Journal of Neuro-Ophthalmology, September 1985, Volume 5, Issue 3

Mixed arteriovenous occlusive disease of the fundus.

Sudden loss of vision occurred in two young healthy patients who had the clinical findings of partial retinal artery and partial retinal vein occlusion. This might represent a severe variant of papillophlebitis.

f. Clill. NCl/r"'''I,hlllllllll"l. 5: loot-loR, IW6 © 1985 Raven Press, New York Mixed Arteriovenous Occlusive Disease of the Fundus JOYCE H. CASSEN, M.D., Ph.D. ROBERT L. TOMSAK, M.D., Ph.D. VINCENT P. DeLUISE, M.D. Abstract Sudden loss of vision occurred in two young healthy patients who had the. c1inic.al find.ings of partial retinal artery and partial retinal .vem occlu­sion. This might represent a severe vanant of pap­illophlebitis. Two healthy young adults developed the sudden onset of unilateral visual loss. They were found to have characteristics of both par­tial retinal artery and partial retinal vein occlu­sion. This peculiar ocular ischemic event might be a serious variant of papillophlebitis. Case Reports Patient 1 A 32-year-old man with a history of classic migraine suddenly lost vision after bending over and feeling Iightheaded. Examination at the Cleveland Clinic Foundation 4 h later dis­closed right eye acuity of counting fingers. Left eye vision was 20/25 at distance and Jl + at near. Ocular motility was normal. Goldmann vi­sual fields were normal on the left but showed generalized constriction, especially in the upper nasal field, and a small but dense central sco­toma on the right (Fig. 1). Moderate venous en­gorgement and early optic disc edema was seen in the right fundus (Fig. 2). The left fundus was normal. Intraretinal circulation time was determined by intravenous fluorescein angiography. The first evidence of venous filling occurred 68 s after the early arterial phase (normal, 6-8 s) (Fig. 3). Poor filling of the optic nerve capillaries From the Department of Ophthalmology (jHC, RLT). Cleve, land Clinic Foundation. Cleveland. Ohio; and Southern New England Regional Eye Center (VPdLj, Waterbury. Connecticut. Write for reprints to: Dr. Robert L. Tomsak. Department of Ophthalmology. Cleveland Clinic Foundation. 9500 Euclid Avenue, Cleveland, Ohio 44106. 164 was also noted. The patient was initially treated with 200 mg of prednisone orally. The following day, right eye vision had improved to 201100. The Marcus Gunn pupil was stili present. A small hemorrhage was noted on the optic disc. The retinal veins were not able to be collapsed by digital pressure on the globe ..A second flu­orescein angiogram showed continued prolon­gation of the intraretinal circula~on time. The patient was admitted to the hospItal. He was treated with topical Timoptic and Pro­pine and oral Oiamox in an effort to de,!e~se intraocular pressure and resistance to artenal ill­flow to the right eye. Oral nitroglycerine was given in an attempt to dilate the retinal arteries and a single retrobulbar injection of 3 cm3 of 2% xvlocaine was administered for the same effect. J On the 3rd hospital day, the macular area de­veloped loss of retinal transparency (Fig. 4) a~d a few dot and blot hemorrhages were seen ill the midperipherv of the fundus. A periocular injection of 40 m'g of triamcinalone was give~. All diagnostic tests done were normal m­c1uding transfemoral right carotid angiography, complete blood cell count, SMA-18, VORL, Westergren sedimentation rate, fibrinogen, an­tinuclear factor, prothrombin and partial throm­boplastin times, and crvofibrinogen. The patient was discharged with instructions to continue ocular hypotensive therapy. Repeat examination 12 days after the onset of visual loss showed right eye vision to be 20/60­2. The fundus picture was essentially un­changed. Two weeks later visual acuity was 20/40. Fun­duscopic examination showed resolution of the disc hemorrhage and the appearance of an op­tociliary shunt vessel (Fig. 5). Intraretinal cir­culation time had improved to 15.5 s. The im­provement in intraretinal circulation is graphi­cally shown in Fig. 6. Examination 6 weeks after his initial presen­tation documented right eye vision of 20/25. Goldmann visual field was also improved, with only a small paracentral scotoma remaining. His vision has remained unchanged 1 year later. Journal of Clinical Neuro-ophthalmology Cassen, Tomsak, DeLuise LEfT RIGHT Figure 1. Goldmann visual fields (patient 1). Patiellt 2 A 38-year-old woman who was in previously good health \-"as examined because of sudden visual loss in the left eye. She had previously experienced several episodes of transient blurred vision lasting 5-45 min in the preceding 2 weeks. Visual acuity was right eye 20/20 and left eye counting fingers at 2 ft. An afferent pupil was present on the left. Slit lamp examination and intraocular pressures were normal. Hemorrhagic swelling of the left optic disc Figure 2. Venous engorgement and early optic disc edema 00 (patient 1). September 1985 was seen in combination with loss of transpar­ency of the macular retina. Dot hemorrhages and microaneurysms were also seen in the pos­terior pole (Fig. 7). Fluorescein angiography showed a swollen optic disc and a prolonged intraretinal circulation time. A complete neurologic and rheumatologic evaluation, including an enhanced high reso­lution computed tomography scan of the head and orbits, antinuclear antibody, VORL, com­plete blood cell count, PT, PIT, cryoglobulins, quantitative immunoglobulins, and Westergren sedimentation rate were all normal. She was hospitalized and treated with meth­yl- prednisolone intravenously for 3 days and subsequently with oral prednisone. When this treatment failed to improve her vision, a left ca­rotid arteriogram was done. This was normal, with good visualization of the ophthalmic artery and choroidal blush. Repeat fundus photographs done 7 days after the initial examination showed persistent hem­orrhagic papillitis and macular edema (Fig. 8). A marked delay in fluorescein transit during both arterial and venous phases was confirmed by repeat intravenous fluorescein angiography. The final impression was a combined partial retinal artery-vein occlusion. No restitution of visual function occurred, and neovascular glau­coma developed. Discussion Lyle and Wybarl first described retinal vas­culitidies at the level of the optic disc in six healthy patients. Significant visual loss did not occur and the course was benign. Marked ve­nous dilation was accompanied by a variable amount of retinal hemorrhage. Lonn and Hoyt2 165 Disease of the Fundus Figure 3. Intravenous fluorescein angiogram showing delayed intra­retinal circulation (patient 1). termed this condition papillophlebitis. They noted the absence of an afferent pupillary defect in the affected eye. Hart, Sanders et aJ.3 de­scribed fluorescein angiographic findings in nine patients. In general, normal arterial filling occurred but venous filling was delayed. How­ever, intraretinal circulation times were not re- Figure 4. Increased optic disc edema and loss of retinal transparency (patient 1). 166 ported. Ophthalmodynamometry was equal in the affected and unaffected eyes. Ellenberger and Messner called attention to the lack of sig­nificant impairment of color vision and visual field in papillophlebitis. Many authorsl - 6 have mentioned the super­ficial similarity of papillophlebitis (retinal vas- Figure 5. Small shunt vessel on disc and resolution of disc hemorrhage (patient 1). Journal of Clinical Neuro-ophthalmology CIRCULATION TIME 70 u C1I 1II C1I E... c: 0 ... ~ ::J U !.. U .~c.:. C1I !.. ~ .!.... c: 10 10 20 30 Days Figure 6. Improvement of retinal circulation (patient I). Cassen, Tomsak, DeLuise Figure 7. Early optic disc edema (patient 2). culitis) to central retinal vein occlusion but have noted the disparity in age, extent of retinal hem­orrhage, and predisposing factors such as hy­pertension and atherosclerosis. However, the presence of shunt vesselsu ,5,6 in some cases suggests that obstruction of venous outflow from the eye is a prominent component in pap- September 1985 Figure 8. Disc hemorrhage and loss of retinal transparency in pos­terior pole (patient 2). 167 Disease of the Fundus illophlebitis. It is interesting that, in central ret­inal vein occlusion, the presence of shunt ves­sels might be predictive of a better visual out­come.? None of these authors mentioned symptoms of retinal arterial obstruction, as seen in our cases (sudden, severe decrease in vision, loss of retinal transparency, afferent pupillary defect, and severe visual field deficit). However, Katz and SmithH reported cilioretinal artery occlusion complicating papillophlebitis in a 26-year-old woman. Sluggish intraretinal circulation with­out significant visual loss has been described as "papilloarterial ischemia". 9 Our first patient suffered from classic mi­graine and had persistent visual loss brought on by bending over, as sometimes occurs in pa­tients with migrainous transient monocular blindness (Tomsak, R.L., unpublished obser­vations). In this regard, it is known that vaso­constriction occurs in the initial stages of mi­graine, lO and might lead to ischemic optic neu­ropathy. ll,12 We propose that prolonged, possibly migrainous, arterial vasospasm led to retinal hypoxia and acute visual loss in our first case. The resultant low-flow state led to venous stasis and partial venous thrombosis. As the va­sospasm abated, either spontaneously or from therapeutic intervention, reperfusion and, pos­sibly, recanalization of venous thrombosis oc­curred, eventually leading to partial restitution of visual function. It is noteworthy that Green and co-workers13 have histologic evidence of thrombosis and recanalization of central retinal vein occlusion. In our second patient, evidence of a combined retinal artery and retinal vein occlusion was also present. Although a clear-cut history of mi­graine was not elicited, the prodromal symp­toms of transient monocular blindness were s~ggestive of a vasospastic etiology, especially since a complete diagnostic evaluation was normal. Unfortunately, permanent loss of vi­sion occurred in this patient. We believe the symptoms and findings doc­umented above are consistent with a mixed ar­teriovenous occlusion of the retinal circulation, a~? repr.esent a ser.ious variant of papillophle­bltls. ThIS entity mIght be similar to Cogan'sl4 "prima.ry, sever~'.' retinal vasculitis, although systemIc vascuhtldes and bilateral ocular in­volvement are often present in this condition but are absent in our two patients. 168 Although we have no proof that therapeutic intervention improved the vision in patient I, the use of systemic vasodilators and ocular hy­potensive agents might have been useful. Steroids4 might have also helped, as Appen and others noted histologic evidence of phlebitis in an eye with optic disc vasculitis that went slowly blind and was subsequently enucleated for neovascular glaucoma. IS References 1. Lyle, K T., and Wybar, K: Retinal vasculitis. Br. /. Ophthalmol. 45: 778-788, 1961. 2. Lonn, L. 1., and Hoyt, W. F.: Papillophlebitis: A cause of protracted yet benign optic disc edema. Eye, Ear, Nose, Throat Month. 45: 62-68, 1966. 3. Hart, C. D., Sanders, M. D., and Miller, S. ]. H.: Benign retinal vasculitis. Br. /. Ophthal. 55: 721­733, 1971. 4. Ellenberger, c., and Messner, K H.: Papillo­phlebitis: benign retinopathy resembling papil­ledema or papillitis. Ann. Neural. 3: 438-440, 1978. 5. Laibovitz, R. A.: Presumed phlebitis of the optic disc. Trans. Am. Acad. Ophth. Otol. 86: 313-319, 1979. 6. Hayreh, S. S.: Optic disc vasculitis. Br. J. Ophthalmol. 56: 652-670, 1972. 7. Priluck, 1. A., Robertson, D. M., and Hollen­horst, R. W.: Long-term follow-up of occlusion of the central retinal vein in young adults. Am. /. Ophthal. 90: 190-202, 1980. 8. Katz, R. S., and Smith, ]. L.: Papillophlebitis causing cilioretinal artery occlusion. In Neuro­ophthalmology Focus 1980'. Masson, New York, 1979, pp. 69-76. 9. Kottow, M. H.: Papilloarterial ischemia. Ann. Ophthalmol. 13: 963, 1981. 10. Edmeads, ].: Cerebral blood flow in migraine. Headache 17: 148-152, 1977. 11. Weinstein, ]. M., and Feman, S. S.: Ischemic optic neuropathy in migraine. Arch. Ophtha/mol. 100: 1097-1100, 1982. 12. Cowan, C. L., and Knox, D. L.: Migraine optic neuropathy. Ann. Ophthalmol. 14: 164-166, 1982. 13. Green, W. R., Chan, C. c., Hutchins, G. M., and Terry, ]. M.: Central retinal vein occlusion: a prospective histopathologic study of 29 eyes in 28 cases. Retina 1: 27-55, 1981. 14. Cog~n, D. G.: Ophthalmic manifestations of sys­temic vascular disease. In Major Problems in In­temal Med/cme, Vol. III. Saunders, Philadelphia, 1974, pp. 135-137. 15. Appen, R. E., DeVenecia, G., and Ferwerda, J.: Optic disk vasculitis. Am. J. Ophthalmol. 90: 352­359, 1980. Journal of Clinical Neuro-ophthalmology