly th .... v"rtf' "elM figure 1. lunj(iludinJI,cdiun t"roo,,'" m{"nin",,,,,,,, ,,( "pi'. n('('V,' Ih.ll h.I' ,·,m'p,,,,..,,d tl>e nt'''''' JJ\d '"Ju"'d e,li", ...,;"..1,hunt vn"'1 "" tl> "{'No' h('Jd (Jfr.,w) (,...do.{'d fwm Xl51 Armed rurn" In,t,l"'" "f I'JlhuJ"l':Y 1\ ,,'" numb<'r 1~!\10n .lnd nej(Jhvc -,q.I057J. H.. mJh,~ylln Jnd ~;n ".lin. June 1981 lOS figur.. 8. J'h<,t,',;r~ph (>f (>1'1', ,,~rv~ d~m(>"SI'~t"'l': p~l .. d,sc ~""..tl",;; wllh 1" ..5.. " .... (,f uph...·d,M)' shun! V~IIlS r~rr0wSJ ,,, ~ p.'l, .."t w,lh p"m~ry "'lr~",btl~1 m"'''''',;;o'''J P.llhology Only two histop<lthologic forms of meningiomd dre ch.lTdcteristic of prim<lT)' intraorbit<ll origin. The meningothelial or syncytial type and the mixed or tr,msitional type with or without psammom.'l bodies Me these two cell types found in the orbit. From his broad experience .at the Anned Forces Institute of Pathology, Zimmerm,m1i . I~ is loathe to accept ,lfly meningiomd Mising within the orbit as primaT)' unless it is One of these two cell types. The meningiotheliom<ltous type (Fig. 10) consists of clearly defined poylgon<ll cells .ur.mged in sheets separ<lted by vascular tr<lbecul'le. The cyto­pldsm is homogeneous dnd the nuclei,He sphericJ!. Often the cells are divided into Idrg(' lobules by bundles of vdscular tissue or hYdlinized collagen. Mitoses .He uncommon and r('tkulin fibers .lre Sp.Hsc. The mixcd or trdnsition.ll (Fig. II) typ(' IS chdr,Jctcrized by whorl form<ltion of concentricJlly dTranged .ells which Me spindle or (lv,Jl in Sh,lpt'. The whorls may nmsist of J number <,f cells .H­TJnged Mound e,Jch other or Jround str,lnds of conn('ctivl' lissure or blood vessels. I\;,lmnwm.l bodies (Fig. l2J .He found in both types but Jre more common in the trJnsitillll,ll f'>rm. They r('sult from hyJlini1.dtion dnd deposition of (..Ikium SJlts in the degenerJt('d centers (,f the whorls. They Jre not diJgnoslic of meninginm.l JS su(h by them­selv('~ Jnd <ln', in fJct, found in other M('.IS sudl ,IS in C<lTcirHlmd of the thyn'id ,md 'lV,IT)'. The (lthl'T forms of menin~l(lmJs d.lssified by Ru~<,dt J<, fibrobl.lstk .Illd .1ll!'\lllbIJsttc types Jn' h'und only III th(' ,.rbit by inv,lsi,Hl fr,lm their sites "I "nl-:ltl wllhill Ill(' illtr,l(T.ll,i,11 (,Wlty. rigur.. 9~. ~Mm.' p~ll(""l JS ,n f,g 8. An.... IJI phJSC oilluo,..s,ci" ~"~I0!,:'Jm rigu,(' 9b. 5Jm~ r~r,ctll ~~ r,~~ :' J"d"~ EJrly \"~""us phJs('. 1\:"IC IhJt <'pr,'nh~,,· ~hUf\l "(,"'5 h~,,~ flll....1 ('Jrh·. whd~ "rhcr b'J".-h~> "f II,.. <cntrJI r~hnJI "cln ,cmJ,n unfilled w,lh flu'" ArJchnoid,l1 hyperpbsi.l m.lY be confused with prill1JT)' orbitJI meningiomJ Jnd hJS recently been point<.>d <'ut bv Co....ling .md Wright I\' as <l possible c..'nfusing p.lthol,,'gic diJ!'\n....sis when dssociated with <1ptl( n(,l"\,e gliomJs. However. ar<lchnoidal hyperpl.\si.l rl'm,lins intrJdur.l1 even though it gr<)ws exuberJntly. whereas growth in most men­ingiomJs invJdes through the dura into the sur­wunding orl,-litJI structures. In addition, following surg('f)' <)fbit.,1 re(u rrence with or without intra(ra­ni, ll t'xtensi,)ll is,J common complicJlion for orbital ll1eningi,)mJS, whereas this never hdppens with .IrJchnoid.ll hyperplJsia. I~. ~~>. ~I [{ecenlly, IJkobiec <lnd co-workers~~ have pointed out that in optic nerve gliomas electron­microsc0py r('veals acluilJ invasion of the leplo­mt'ninges with tumor cells instead of re<lctive ar- Journal of ClinicJI Neuro-ophthalmology .ldllhlid.ll hypt'fpl.i:oi.l. This 1:0 ,I (urth.-r Jr((l'fl'll­tidlin~ ((".llur(' in ':.lSI·:O llut h.l\'l· bl'l'n 1'"n(u"I'J with m('nin~il'nl.\Sby s..'nll' p,lth.,I"~I:O"', Other .:.'nJiti"ns wh..-h lUuSt bl' dit(t'fl'ntl.lh'J from .,rbll.11 ml·nin~i"n\.1 hbl"I',lth"l.~i,·.III\' .In' jU\,("lllk .l,"li\·(' .>ssifyin~ flbn'lII.l, mi"'d '·l·il Iu­m.' rs "f thl' [.l.:rim.l[ ~l.lIlJ.•111.1 ht·1l1.Ill~l,'pl'rK)'­t(' Im.'s. Alpn Clinical rresenlation Sp,,'I1\'('r' FriS<'11 ('I .IL.:!' Jnd l,tht'rs"\'!!' rt"'C("nlly d('(lIwJ .1 dinie.11 tnJJ which th('y fclt to b(' diillo;­11"... 11. of pt'nuptic n('rvt' !>ht'Jlh m('nin~lOmils. This tri,lJ tlm"l!>t.. of lun~-SI.lnJinlo:visudiloss. pJle disc I'Jt'm,I, ,1I'IJ llptllciliMy :ohunt vdn:o. SUSoiC ('t dl. c ., J("scriNd .1I1(1th('(" typl' of ml·ningiom.l which oc- Figur. 10. MN,.n~,,,,h.lt ...m..I,'us l,yo,-yh..l, o,~nmt:,,,m .. "~I ,'rl,( n..,v... one (If Iw" h,.I,'p.. th. ol,~l( IVJ>f" >f'('O ,n ",b,1 lXl00l Armed r ..,,(t"> Inshtute of PoOth"l~y n"R.. t,.... 70_5.:l~"l Hem"lolyl,n ..nd ..",m sloO,n Fig\l'" 11. Th\' "tl\l" h"IUr'-'lhul,-.:" lVf'<' "f 1'''01.,,,, "rh, Il.'tv\' n"'''''',:,,·m.. " Ih .. n,,, ..d (rr..o"I"m..h m('n,n.:,,,moO lXIISI. A,m,..t rllll'" 1'''IIIlH,· ", l'.,th"I".:v '''',:''II\'(' 71_.0(>711. H\'m"lo~yho ..od ....,m .r.'''' June 19!'or 107 Figur.. 12. rh"t"microj(r~ph "j SP'('<"I",~n ,hu"'o '0 fij(. 4 Wh(>r1~ ,,( ~bo('rm~1 ~,~chn(',d crll~ ~rp~r6jrd by f;bH'U~ S('pl6r ""th p.s6mm"m6 bcodir~ Ch6rJClero,l,c <>f m"rd (lr60s,I,on6lJ ml'n;o!t,omJ dr<' ooll'd (X IISI. Armrd r ,,,crs (OSI,lulr ,,( r Jlholo!tY Accrss,,:lO oumbrr 1310;"Z. N..gdl;Vl' numb... 7I·407S. Hl'mdlo'yltn Jnd ros,o sld,n curs in the orbital ilpex in middle-aged women, presents with unilateral progressive viSUdlloss dnd typical signs of optic nerve compression. and is dssociated with norm<l) neuroradiologic studies. They entitled this type of meningioma "th(' impos­sible meningiom.l" <lnd reported their findings in the Archives of Neurology in 1977. From the clinical work of W<llsh~~'~~ dnd the report of Kdrp et al.,l' who studied 25 cas('s of meningiomil ilt the Armed Forces Institute of Pa­thology, I have summ<lrized their findin~sof orbital meningiom<ls in young patients. Their combined conclusions were th<lt these lumors Me infrequ('nt but not rMe under 20 yeMs of dge. Ten of 25 ("If 40% occurred under twenty yeMs in the A.F.I.P. series. There is often a delJy in diagnosis. Gli,'m,l is usudlly the suspect('d di.l~nosis, Th(' prognosis for retention of vision is poor and radical surgl.'ry is th(' tredtm('nt of choice. In IQ77. from.1 study of pdtients at the MdYO Clinic, Hollenhorst, Hollen­horst <lnd MdCC<lrty'" noted that prognosis for retention of vision WdS poor in this syndroml' with or without tre<ltment. Case Reports Our first ('xperil'nce with this «mditi<m occurred in IQ67 in a 17-Yl'<lr-old white female who pre­<' l'ntl'd with right progressivE' visudl 1<'55 and in-duced hyperopid of +4.00 sphere which corrected the vision in this eyt> to 20/30. Right monoculM exophthalmos of 3 mm W<lS present. There was p<lle swelling of the optic disc. dnd a cilioretin<ll shunt vessel WdS present on its surfdce. All routine studies including skull x-rays with views of the optic fordmina were normdl. Venogrdpy and orbi­tography. howevt>r. demonstrdted iI mass within th(' muscle conE' repldting tht' optic nt'TVt'. Of cours(', in those d.lvS we did not h.lve computed tomogr.lphy sC.lnning which would undoubtedly h<lve shown us th(' diagnosis (Figs. 13-ltO). Consultation was sought with Dr. Fr<lnk Wdlsh and oth('rs. A differentidl di<lgnosis favored d gliom.1 o\'t>r a possible infldmmdtory orbitdl pseu­dotumor. No one thought of a perioptic menin­~ ioma. Aft('r prl.'sentation to Dr. Walsh's Sdturd<ly m~lrning conferl.'nce dI the Wilmer Eye Institute, it was dl.'cided to biopsy the mass. This was dccom­plished and a lypic<l1 mixed~c(>ll-type meningiomd W.15 di.lgnosed on histop<lthologic examination Since none of us had ever tre<lted dn orbitdl nll.'ningioma in dn ..dolescent, w(> were faced with .1 dilemm.l in m<lndgement. A review of the literd­ture rE'vedled <l simildT lack of experience. The patient W<lS once dgain presented to Dr. Wdlsh's nt>uro-ophthalmology clinic for opinions as to fur­ther m<lndgement. Severdl courses of therapy were advOCdtl.'d. The neurosurgeons suggested d crani-laurn.! 1 of Clinicdl Neuro-ophthdlmalogy F;!t.. r~ l.l..... r;·.y,· ...."ld t('mJI(' "',lh I',,\.:r('"~,,·,· ,·i'u.,1 1,\"~ JnJ .'·n,n, (""I'h,h..lm,'" "j Ih(' n.:ht ,'\'(' Alp<'r figur... 14. f'Jk <w"II('n "fo:hl "phr d"c w,lh "1o,,'N,,,.. 1 ,hunt \"',"('1 On ~urf....('. .>nd 'Iri .... in th .. p<'Sl<'""r plOl,', Indu,('d hyp<'ropi.> of +.HlO. "me 1981 Hgur~ IS~. I'A ",ew 01 orb,I.>1 p"'e~r ..m or p'>',e"t S"Own on lifo:. U demonst.dlE'S oulwdrd displ.rE'ment of ,hE' s<"<ond portion of Ihe sUp<'rio. ophthdlmoc VE'on (d"owl indicdtonl': dn inlr..conJI m..S'. Figur... Isb. Lll('r.. 1vww "f _ublr.. <"t('d "rbi!.>1 pl'tI,·b''I\r.'m ,h,'wl\ on f'l\_ IS., dE'm"nstr.>Ie, d('"..I",n uf Ih,· ,,,,,,und J'<,rti"n "I th .. ,up<'ri", "phthJlmir vtin (.'fOW) ;ndicdtinl\.>n onlrdcun.. t mdS', 109 Figure 16~. AP view of conlr"st orb,togr.om demonstra,c~ a f,lhnl\ defecl in the muscle cone (drfOwj rep!.octng lh.. nonnal oplic n..rv" p"nem. tigure 16b. L.o'<>r.or vi ..w of conlr.ost orb,'ogr..m demonSlr..,es an ",'raconal filling defee' r<>pl"Clng ,he norm,,1 p.ollcrn of ,he oplic n"rve (arrowl,ndic"'ing.on inrr"cor",l m.oss, otomy with either stripping of the tumor from all struclures involved or total excision of the optic nerve from in front of the chiasm to immediately behind the ~Iobe, Dr. eh.Hles Iliff recommended exenleriltion to be followed by a craniotomy for removdl of dny inlr<lCrJniJI tumor rem<linin~, No one ddvoc<lt('d a conservative approach. We dected 10 strip the tumor from th(' nerve. A crilniotomy W<lS performed. the orbit and optic Cdn<ll wer(> unroofed, <lnd the tumor w.1s noted to ('xtend from Ihe b,lCk of th(' globe to Ihe apex of the orbit <lnd into Ihl' optic can.J!. Th(' ('ntir(' tumor was stripped from the nerve (Figs. 17 ,md 1:1). Th(' p<ltient r('covered promptly but was quite blind in the eye foll\lwin~ the oper<llion. She was followed ,1,,~,'lv f"r th\' Ole'lt wver.ll y,'.:JrS.•md .Jfter 3 years " I." .1"" 'l'I,.H,'nl th.•1 th,'r(> W.IS.l r,'rurrence in the orbit IFig. 19). Because she had mdtriculated to Duke University she was referred to Dr. Joseph Wadsworth for further cMe, He exenterated the orbit and ,I repeilt cr<lniotomy was performed. The tumor had recurred within the orbit and had in­vaded the posterior globe of the remaining blind eye {Fig, 20). The patient remains alive and well ill this time, some 13 years after our initial encounter, without any evidence of recurrence. Since the advent of CT scanning we hdve oper­aled on eight more primMy orbital meningiomas. Coincidentillly, all of th(>sc piltienls have been un­der 35 years of dge. They have illl been blind or almost blind in the affected eye prior to surgeI)' and hilve been chdTdcterized bv the following find­ings: I Progressive long-sldnding ViSUdl loss. 2. Primary optic atrophy with dbsence of opto-cilid!)' shunts. 3. Young ddults in the third decade. 4. CT scan demonstrilted the tumor. 5 Polytomogrilphy demonstrated either an en­larged or normdl size optic cdnal without hyper­ostosis. b. There \\IdS dbsence of chiasmdl involvement. 7, All were operated by transorbital biopsy, which consisted of ,I modified Kroenlein ilpprodch and excision of the optic nerve and tumor from the globe to the orbital dpex. The diagnosis WdS established in this manner. All showed extension of the tumor to the edge of the specimen from the apex of the orbit. The p.1tients were then definitively treated by crani­otomy with removdl of the intracranial portion of the tumor in front of the chiasm and up to the area of surgery performed in the orbit. There were no sur~ical complications, and ,Ill patients have re­tained their blind eye without evidence of phthisis Joornal of Clinical Neoro-ophthalmology Alper ~ - ----- ~, ..f'~·· ' ..... .!J.. i' "/',' " '. • figu~ 17. ::'~m~ pJhent JS in Hll.s. lJ-lo. View ot th.. sur~iallieJd shows J pnm~ry oph( "cr...... me"in~iom .. surroundinll. the optir n.. rve. Thl' surg..on 's stnpplng th~ rumor from rh .. n.. rv.. in rhis vi ..w, figur.. 18. Gro~s speClm..n of rumor show" in fig. J6 d..mon­Slr. t.. s Ih.. groo"e left on rh~ rumor m.ss by th .. oplir n.. rv.. Figur.. 19. 5.om.. p..h.."t ..~ in FiK_ 13. with rerurfCnc{' 01 the prim.ry opric n..rv.. m..ningiom.. Not.. fulln ..ss in Ih.. upper lid of rh.. njtht ey... bulbi. All patients are alive and well at this time with the exception of one recurrence, which nect's­sitated exenteration and reoperation by craniotomy to remove the intracranial recurrence. June 1981 Diagnosis In the author's series of nine patients with pri­mary intraorbital meningioma. diagnosis was es­tablished by CT scanning. although hypocycloidal polytomography and arteriography were also per­formed in all to study the optic canal and intracra­nial ch,mges_ Pneumoencephalography was per­formed in only two of these patients. The CT scanning characteristics nott'd in tht'se patit'nts revealed the following: I. Tubular t'nlargement of the optic nerve (Fig. 21). 2. Bulbous enlargement of the optic nerve at the apex with distal tubular enlargement (Fig. 22). 3. Midorbital fusiform enlargemt'nt of the optic nerve with extension forward into the eye or back to the optic canal (Fig. 23). 4. Enhancement of the lesion after contrast. 5. On coronal section of the optic nerve there is an area of hypodt'nsity surrounded by a mort' peripheral dense area which is quite marked when the instrument is placed in the measure mode (Figs. 24J and 24b). We feel that this latter finding is pathognomonic of a primary perioplic meningioma. Treatmenl The signific.ant problems which f.lce us today in treating the individual p.ltient were considered by earlier authors such.ls Byers" in 1901 Byers stdted thdt the "d.lOger is not from recurrence in the strict sense of the term but from tht' continued devel­opment of tht' intracranial portion of the tumor which is impossible to remove at the time of the operation." Arnold Knapp,' in 1915, stdted that "these tumors dre generdlly regarded as benign, but it seems to me that Byers is quite correct in warning against this assumption even if it is years III fi&url' 20. 5.oml' p~lll'nl ~~ In fll(. 10 [~l'nttr ..'OI'd ~JH''Oml'n ~ho,,"!o In"~~lon of Iht po!.lt"Ot Jl:lobc- by Ihl' lumo. IxSI. AnnOI'd fmct"!o [nsIIIUII' "I P~lholC'gv Acc....."'n num~r 1~7.wt.. NtJl:olllvt "'74·11027 filtu,t 21. r'.,n","ull T .... ~" ,kn"'",'r••lt·' lul•.,I.....nl~f):ml'nt .'1 Ih.. I.. fr "J'l" n,'''''' t"'m ,h., uri-lI.,1 .,."." h' Ih.. 1o:1,>l>t- t~rr,'w) $" .." red,'nnl'd bv f,.unh'Io:,·n,·r~II""l •.l :<,',,-1 ... ~",..·r ,·nh~".N w"h ,·,""r~.' Figure 22. CT sun "f ~g·v..~r-,'Id fem~le demonslr~tes bulbous enlul'ement of leM opl'c nerve ~t orbll~1 ~pe, wllh tubul~r enlJI)!:l'ment to Ihe globe_ Xdn po:rformed by second-f\<,nerallOn [~11 t>ud sc~"ne. enhanced ",".th cOnlfJst. Alper bd\'r(' the intr,lcr.lni,\1 extension seems to show itself, " W,llter D,mdy,~'~' one of the most original of all n(>uwsur~(,\lns, st,lted in [922 th,lt "thl're .He two rroblems to mect in trcatm('nt of optic nerve men­in~ iom.ls_ First, .Ind most import.mt, r('store that which is ~one." H,lrvey Cushing,l" in 1937, stated th.lt "it is thc instincts .Ind tr,lining of the neuro­sur~ eon to ~et to the windw,lTd of the lesion with which he is to dcal .It the onset of tredtment." D.lndy probably st.lled it best when he sdid, "when .1 10c.lI operJlion hds been performed for .'In orbitdl tumor .lnd it is found impossible to complete its remavdr, the intrdcrdnidl operation must thl'n be done for unless th(' tumor can be complett>ly re­moved, totill blindness and dedth are inevitilble." Since we believr<> primary optic nerve m('nin­giomas Me potentially life-threatening tumors, in the past our indications for surgery hold been to perform total extirpation of the optic nerve and tumor in ol blind or almost blind eye i1nd to remove the intrJ.cranial portion by CTJ.niotomy. We had advocated more radical surgery in the young and adolescent. Because of recent controversy in tre.lt­ment, however, we dr<>cided to r('think our indica­tions for surgery. Bearing in mind the admonitions of our neuro­surgical .lncestors noted Jbove i1nd with our own June 1981 Figure n. CT scan of Idt optic n<''''e nwnon~ion,., in J ~5·VNr·,'ld (l'm.,ll' w,th J .l_yl'Jr history of pr"l':r<'losivl' v,suall",-. NOll' Ih... l>ulb.'u~ l'"IJr~e_ ment of th .. optic n.. rve w,th enh~ncem<'nt"f the uptic d,sc with ,",'nlrJst (arrowsl_ Tr~nsa~'JI "i('w p<'.furmrd by f"urih-g('n....aflon G.L tIlIOO SCdn_ n('r ('nhM.ced wilh contr.SI. 113 figure~. CoronAl ~An demonstrAtes enlArged left optic nerve with .I low-density center surrounded by .I mOre den~ per;ph­" y experience, we designed a study to answer the following questions: 1. What is the course of primary intraorbital meningiomas? 2. Is primary meningioma as aggressive in other age groups as is reported in the group under 20 years of age? 3. Since orbital surgery of primary intraorbital meningioma, in our hands, invariably blinded the eye when total removal was attempted, when is surgery indicated? 4. And finally, what Me the recurrence and sur­vival rates after surgery has been performed? Methods and Materials of Study A study of primary optic nerve meningiomas was undertaken at the Armed Forces Institute of Pathology (AFJP), A review of the material on file in the Registry of Ophthalmic Pathology revealed that 96 cases had bC'('n didgnosed JS orbital m('n­ingiomas. Thcs(' included the 25 patients alrcady rcported by Karp cl al." Thc follow-up course of thes(' 25 pati('nts was mJde current. An additional ~ I 'd~"~ Wt'rt' ,tddt'd 10 th('s<, in whi,h tht· tumor , .,,, ..... ! I·· I,,· pnrll.lrilv intr.lorbitdl by c1iniCJI Figure 24&. 5.ome ~An AS in Fig. 2>&.1 The instrument hAS linn plACed in the "meASure mode" to further demonstrAte the low denSIty center of the enlArged left optic nerve. Note enh.nce­ment of the optiC nerve circumfuence, evaluation and histopathologic criteria. The intra­dural origin of these additional cases was further demonstrated by histopathologic examination. To these 46 cilses were added nine p3tients from the author's series, so that a totill of 55 piltients were available for study. Results of Study We found that the age distribution ranged from 3 to 83 years. In the 3- to 20-yeM age group there were 15 patients, or 27.3%. In the 20- to 35-year age group there were 14 patients. or 25.5%. In the 35- to 50-yeM age group there were 16 patients, or 29%. In the 50- to 83-year age group there were 10 patients, or 18.2%. Th(' average age of the entire group was 35.32 years, with the average for females 37.68 and for males. 29.56. There were 48 white patients or 87.27%, and seven black patients or 12.73%. Females predominated overall, with 39 to 16 m<1l('s or 70.9% females to 29.1% males. In the age group under 20 years, however, there were nine females and six males with a percentage of 60% to 40%. The right orbit was affected in 30 cases and the left in 22 cases, with no notation made as to the affected orbit in three cases. Joumal of Clinical Neuro-opnlhalmology In E"volluoltin~ th(' c1inkoll presE"ntoitilm. th(' re­spondE" nlS wer(' n'~l unih~rm in thl'ir 1~I:>Sl'lV.llions. Howl'v(>r. 01 c1(',lT diniColl pktur(' eVI~lv('d frum th(' moltE"Tidl on holnd .md from folluw-up l'l~rres~xlO­dE" nce. Visuoll loss oInd ("I~phth"lmns were thl' prim.uy presentin~ findin~s. In .''''' c.lses visu.11 IllSS ,11'­peolrE" d bl.'fort" th('Te W..lS ("I~phth.llml~s. while 10 p.rtiE"nts presented with e'llphth.llmos bl.'fllTC thcrc WolS visuoll loss. In five p.ltients therc WJ-S visuoll loss dnd exophthoilnulS slmult.lnelluslv. Am,mTllSis fU~d)' oInd hypeTl'ri..l WE"U' pr('s('nt in thre(' pJtients e"ch. E.....orhth"lmllS volrird frl'm Z.5 III II;> mm ..Ind. in some p.rtients. WolS .1SSlXioltE"d with chemosis .md lid edem... MolTcus-Gunn pupil Jnd E').trJocul.. r motility disturbolnces. The most common e-xtrolocu­I.. r motility disturbolnce W.1S inolbility to look up. In three p"tients 01 p.1lp"ble molSS W.1S prE'sE'nt. ThE' most common diolj'tnosis W.1S optic nE"urilis. .1nd in somE" p.rtiE"nts .1 diJj'tnosis of millign"nt mE'l.anomd or solid retin"t detdchmE'nt WdS mdde. Gliomd WdS the suspE"<tE"d di.Jj'tnosis in .mothE"r number of pdtients. Three pdtie-nts prE"SentE'd with hE'morrhdgic glucomil. TWE'nty-threE' of the pdtiE'nts we-rE" blind dnd presented with chronic disc sWE"lting dnd .Jbnormdi vessels describE"d on thE" surfdCE" of the swollen optic neve. Primdry optic dtrophy WdS prE"sE"nt in II pdtients. i1nd othe-r volsculu phenomE"nJ such dS centrdl retindl vein occlusion oInd hE"morrhd~ic glducomd were notE'd. All types of surgery were performed on thesE' 55 pdtients. In 33. only orbital surgery WdS perfonned. mostly enucleations dnd in somE', exentE"rations. Eight undE'rwent cr.l.niotomy only. Fourteen undE'r­went a combined orbital ope-ration with crani­otomy. It is interesting to note thdt the interval between onset of symptoms and surgery ranged from 2 months to 50 years. Under the dgE' of 20, 63% of the patients were reoperJted. The following surgiul compJic.ltions W('Te noted: I) .Ill pdtiE'nts WE're blind in the operat('d eye, 2J recurrence in the orbital dpeX, 3) introlcr.lniJI extension, 4) extraocular muscle palsies,S) phthisis bulbi, 6) cerebrospinal fluid ledk, and 7) meningi­tis. It must be remembered, howevE"f, that thl'se pAtients were operdted over .I period of the last 50 yeArs. Surgiul techniques holvE" chAnged quite markedly in thAt period of timE". NE"verthelE'Ss, it WAS appArent that there WdS no undnimity of sur­gicoll philosophy in the USE'S taken from thE' files of the AFlP. Survival Rates Attempts to follow up theSE" poltienis werE' moldE" by the AFIP personnE'l .Jnd by mysE"lf persondlly rr..ble I). In the 15 pAtiE'nts undE'r 20 yE'drs of dge, no follow-up WAS nAilAble on thrE'e poItients. At Junl!' t981 AJpt" rAlit I: , R«u...·ncl'~ .nd !'urviv.1 R.t.... in 5S C.sn of Orbit.1 Ml'ninjl;ium. ... ~.·l.",up ... .xl .xl-J~ n-so SO·,!.) Nun.l,,·, ,of "JIl.·"" " " ,. ,. N,.I"Il"w_ul' , Z , • Ahv,·-N•• 'h... u,rt·n.o· , , , , Al.n w•• h K.... ur........ , • , • t 'k-Jd-{lrto,t 101.... u"'......·I'" 1o.. ,.... 'Jn.... l.t..n~" .... , • • 1l...JJ-lnU""Jn'J' r-;..u"of,. " ....... ~" Mull,u'ntn( M,·n.nlo:".mJ , • • • t>C-Jd-A_,,- •.oIC'd l...uw-. • • , n.:-.od_A~St....ot...:l <'.u_ Au... ~v_lntr.ll'un••J Ro" (ullc-nc.. • • , • R.....po.·rJIlOn~ ,. , , , the time of writing. two .Ire Alive without recur­rence; five are Alive with recurrence; four holve diE'd with introlcrdnidl extension; dnd onE' patient died from Reye's disedse. In thE' 14 patients in the 20- to 35-year age group, no follow-up was obtolinE'd in two. 5E"ven ue alive without recurrence; four .Ire living with recurrencE'; ,!Od one poltient hdS died from introlcranial extE'n­sion. In the 16 pdtients in the 35- to 50-yeu age group, IhE're WE're three lost to follow-up. SevE"n paliE"nts are alive without recurrencE'; two arE' alivE' wilh recurrence; oInd four have diE'd from AssocioltE"d causes UnrE"lolted to thE' tumor, dlthough threE" of these pdtients hold introlcranioll meningiomas. It is inlNesting to note that two of thesE' loIttE'r three patients showed multicentric mt'ningiomas olt oIU­topsy. Both of these poltients displdyt'd olf.lctorv groove menin~iom.lson tl1(' opposite side from th~ involved orbit. In tht> 50- to 83.yNr .1~l' ~roup. thNE" werE' 10 patil'nts, of whom wt'r(' lost to follow-up. FivE" of these poltients ,ue still living without recurrencE' olnd onE' hols died from olSsocioltt>d CJuses. Reoperations It WolS necess.lry h> reop<'rJte (In 10 of tht> 15 pJtients in thl' ZO-yNr .md undN .l~e ~roup, thrE'E' in thl' 20- to 35-yNr .I~e ~roup. two in thE" 35- to SO-ye.'lr oIl(e group, .1nd one in the so- to 83-year .'IgE' group. Tht>st> fil(urt>s indiColt(' that a primary orbitJI mt>ningiomJ is VE'ry dggrE"ssive and potE'n­tidily life-lhrE'Jt('nin~ in thE' dg(' group under 20 yedrs. It is Also dAAressivE" .md Iife-Ihredtening in thE" ~roup betwE"en 20 dnd 35 yE'drs of Age, dlthough 10 A lE'sS degree. In the group oldN than 35 yE"ars of .'Ige. the tumor oIPpE'drs to becomE' more indolent us l'riOl.lry l.)ph< Nt'rvt' Mt'llill~i,,,n,'~ in it~ ~wwlh p.ltter!l ,md m,'ny of the pMients h.:lve "utliv('d their dis(',lSC', dying of otllt'r ,-.lllses. Discussion Fwrn Ihis study, it is .lpr,m'nl th,lt thC' Ji,,~ntlstic ev.,lu.:Iti,ln must induJe nlutine s"ull x-r,IYs with vi<'ws "f the ,)ptir fllr.Jmin.1 .mo hypocydoid'll p"lyt\)mogr.:lphy I,f the I.rbil, ('pti,' r,m.Jl~ ,Hld Srhl.'nl'id rid~C's. The sine qU,l nOll Ilf di,lgno~i ... h,lw('v<'r, r,'sts uron computed tornllgr,'phic ..r,m­ning with .1nd withl'ut {"(llltr.Jst m,lteriJ!. Before perfurming intr.l(TJniJI sur~('ry, ilTteriogr.:lphy is necess.:lry. None of our p,ltients in recent yeJrs hJv(' h.ld pneumoenceph,llogrJphy. Surgery is indic.lted for primJry intr,lOrbitJI meningioma in p.1tients under the Jge of 20 JS soon JS it is suspected, especiJlly if there has been pro~ressive visu<11 [ass. In this Jge group we ad­VOCJte In orbitJl biopsy includinl': both nerve and tumor. If J meningioma is present, transcrJni,ll surg<'ry to remove the intracraniJI portion should be done to be followed by an exenterJlion to assure complete removJI of the remaining turrnor in the orbit. In the age group of 20-35 y<'ars, the pJtients should be operated for diagnosis if there is pro­gressive visual loss. Again, wE' advocate In E'xci­sional biopsy of the optic nerve and surrounding tumor for pathologic diJgnosis. If the diJgnosis is meningioma, it should be followed by craniotomy to remove the intracranial portion of the tumor. This should include unroofing the orbit and optic can.:l!. If at craniotomy. it is felt that Ih(> tumor has escJped from the dural she<1th into the orbit, ,m exenteration is then advocat(>d to remove the re­mainder of the orbital portion. In the older Jge group, several cases hJve now been reported to have been successfully removed with restoration of vision. I. ~, Although it hJS not been our philosophy to do this for feJr of seeding the tumor, an attempt moly be mJde to remove the tumor from Ihe orbit if the CT SCJn reveJls th.:lt it is small in size, is confined to mid- or .Interior orbit, the apex is sp3frd and useful vision rem,'ins. Surgery consists of splitting the dur.11 sheJth ,md sucking out the tumor JS W.:lS Jccomp[ishl.'d in the reported CJses. Of course, dose foll,'w-ur must b{' performed postop<'rJtivf'ly for m.my ye.Hs with high-resolution CT Sf.:lnninK tll d('tecl loc.11 rf'CUf­rence Jnd/or inlrJCfJnidl invJsion. Wright and co~workf'rs~ recently revif'w"d th"ir r"sults in 27 Cdses Jnd hJve concluded Ih.1I such attempts to remove the tumor from within the dur<11 sheJlh should bl' ,lb,mdont'd b('~·.,uS(' l,f spreJding the tumor into the orbit with fre,!uf'nt Jnd rJpid rf'rurr('nn'. Thf'Y .IKr('e with <HIT phill'S­( lrhy of w,litinK until vision is quite b,ld ,md then r"Ill<lving Ih" "plic rwrvf' l"g£>lh,'r with thl' tUlllor, .", \", 1... \,,· .Idv." .1t..J in Ilw "IJ,'r p,lIil'nts. If the vision is good in this older age group. it h,1S been our custom to follow the patient without surgcry every 3 months with visual fields and every 6 months with CT sCianning. At the present time we hdve six older patients under our observdtion for presumed primary optic nerve meningiomas who .:ITt' being followed in this manner without pwgressive loss of vision. None of these patients h.Js evidence of intracrilnial extension at this writ­inK R<1dioth(>rJpy has been advocated recently by Smith I ,IS definitive trE'<1tment when vision has become usel..ss. He ciles one patient whose vision had detrrior.-lted to hand motions. After a dosE' of 5220 rads of irrddiJtion therapy WdS rendered 10 th.. Jffected orbit. vision WilS recovered to 20/70 for 18 months. Subsequently, the vision Jnd visual field dC't<'riordled. There was no follow-up reported aftN 42 months Others have reported the us€' of rddiation IherJpy for intrdCrJnidl meningiomas with variable results in those that have been incom­pletely removed. Me histologically md[igndnt or clinicdlly invasive.-'" In generill. we hJve reserved rddiolherapy for orbitill recurrence of intrdcrani<11 meningiomJs bul h.lv{' not had experience with this moddlity as deFinitive inili<11 therdpy in orbital meningiomds which Jre primary in the optic nerve. We have trealed primJry optic nerve meningiomas only by the surgical technique noted above. Conclusions In conclusion we have presented the pathology, the dinicJI picture Jnd the computed tomographic findings of primdrv optic nerve meningiom<1. We hJve concluded from J study of nine of our own pJtients .md 46 cases from the files of the Oph­thJlrnic Division of the Armed Forces Institute of Pathology that Ihe tumor is more JAAressive and life-threJtening in the p.lIients under 20 years of age thJn in those over this Jge group, [n the age group l,f 20-35 ye,lTs, Ihe tumor is somewhat less .lggressive but still life-threatening. whereas in pa­tients over 35 "e,us of a~e the lumor becomes more indolent in its charJcter. In thC' Y~'unKer .1ge group, we recommend that once vision is lost Jn orbit<11 operJtion is perfonned with r,'m,w.J1 of the optic nerve and the menin­~ il,mJ. This .Issures In Jdequate specimen for di­.1~ nl1sis ,md ,11 the Silme time allows the pathologist II' df'tNmine whether the tumor has escdped from th(' dur.ll she,lth. This should be followed by a cr.mi<l!l'my with unroofing of Ihe orbit ilnd optiC c,m,ll to r('mov(' tholt portion of the tumor which h,ls invJded intr,lCfanially. If at craniotomy it is noted tholt the tumor hds escaped into the orbit, the Cf,miotomy should be followed by an exenteration to remove Ihe life-thredtening ddnger from the tumor remdining. lournal of Clinical Neuro-ophthalmology In thE' oldcr .1\':C ~n)Ur. if Ih(' vIsion r('m,lins ~ood, w(' follow Ih(' r,llll;>n! wilh ('1' s.:,mning ('v('ry b monlhs ,:and visu"l fidds with d,)S(' dinic,,1 ('v,ll­U., llion every 3 m,~nlhs. If vis"'11 ht'l.',)Oll'S pn~r('s­SivE'ly 1,)St in ,10 "Id,'r r.lliCnl ,mJ Ih,' .If><'' of thl' orbit is sp_Ired, So.)l1ll' surg''\'lls h,wl' ,I!tcmpll'd remo\'.11 by.1 KWf"nll'in Jrrr".Kh, slIlIing Ihc JurJI she.1!h ,:and sud,.. in~ "u! thc IUnl,)r h) d"n)mpn's!> Ih(' n(',,'c. If this is oJ,)nc, Ih(' p,llknt OlLIS! Ihell l:>c f"II"we,J d,'s('ly h)r e\'id,'n,'C "f n','urrellC,' ,'r in­lr., l,r.,ni.11 (',!('nsi,'n. It is ,'ur ph,I,)s,'ph)', h,w..('v('r, I,' f,'II,'w thE' p,lli"nt until \'isi,)n is I,'sl ,lIlJ Ih('n III r('nhW{' Ih{' ,'pli,' ne,,'(' l\,t"lIy ,ll,'n~ with th,' IU­m,' r. Th(' r.1li('nl is th,'1l ",Hdully f"II,'weJ f,'r I,X,II ,'r 1Illr,lCrJIli,ll r,','urr,'I1\'(', If Ihere is recurr('n,',' in Ih{' ,I~"'\ ,)f Ihe orbil or in Ir.lcr.1ni,ll (',tcnSI,)Il. (',,'nt('r,lli,m ,lIld crJni­,' t,1my musl Ihen be pert','rm{'J ,IS W,lS indicJt"d in thc y"un~{'r c.1scs. R,ldh'thNJpy. which hJS been JJnx"led by Smllh .IS ddinitivl' thcr.1py. rn.,lY SE'r"\'E' .,lS .In .lllemJt!\,(' I,) IhesE' m"r(' r.ldiCJI pro­,(' durl's in tht' .1dult. Thc ~cncr.11 rul(' for Ih(' older p,1l1ent. h,'\\'('\'E'L is c,'ns('rv.lliv(' mJ.nJ.~ement dS long ,1S Vision rern.lll\S useful Jnd therE' is no evidence of mlr.1cr.lmoll (',tcnsion. RE'fE'rE'nc6 I Smith. Il' ~E'uro-ophth.llmol,"8Y updJtlP'. Tr,lns. Am Ac.td. Ophth.tlme>/ Of"I.uyngol 86: 303-307, '"'' :!. Wnght. I.E.. Cdll, ~ B.•lnd LldrICos. 5.: Pnmdry OptiC nervlP' mlP'nln¥-IomJ Br J Oph'h,dme>1- 64: 553­~ 58, 19e.o. 3 nlper, M.G.: P<lthol~y dnd sur¥-Iul mJnJ¥-emlP'nt of prlm<l!)' Intuorb,ul menlnglom<ls. (In presq -to Ebers. G.C. GIrvin, I P.. dnd Cdnny. CB., A posSIble optic nervlP' menlllglomd. Arch. Neural 37; 781-783, 1980. 5, MJI'k. L.C Kenerdell. /.5., MJfllon. I-e., Rosen­b< lum. AE. Helllz. R.. Jnd luhn50n. B,L M'(I'05Urll­icdl remov<ll of .I p"mJry IntrJurbltJI mtnm~i"mJ. Am. 1- OphthJlmol 86; 7O-t-70C1, 1°78. 6. Byers, W.G.M; r"mJry 'nfr.-.dut.J1 Tumo~ "I thr Optic NlP'rve. ROY<lI VICtorl<l HospIU1. Montre.11. 1901-1903, vol. t. pp 3-82 7. Kn.pp. A.: A p"m.ry tumor of the optic nerve successfully removlP'd WIth prew!"V<lhon of the eye­bdll by th~ KroenllP'IIl method Arch. Ophrh.. lm,.' oW: 660-665, 1915. 8. O.ndy. W.E.: Prf"Chl.llsm.1 IIltun<ln'dl tumtlrs "f tht' OptIC nlP'rves. Am. J. Ophth.tlm..'. 5: 10Q-ISS. I1l2:!. 9. O.ndy, W.E.: Resulls followln~ tht' tr.. nscrJnJJI "p­er. lltlve .lI.ck on orblt<ll tumo~. An-h. Ophfh.llm"'. 25; 191-220, 19~1. 10. Cushing. H., dnd E,wnh.rdt, L.: Mf'nmj{wm.lS: Thf'ir Olss;(;c.'ion, Reglondl BehdvlOr, Lilf' Hi!'tory. ,md Surgiul End Results. Chules C Th..md~, 5prinll' field, Ill., 1938, pp. 19-69, 250-232. U3-3IQ. II. Henderson, I.W.; Orb,'.I' Tumors. W.B. Sdunders. Phil.delphi<l, 1973. pp. 527-554. 12. Ret'SE', A.B.: Tumors 01 tht' Ey(" 13rd ed.). Huper &. Row, New York, 1976, pp. 148-153, June /961 AlpN l.\ H"lI,·nh,'r'l. R.W, Hulll'nh"r,t, It W., Ir., Jnd M"CJfty, <.".5.: VI"uJI pnll\nt>"" "r I,ptic nE'I'VE' ,h,'Jth mrnlll~I"mJ' pr,,-.lm 1Ill\ ..hunt v('<;sds on thlP' "pt,.- -.II...... Th(" H"yt-~J"t"nn'r 'yndr"m(" Tr.ms. Am. Orhth.l'n",1 ~"'. 75: 141-loJ, 1077 l-t Wrl'N·r.:, LA, JnJ IJ\\.b.., L.I) OrbltJI Ct>mputed t"n"ll\rJphy: Anuvrrvu'w I (,.n' Cd Oph'h.dmol. 40: 1_\-2~, I~. 15 Alpc'r, M.e.: I'rim.-.ry urb,...1 m('nlnl\IUmJl-' Prob­lrm~ III d'J~nosi!- ..nd mJnJl\I'm('nt Th(' -..('cond rrJnCl' HN"J AJlrr Ir\lur(". Unlvrr"'yof PE'nnsyl­V. Irl'J, MJy 1070. 10. W,,~ht, I C. I'l'ImJry ,'pile n("!"VI' mtnml\lnm<l5' elm. ....11 rrt'~nl.-.hon ,lIld m.-.n'-'~I'm(·nl. T,.In'. Am. A,·,ld. OphthJ/mu/. Ot"I,JrynX"I, 83: 017-025. 1977. 17. K,up, LA., ZimmermJn. L.L, BI,,,I, A., .. nd Spencrr. W,H" I'rim.lry intr.1,'rb'IJI mrnlnjo:l()m.l~ Arch, Ophlh.lln",/. 91: 24-28, 197-t. It'. ZlmmermJn. L.E.: I'rrsonJI communic.lllon IQ. CO\,lin~, R./.. .-.nd Wright, I.E.: AuchnOld hyperpl<l­SIJ m optic nervI" gl,om.1: ConfUSIon with orblld! menin~lOmd. Br. /. Ophth.. lmo/. 63: 5%-599, t979. 20. Hogdn, M.J.. <lnd Zlmmerm<ln. LL Ophth.. lmic P<l­, h(l/O)ty, An A".ls .lnd Tpv'book 12nd ed.,. W.B. SJunders, rhil.ldetphl<l, 1902. 21. YJnoff, N.. OdV'S. R.L Jnd Z,mmemun, L.E., Ju­vE' 1lI11P' PilocytlC astrocytom. r'¥-I,om<l", of optl( nervE': Clmico-p.ltholop;ic study of sl'\ty-thrE'E' (.l~. In Ocul.lr Jnd AdnE''\JI Tumors, r A, !dkob,K, Ed_ AE'SCul<lpius, Birmmsham, 1978. PP 085-707. U. Stem, J., Idkobif"C, LA.. and Houwpldn. LM.' The drchltKture of OptIC nerve gl,omds WIth <lnd Without n("urofibromaIOSls. Arch. OphlhdlmoJ. 98: 505-511. ,,,'" 2.:\. Spt"nclP'r, W.H., Prim<lry neoplJsms of the e>ptlc nervE' <lnd its shedth: CI'llIul fe<lturf'S Jnd current concepts of p.l1hOllenetic mKhdnlsms. Tr.lns. Am. Ophthdlmo/. Soc. 70: -tQ(l-521'. IQn 24 Fri~n, L., Hoyt. W.F., .md Tengroth. B.M.: Optico­cili< lry v('ins, disc pollioI' dnd visUJI lcoss: A tOdd of SIgns indiC<lting spheno-orbil.-.I menlnglomJ. A(IJ Oph'hJ/mol. 51, 241-:!.4/1. IQ7J. 25. [ll('nberg('r, C: Pl"rioptic m('ning".... m,IJ', Arch, Neu­1'<. 1 33: b71-b7-t. I Q70, 20 Su~.-.c. 1.0.. Smith, I.L., Jnd WJIJ'h, F.B.. Th(' Imp"!'­Sibil' m('ningium.l. Ar.-h. Nf'ur,'/ J-t: .?oo-J~. r~77 :!.7. WJJsh, r.B.: M('nin~I<'m..', I'rml,lry "'Ithm the ,'rbll. <lnd "ptle eJnJI. In N(,ul'<'-"rhth.llm,.I<'~y ~ymp,,­Mum "I tht Uni"f'rsi'\' "t M •.lm, .IIlJ th(" BJ"",'m PJlmf'r eve In~litutf'. I.L. Smith. (.1 HuffmJn Pub· IIshing C~'" HJlIJndJle, r1.. IQ70. rr. :!.-to-~. U. W..lsh, r.B.: Memngwm.ls, pnmJrv wlthm the ur­bit .lnd "ph( (In.1l. In NE'ur".,'phth.llm,>/,~y, 1.5. CIJ~r, ,md I.L. SmIth, (ds. M,"l'-l-oy, 51 Ll;'luis. I<17S, v"I/I, pp. 100-1"0. :!.~. W.1r... W.M., Shdm(". C (, Nrwm,m, H.. T,)wn!'('nd, II, .1nd tk,IJrlP'y, LB.: R.lJ",ti,'n Ih('rJpy "f memn­/( llImJs. Am. 1_ R,>("ntxf'n,'1. 123: -tSJ--t51'. 1<175. Wri'r (or r('prints f,': M("lvin G, Alper, M.D., 5454 Wiscnnsin Av("nur, Suitr 050, Ch('vy ChJSt', M.lI'yl<lnd 20015. 117