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Show J. Clin. Neuro-ophthalmol. 3: 185-189, 1983. Bitemporal Palsy of the Pupillary Sphincters A Sign of Diffuse Circulatory Insufficiency AVINOAM B. SAFRAN, MD. YVES JANIN, MD. ANDRE ROTH, MD. Abstract Two patients with generalized arteriosclerotic changes presented with oval-shaped pupils in both eyes. Sectorial palsy of the iris sphincters, essentially in the lower temporal segments, was demonstrated. Paralyzed sectors did not react to 2% pilocarpine. There was no dilator weakness, and the iris did not exhibit atrophy or synechia. We consider the sectorial palsy of the sphincters in these patients to be due to circulatory insufficiency of the iris. We reviewed previously published cases of diffuse or multifocal circulatory insufficiency with ischemic iris changes, and analyzed the accompanying photographs. We found that temporal segments of the iris are predominantly involved in these patients. Predisposition to this sectorial palsy, especially in the pupillary temporal region, might be explained by several anatomical particularities of the vascular network in the iris. Bitemporal palsy of the pupillary sphincters may be a sign of generalized circulatory insufficiency. All tissues of the anterior segment of the eye can be affected by ischemic injury.l Clinical features of circulatory insufficiency of the iris include functional impairment of iris muscles, sectorial atrophy of the stroma, and neovascularization.2 We describe here two patients with diffuse arteriosclerotic changes and presenting with sectorial palsy of both iris sphincters. Palsy affected essentially the lower temporal sectors and induced a lower temporal elongation of the pupil. Case Reports Case 1 A 75-year-old man was examined at the Geneva University Eye Clinic. Clinical signs included right exotropia with right amblyopia. In addition, he had high blood pressure for 16 years and at 63 years of From the Clinique d'Ophthalmologie, Hopital Cantonal Universitaire, Geneva, Switzerland. September 1983 age had suffered a transient ischemic attack with right hemiparesis. For 6 months before consulting, he experienced memory loss and intermittent claudication. Doppler sonography revealed severe impairment of vascular flow in both internal carotid arteries. Best-corrected visual acuity was R.E. 20/200 and L.E. 20/50. The patient showed right comitant exotropia. Slit lamp examination revealed that both pupils were irregular in shape and that there was an incipient corticonuclear senile cataract. Ocular pressure was 15 mm Hg in both eyes. Fundi examination disclosed attenuation of the arterioles and numerous arteriolovenous crossing changes. The macular area showed slight mottling of pigmentary epithelium. Both pupils were elongated toward the lower temporal quadrants. Partial palsy of the pupillary sphincters was observed in the right eye in the area from 5 to 11:00 (Fig. 1), and the sphincter was paralyzed in the left eye in the area from 1 to 7:00 (Fig. 2). Transillumination did not reveal any atrophic area of iris pigmentary epithelium. The pupils failed to react to 1 drop of 0.2% pilocarpine but became regularly dilated in darkness or with 2 drops in each eye of 10% phenylephrine. One week later, topical application of 2 drops of 2% pilocarpine in each eye caused the pupils to contract with the exception of the area from 5 to 11:00 in the right eye and from 1 to 7:00 in the left. Case 2 A 68-year-old man was admitted to Geneva University Hospital for evaluation of various vascular problems. This patient presented with ostium primum, polycystic kidneys, high blood pressure, angina pectoris, auriculoventricular block and cardiomegaly. Ten years earlier, the patient had sufferred from obstruction of the superior temporal retinal vein in the left eye, for which he had not been given any local treatment. Best-corrected visual acuity was R.E. 20/20 and L.E. 20/50. Eye movements, corneal sensitivity, and ocular pressure were normal. Slit lamp examina- 185 Palsy of Pupillary Sphincters Figure 1. Patient 1. The right pupil is elongated towards the lower temporal sector. The sphincter shows partial palsy in the area extending from 5 to 11:00. Figure 2. Patient 1. The left pupil demonstrates lower temporal elongation with sectorial palsy of the sphincter in the area extending from 1 to 7:00. "Streaming of stroma" points to 12 and 8:00 positions. Figure 3. Patient 2. In the right eye, the sphincter shows a sectorial palsy in the area extending from 5 to 11 :00. tion showed sectorial palsy of the iris sphincter in both eyes and, mainly in the right eye, cortical lens opacities. Both fundi showed generalized attenuation of the arterioles and arteriolovenous crossing defects. Capillary dilatation, microaneurysms, and Figure 4. Patient 2. In the left eye, sectorial palsy of the sphincter can be seen in the area extending from 1 to 7:00. hard exudates were seen in the area of the left superior temporal vein. Both pupils were elongated, with dilatation in the lower temporal quadrants. Sphincters were paralyzed, in the right eye in the area from 5 to 11:00 (Fig. 3) and in the left eye in the area from 1 to 7:00 (Fig. 4). Transillumination revealed no iris atrophy. Pupils failed to react to 1 drop of 0.2% pilocarpine, whereas darkness or 2 drops of 10% phenylephrine caused a regular dilatation of both pupils. One week later, bilateral topical application of 2 drops of 2% pilocarpine induced a contraction of the iris sphincter in both eyes, sparing however the area extending from 5 to 11:00 in the right eye, and 1 to 7:00 in the left eye. Discussion In both patients, pupils were elongated in the direction of the lower temporal quadrants. This pupillary distortion was associated with sectorial sphincter palsy, essentially localized in the lower temporal quadrant. Dilator integrity as well as absence of either iris atrophy, cicatricial retraction, or synechia indicated that the pupillary anomaly was probably due to the sectorial sphincter palsy. Sectorial palsy of the iris sphincter can occur in several conditions including lesions in the midbrain, third cranial nerve, ciliary ganglion, short ciliary nerves, or the iris tissue.:J In the present study, no patient showed any other clinical signs of mesencephalic involvement. Furthermore, normal eye and lid motility makes a diagnosis of third nerve involvement unlikely, particularly considering the usual relative sparing of the pupil function in ischemic third nerve palsies.4 Sectorial failure to react to 2% pilocarpine is not consistent with ciliary ganglion or short ciliary nerves involvement.5 In fact, the sectorial failure of the sphincters to contract in response to 2% pilocarpine was a positive indication that the lesion was located in the Journal of Clinical Neuro-ophthalmology iris itself. Neither patient had any history or signs of previous ocular trauma, or any local inflammation. Since it has been demonstrated that sectorial palsy of the sphincter can be the only sequela of hypoxic injury of the iris,6 we considered the history of generalized arteriosclerotic changes in our patients, and we concluded that the sectorial palsy of their iris sphincters was probably due to some circulatory disturbance. Although ischemic lesions of the anterior segment were recognized by Schmidt in 1874,' detailed descriptions of ocular changes due to local interference with the blood supply of the anterior segment only began to appear in 1941 with the studies of extraocular muscles transposition by leinfelder and Black.8 Most studies of iris ischemia were done on patients operated on for strabismus9 - 16 or retinal detachment,17-21 or on diabetic subjects.2. 22. 2:1 Other studies on iris ischemia were made on patients with aortic arch syndrome/:1 carotid occlusion, I. 2~ carotid cavernous fistulae,6 actinic angiopathies, I sequelae of retinal venous obstruction/ sickle cell disease,25 zoster/" or rubella l infections. We attempted to determine by examining previously published cases whether the temporaland in particular, the lower temporal-iris sector is more vulnerable to hypoxic injury than other iris sectors. It has been established that temporal sectors of the iris are predominantly involved in patients operated on for strabismus or retinal detachment/ 7 but no study seems to have been made to determine the topography of iris lesions in patients with diffuse ischemia of the eye. Although postoperative hypoxia of the iris is essentially comparable to hypoxia due to global iris circulatory failure, these two phenomena are not necessarily identical in their physiopathology: surgical procedure may cause localized vascular failure, whereas iris circulation, theoretically, would be more globally impaired in patients with generalized vascular conditions. Therefore, in order to determine a particular susceptibility of temporal iris tissues to hypoxia, the predominance of temporal lesions would have to be established in patients with generalized circulatory disturbances. Among the published papers dealing with sectorial lesions of the iris of such patients, descriptions of the topography of these lesions are rare. Therefore, we decided to analyze the illustrations of these articles, in which it was possible to determine which were the temporal and which the nasal sides. We collected the cases in which a sectorial predominance of the lesions could be observed; we were able to obtain adequate data in seven papers"' 14,22-2",28 involving 13 eyes in all. Considering the four eyes described in the present study, the final analysis was made on 17 eyes. In 1959, Vergez2H described a patient with signs of anterior segment ischemia due to carotid throm- September 1983 Safran, )anin, Roth bosis. Subsequent examination of the iris revealed nasal sectorial atrophy. Several patients with ischemic ocular inflammation were described by Knox2:1in 1965. He noted that in one patient the right pupil in the upper nasal quadrant was dilated. In 1969, Sanders and Hoyt" reported several cases of anterior segment ischemia secondary to carotid cavernous fistulae. In most of them, iris changes were noted. On one of the accompanying photographs, a sectorial temporal superior atrophy of the iris could be distinguished. In 1973, Galinos et a1.2" described patients with sickle cell disease. In three eyes, sectorial atrophy was predominantly temporal. In 1973, Easty and Chignell 14 published a number of fluorescein angiographies of patients with anterior segment ischemia. The iris of one glaucomatous eye showed lower temporal atrophy (Fig. 2a of Easty and Chignell's study) and that of another eye, nasal sectorial atrophy (Fig. 3 of Easty and Chignelt's study). A study of rubeosis iridis in 33 diabetic eyes was made by laatikainen22 in 1979. In two of these eyes, we were able to recognize a lower temporal elongation of the iris (Figs. 3 and 4 of laatikainen's study). Hayreh and Podhajsk/4 recently published iris fluorescein angiography photographs of two patients with unilateral occlusion of the central retinal artery and stenosis or occlusion of an internal carotid artery. In the first patient, the left iris showed numerous avascular areas, the larger located in the lower temporal sector. In the second patient, the right iris presented a large avascular area, predominantly located on the temporal side. A few spots of neovascularization, most of them in the superotemporal sector, were observed in the left iris. Therefore, of 17 eyes with sectorial lesions of the iris, 14 show a predominantly temporal involvement, whereas three show changes mainly in the nasal sector of the iris. In eight eyes, the injury was predominantly inferotemporal. Certain anatomical data can explain why the temporal iris sectors are predominantly affected in patients with diffuse circulatory insufficiency. The arterial anastomosis network of the iris is incomplete/~·:llI and, therefore, collateral blood supply cannot always be provided in case of sectorial reduction of the blood flow. The temporal and the nasal sectors of the iris are not supplied by the same arteries. The temporal sector of the iris seems to be essentially supplied by the superior and inferior anterior ciliary arteries, while the nasal sector by the medial long posterior ciliary artery.27 It is conceivable that in certain vascular diseases, the blood supply would be more easily disturbed in the network of the anterior ciliary arteries than in that of the long posterior 187 Palsy of Pupillary Sphincters ciliary arteries. The function of the temporal sector of the iris would then be affected before that of the nasal sector. The temporal sector of the normal lflS seems less vascularized than the nasal sector. In one study of the normal iris, the vascularization was described as maximal in the superior nasal sector.~1 In another study, it was found that in some normal eyes the iris vascularization is much greater in the nasal segment than elsewhere. In others, no regional variation of density of iris vessels was detected. 30 It was also found that in a few normal eyes, the vessels in one segment of the pupillary region (usually the temporal part) showed a substantial delay in filling, so that it was the last part of the iris to fill. 30 This could explain why it is predominantly the temporal sector of the iris, especially in the pupillary region, that is affected by a decrease of the blood flow in the iris vascular network. The selective sphincter dysfunction observed in the present study might be further explained by the fact that ischemic injUry of the iris is more likely to occur in the vicinity of the collarette, since its vessels are of small caliber and are often partially obliterated. 29 Fisher32 recently reported oval pupils in one or both eyes of 17 patients with serious cerebrovascular illness. The oval shape of the pupil was transient in several patients, and permanent in others. The orientation of the long axis was described in 16 eyes using the face of a clock as reference. However, the location (nasal or temporal) of the iris dysfunction cannot be deduced. Fisher suggests that in most of his cases the distortion of the pupil was due to a midbrain lesion. Although it is probable that in a number of his patients a midbrain lesion was the cause of elongation of the pupil, it seems likely (based on the findings of the present study) that several subjects of Fisher's study had sectorial palsy of the iris sphincter, secondary to an ischemic injury of the iris and probably located mainly in the temporal part. Sectorial palsy of the iris sphincter, especially in the temporal part, may be a sign of global circulatory insufficiency of the iris. It is probably much more frequent than would appear from the literature. References 1. Crock, G: Clinical syndromes of anterior segment ischemia. Trans. Ophthalmol. Soc. u.K. 87: 513-533, 1967. 2. Kottow, M.H.: Anterior Segment Fluorescein Angiography. Williams & Wilkins, Baltimore, 1978, pp. 177-214. 3. Thompson, S.H.: Segmental palsy of the iris sphincter in Adie's syndrome. Arch. Ophthalmol. 96: 16151620, 1978. 4. Goldstein, J.E., and Cogan, D.G: Diabetic ophthalmoplegia with special reference to the pupil. Arch. Ophthalmol. 64: 492-500, 1960. 5. Thompson, S.H., Bell, R.A., and Bourgon, P.: The natural history of Adie's syndrome. In Topics in Neuro-ophthalmology, H.5. Thompson, R. Daroff, L. Frisen, JS Glaser, and MD. Sanders, Eds., Williams & Wilkins, Baltimore, 1979, pp. 96-99. 6. Sanders, MD., and Hoyt, W.F.: Hypoxic ocular sequellae of carotid cavernous fistulae. Br. I. Ophthalmol. 53: 82-97, 1969. 7. Schmidt, H.: Beitrag zur Kenntnis der Embolie der Arteria centralis retinae: Albrecht v. Graefes. Arch. Ophthalmol. 20: 287-306, 1874. 8. Leinfelder, P.J., and Black, N.M.: Experimental transposition of the extraocular muscles in monkeys. Am. I. Ophthalmol. 24: 1115-1120, 1941. 9. Stucchi, C, and Bianchi, G: Depigmentation en secteur de l'iris consecutive a des transplantations musculaires. Ophthalmologica 133: 231-236, 1957. 10. Forbes, S.B.: Muscle transplantation. Am. I. Ophthalmol. 48: 248-251, 1959. 11. Audouienex, M.E.: Atrophie de I'iris en secteur apres intervention d'Hummelsheim O'Connor. BuJJ. Soc. Ophthalmol. 60: 638-642, 1960. 12. Girard, L.J., and Beltranena, F.: Early and late complications of extensive muscle surgery. Arch. Ophthalmol. 64: 576-584, 1960. 13. Uribe, L.E.: Muscle transplantaton in ocular paralysis. Am. I. Ophthalmol. 65: 600-607, 1968. 14. Easty, D.L., and Chignell, A.H.: Fluorescein angiography in anterior segment ischemia. Br. I. Ophthalmol. 57: 18-26, 1973. 15. Von Noorden, GK.: Anterior segment ischemia following the Jensen procedure. Arch. Ophthalmol. 94: 845-847, 1976. 16. Jacobs, OS, Vastine, D.W., and Urist, M.l.: Anterior segment ischemia and sector iris atrophy after strabismus surgery in a patient with chronic lymphocytic leukemia. Ophthalmol. Surg. 7: 42-48, 1976. 17. Boniuk, M., and Zimmermann, L.E.: Pathological anatomy of complications. In Controversial Aspects of the Management of Retinal Detachment, CL. Schepens and CD,J. Regan, Eds. Little, Brown and Co, Boston, 1965, pp. 263-287. 18. Duguid, I.M.: Anterior segment necrosis following retinal detachment surgery. Trans. Ophthalmol. Soc. UK 87: 171-178, 1967. 19. Ryan, S.J., and Goldberg, M.F.: Anterior segment ischemia following scleral buckling in sickle cell hemoglobinopathy. Am. I. Ophthalmol. 72: 35-50, 1971. 20. Robertson, D.M.: Anterior segment ischemia after segmental episcleral buckling and cryopexy. Am. I. Ophthalmol. 72: 35-50, 1971. 21. Eagle, R.C, Yanoff, M., and Morse, P.H.: Anterior segment necrosis following scleral buckling in hemoglobin sickle cell disease. Am. I. Ophthalmol. 75: 426-433, 1973. 22. Laatikainen, L.: Development and classification or rubeosis iridis in diabetic eye disease. Br. I. Ophthalmol. 63: 150-156, 1979. 23. Knox, D.L.: Ischemic ocular inflammation. Am. ]. Ophthalmol. 60: 995-1002,1965. 24. Hayreh, 5.5., and Podhajsky, P.: Ocular neovascularisation with retinal vascular occlusion. II. Occur- Journal of Clinical Neuro-ophthalmology rence in central and branch retinal artery occlusion. Arch. Ophthalmol. 100: 1585-1596, 1982. 25. Galinos, S., Rabb, M.F., Goldberg, M.F., and Frenkel, M.: Hemoglobin sickle cell disease and iris atrophy. Am. ]. Ophthalmol. 75: 421-425, 1973. 26. Walsh, F.B., and Hoyt, W.F.: Clinical Neuro-ophthalmology (3rd ed.). Williams & Wilkins Co., Baltimore, 1969, p. 1357. 27. Hayreh, S.5.: Ischemia of the anterior segment of the eye. Ir. Fae. Ophthalmol. Yr. Bk. 12: 21-31, 1980. 28. Vergez, A.: Syndrome oculaire rare au cours d'une thrombose carotidienne spontanee. Ann. Oculist 192: 376, 1959. 29. Duke-Elder, St. Sir, and Wybar, K.C: In The anatomy of the visual system. System of Ophthalmology, September 1983 Safran, Janin, Roth Vol. 2. Henry Kimpton, London, 1961, p. 350. 30. Hayreh, S.5., and Scott, W.E.: Fluorescein iris angiography I. Normal pattern. Arch. Ophthalmol. 96: 1383-1389, 1978. 31. Amalric, P.: The angiography of the anterior segment of the eye. In Proceedings of the International Symposium of Fluorescein Angiography, Tokyo 1972, K. Shimisu, Ed. Igaku Shoin Lt., Tokyo, 1974, pp. 281318. 32. Fisher, CM.: Oval pupils. Arch. Neurol. 37: 502503,1980. Write for reprints to: Avinoam B. Safran, M.D., Clinique d'Ophthalmologie, Hopital Cantonal Universitaire, CH-1211 Geneva 4, Switzerland. 189 |
