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4 April 2011
Posner-Schlossman Syndrome
Glaucomatocyclitic Crisis
John R. Martinelli, OD, FAAO
St. Georgeʼs University School of Medicine
Abstract
Posner-Schlossman syndrome (PSS) or Glaucomatocyclitic Crisis is an
infrequent and typically unilateral, recurrent, inflammatory, open-angle ocular
hypertensive disorder. The condition normally affects individuals between 20 – 50
years of age and has no racial or sexual predilection. PSS is marked by an acute
marked elevation of intraocular pressure (IOP) associated with mild uveal
inflammation. The uveitis and symptoms at the initial presentation may be
negligible and care must be taken when considering differential diagnoses.
Medical treatment is indicated to control inflammation and lower intraocular
pressure thereby preventing secondary glaucomatous optic nerve damage and
vision loss. Associated systemic conditions may be absent; however, generalized
inflammatory and auto-immune etiologies should be investigated.
Keywords: Glaucoma, Glaucomatocyclitic Crisis, Inflammatory Glaucoma,
Inflammatory Disease, Posner-Schlossman Syndrome, PSS, Uveitis, Uveitic
Glaucoma

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Introduction
Posner-Schlossman syndrome (PSS), inflammatory glaucoma, uveitic glaucoma,
or glaucomatocyclitic crisis are terms used in the diagnosis of ocular
inflammatory disease contributing to increased intraocular pressure (IOP) with
the risk of associated glaucomatous optic nerve damage. Often, the IOP rise can
be quite dramatic ranging upwards from 40mm Hg to 60mm Hg.1
Of course, IOP
at this level can and will create a pressure related secondary optic neuropathy
and visual field defect if not treated promptly and appropriately.
First explained in 1813, Joseph Beer suspected an association between uveitis
and glaucoma and described it as an arthritic iritis followed by glaucoma and loss
of vision.2
In 1891, Priesley Smith proposed a modern classification of uveitic
glaucoma.2
Specific forms of uveitic glaucoma were described by Fuchs in 1906
including heterochromic uveitis.2
In 1948, Posner and Schlossman coined the
term glaucomatocyclitic crisis.2
Worldwide, PSS is considered rare and is primarily found to be a unilateral
disorder; however, several bilateral cases have been described in the
literature.4,7,8
Very often the etiology is unknown or idiopathic; however,
associations with systemic conditions such as the herpes virus, HLA-Bw54
defect, peptic ulcer, allergy, and others have been described.1,5
Historically, there
appears to be some discrepancy in the literature whether “true” PSS can be
associated with systemic disease or if it is indeed purely an idiopathic primary
ocular condition. The actual diagnosed glaucomatocyclitic event may be one of
many undetected exacerbations over time being that patient symptoms can be
mild and thus sub-clinical.1,3,5,9
Regardless of etiology, the ocular manifestations
must be treated aggressively to prevent the potential additive effects of this
condition on ocular tissue and the optic disc.
Additionally, various ocular differential diagnoses should be considered such as
inflammatory open-angle glaucoma, acute angle-closure glaucoma, mixed
mechanism glaucoma, neovascular glaucoma, pigmentary glaucoma, Fuchs
heterochromic iridocyclitis, and herpes simplex or zoster keratouveitis.
Case Report
A 66 year old Caucasian woman presented to our center for the first time on
January 15th
, 2007 with a chief complaint of recent blurred vision and mild
achiness of her right eye, of two days duration. She denied any previous similar
episodes and reported an otherwise unremarkable ocular history. Her systemic
history was significant for hypertension, osteoporosis, and osteoarthritis
controlled with Lotrel 5/20 (Amlodipine/Benazepril), Actonel (Risedronate), Evista

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(Raloxifene), and Piroxican. She denied any breathing difficulties. She reported
an unremarkable systemic and ocular family history.
Her uncorrected VA was 20/60 PH 20/50 OD, 20/40 PH 20/NI OS. Pupils were
unequal OS > OD with increased asymmetry in dim illumination. There was no
evidence of ptosis OU or facial anhydrosis. Versions were full and unrestricted
without subjective symptoms of diplopia. Confrontation visual field showed a
slight generalized 360 degree constriction OD but was full OS.
Biomicroscopic examination OD revealed slight episcleral injection with grade II
stromal corneal edema and associated descemet folds. There was early inferior
EBMD present. Trace fine keratic precipitates were evident on the corneal
endothelium. Grade I anterior chamber cells and flare were present. A partial
posterior synechia was evident in the inferior and nasal quadrant creating the
anisocoria. Early to mild nuclear sclerotic and cortical lens changes were
apparent. The angle remained open, grade III by Vonherrick technique, with
some slight nasal narrowing secondary to the synechia creating an anterior step
approach of the iris. Applanation intraocular pressure was 52mmHg OD and
10mmHg OS. Although difficult through a mildly edematous cornea, Goldmann 3-
mirror gonioscopy was performed showing grade I trabecular pigment without
evidence of exfoliative material or peripheral anterior synechia in any of the four
quadrants. All angle structures including schwalbeʼs line, trabecular meshwork,
schlemmʼs canal, and scleral spur were successfully observed with the ciliary
body being the deepest visible structure. The inferior and nasal quadrants did
appear slightly narrow due to the posterior synechia and slight iris step; however,
all angle structures remained in view without evidence of secondary pupillary
block angle closure. There was no evidence of iris transillumination or iris
neovascularization. Through the hazy cornea and lens changes, dilated 90 D
evaluation demonstrated a healthy appearing optic disc with healthy rim tissue
and a 0.3/0.2 C/D. Vitreous, peripheral retina, posterior pole, vessels, and
macula were unremarkable. There was no evidence of vitreous cells, vasculitis,
or posterior inflammatory disease such as peripheral pars planitis.
Biomicroscopic examination OS was completely unremarkable aside from the
early to mild nuclear and cortical lens changes. There was no evidence of
anterior inflammatory disease with an open and quiet anterior chamber. Dilated
90D evaluation revealed a healthy optic disc and rim tissue with a 0.2/0.2 C/D.
Vitreous, peripheral retina, posterior pole, vessels, and macula were
unremarkable. There was no evidence of vitreous cells or posterior inflammatory
disease.
The reduced uncorrected pinhole acuity was attributed to the presenting
condition as well as mild lens changes apparent in each eye. Considering the
obvious pathology present and patient discomfort, refraction and keratometry
was deferred until resolution or stability of the corneal edema and inflammation

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became clinically evident. For the same reason, color vision and stereopsis was
to be performed subsequently.
The delineating characteristics of inflammatory open angle glaucoma or uveitic
glaucoma and Posner-Schlossman syndrome as defined in the literature reveals
two separate entities. PSS is marked by significantly elevated IOP in the
presence of negligible to mild inflammation.1,3,5
Inflammatory glaucoma can
present with dramatic uveitic conditions including significant episcleral injection,
excessive cell and flare, moderate keratic precipitates, posterior and anterior
synechia, and pain.2
The evaluation, treatment, systemic considerations, and
follow-up care are similar although inflammatory glaucoma is more likely to be
found with systemic contributors.2
PSS is somewhat paradoxical being a typically
cyclic condition but more likely to be idiopathic without a known etiology.1,3,5,9
Therefore, a treatment plan of the following was prescribed with a scheduled 1
day follow-up:
Pred-Forteʼ (Prednisolone Acetate 1%): 1 gtt Q2H OD
Homatropine 5%: 1 gtt Q12H OD
CoSopt (Dorzolamide 2%/Timolol 0.5%): 1 gtt Q12H OD
Alphagan P (Brimonidine 0.15%): 1 gtt Q12H OD
The avoidance of a prostaglandin agonist in the treatment must be noted and is
of significance so to not enhance the inflammatory cascade.
Optic disc HRT, photos, and 30-2 threshold visual fields were scheduled.
Bloodwork including CBC with differential, SMA-20, ANA, ESR, RF, ACE, and
HLA-B27 was ordered. A chest XR was also requested.
Figure 1.1 Figure 1.2

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Figure 1.3
Follow-up #1
She returned the next day as scheduled for follow-up. Uncorrected VA was
unchanged at 20/60 PH 20/50 OD. OS was unremarkable. OD biomicroscopic
evaluation was relatively unchanged with persistent stromal corneal edema and
descemet folds, trace KPʼs, mild anterior chamber cells and flare, as well as the
partial nasal posterior synechia and slight anterior iris step. The synechia was
still apparent despite the initial homatropine treatment which had now created a
resultant mydriatic ovoid pupil. The angle remained open. Fortunately, however,
the intraocular pressure dropped dramatically to 8mmHg.
Considering the rapid reduction of IOP, she was instructed to continue the
medical treatment regiment as prescribed. It was stressed that complete
resolution of this episode may take from days to weeks and that she would
require a slow taper of the treatment. She was advised of the possible need for
oral steroid treatment if the topical regiment proved to be inadequate. She was
also explained of the possibility of an extended maintenance period on the topical
steroid in the case of prolonged inflammatory activity. Pending her lab results, an
appropriate referral would be made. She was asked to return for follow-up in 4
days.
Follow-up #2
She returned as scheduled for her 4 day follow-up visit showing a marked
improvement. The left eye continued to be unremarkable. The uncorrected VA
OD was stable 20/60 but now with improved 20/30 PH acuity. IOP remained
controlled at 8mm Hg. Significant resolution of the corneal edema and folds was
apparent as well as a reduction of the anterior chamber reaction. Trace - grade I
stromal corneal edema with trace descemet folds were viewed along with trace –
grade I cell and flare in the anterior chamber. Keratic precipitates were absent.
However, the nasal posterior synechia and associated iris step continued to
remain unchanged within an open anterior chamber. Hoping to also break the
synechia, the treatment was continued for an additional 5 days.

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Figure 2.1 Figure 2.2
Follow-up #3
She returned as scheduled for her 5 day follow-up visit with continued
improvement. Corrected VA with her current spectacle prescription was 20/30
OU without PH improvement. The left eye was again unremarkable. OD IOP rose
slightly to 15mm Hg. The previous corneal edema with descemet folds was totally
resolved. One cell was viewed in the open anterior chamber. There was now no
evidence of persistent posterior synechia or iris step. A mydriatic pupil was
apparent from the Homatropine therapy; however, an ovoid pupil remained.
Nasal anterior lens capsule pigment dusting was observed secondary to the
broken synechia. She was instructed to discontinue Homatropine and Alphagan,
taper the Pred-Forte to QID, and continue the Cosopt Q12H. A 1 week follow-up
was scheduled.
Figure 3.1 Figure 3.2
Follow-up #4
She returned as scheduled for her 1 week follow-up. Corrected VA was stable at
20/30 OU. The left eye was unremarkable. A slight ovoid pupil remained evident
OD without posterior synechia. IOP was 10mm Hg. There was no evidence of
continued ocular inflammatory disease. Trace pigment dusting of the nasal

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anterior lens capsule also remained. She was instructed to discontinue Cosopt
and slowly taper Pred-Forteʼ over a 30 day period. Also on this visit, 30-2 visual
fields were full OU. Optic nerve head HRT revealed no secondary glaucomatous
optic cupping or NFL defect OU. She was asked to return for a 1 month follow-up
visit with refraction, keratometry, color, and stereopsis testing scheduled.
Figure 4.1
Follow-up #5
She returned for her 1 month visit as scheduled. She had stopped all topical
medications. With a new refraction, best corrected VA was unchanged at 20/30
OU without PH improvement. Color plates and stereopsis was normal.
Autokeratometry readings did not reveal associated secondary atypical corneal
cylinder or distortion remaining in the right eye. Without evidence of other visually
significant ophthalmopathy, the slightly reduced VA was attributed to her mild
lens changes in each eye. The slight ovoid pupil and trace anterior lens capsule
pigmentary debris were the only indicators of previous activity and were not
considered visually significant. OD IOP was stable at 10mm Hg. OS remained
unremarkable. There was no evidence of recurrent or chronic inflammatory
disease in either eye. She was advised that should her vision decrease due to
progressive cataractous lens changes or if her current best corrected visual
acuity was not sufficient, she may consider cataract surgery provided there was
no evidence of active inflammatory ocular disease or systemic contraindications.
Laboratory
Subsequent laboratory results proved to be negative without suspicion of
contributing infectious or inflammatory systemic disease. Chest XR was also
negative without sign of prior or chronic pulmonary disease.
Discussion
As with this case, the etiology of Posner-Schlossman syndrome (PSS) remains
difficult to target and is rarely uncovered. Worldwide statistics reveal the rarity of

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the condition although there is a prevalence of 1.9 per 100,000 within the Finnish
population.1
The initial diagnosis normally falls between 20 – 50 years of age;
however, as exemplified by the preceding report, cases have been described
over age 60.6
A suspected case of PSS in a child has also been reported in the
literature.1
Unilateral involvement is found in the majority of cases although it is
possible to have alternating or simultaneous bilateral involvement.1,3,5,7,8,9
PSS
tends to be recurrent with or without mild episodic symptoms continuing for
hours, days, or weeks.1,3,5,9
Therefore, chronic subclinical disease may be
unbeknownst to the individual.
Characteristically there is an acute dramatic rise in intraocular pressure out of
proportion to the uveal inflammation.1,5,9
The IOP rise can be present several
days before discernable biomicroscopic evidence.1
It is not uncommon to find
peak IOP levels reaching 40mm Hg – 60mm Hg during the active phase.1
IOP
levels may also vary rapidly and widely between exacerbations.1
If the diagnosis
is not made and treatment is not instituted with careful monitoring, it is only
reasonable to conclude that individuals susceptible to recurrences are most likely
to develop permanent pressure related glaucomatous optic neuropathy and
visual field loss.
It is theorized that recurrent inflammatory pathophysiologic changes occur within
the trabecular meshwork leading to a corresponding decrease in aqueous
outflow.1,5
Of course, restricted outflow contributes to an increase in intraocular
pressure. Electroretinogram studies during IOP peaks have shown a diminished
S-cone b-wave.1
Studies have also confirmed associated transient retinal blood
flow alterations as well as transient optic nerve disruption within the acute phase
of PSS.1
Additionally, there have been isolated cases of secondary non-arteritic
anterior ischemic optic neuropathy (NAION) presented in the literature.7,8
Possible contributors to trabeculitis or trabecular inflammatory disease are
abnormal micro-vascular metabolism, autonomic nervous system deficits,
intraocular disease such as CMV or herpes simplex, and corneal disorders such
as herpetic keratitis.1,5
Systemic inflammatory or auto-immune disease may co-
exist with examples including but not limited to rheumatoid arthritis, ankylosing
spondylitis, sarcoidosis, lupus, gastro-intestinal disease, immunologic factors
including HLA-Bw54, and allergy.1,5
As described in the case, diagnostic
laboratory and radiologic studies are imperative. With evidence of systemic
disease, the formation of a long-term treatment plan can be established within
the appropriate specialties.
It is of interest to note that upon histological examination, atypical non-
granulomatous keratic precipitates are characteristic of PSS. Corneal endothelial
KPʼs tend to aggregate together as clusters with the formation of pseudopod

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extensions between them.1
This is in contrast to scattered or mutton-fat focal
KPʼs present in other uveitic disorders.2
With recurrent or longstanding uveitic disease, a secondary iris vasculopathy
consisting of tortuous vessels and leakage upon fluorescein angiography may
develop.1
Consequently, secondary iris ischemia and atrophy may be apparent in
longstanding cases.1,5
Ocular inflammatory disease, particularly uveitic disease, has been known to be
mediated by prostaglandin involvement.1,5
Specifically, prostaglandin E has been
found in elevated concentrations within the aqueous during the acute phase of
uveitic crises with increased IOP.1,5
Prostaglandin E levels have been shown to
return to normal levels between episodes of activity.1,5
Understanding the role of prostaglandin mediated uveitic glaucoma including
Posner-Schlossman syndrome, the therapeutic approach can be better
understood. The disease process must be strategically controlled by first
considering the reason for the ocular hypertensive event and providing proper
countering medical treatment. Therefore, as reported in the case study, steroidal
anti-inflammatory and ocular hypotensive agents are utilized in conjunction with
each other. Mydriatic agents are also utilized to mechanically stabilize the iris. In
the event of decreasing uveal inflammation but with persistent ocular
hypertension, the possibility of the patient being a “steroid responder” must be
considered. In this situation, topical NSAID treatment will become the superior
choice and can also be effective therapy without the potential for secondary
ocular hypertension. Considering that the mechanism of NSAID agents involve
the inhibition of prostaglandin synthesis, this is another powerful treatment
option.
Miotics such as Pilocarpine are contraindicated due to their constrictive effect on
the iris potentially exacerbating the uveitis. Ocular hypotensive prostaglandin
agonist therapy is also contraindicated due to the possible enhancement of the
inflammatory cascade. Rarely is surgical intervention indicated but can be utilized
in atypical unresponsive ocular hypertensive situations.1
Threshold visual field testing, optic disc photos, and optic disc analysis by HRT,
GDx, or OCT must also be part of the treatment plan. Depending on the absence
or presence of associated glaucomatous optic cupping, NFL defect, and/or visual
field defect, the aggressiveness of treatment and corresponding recall schedule
can be evaluated and instituted properly.
In the event of associated systemic disease, proper communications and
referrals to the appropriate specialties is of priority. Treatment of underlying

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causative systemic conditions can ultimately lead to better control of secondary
ocular inflammation and IOP.
Conclusion
Understanding and differentiating uveitic glaucoma, inflammatory glaucoma,
glaucomatocyclitic crisis, and Posner-Schlossman syndrome in the literature, we
are consequently provided with opportunity for thought provoking analyses of
these rare and potentially blinding conditions of unknown or conflicting etiologies.
This case calls upon our understanding at multiple levels. Knowledge of the
inflammatory process as it relates to uveitic disease, the pathophysiology of
ocular hypertension and its effect on the integrity of the optic nerve, and the
associated patterns of visual field loss. Knowledge of the pharmacodynamics of
medical treatment to reduce ocular inflammation and secondary ocular
hypertension. Finally, knowledge of possible associated systemic disease with
the proper recommendation and interpretation of laboratory and radiologic
testing.
Unlike the idiopathic PSS case described, the possible association with systemic
disease allows us as clinicians to be in a unique and exciting position. Many
ocular conditions put us on the front lines to probe and initially discover treatable
co-existing disorders. By understanding and utilizing laboratory and radiologic
testing, very often the initial systemic diagnoses can be first made in a primary or
medical eyecare setting. This allows for prompt, efficient, cost-effective, and
appropriate referrals. Not only are we in a position to save vision, we are
sometimes in a position to save a life. Contributing in this way to our patientsʼ
overall health care experience can be extremely rewarding and is part of our
responsibilities as primary and medical eyecare providers.
Bibliography
1. Oakman J. Posner-Schlossman syndrome, eMedicine. Retrieved March
31, 2007, from www.emedicine.com/oph/topic137.htm.
2. Herndon, Jr L. Uveitic Glaucoma, eMedicine. Retrieved March 31, 2007,
from www.emedicine.com/OPH/topic145.htm.
3. Scott KS, Wade NK. Posner-Schlossman syndrome, American Uveitis
Society. Retrieved March 31, 2007, from
www.uveitissociety.org/pages/diseases/pss.pdf.

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4. Teoh SB, Thean L, Koay E: Cytomegalovirus in aetiology of Posner-
Schlossman syndrome: evidence from quantitative polymerase chain
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5. Ralph J Green DipAppSc(Optom) (2007)
Posner-Schlossman syndrome (glaucomatocyclitic crisis)
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Complications of Neodymium: YAG laser goniopuncture after deep
sclerectomy
Acta Ophthalmologica Scandinavica 81 (6), 573–576.
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neuropathy associated with acute glaucoma secondary to Posner-
Schlossman syndrome. Arch Ophthalmol 2003 Jan; 121(1): 127-8.
8. Irak I, Katz BJ, Zabriskie NA, Zimmerman PL. Posner-Schlossman
syndrome and nonarteritic anterior ischemic optic neuropathy.
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