Journal of Neuro-Ophthalmology, December 2003, Volume 23, Issue 4

Posner-Schlossman Syndrome and Nonarteritic Anterior Ischemic Optic Neuropathy

A 41-year-old woman with acute OD pain and decreased visual acuity presented with anterior uveitis, an intraocular pressure of 56 mm Hg, an open angle, ipsilateral nerve fiber bundle visual field defects, and optic nerve edema. With control of intraocular pressure and uveitis, visual acuity improved to 20/25, visual field defects persisted, and optic disc pallor developed. She has remained stable over 23 months of follow-up. This case represents a concurrence of glaucomatocyclitic crisis (Posner-Schlossman syndrome, PSS) and nonarteritic ischemic optic neuropathy (NAION). Although this combination occurs rarely, patients with PSS and other risk factors for NAION, including an optic disc that lacks a physiologic cup, should be protected against NAION by prophylactic treatment with ocular antihypertensive medications.

ORIGINAL CONTRIBUTION Posner- Schlossman Syndrome and Nonarteritic Anterior Ischemic Optic Neuropathy Inci Irak, MD, Bradley J. Katz, MD, PhD, Norm A. Zabriskie, MD, and Paul L. Zimmerman, MD Abstract: A 41- year- old woman with acute OD pain and decreased visual acuity presented with anterior uveitis, an intraocular pressure of 56 mm Hg, an open angle, ipsilateral nerve fiber bundle visual field defects, and optic nerve edema. With control of intraocular pressure and uveitis, vi­sual acuity improved to 20/ 25, visual field defects persisted, and optic disc pallor developed. She has remained stable over 23 months of follow- up. This case represents a con­currence of glaucomatocychtic crisis ( Posner- Schlossman syndrome, PSS) and nonarteritic ischemic optic neuropathy ( NAION). Although this combination occurs rarely, pa­tients with PSS and other risk factors for NAION, including an optic disc that lacks a physiologic cup, should be pro­tected against NAION by prophylactic treatment with ocu­lar antihypertensive medications. ( JNeuro- Ophthalmol 2003; 23: 264- 267) Posner- Schlossman syndrome ( PSS), also known as glaucomatocyclitic crisis, is characterized by recurrent attacks of anterior nongranulomatous uveitis and elevated intraocular pressure ( IOP). 1 It is generally considered a be­nign, self- limited disease, and short- term use of corticoste­roids and antiglaucoma medications controls the attacks. 2 In a recent series of PSS cases, Jap et al3 found that 14 ( 26.4%) of 53 eyes had glaucomatous optic nerve damage. We report another complication of PSS, nonarteritic ante­rior ischemic optic neuropathy ( NAION). CASE REPORT A 41- year- old Hispanic woman presented with a 4- day history of mild redness, pain, and blurred vision of the OD. She reported that a similar episode had occurred From the Department of Ophthalmology and Visual Sciences and the John A Moran Eye Center, University of Utah Health Sciences Center, Salt Lake City, Utah Reprints: Bradley J. Katz, MD, PhD, John A Moran Eye Center, Uni­versity of Utah, 50 N. Medical Drive, Salt Lake City, UT 84132. E- mail: bradley. katz@ hsc. utah. edu This work was supported in part by an unrestricted grant to the Depart­ment of Ophthalmology and Visual Sciences from Research to Prevent Blindness, Inc., New York, NY. 3 months earlier and had improved with eyedrops pre­scribed at another institution. Her past medical history was significant for hypercholesterolemia and hypertension. She was using no systemic medications. Visual acuity was 20/ 50 OD and 20/ 20 OS. There was a small relative afferent pupillary defect OD. Color vision was normal. She had some fine, round keratic precipitates and trace anterior chamber cell OD. Intraocular pressure was 56 mm Hg OD and 18 mm Hg OS by applanation. On gonioscopy, the ciliary body band was visible for 360 de­grees OU without peripheral anterior synechiae or inflam­matory deposits. Dilated funduscopic examination showed optic disc edema and flame hemorrhages on the disc margin OD and a normal appearing disc with no cup OS ( Fig. 1). The patient was immediately treated with acetazolamide 500 mg BID PO, timolol 0.5% BID OD, latanoprost 0.005% QHS OD, and prednisolone acetate 1% QID OD. On the following day, visual acuity was 20/ 50 and IOP was 13 mm Hg OD. Automated perimetry revealed dense superior and inferior arcuate defects OD and a full visual field OS ( Fig. 2). One week later, visual acuity OD had improved to 20/ 25, the anterior chamber was quiet, and the IOP was 17 mm Hg. All medications were discontinued except latanoprost. Six months later, optic disc edema had been replaced by pallor ( Fig. 3). Eleven months after pre­sentation, the visual field defects persisted in the OD, while the field of the OS remained normal ( Fig. 4). During 23 months of follow- up, there have been no further signs or symptoms of recurrent inflammation and IOP has been normal. DISCUSSION PSS is an uncommon, unilateral syndrome associated with recurrent anterior segment inflammation and elevated IOP. With acute attacks, the IOP is typically 40- 60 mm Hg. Despite acute pressure elevations, patients complain only of mild discomfort and redness. After an attack, IOP and facility of aqueous outflow return to normal. 2 The etiology of PSS is unknown, although herpes simplex virus has been isolated from aqueous humor of three patients during at­tacks4, and there is an association with HLA- Bw54.5 Glau­comatous optic nerve damage is common after attacks, 3 Copyright © Lippincott Williams & Wilkins. UnauthorizGd reproduction of this article is prohibited. 264 J Neuro- Ophthalmol, Vol. 23, No. 4, 2003 Posner Schlossman and NAION JNeuro- Ophthalmol, Vol. 23, No. 4, 2003 FIGURE 1. One week after presentation, the right optic disc is diffusely swollen with flame- shaped hemorrhages. The left optic disc appears normal, although it has no physiologic cup. and many cases eventually evolve into a clinical picture similar to that of open angle glaucoma. 6 Whether elevated IOP contributes to NAION remains a controversial issue. Some authors have reported mildly elevated IOP in patients with NAION, 7' 8 while others con­tend that IOP is not significantly different in patients with NAION compared with controls. 9 There are reports of NAION after IOP spikes associated with intracapsular cata­ract surgery, 10 extracapsular cataract surgery, 11 acute angle closure glaucoma, 12 and herpes zoster ophthalmicus. 13 It is interesting to note that the loss of vision often occurs some days after the acute elevation in IOP. It is possible that FIGURE 2. One day after presentation, static threshold visual fields show dense superior and inferior nerve fiber bundle defects in the OD ( mean deviation = - 22.19 db) and no defects in the OS. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 265 JNeuro- Ophthalmol, Vol. 23, No. 4, 2003 Irak et al FIGURE 3. Six months after presentation, the right optic disc is pale and the left optic disc remains normal. this delay occurs because acute ischemia leads first to axo-plasmic flow stasis and optic nerve swelling. Swelling of the optic nerve head leads to further compromise of blood flow to the optic nerve, finally resulting in infarction. This mechanism has been proposed by Hayreh, 14 who observed NAION preceded by optic nerve edema in four patients with bilateral, sequential NAION. Because the choroidal contribution to the circulation of the optic nerve is most susceptible to elevated IOP, 15 Hayreh16 has also hy­pothesized that it is the balance between arterial pressure and IOP, both of which affect optic nerve perfusion, that is upset in cases of NAION. Although most cases of el­evated IOP, including acute angle closure glaucoma, do not result in optic disc edema and irreversible vision loss, • • f 30 FIGURE 4. Eleven months after presentation, the OD has a persistent, dense superior arcuate defect ( mean deviation - 15.92 db). The OS has a full field. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. 266 © 2003 Lippincott Williams & Wilkins Posner Schlossman and NAION JNeuro- Ophthalmol, Vol. 23, No. 4, 2003 variations in the vascular supply of the optic nerve head, 15 along with other ocular and systemic risk factors, may pre­dispose certain individuals to NAION during periods of el­evated IOP. Despite her young age, our patient had two risk fac­tors for NAION: systemic hypertension and a small cup- to-disc ratio, or a " disk at risk". 17 The temporal association between her acute rise in IOP and the precipitation of NAION leads us to conclude that elevated IOP in this case was likely the final insult that led to compromise of her optic nerve perfusion and subsequent NAION. Our case is similar to another recently reported case of NAION associ­ated with PSS. 18 In that case, a 71- year- old woman pre­sented with acute vision loss, corneal edema, elevated IOP, and optic nerve edema. She went on to develop optic nerve pallor and a stable visual field defect. Most ophthalmologists would agree that a patient with PSS and a large cup- to- disc ratio should be using ocu­lar antihypertensive medications prophylactically because of the risk of glaucomatous damage. We recommend that ophthalmologists treating patients with PSS and other risk factors for NAION, especially a very small cup- to- disc ratio ( absent physiologic cup, the " disk at risk") also consider treating these patients with ocular antihypertensive medica­tions because of the fear that an attack of PSS might pre­cipitate NAION. REFERENCES 1. Posner A, Schlossman A. Syndrome of unilateral recurrent attacks of glaucoma with cyclitic symptoms. Arch Ophthalmol. 1948; 39: 517- 535. 2. Shields MB. 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