Journal of Neuro-Ophthalmology, December 1986, Volume 6, Issue 4

Resolution of optociliary shunt vessels.

A 40-year-old woman developed profound bilateral loss of vision from chronic atrophic papilledema. There were striking optociliary shunt vessels on the right optic disc. Within 1 month after removal of a massive intracranial meningioma, the shunt vessels had regressed markedly. This regression was evident on fluorescein angiograms. We attribute their resolution to the normalization of intracranial pressure.

Jour/wi of Cli"iCIII Nl'IIn>-"I'''tlW[",olo,~1/ 6(41: 205-208, lY8b. Resolution of Optociliary Shunt Vessels Sydney L. Tyson, B.A., M.P.H., and Simmons Lessell, M.D. IY86 Raven Press, New York A -lO-year-old woman developed profound bilateral loss of vision irom chronic atrophic papilledema. There were striking optociliarv shunt vl'ssels on thl' right optic disc Within 1 ml1l1th ,liter remov,ll of a massive intracranial meningilmla, the shunt vessels had regressed markedly. This regression was evident on tluorescein angiograms. We attribute their resolution to the normalization oi in­tracranial pressure. Key Words: Brain tumor-Optic nerve-Optociliary veins- Papilledema. From the Neuro-ophthalmology Unit, Massachusl'tts EyL' and Ear Infirmary, Boston, Massachusl'lts. Address correspondence and reprint rl'LJul'sts III Dr. Simmons Lessell, 243 Charles St., Boston, MA lJ2114, USA 205 Acquired optociliary shunt veins are thought to develop as a result of progressive interference with outflow through the central retinal vein. This interference fosters the development of shunts be­tween retinal and choroidal veins. The evolution of these bypass channels has been well docu­mented by fluorescein angiography (1). However, little has been written on their resolution. We present the case of a patient whose optociliary shunt vessels had resolved completely 1 month after surgical decompression of a large sphenoid ridge meningioma. CASE REPORT A 40-year-old woman was well until June 1985, when she began to complain of transient obscura­tion of vision in her left eye. This was followed several months later by transient obscurations in her right eye and progressive loss of vision in both eyes. By December 1985, she was blind. The pa­tient did not consult a physician until April 1986. His examination revealed bilateral visual loss with optic atrophy. A large contrast-enhancing mass, extending from the greater wing of the sphenoid on the right with edema and subfaIcial and uncal herniation, was imaged on a computerized tomo­graphic (CT) scan (Fig. 1). Neuro-ophthalmologic examination was per­formed 1 week later. Visual acuity was no light perception in the right eye and light perception in the left. Exophthalmometry measured 23 mm on both sides, but the family reported that the pa­tient's eyes had always been prominent. Motility examination was abnormal. The right eye tended to drift slowly during fixation in the primary posi­tion, and the left eye could not fully adduct, ab­duct, or depress. Slit-lamp examination showed normal anterior segments. Her pupils were equal, with no direct response on the right and a minimal response on the left. Ophthalmoscopy revealed bi­lateral secondary optic atrophy with prominent s /./rs( W liNn s. I.LSSLLL FIG. 1. (a) Axial CT scan showing large right hemispheric mass with surrounding edema and subfalclan herniatIOn (b) Coronal CT scan showing right hemispheriC mass with bilateral distention of OptiC nerve sheaths. llptociliarv shunt vessels lln the right llptic di:,c (Fig. 2). No shunt vesseb were seen lHl the left. Phvsical and neurllll)gical t':--aminatillns were nllrmaL In May 1980, a meningil)ma lli the right sphenoid ridge WdS ren1l1\'ed tlltally. Four week:, later, visual acuity was h,lnd motions in bllth eves, and the :,hunt \'essels had disappeared irllm tilt' right disc (Fig. 3). Minimal shunt vessel rl'sidu,) FrG. 2. Fundus of right eye showing chronic atrophic papilledema. Note shunt vessels. could be identitied l)n a ilul)rescein angiogram (Fig . .f). Tht' remainder l)i the e\.amination was unchanged. DISCUSSION Acquired l)ptl)ciliclrv \eins h,l\·e been described mllst irequenth· in cl)nditil)ns assl1Ciated with cllmpressil1n l)t the central rdinal \'ein: chronic FIG. 3. Postoperative appearance of right fundus, Shunt vessels have disappeared, RESOLUTION or OPTOc/UARY SHUNT VESSELS 207 FIG. 4. Fluorescein angiogram (arteriovenous phase) shows filling of small. residual shunt vessels. glaucoma (2--1). chronic papilledema (5). occlu­sion of the central retinal vein (6), and in tumors such as glioma (7) and optic nerve sheath menin­gioma (8-1-1). They also have been reported with drusen of the optic disc (15), arachnoid cysts of the optic nerve sheath (16), and phacomatosis (17). Four publications describe reduction in caliber of shunt vessels (18-21). One describes their re­gression after radiation therapy for optic nerve sheath meningioma (18). Two other reports noted partial regression after surgical decompression of the optic nerve sheath in patients with chronic papilledema (19,20). There is only one reported case of total resolu­tion of shunt vessels (21). This occurred 11 days after surgical removal of a subfrontal meningioma. Postresolution angiography, if done, was not mentioned. No mention was made of the extent to which visual acuity was restored. Our case is quite similar, with nea; total resolution I month after surgical decompression. What is the pathogenesis of these abnormal shunt vessels? Two mechanisms can be postu­lated. Cogan (11) postulated that in optic nerve sheath meningiomas, shunt vessels may represent drainage from the tumor itself. In cases associated with venous obstruction, the shunts presumably represent enlargement of pre-existing capillary vessels bridging the retinal and choroidal circula­tions (10-12,14). Boschetti, Smith, Osher, Gass, and Norton (22) have reported that the fluorescein angiographic appearance of the optociliary shunt vessels in these two groups are characteristically different. In the optic nerve sheath meningioma group, it was noted that the shunt vessels filled earlier (in the arteriovenous phase), the flow drained to central venous tributaries, and the late staining was hy­perfluorescent to other veins. However, in the central vein occlusion group, the shunts filled later (in the venous phase), showed a flow draining to the outer disc margin, and late staining was eu­fluorescent to other veins. In our patient, the fluoroangiographic appear­ance of the vessels was more typical of changes noted in patients with optic nerve sheath menin­giomas. Fluoroangiography revealed early filling of shunts in the arteriovenous phase and drainage to central venous tributaries. We were not able to characterize the staining pattern in the late stages of angiography. Despite these analogous changes, there was no evidence of meningiomatous infiltra­tion of the optic nerve in our patient. This discrep­ancy calls into question the value of fluoroangi­ography in differentiating their pathogenesis. What accounts for the decrease in the caliber of these shunt vessels? We speculate that in normal individuals, these channels are invisible, because pressure in the retinal and choroidal circulations is nearly equal. However, when pressure in the cen­tral retinal vein becomes greater than that in the uveal venous circulation, blood flow is diverted through these channels, and they become ophthalmoscopically and angiographically visible. When the obstruction of the central retinal vein is relieved, the differential pressure which initiated the development of these shunts falls, allowing regression. In our patient and in the reported cases with chronically elevated intracranial pres­sure (18-21), normalization of the intracranial pressure presumably relieved the obstruction of the central retinal vein in the subarachnoid space and restored the balance of pressure between ret­inal and choroidal circulations. The angiographically documented regression of shunt vessels after decompression reinforces the hypotheses that these shunts are formed from pre-existing vascular channels that connect retinal and choroidal venous systems, and that their ap­pearance and disappearance are, in great part, de­pendent upon the balance of central retinal and choroidal Vl'11llUS pressures. REFERENCES 1. 1n1<'s RK, SCh,ltz H, Hllvt WF, Mllntiero MLR, Narahara M. EVlllllti,)Il "' llptllcili,ln; Vl'ins in llptiC nerve sheath menin­gillm, l. :11<// l )l'iJIIlIlllIl<l/ 14HS;1ll3:59-60. , Stellwag C Vlln C. LciJrlJ//ciJ der I'raktisd/ell AIIgCllI/ei/kliIlde. 2nd I'd. Vil'nn,l: Bralillmliller, 11'64:283. 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