Bilateral Contemporaneous Posner-Schlossman Syndrome With Advanced Chronic Open-Angle Glaucoma

The diagnosis of PSS can be difficult to make because the disease shares similar characteristics observed in other forms of glaucoma.

By Austin R. Lifferth, OD

Posner-Schlossman syndrome (PSS) or glaucomatocyclitic crisis is a secondary, open-angle glaucoma with an associated mild and disproportionate inflammatory component. PSS is usually unilateral, has relatively unique and characteristic glaucoma-like features, and presents infrequently.1,2 This case report describes a patient with an even more unusual presentation of this relatively rare form of glaucoma.

CASE PRESENTATION

Recently, I received a phone call from another doctor regarding a 48-year-old white man with significantly elevated intraocular pressures (IOPs) of more than 60 mm Hg in both eyes. The doctor reported that the patient was asymptomatic for pain, was complaining of only moderately blurred vision for the past 3 weeks, and clinically was found to have open angles by Van Herick estimation.

I saw the patient about an hour later and found his UCVA to be 20/60 OD and 20/40 OS with minimal improvement upon pinhole testing. His confrontation visual field testing showed dense constriction, and although his pupils were round and measured approximately 6.0 mm in photopic illumination with equal color and reactivity, there was a trace relative afferent pupillary defect present in his right eye.

The patient’s IOPs were 78 mm Hg+ OD and 74 mm Hg OS with Goldmann applanation tonometry. The adnexa was clear with only minimal bilateral conjunctival injection. The cornea showed 1+ edema with associated overlying 1 to 2+ inferior microcystic edema, which was greater OD than OS (Figure 1) and correlating mild decreased clarity and loss of iris detail. Of importance, there was no appreciable iris heterochromia and no iris transillumination defects. Upon close inspection (Figure 2), there were a few small white stellate keratin precipitates on the inferior cornea in the right eye and only trace cell visible in the anterior chamber OU with moderate movement OU. There was no evidence of posterior synechiae OU, and Von Herrick angle estimation confirmed deep angles OU. Gonioscopy was deferred due to the edematous cornea and likely diminished clarity for the fine-angle structures. The dilated examination showed that the lens was clear, the vitreous was clear and void of cells, and the macula was flat with attached periphery OU. More concerning was that the patient’s optic nerve had diffuse thinning with an estimated cup-to-disc ratio of 0.95 OD and 0.85 OS for an average-to-large nerve size.

The patient’s personal and family ocular history was unremarkable, and this was his first clinical eye examination. He denied any other memorable episodes of similar symptoms of decreased vision. His personal medical history was unremarkable, and the only medication he was taking was an antibiotic for a his resolving respiratory infection. He was a former smoker and, prior to quitting about 18 months from our initial visit, he smoked “a few” cigars per day for the last 20 years.

Based on the patient’s case presentation, symptoms, and clinical findings, I gave him a provisional diagnosis of advanced secondary open-angle uveitic glaucoma with possible bilateral PSS. In the office, I administered one drop of brinzolamide 1% (Azopt, Alcon Laboratories, Inc.), brimonidine 0.2% plus timolol 0.5% (Combigan, Allergan, Inc.), and bimatoprost 0.01% (Lumigan, Allergan, Inc.) as well as a double dose of difluprednate 0.05% (Durezol, Alcon Laboratories, Inc.) topically and two 250-mg acetazolamide tablets orally. After 35 minutes, his IOPs began to decline, and he left the office with IOPs of 71 mm Hg OD and 64 mm Hg OS. I directed him to use one more drop of brinzolamide and brimonidine plus timolol in both eyes later that evening, as well as difluprednate q.i.d. OU every day and bimatoprost q.h.s. starting the following day. He was to see me in 2 days for re-evaluation. When the patient returned, he reported slightly improved vision, and he was still asymptomatic for ocular pain, significant redness, or photophobia. His vision had improved to 20/40 in both eyes without correction, and the IOPs were significantly and remarkably lower at 19 mm Hg OD and 17 mm Hg OS with Goldmann applanation tonometry. Gonioscopy was performed and showed that the ciliary body was visible in all four quadrants with normal iris approach and characterized by few iris processes OU but no peripheral anterior synechiae. The corneal edema was nearly resolved, and the few inferior keratic precipitates were more distinct in appearance but still remaining. There was no cell or flare in the anterior chambers.

Because the patient’s IOPs were more stable for his advanced glaucomatous stage, I instructed him to discontinue bimatoprost and difluprednate in 2 days to minimize the possible exacerbation of an underlying inflammatory condition and prevent an unwanted potential ocular hypertensive steroid response. He continued brinzolamide and brimonidine plus timolol OU b.i.d. and started nepafenac 0.3% (Nevanac, Alcon Laboratories, Inc.) OU b.i.d. for continued anti-inflammatory control.

One week later, the patient’s UCVA improved to 20/30 in both eyes with BCVA 20/25. The IOPs were further reduced to 13 mm Hg in both eyes as revealed with Goldman applanation tonometry. The patient underwent baseline Humphrey visual field (HVF; Carl Zeiss Meditec, Inc.) threshold testing, which showed severe constriction OD greater than OS (Figure 3) with moderate reliability OD greater than OS. This finding correlated with the dilated stereoscopic disc photographs (Figure 4) which were obtained that day. His pachymetry measurements were 577 μm OD and 578 μm OS. I explained these findings to this patient in detail, and we discussed his prognosis, visual disability, and the impact his condition will continue to have on his personal life and his profession.

To better understand the patient’s central vision, functional peripheral vision, and to prepare for probable employment disability, I asked him to return in1 month for HVF 10-2 and Goldmann visual field testing with the HVF 10-2 test results shown in Figure 5. Since these initial visits, the patient had another recurrence with associated elevated IOP despite brimonidine plus timolol, brinzolamide, and nepafenac b.i.d. OU. I retreated aggressively using topical steroids in place of nepafenac followed by a reasonable taper schedule. Currently, he has stable IOPs in the midteens with a maintenance regimen of brimonidine plus timolol and brinzolamide b.i.d. OU and prednisolone acetate 1% OU every morning. Despite his advanced visual field presentation, maximum appropriate medication therapy, and his relatively younger age, the patient has declined any recommended surgical treatment at this time. Therefore, I see him every 3 months for close monitoring and treatment as indicated for his advanced and fragile glaucoma.

“PSS is ‘. . . an odd entity, which has the nature of both primary and secondary glaucoma. In appearance, it may resemble primary glaucoma, but its treatment is that of mild secondary glaucoma.’”

PHYSICAL PRESENTATION

PSS was first presented as a unique syndrome by Adolph Posner and Abraham Schlossman in 1948.3 Posner and Schlossman reviewed the few previous reported cases, made correlations with at least nine new specific cases that they had also identified, and framed the syndrome as a distinct entity that had the following uniform specific characteristics:

  • The disease is unilateral.
  • If symptoms are present, they may include slight [ocular] discomfort, colored halos, or blurring of vision.
  • The eye is white or a few dilated conjunctival vessels may be visible. Ciliary injection is never present. If tension is very high, mild congestion of the sclera or edema of the corneal epithelium may be noted.
  • Ocular hypertension may appear a day or so before, or simultaneously with, cells in the aqueous.
  • The severity of the glaucomatous attacks [are] out of proportion to the cyclitic manifestations.
  • The individual attacks of ocular hypertension [last] a few hours to 1 month but rarely more than 2 weeks. Attacks that have been observed without treatment have cleared within 2 weeks.
  • Episodes may occur with varying frequency and without any apparent cause.

Soon after Posner and Schlossman’s summary was published, another contemporary commented that the condition was “. . . an odd entity, which has the nature of both primary and secondary glaucoma. In appearance, it may resemble primary glaucoma, but its treatment is that of mild secondary glaucoma.”4

Since that time, these characteristics have remained relatively constant with the exception of the following modifications and additions. The following information is an update of the literature regarding these refinements as well as a brief supportive commentary.

  • Although the disease is unilateral in 92% of the cases,5 it may manifest in both eyes at separate distinct times and can, although rare, occur contemporaneously.4,6
  • The disease may have an underlying primary, open-angle glaucoma component that can lead to glaucomatous optic nerve damage with associated characteristic glaucomatous visual field loss.7 Studies have also shown that there is an association between primary open-angle glaucoma and PSS with as many as 45% of PSS patients having concomitant, permanent, and even progressive glaucomatous optic nerve cupping.8 From this report and others, it is clear that there is a risk of subsequent glaucomatous development with repeated glaucomatocyclitic episodes.9
  • The disease course and severity can be shortened and controlled with the use of topical anti- inflammatory and/or ocular hypotensive medications and may require continued ocular hypotensive medical or surgical therapy to effectively manage any resulting associated glaucoma.
  • The IOPs are usually 40 to 60 mm Hg, and this is related more to the duration of the uveitis rather than its degree.1
  • The attack is usually followed within the next 24 hours by one to 20 small, well-defined nonpigmented precipitates on the posterior surface of the cornea (usually centrally or inferiorly),8 and the precipitates fade away within a few days to a month. Although precipitates are present as a rule, they may be absent during some of the attacks. At no time is there more than a trace of aqueous flare. Posterior synechiae are never formed.10
  • The affected eye may show mild heterochromia and mydriatic anisocoria. Heterochromia may present in one-third of the cases and anisocoria in one-third of the cases.10
  • The disease almost exclusively occurs in patients 20 to 50 years old, with very few episodes reported outside of this range, and it usually decreases in frequency with advancing age.1,2
  • There is never any vitreous involvement and no cystoid macular edema.8
  • The attacks usually last 2 to 3 weeks and can both resolve spontaneously and recur randomly. The eye is normal between attacks.8

DIFFERENTIAL DIAGNOSIS

The diagnosis of PSS is sometimes difficult to make because the disease shares some similar characteristics with other forms of glaucoma. Specifically, this condition should be differentiated from the following and somewhat similar conditions.

Fuch heterochromic iridocyclitis (FHI). This condition is similar to PSS in that both secondary open-angle inflammatory glaucomas have a mild non-granulomatous anterior chamber reaction with no posterior synechiae formation. PSS is different from FHI in a few distinct ways. First, PSS is acute and, although potentially recurrent, it is responsive to steroid treatment, whereas FHI is more chronic, responds poorly to steroid treatment resulting in persistent cells, and is therefore usually refractory to medical therapy. Second, the keratic precipitates in PSS are usually small, relatively few in number, and are located in the inferior/central cornea, whereas the keratic precipitates in FHI are more numerous and usually cover the entire corneal surface. Furthermore, and unlike PSS, patients with FHI are more symptomatic due to an increased likelihood of cataract formation, vitreous floaters, increased risk of cystoid macular edema, and actual glaucoma damage.2,5

Uveitic glaucoma. This condition is similar to PSS in that both conditions have an underlying inflammatory component. Many times, however, patients with primary uveitis will not only be more symptomatic with ocular redness, pain, and photophobia, but initially their IOPs are low due to depressed aqueous production. If treatment is delayed or insufficient, however, the aqueous production levels regulate and resume but also with gradual increased outflow obstruction with resultant increased IOPs. During this sustained inflammatory process, corneal keratic precipitates may develop, and both posterior and peripheral anterior synechiae formation occurs, further complicating the normal aqueous flow and drainage, respectively. Although most cases may be idiopathic, an important percentage may also be due to other systemic causes, infectious agents, or intraocular tumors and may require additional testing and treatment beyond the scope of this review.2,8,9

Primary angle-closure glaucoma. This condition is very similar to the secondary inflammatory conditions described previously in that this too can also cause markedly increased IOP. In patients with acute primary angle-closure glaucoma, however, the symptoms appear more rapidly, and they are more extreme in terms of ocular redness, intense boring ocular pain, nausea/vomiting, foreign body sensation, lacrimation, and very blurry vision due to diffuse corneal edema. In patients with chronic primary angle-closure glaucoma, the presentation is elevated IOPs and a dull ache over the affected eye. Because these characteristic symptoms may be variable depending on the chronicity of the condition, primary angle-closure glaucoma is further differentiated more definitively from PSS by gonioscopy, which will readily show anatomically closed angles. In other words, the important diagnostic difference between PSS and acute angle-closure or chronic angle-closure glaucoma is that the angle is completely open and unobstructed in patients with PSS. As an important reminder, patients with PSS are usually relatively asymptomatic and are treated with anti-inflammatory medications and/or ocular hypotensive medications as discussed, rather than laser peripheral iridotomy.2,9

TREATMENT

The preferred treatment for patients with PSS is a logical combination of topical anti-inflammatory and topical ocular hypertensive medications. Specifically, a steroid is very effective for rapid inflammatory control followed by a reasonably rapid taper of the steroid to minimize steroid response and may even be replaced by a nonsteroidal anti-inflammatory agent in certain settings. The steroid alone can dramatically reduce the elevated IOPs, and additional topical ocular hypo-tensive treatment may commonly be indicated as adjunctive therapy. The best choices are topical beta-blockers, alpha-agonists, and/or carbonic anhydride inhibitors. Prostaglandin analogs should be used with caution, as their efficacy and safety in PSS is not well established.2,6 In most cases, surgery is not necessary, and in fact, treatment with the drops is usually not necessary during remission and does not influence the frequency of attacks.9 Well-informed patients can sense an impending attack and may be able to self-treat with a nonsteroidal anti-inflammatory drug and an aqueous suppressant.2

If patients fail to achieve adequate IOP reduction, the next step is surgical treatment with a trabeculectomy; trabeculoplasty is contraindicated.2

CONCLUSION

Because PSS is such an elusive disease, the literature advises that care should be taken in making the diagnosis, and patients should be closely followed up, as the course of the disease is not well understood2 and because of its episodic and recurrent nature and the risk of progression to primary open-angle glaucoma.9 PSS should be considered in any patient with increased IOP because the inflammation may follow the acute rise in IOP by some days or be so mild as to be overlooked. The etiology of the IOP rise may not be clear at first presentation.9

Posner and Schlossman were ahead of their time when they said, “In order to form a complete picture of a case of glaucoma, it is essential to observe the patient at frequent intervals and over a relatively long period.3” This proverb-worthy motto not only applies to glaucomatocyclitic crisis but should also be our personal approach for all patients with all forms of glaucoma.

Austin R. Lifferth, OD, is a consultative optometrist at Bennett & Bloom Eye Centers in Louisville, Kentucky. He acknowledged no financial interest in the products or companies mentioned herein. Dr. Lifferth may be reached at (502) 403-6422; austinlifferth@yahoo.com.

  1. Oakman JH, Hampton R. Posner-Schlossman Syndrome. http://emedicine.medscape.com/article/1205949-overview. Accessed January 2, 2013.
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  9. Posner A, Schlossman A. Syndrome of glaucomatocyclitic crisis. Society Proceedings. Am J Ophthalmol. 1948;31(6):735.