Journal of Neuro-Ophthalmology, December 1982, Volume 2, Issue 4

Diminished vision and painless proptosis.

/. ("Jill. N"Ufl l -"l'hth,lllllpl. 2: 273-278, 1<)82. Neuroradiological Clinical Pathological Correlations Diminished Vision and Painless Proptosis ISHMAEL I. TORRES, MD. ROBERT M. QUENCER, M.D. Case History ;\ C'~-\'l'.If-l)ld fem,lle presented with diminished \"lsil)n .1I1d p.linless proptosis of the left eye of 2­\\" feb dur.ltil>n. She h.ld undergone .m uneventful C,lt,H,lC! e"tr,lction .md intr.lOcular lens implanta­til) n 2 months prior to the onset of the present svmptl)ms. On ex.lmin.ltion, the patient was noted tl' have a proptosed left eye with markedly chem­l) tic con junctiva (Fig. I). There was arterialization l,f sm.ll1 conjunctival vessels .md a retinal detach­ment was seen in the left eye. There was no ocular motor disturbances, diplopia. or cranial nerve def­icits. No bruits could be heard on auscultation. Tension measurements were 19 mm Hg in both eves Past medical history was unremarkable ex­cept for a hemithyroidectomy 20 years ago for thyroid disease. She is presently taking synthyroid From the \:eurorJdiolo~y Section, DepJrtment of RJdiolo~y, LnlverSltv (If M,Jml School of MediCIne, MIJmi. Florida. .md is well under control. The radiographic eval­uation included a skull series with optic foramen views which were taken at another institution and reported as normal. Other studies included a com­puted tomographic evaluation (CT) of the orbits and parasellar region (Figs. 2£1 and 2b), and select­ive angiography of the internal (Figs. 3a -3c) and external carotid circulation. All laboratory exami­nations were otherwise normal. Discussion Contrdst enhanced computed tomography (CT) obtained in the axial and coronal planes (Figs. 2£1 and 2 b) shows an enhancing mass in the left cav­ernous sinus. Kline et al. I have stated that the CT criteria that suggest an abnormality in the cavern­ous sinus are: I) asymmetry of size; 2) asymmetry of shape, particularly the lateral wall; and 3) focal areas of abnormal density within the sinus itself. Fil\ure I. ".'11t'1I1 i' .1 NI-y..",-ol<l 1<'11I.,i<' wilh p.,illl<'" l''''l'h"i, "l th .. letl ..y... ("h,',,· i, m.H"ed l h('mo...,i ... 'lnd ,Htt'ri.llil..llioll 01 the umjundiv,ll vel" .... 273 '.I (bl rigurl·... !.J dnd 2,"'. ll'l11pull'd tlll1lpgr.lphy (.J) r'nstlnfuSH"11 J,i.ll CT 5CJ,11 shows J.n area of ('nh,n\< ,'n\<'nl (",r"\\'h,',,d,) ,11 Ihl' Il'vel "f the left (.l\'l'rn,'Us SInus. It bulges into the left Illlddl(' ItI........' .1 .... llPPll... t:d ttl the 11l1rm.ll H.lt l'r slightly (on(,)\,(' sinus on the opposite side. 1';"1,, " n"rlll"I-,'pp(',H1ng 'uf1l'nt,," "phth,llmic vell1 (curved .Jrr,'IV) in the left orbit. (b) A ( lIlt lll.d ( T dl'IllPIl .... t r.,teo., the I11.Hked convexity of the left lJ,VCrllllU5 sinus (.:Ufo\vheads). ~''''l Journal of Clinical Neuro-ophth,llm,)logy Torres, Quencer Figures 3a-3c. CerebrJI JngiogrJphy. (J) The eJrly JrteriJI phdse of In internJI cJrotid JnglllgrJm shows .1bnormJlly prominent arterial branches of the cavernous portion of the internJI carotid artery: meningohypophyseJllrunk (curved drrows), inferior cavernous sinus drterv (single arrow), dnd cJpsulJr arteries (drrowheJd). The recurrent meningeJI brJnch of the ophthJlmic artery (open JrrowheJds) is "Iso enldrged. (hI The I"te Jrteri.11 phJse shows intense op"cificJtion of the CJvernous sinus (Jrrows) and eJrly filling of a middle cerebrdl vein (JrrowheJds). (c) Left externdl cJrotid JngiogrJm shows In abnormJlly prominent artery to the forJmen wlundum (single curved Jrrow), which is J br.lnch "f the internJI mJxillJry drtery and prompt opJcificJtion of the C.lVernous sinus (.Hrl1wheJds). In our case, the asymmetric bulging of the wall of the left cavernous sinus into the middle fossa is the most sensitive indicator of pathologic involvement of the cavernous sinus. Cavernous meningiom.:ls, .:15 .:I rule, exhibit rel,l­tively homogenous enhancement .:Ind are well cir­cumscribed. Even though th(' degr('(' of 10c,11 ('xt('n­sian by the meningioma vari('s at th(' tim(' of presentation, there is usually extension posteriorly into the cisterns anterior Jnd later,ll to th(' brain stem or laterally into the medial aspect of the floor of the middle fossa.~ In the present case, however, there was no evidence of extension of the enh'lnc­ing lesion onto adjacent dural structures. Vascul,H lesions of the cavernous sinus such ,IS ,In intr,lc,lV­emous carotid artery ,lneurysm or ,In ,Hteriovenous December 1982 m,llformation m,ly disclose .111 enl.Hged C.lvcrnl'us sinus with ,I smooth bulging l.lter.ll w.lli. such ,IS we see in this C,lse, .lIld for this re,lSl1n cerebral angiogr.lphy would be necess.H-y to est,lblish either of these di,lgnoses. A neurofibrom,l within the C,lV('rnous sinus m,ly ('nh,lnce ,lnd show ,1 bulging wall, but these .He usu,ll1v hHlIld in p.ltients with n('unlfibnlm,ltosis. Prim,lry llI' sCCl1nd,H)' neopl.ls­tic proccsses wl1uld be less likclv in this Clse bec,luse of the I,ll'k llf bony destruction. Eccentric growth into the C,lVernous sinus frllll1 ,1 prim,lry pituit,Hy tunlllr Wl1Uld ,11sl1 be unusu,ll bec,lus~ there is tlll evidence of sell,l turciC,l enlargement or ,1bnorm,11 intr,l- l'r supr,lselbr enhancement. Ex­tr. ldur.ll epidernlllids h.lVc been shown to present ,IS .In enh.lncing bulging C.lVernous sinus mass:l ; 275 Figure 3. (C"ntmucJ.) hlllvever, these tumors are rare and frequently will h,we low density areas within them, a finding not pre<.,ent in llur C,15e. Cerebral ,mgiogr.lphy (Figs. 3,1-3c) shlHved en­larged br,lllches of the c,wernous carotid artery including the meningohypophyse,ll trunk. the in­ferillr c,wernllUS sinu<., artery ,llld l-,lpsuI.H .Hteries. ,m enl.uged recurrent Illeninge,ll br,lllch l)f the llrhth,lllllic ,lrtery, ,llld ,111 enl.uged .Hterv tl) the fOr,1I11('n nltundulll Thert' W,lS denst' l)p,lcificltil)Jl of thl' l,lvernou<., Sillll<., ,llld ,lll e.Hly dr,lining Illiddle lerl'br,ll vein, Thl' ,lngiogr,lphic findings in C,lVerrwus Illenin­giolll, l" include vi"ualiz,ltilln llf .1 Illl)der.ltl'ly dense tumor blu"h in the tllrogr,lphv of the sinus which I,l>'h into the vcnou<., ph,lSt'. Hvpt'rtrllrhic ,Hteries, ,I>, in thi>, LI>'(', .Hise fWIll the (.lVernl'lIS segml'nt of the intl'rn,ll l',Hotid ,lrtery, ,llld Clll1tribution trolll tl1(' extl'rn,ll c,uotid ,Irtery m,ly also be pre­>,(' nl. TI1('re m,ly be enC,l<.,elllt'nt t)f thl' c,Hotid (bl ,uterv bv the tumor mass along with extension of the tunwr blush I,lterallv .lnd posteriorly, similar to wh,lt is seen l)n CT; ht~\Vever, rapid arterial to Venl)US shunting is nl)t a feature of the common ft1rms l)f meningiom,ls. In the present case, there is ,111 intense ,1l1d well-circumscribed opacification t)f the ClVernt1US sinus without evidence of a tumor blush, l)r enc.lsement of the cavernous portion of tht' c.Hl)tid .lrtery. An intracr,1I1i,11 arterial-venous fistula commonly invl)lves .l direct connection between the internal c,Hotid artery ,lnd the cavernous sinus secondary tt) tr,H1ma or ,1S a result of rupture of an aneurysm inh1 the c.1Vernous sinus. A high-flow shunt of the contr.lst m,lterial into the cavernous sinus is evi­dent in the early arterial phase of an angiogram with enlarged and early draining veins visualized, Reversal of flow into the ophthalmic vein is a prominent feature of this type of fistula. These patients present with pulsating exophthalmos and Journal of Clinical Neuro-ophthalmology Torres, Quencer Figure J. (C'nIIlHlcJ.) a loud bruit which is secondary to the high flow, through the abnormal communication. These pa­tll'nts mav also exhibit chemosis, extraocular pal­sies. and loss of vision. In contrast to these high-flow fistulas which involve a direct communic,ltion between the ca­rotid artery and the cavernous sinus, some fistulas exhibit a slower flow state with an indirect com­munciation between these two major vascular structures by way of a dural arteriovenous malfor­mation. There is no direct communication between the carotid artery and cavernous sinus; instead, multiple dural branches of the internal and external carotid arteries are hypertrophied and fill the cav­ernous sinus. The typical c1inic.11 signs th.lt com­prise the syndrome of these types of dural c.lVern­ous fistula include mild dilatation of the conjunc­tival veins, mild ipsilateral proptosis, increased intraocular pressure, and an inconst.lnt but often high-pitched and focal bruit he.Hd over the ipsil.1t­eraJ closed eye or temple.' Our p.ltient under C<1Il­sideration did not exhibit .1 bruit; howev('r, .IS indicated by Newton et ,11,' ,I bruit nl,ly be ,Ibsl'nt in one-half the p,ltienh in their series of dur,ll cavernous fistula. In the<,e dur,lJ (,IVernOUS fistul,b, the main feeding branches from the intern,lll-.ulltiJ artery originate from the c.lvernous portion <If th,lt vessel and include the meningl1hypophyse,11 trunls, the inferior cavernous sinus ,Irtery, ,mJ the C,lp­sular arteries of McConnell. There is ,In ('xten ... ive vascular basilar plexus in which each of the branches of the cavernous carotid an.1stonwse with December 1982 Ie) its mate from the opposite carotid artery, as well as with meningeal br,mches of the external carotid, the ophthdlmic, and the vertebral drteries. This network of durdl vessels is referred to as the rete mirable." The cavernous branches of the internal carotid artery are usually filamentous and difficult to visualize during normal angiography; they are considered to have pathological significance when they exhibit an increase of caliber, marked undu­Idtions in their course, have multiple branches. or result in d tumor stain.'-' The first major branch of the cavernous internal carotid artery, the menin­gohypophyseal trunls, trifurc,ltes shortly after its llrig·in. P.ukinson'; desffibed its three br.1·nches: the tentlHi.11, the dllrs.11 lllenin~e.11. ,md the inferior hypllphyse,ll ,Irteries. They supply p,lrt llf the wllf llf the CIVerl1lIUS sinus .1nd the C,lVerlll'US sinus pwper, in ,Idditilln tll the dur,l llf the tentl)riUIll, f.llx, .1I1d clivus. The secl)nd m,ljllr br,1I1ch, the .Htery to the inferillr C,lvernlHIS sinus, ,1Isl) supplies structures within the sinus ,1S well .15 its dur,11 l·,)Vering. 7 This vessel ,11sl1 trifurc1tcs sllllrtiv ,1fter ih llrigin. TIll' first ur SU~1('rillr br,1I1ch suppli~s p.lrt llf the tentllriulll ,md supplies the third .1nl! fl)urth IT,mi,11 nerves. The sl'«lnd lll' ,mtl'ril)r br,lnch has .1 I.lte 1'.1 I r,lI11US whit'll p,lrtly V,l<;cul.Hizes the floor of the Illiddle fllSS,l in the vicinity of the fllr,lI1len !'lltundulll, wlll'rl' it ,1I1,lst"lllllses with the .1rtery to the fllr,1I1H'n !'lltunJulll which is .1 branch of the intern,ll 1ll.1xill,Hy .utery. The medi.11 r,lmus of this ,lI1terior br.lI1ch supplies til(' third, fourth, .1I1d sixth IT,mi,ll nerves ,md .m.1st,'ml)Ses in turn with the 277 I)illlilli~lH'd Vi~i"l1 ,lI1d I>r"pl\l~i~ I.AIII I I. Br.lIldll's lit 11ll' Cdv"rnllUS Int"rndl Cdrolid !lrl"ry ~ ll'111 rl~~' ,I, VlllII,I, V<.,t ',11 Irul\~ IlllI'IitH l.l\'t·f11Pll .... "'IIUb .Irll'!"\, 3, ('.lp"UI.H .Htt'ril'''' of M,l "nlll·1I .1. T'·I1I"ri.1I.1. .1. 3rd .Jnd 4th, 1'.111;.,1 NN: n>uf of l.JverI1Pu" .... IlU",; oureJ of t('o­Itlriulll .JnJ f~llx b. I )pp...,1 11Il'lIillgt·.d .I. b. (lth l r.1I1i.ll N; JurJ of clivul, Illl"fltlf hvptlphy.... ·.11 !\:f'lIrt thyp"rh V""<" •• rll'ry .1. ~lllpl'ripr l,. .1. ~rd ,lOJ ,11h .. r.IIlI ...1T\:f\.: Jlld <.,urrllunJlng dur.), fre(' rTlJr­gill lIf t{'n!PrJum h. IInll'ri,'r hI' h M,·d,.,1 r"mu' 3rd. 41h, ,'nd bth ( r.lf'I.,1 r--..''' It, ""urt'r1or orbltJ! f,''lJrl': 1.'Il'r,1I I'd""" MIddle to......., ([(lllr dur.1 I\l.. t('rillr br MI'JI.l! r.lrTlU<.,' M('dl.d I , gJ ...... ('- rI.,n g,lnglll,n, Idlerdl rdmu,' L.,ter,,1 :, (of gd"erldn gdn­glilln .1nO durJ .1. Anh"ri,lr br. .1 Dur.l ..A ,lnt('(Hlr <;{'II.H \'\'JIJ b. Inf(,'ripr br. b Dur., «A ...('JI.,H (I(I.,.r..Jnt{'nnr I"be ",. hvp,.phv'l' d. Cunlrdldterdl lentl.>rI,,1 d. b. Conlrdldll.·rdl d(lr~~1 meningedl Ulery; vcrlebrdl d. meningedl brdnche' c. Conlr~ldlcrdl Inferior hypo­physedl d; c~psul.H drteries d. Tentoridl d. of meningohypo­phvsedl trunk b Recurrent emeningedl from I.'phlhdlmic utery: fordmen rolundum ulery of the ,nter­ndl mdxilldry ulery ACll'''Ory menJngedl drlerv of lhe intNndl mdxilldrv Ulery: middle meningedl drlery d ConlrdldlerdJ cdp<ulM d. b Ip~i1~lerdl 'nferoor hvpophvsedl d recurrent meninge,ll br.1I1ch of the ophth.llmic ar­tery. The third or pl.'sterior branch of the artery to the inferior C.lVernous sinus has a medial ramus that communicates with the accessory meningeal artery in the vicinity of the foramen ovale and a 1.1ter.ll rdmus which forms anastomoses vvith branches of the middle meningeal olrtery dt the level of the foramen spinosum. Both the medi<ll and 1.1ter.11 ramus of the posterior branch of the inferior C.lVernous sinus artery supply the g.1sseri<ln ganglion .lnd the surrounding dura. The third ma­jor group of branches from the C.lVernous cMotid, the c.lpsular arteries of McConnell, supplY the dura of the sella turcica and the anterior lobe of the hype'physis. Table I summ.uizes these br.1I1ches and their interconnections. The C,lVernous sinus norm.llly receives venous tribut.uies from superior and inferior ophthalmic veins, centr.ll rdin.11 veins. middle .lnd inferior cerebr,ll veins, ,lIld middle J1)eninge.ll veins. In .lddition, it Clllllmunic.ltes with the superior .1I1d inferillf pet rosal si n uses. the pterygoid plexus.•1Ild the Clllltr.ll.lter.ll cavernous sinus.' In their series, Newton PI .11.' found th.lt velHHIS dr.lin.lge frl.lJ1) the l,lVerlHlU~ sinu~ in C,1ses I.lf dur.ll c.lvernl.1US fisted.ls l'xtended .1Ilterillrly inlll the superil.lr ,lI1d inferillr llphth.llrnic veins in .1 1ll.1jllrity llf c.lses. In .1 few "ISI'~ there were bl.lth .1I1teril.lr .lnd posterior dr.lin.lge, while llnly lllll' C.1se dr.lined pl.lstcril.lrlY I·Xllusively. TIll' present l'.1~1· exemplifies .1 typic,ll "spon­t. lI11'llUS" dur.11 C.IV1'f1HlUS sinus fistul,\. The etiol­ogy llt ll111st dur.llc,lVerllllUS sinus fistul.ls rem.lins "h"cufl·. It i~ prllb.lbly the result I.lf rupture of the sm.11I I..Hlltid I. .lVl'rnllU~ br,lIlrill's .1S they course through the sinusllids that cllmprise the cavernous sinus. C<luses 'If the rupture may include predis­posing vascular diseJse, straining. llr trauma. It h<ls been observed that J large number of these slow­flow fistulas clclse spclIltaneously; definite tre<lt­ment sh,~uld be delayed. I References Klinc. LB. Ad.cr. 10. PL1St. MID . .md Vitek. 1.1: The c.l\'crn,'US s,nus: A cI.lmputed tl.'mLlgraphic study AJ.\'R 2: 2°°-30.5. 10SI. , P,'st. t\lID. CI,lser. IS. ,lnd Twbe. 1.0: The radio­gr. lphic di,lgIlllSlS I.lf C,l\'CrllllUS meningil.'m,ls .1Ild .lnl'u,,·,m, \\'Ith ,1 rC\'Il'\\, I.,f the neur<wascular anat­I.' Ill\' I.,f the C,l\'CrnL'US Sinus. CRC Cril. Re\·. Di.lgn. Im,J,~ 12: 1-3~. 1070 3 Kline, LB. ,md C.llbr.1ith. I.C .. Pr,lsell.H tumor pre­scntlng ,15 p.lInful ,'phth.llnlllplegia. r .\,'eurosurg. 5~: I 1-"- 117. lOS I. ~. :\,'\\'I,'n. T.H ..md HI.'vt. \\'.F.: Dur,ll .lrteril.wenous ,hunt~ 111 the rcgil.'n ,i the C,l\'ernl.)US sinus. tVeuro­r. ldi"I,'g\· I: 71-SI. 1070. :.. \V.IIL,CI.'. ~. CI.lldbcrg, HL.. Leeds. N.E., and Mish­I.. in. t\1.t\1.. Thc (,l\'CrIWUS br.1I1ches of the internal c,Hl'tid .Hte,,'. A../R. 101: 3~-~(>, (0(>7. l'. I'.Hl..inSI.'Il. D.: CI.11I,lter.11 circul.ltil.)n of cavernous c.HI.ltid ,Hte,,': AIl,ltl.'mv. CIll. I. Surg. 7: 251-208. 101'~' '. M,Hgl.lli,. M.T...111.1 Newton, T.H.: ColI'lteral path­W, IV' between the c.l\'l'rn,'US portion of the interndl c,lHllid .1nd c'-tern.11 c.Hotid ,Hteries. Radiologv 93: S3~-S31', 10l'o, . I Vrilf' {"r reprinls I,,: Robert M. Quencer, M.D., Dep.Htment n~ R.,diology (R 130), University of Miami Scllllnl I.l~ Medicine, P.O. Box 016900, Midmi, Floridd 33101. lournal of Clinical Neuro-ophthalmology