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Show Anterior Ischemic Optic Neuropathy Occurring in Association with Carotid Artery Obstruction Gary C. Brown, 7\1.11. The C,l~l' l,l ,1 b;"-\'e.1r-llld 111,1n \\'Ith ,KlIll' ,1I1tl'fillr i~cht'mic l'ptIC Ill'lIrl'p,lth\' llcclIrnng 111 l'l1I11111Ktilln \\'Ith till' l)cul.1r I~Cht'I11IC ~\'ndn)Illl' pnll'rillr ~egl11ent ,1nd tundll~ 111,1nIte~t,ltll)n~ ~ecllneLHV tll ~l'\'l'rl' Ip~il,lll'r.11 c.1wtId Mten' l,b~truCtIl1I1) I~ ~'rl'~l'nll'd Althllugh the ,1SSllLi,ltilln ,1~'pear~ tl' l'l' qUIll' UnLl)l11l11l1l1, l)cca~illnallv a CMlltid ,1ftt'rv l)b~truLlI\'l' kSll)n 111.1\' predi~pl)~e ,1 p.1tient tl) the de\'e!l)pl11ent e)t ,1LUll' l~chl'I11IC ch,lngl's in the .1nterillr l'ptlC nerve, From the Retina Vascular Unit, Wilb he Ilo~Plt"l, f'hil"delphia, Pennsylvallla, Address 'correspondence and reprint n'que'~h to C, C Brown, M,D" 910 E, Willow Grow Avenul', Wyndmoor, ['i\ 19118, USA 39 Patients with acute anterior ischemic optic neuropathy characteristically present with a history of rapid onset of visual loss and pale swelling of the optic disc. The disc swelling mav be localized or extend to involve the peripapillary tissues (1). Histopathologic correlation reveals infarction of the optic nerve within or around the region of the lamina cribrosa (2), and it is generally believed that the pathophysiologic process responsible for the entity is impairment of blood flow through the posterior ciliary vessels that supplv the anterior nerve (3). Anterior ischemic optic neuropathY ma:' occur secondary to giant cell arteritis (1-l'7c of cases) but more commonly presents as the nonarteritic variant (86'i( of cases), in which significant svstemic associations include arterial hypertension and diabetes mellitus (-l,5). While instances of ischemic optic neuropathy in conjunction with carotid artery occlusion have been described (3,6-8), this association appears to be mllst unusual (1,3-5). Reported herein is the case of a patient with the ocular ischemic syndrome (anteril1r segment and fundus signs secl1I1darv to severe C,lrotid obstruction) who developt'd dnteril,r ischemic optic neuropathv. CASE REPORT A o7-vear-old man presented with d 6-dav history of aching pain dnd decreased vision in the right eve. He hdd e,pt'rienced 11l) previous episodes of visual loss or transient ischemic attacks but was known to have diabetes mellitus and svstemic arterial hypertension for 3 years. The initial ocular e,amination revealed a visual acuity of 20/20lJ in the right eye clnd 20/20 in the It'it eyt'. The intraocular pressure was 20 mm Hg in the right eVL' and 18 mm Hg in the left eye. Slit lamp examination of the right eye disclosed ru- .J(} (; ( 11/,(lWN FIG. 1. Right fundus at the time of initial examinatIOn Several small Intraretlnal hemorrhages are present and the optiC diSC appears normal bl'll..;i..; iridi", ,1I1d gl1nilhCl1pl' rt'I'I'alt'd nt'\\' 1'1'''''1,1" In tht' ,1lllt'ril1r Ch,l mb,'r a ngll', bu t nl1 pITI~'lwr,ll ,1lltl'nl1r "1'11t'r111,W \\'t'rl' nl1lt'd, lilt' Idt al1lt'rIllr "t'gnwl1t \\,.1"; 1111rm,11, A ljut'"tJl1l1abk nght alkrel1t pupillMI' ddt'rt \\',1" l1b";"r\,t'd Ophth,llml1..;n lpl' 111 tht' right I'\'t' dl'mlll1"tralt'd a nl1rma!-,lppt',uing l1ptic di"c ,111d "light", l1arw\\' ed rdil1a! arlt'rit''';, Intrardil1,ll ht'l1111rrh,lg"" \\'ere prt'st'l1t. prt'dl1minant'" in tht' 11lIdp,'riph"r\', althl1ugh ",'I't'ral cuuld ,1bl1 bt' "1'1'11 il1 the pll";lt'riur pult' (Fig, 1), Tht' Idt iundu" ,lppt',H,1llCI' \\',1"; unremark,lbl,', Intra\'t_'l1l1u,,; iluurt'..;cein 'lngil1gr,lpll\' III tht' right l'\'t' rt'I't'alt'd a prllillngt'd arm-tl1-rdin,l clrcu-latil1l1 tinlt', ,1" \\'1'11 a" an increased arteriovenous Iral1"it time, Filling lli the choroid \Vas delayed ,1I1d unl'I'I'I1, a" \Va..; that 111 the uptic disc (Fig, 211), 111 lilt' lalt'r pha"l'" l1i the "ludv, marked leakage oi dvl' Irllm thl' rdinal bll1l1d ves..;els cl1uld be seen (hg 2/,) lilt' p,ltiel1t \Va" thl1ught to have the oculdl' I"dll'rnic "1'l1drllml' (h), Bl'ldU"I' 1I1 the iri" nl'lll'ascularization, panrdll1al phlltllu1agulatilln 1\,.1.., begun immediately 111 llll' nght l'l'l', (J\'t'r tWll "es"ions, -1 days apart, tilt' pdtll'l1t rl'n'l\ed apprll\lmatelv 1,200 500-flm "plll'''I/l' bu rl1" l lilt' I\l'l'k alll'r tilt' 111111.11 e\amination the pallt'l1t \\.1" "1'1'11 dgall1, and ,1t that timt' he related a hl"tllr\' II! dbrupt l'i"u,lll11"''' 111 the right eve occurnl1g I ddl pr"llllu"h-II1l' l'l..,ual acuity in the n,~ht I'll' \\.1" h,1lld mlltilln.." and the II1traocular pfl''''''Ufl' \\.1" 211 mm fig A pwmil1l'nt aiierent pupillar' dl'kd \\,1" lIb"l'r\,'d In the eve, Right tundu" 1'\,1111111,ltI1l11 dl"C!lI"l'd ,1 "\\llllt-n, chalk\\ hltl' llptll dl"l and pl'np,lpill,HI' rl'gll1n (Fig, 3) 1,lullrl'''ll'1I1 ,111,~llI,~raphl' rl'I",llni hl'pl1periusion lIt tilt' I1lTIl' 11l'dd ,1lld "urrllul1dll1g rl',~ll1n (Fig, -1), :\ \\'t'''tl'r,~rl'n l'r\,thflll'\'tl' "1'dInwntatllln rate \\'.1" llbtaillt'd ,111d nlt'a"ur,'d tIl bl' :; mm h T\\'ll d,ll'" ,ll!t'r tilt' "udd,'n d,'cr,',bl' in \'ision in the nght l'\'t' tilt' ~'atll'l1t ul1dtT\\'t'nt carutid artenl1gr, lpll\' bl'cau"l' III ,1 "u"p,'clt'd n,~ht-sided ub" trurtllll1 :\ lilli', Ill,,,trudllll1 ul the nght internal cawtJd ,Htl'r\' \\a" prl'''I'nt. ,lnd UI1 the leit side a \..j:;', ''It'l1lhl'' \\',1" ,1p~'arent \\'lthll1 the Internal caflltJd 11'''''1,1 ,-\ nght ,-,Hlltld "l1d-arterectumv \\'as ,ltlt'mplt'd l'ut \\',1" ulbucc"""tul bl'cau"t' oi pru\- FIG. 2. Intravenous fluorescein angiogram of same eye as In Fig, 1 (a) At approximately 1 min after inJection, the study IS stili In the retinal laminar venOUS-filling phase Hypoperfusion of the superonasa~optiC diSC IS eVident. as IS a superior peripapillary choroidal filling defect (arrow) (b) At 3 .. min Into the study. marked leakage of dye from the retinal vessels. particu-larly the arteries. can be seen 4/ FIG. 3. Right fundus at 1 week after that shown In Fig 1 The disc IS swollen and extremely pale, as IS the peripapillary region Laser burns can be seen outside the macula Im,,1 pwpagatwn L1t thl' thrL1mbu~. One week later. a ~ucce""tul left endarkrl'ctllm\' wa" pertormed, but the \'i"ual acuity in the right eve tailed to impwye. DISCUSSION Anterior i"chemic L1ptic neuwpathv C,1I1 be Clln\' enienth' di\'ided intll nllnartnitic and arteritic torms. Apprllximatelv t'\6'; llt ca"e" are ot the tormer h'pe, \\·hile about l-l'; are in the latter group and a""ociated with giant cell arkriti" (-l). Among the group ot patients with the nonarkritic \'arietv, arterial hypertension and diabetes mel- FIG,.4. Fluorescein angiogram corresponding to that In Fig 3 at 81 s after injection reveals marked hypoperfusion, demonstrated by hypofluorescence, of the optic disc and peripapillary regions. litu~ appear to be thL' mo~t frL'qm'ntly associated ~v~lL'mic di~llfdL'r~ (1,:1,4); and, indeed, Repka and culll"lgUl'~ (-l) tound v,duL'~ ot 38'Ir and 15'1r, re~ pl'cliv,'lv, lur thL'~e L'ntitiL's. ThL' ,1ssoci,ltion of carotid arkry obstructive disL'ase and nunarlL'ritic ankrior ischemic optic neuropathv h,lS been notl'd previously but thought to be no higher th,1I1 that seen in an age-matched l'llntrol gruup (-l). Eagling dnd co-wllfkers (3) obSl'r\' l'd artl'riographic evidence in 3 ot 33 ((Fir) l'<lSl'S, ,1lthough it is uncertdin how many of these patients were spL'cificallv evaluated for carotid arteri, 11 cllmpromise [n ol~e ut thl'se three instances, a c,Hutid Ucc!usilln was tuund in cllnlunction with a cllntralatl'ral hemiplegia. The abnormal optic disc in the p,ltient de~nibed herein appeared quite "imilar tL1 the latter case. Waybright and associates (6) de"nibed three patient" with anterior ischemic optic neuropathy and an ipsilateral carotid artery L1bstructilln, but in each eye other teatures of th~ llcular i"chemic svndrome were not noted. However, in all ot these cases there was a history of previous or concurrent hemiparesis and or other localizing neurologic abnormalities attributable to carotid artery stenosis. Failure to maintain an adequate perfusiL1n pres" ure within the deep capillarie" of the optic nen'e head is believed tll be the pathoph\'"iologic mechani" m re"ponsible for the occurrence of acute anterillr ischemic optic neuropa thy (1). These vessels are "upplied by the pllsteril;r ciliary arteries, which, when LOmpromised, lead to i~farctil1J1 of the nerve in and around the lamina cribrosa (2). In cases of severe carotid arterial steno"is, a" wa" the situation in the patient de"cribed, a distallv transmitted dl'crea"e in perfusil1J1 pre"sure n1L1~t likelY It'd to "mall-\'essel in"ufficiencY and subseque~t infarction within the anterillr optic lwnT. While rdin,11 arlL'ri,ll cllmpromi~e, in the fllrm ot central rdin,,1 artl'ry ob~truction, appear" to be a coml110n Iy enCllU nlL'red m,lll ife~t,l tion L1t the l1Cular i~chemic ~yndron1l' (9), ~uch wa" l1L1t the case in this pdtient. rre~ul11ably, preferential athero~ dt'ro~is within the po~terillr ciliary \'e""els, a" cllmp,Hed tll the cl'ntr,11 retin,11 ,Hter~', might well hdve rl'nlkred the tllrn1l'r rel,ltively more "u"ceptible tll Sl'Yl'fL' cllmpromi~e upon expL1~ure to a low perfu~illn prl's~url'. The ,1~~llci,ltilln llt rubeo~i~ iridi~ with severe carotid artl'n' ~tl'l1L1~is hdS prl'Yi'1u~ly been described (10). The prl'~l'ncl' llf iris Iwo\'ascularization in cllnjunctilln with ,1I1tnior i~chemic nptic neurop,1thv, in the ab~l'IKe of diabetic retinnpathv or central rL'tinal vein llb~tructinn, strongly suggests that CllnClll11itant carotid obstructive disease exists I CIIIJ Nl'lIw-ophthlllllllll, \/01. 6. No.1, 1986 C, C. BROWN (l), Ill), Uthn c1inic,d fe,llun's th,11 supporl the di,lgl111sis of m,Hh.l'd c,Holid com prom ise include midpl'riplll'r,11 rdin,11 lll'nHlrrh"ges ,1nd n,lITowed rdin,ll ,Hkril's, In nllllr,ls1 10 this c1inic,,1 sdling, eves wilh lhe usu,ll forlll of ,1Ilkrior ischemic optic l1l'urllp,llhv (eillll'r ,Hteritil' llr non"rkrilic) do nol m,1l1ifl'st such signs, In IIll'sl' insl,1fKl's, rdin,l) 1ll'lllllITh,lgl's 1ll,1V bl' presenl in lhe imnll'di.1!l' ~'erip,lpill,H\' region bul are cllnspicuouslv ,1bsl'nl l,lsewhl'rl', Fnlm the d,ll,l presenll'd herein ,1I1d inforll1ation reporll'd bv oll1l'r ,H1tllllrs (3,h), it appl'.HS th.1t ev,1llh1tion for c,lrotid arll'rial obstruction should be considl'red in lhllsl' C,1Sl'S of anll'rior isdll'l1llc t1ptic nt'uwp,1thv ,1ssociall'd with l'itht'r till' c1inic,ll ma nifesta tit)ns lit Ihe tlcul,H ischell1 ic synti rome or focal neurologic deiicits, Howevn, uncntaintv exists as to whether patients with ilnll'rior ischemic optic neuropathy ilnd tIll' absence of thest' concomitant abnormalities should be subjecll'd routinely to carotid artery studies, Although St'vere carotid arll'ry obstruction ilppears to be a rare precipitating cause of anterior ischemic optic neuropathy, the aSSllCiatillil should be recognized, The presence of a pall', swollen optic nerve in conjunction with rubeosis iridis, predominantly midperipheral retin,ll hemorrhages, and a marked delay in retinal and choroidal filling with fluorescein angiography should arouse suspicion of this possible relationship, Acknowledgment: This work was supported in part by the Retina Research and Development Fund, Philadl'lphiil, Pennsylvania, REFERENCES f'"uld" W..... 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