Glaucomatocyclitic Crisis (Posner Schlossman Syndrome) 

Glaucomatocyclitic Crisis (Posner Schlossman Syndrome)

 

Description

• Glaucomatocyclitic crisis (also known as Posner-Schlossman syndrome) is a rare ocular condition characterized by unilateral, recurrent episodes of elevated intraocular pressure and mild nongranulomatous anterior uveitis^(1,2)

Also Called

• Posner-Schlossman syndrome
• PSS

Definitions

• Glaucomatocyclitic crisis refers to the acute (not recurrent) episode⁽²⁾
• Iridocyclitis is inflammation of the anterior chamber, iris, ciliary body, and anterior vitreous

Epidemiology

Who Is Most Affected

• typically more common in adults aged 20-50 years, but mean age at diagnosis reported to range from 32 to 58 years^(1,2)
• higher male predominance reported⁽²⁾

Incidence/Prevalence

• Rare⁽¹⁾
• mean annual incidence and prevalence
  • mean incidence 3.9 per 100,000 annually and prevalence 39.5 per 100,000 in China
    • based on retrospective chart review of 576 inpatients and outpatients at eye clinic in Lucheng District of Wenzhou, China from 2005 to 2014
    • Reference – Br J Ophthalmol 2017 Dec;101(12):1638full-text
  • mean incidence 0.4 per 100,000 annually and prevalence 1.9 per 100,000 in Finland
    • based on retrospective chart review of 1,122 patients with endogenous uveitis treated at eye clinic in Turku, Finland from 1980 to 1982 and 1988
    • Reference – Acta Ophthalmol Scand 1997 Feb;75(1):76full-text
• glaucomatocyclitic crisis accounted for 1.7% – 4.3% cases of uveitis in Japan from 2007 to 2013
  • 1.7% of cases of uveitis in cohort of 304 cases of uveitis in Japan in 2010-2013 (Br J Ophthalmol 2014 Jul;98(7):932full-text)
  • 1.8% of cases of uveitis in cohort of 2,556 cases of uveitis in Japan in 2009-2010 (Jpn J Ophthalmol 2012 Sep;56(5):432)
  • 4.3% of cases of uveitis in cohort of 468 cases of uveitis in Tokyo, Japan in 2007-2009 (Ocul Immunol Inflamm 2015 Aug;23(4):291)

Risk Factors

• viral infection in anterior chamber due to cytomegalovirus associated with increased risk for glaucomatocyclitic crisis ^(1,2)

Associated Conditions

• potential associated conditions include
  •  nonarteritic anterior ischemic optic neuropathy (Arch Ophthalmol 2003 Jan;121(1):127, J Neuroophthalmol 2003 Dec;23(4):264)
  • primary open-angle glaucoma may be concomitant or a differential diagnosis⁽¹⁾

Etiology and Pathogenesis

Causes

• underlying cause of glaucomatocyclitic crisis is unclear; condition likely to share etiologies associated with other acute uveitic conditions^(1,2)
• proposed etiologies include
  • infection from
    • cytomegalovirus in anterior chamber (leading cause); location of initial infection unknown but may include iris, trabecular meshwork, or endothelial cells of cornea^(1,2)
    • herpes simplex virus and Helicobacter Pylori identified as possible causal factors in case reports, but evidence for causal link is lacking⁽²⁾
  • vascular endothelial cell dysfunction^(1,2)
  • immune-mediated inflammatory mechanisms, potentially with genetic component⁽²⁾

Pathophysiology

• pathophysiologic processes leading to glaucomatocyclitic crisis may involve^(1,2)
  • inflammation of trabecular meshwork, leading to rise in intraocular pressure, possibly due to
    • trabeculitis (development of thick, edematous trabecular band)
    • obstruction to trabecular outflow by chronic inflammatory cells and pigments
    • development of peripheral anterior synechiae leading to secondary angle-closure glaucoma
  • vascular endothelial cell dysfunction, resulting in disruption of retinal and optic nerve head autoregulation and subsequent fluctuations to oxygen supply
  • genetic variants of immune regulation (such as human leukocyte antigens [HLA] polymorphisms), leading to alterations in immune-mediated inflammatory responses and cytokine profile

History and Physical

History

Clinical Presentation

• glaucomatocyclitic crisis may be asymptomatic or relatively mild; elevated intraocular pressure may be present prior to the development of acute symptoms (⁽²⁾Ann Emerg Med.2006 Feb;47(2):167)
• clinical characteristics of glaucomatocyclitic crisis⁽²⁾
  • usually unilateral, but can be bilateral
  • recurrent episodes (individual episodes may last days to weeks)
  • mild anterior uveitis with ocular redness
  • elevation of intraocular pressure (> 40 mm Hg); out of proportion to inflammation
  • mild eye discomfort (rarely pain), blurred vision or halos (usually due to corneal edema)
  • open anterior chamber angles, with fine keratic precipitates on central and inferior cornea
  • visual fields and optic discs appear normal
  • in some cases
    • diffuse iris atrophy may be present
    • may have glaucomatous cupping or other optic nerve changes

History of Present Illness (HPI)

•  ask about duration, attacks generally self-limited⁽¹⁾
• ask about frequency, episodes may last days to weeks⁽¹⁾
• ask about vision in between attacks, affected eye usually appears normal between attacks⁽²⁾

Physical

Overview

• perform ophthalmic evaluation focusing on specific elements of comprehensive adult medical eye evaluation including
  • visual acuity measurement, pupil examination, and confrontation visual fields
  • slit-lamp biomicroscopy
  • intraocular pressure (IOP) measurement
  • gonioscopy
  • optic nerve head and retinal nerve fiber layer (RNFL) examination
  • fundus examination

Slit-lamp Biomicroscopy

• evaluate anterior segment examination with slit-lamp biomicroscopy; finding may include
  • diffuse iris atrophy⁽²⁾
  • iris heterochromia⁽¹⁾
  • fewer inflammatory cells in anterior chamber than expected
  •  occasional small nonpigmented keratic precipitates on corneal epithelium
  •  diffuse white keratic precipitates
  •  corneal haziness
  • Reference – Ann Emerg Med 2006 Feb;47(2):167

Intraocular Pressure (IOP) Measurement

• measure IOP in each eye; IOP is typically elevated out of proportion to inflammation in anterior chamber^(1,2)
  • IOP may reach > 40 mm Hg
  • IOP reported to range from mean 42.77 to 49.2 mm Hg at presentation

Gonioscopy

• evaluate anterior chamber angle using gonioscopy; findings may include^(1,2)
  • open angles (classic presentation); partially or completely closed angles suggest angle closure glaucoma
  • keratic precipitates on trabecular meshwork; findings characteristic of cytomegalovirus infection include coin-shaped lesions comprised of medium-sized, circumferential keratic precipitates and associated stromal edema

Optic Nerve Head and Retinal Nerve Fiber Layer (RNFL)

• examine optic nerve head and RNFL for structural signs of glaucomatous nerve damage and reduced retinal blood flow⁽¹⁾
• visible structural alterations of optic nerve head or RNFL and parapapillary choroidal atrophy may precede onset of visual field defects in early glaucoma (Ophthalmology 2021 Jan;128(1):P71, correction can be found in Ophthalmology 2021 May;128(5):P805)
• document optic nerve appearance and measure cup-to-disc (C/D) ratio
  •  optic discs usually appear normal between episodes of glaucomatocyclitic crisis (2)
  • optic nerve cupping has been reported in severe cases of glaucomatocyclitic crisis ⁽¹⁾
  • large C/D ratio suggests increased risk for glaucoma; nearly 3-fold increase in glaucoma progression reported in patients with glaucomatocyclitic crisis for ≥ 10 years⁽²⁾

Fundus Examination

• examine fundus through dilated pupil (when feasible), looking for other signs that may account for optic nerve changes and/or visual field defects such as
  • optic nerve pallor
  • disc drusen
  • optic nerve pits
  • disc edema
  • macular degeneration
  • retinovascular occlusion
  • other retinal disease
  • Reference – Ophthalmology 2021 Jan;128(1):P71, correction can be found in Ophthalmology 2021 May;128(5):P805

Diagnosis

Making the Diagnosis

• suspect glaucomatocyclitic crisis in patients with⁽²⁾
  • mild eye discomfort (rarely pain), blurred vision or halos (usually due to corneal edema)
  • mild anterior uveitis with ocular redness
  • elevation of intraocular pressure (> 40 mm Hg) out of proportion to inflammation
• diagnose glaucomatocyclitic crisis based on clinical findings, including⁽²⁾
  • usually unilateral, but can be bilateral
  • episodes recur, with duration of attacks lasting last days to weeks
  • anterior chamber angles are open, with fine keratic precipitates on central and inferior cornea
  • be aware that visual fields and optic discs may appear normal during acute episode, but chronic recurrences may result in glaucomatous cupping suggesting optic nerve damage
  • absence of other known explanations for ocular inflammation and intraocular pressure such as angle-closure glaucoma, primary open-angle glaucoma, Fuchs heterochromic uveitis, uveitic glaucoma, and other systemic conditions

Differential Diagnosis

• other causes of uveitis such as^(1,2)
  • Behcet syndrome
  • human leukocyte antigen (HLA) B27 uveitis
  • human T lymphotropic virus type 1
  • herpes simplex virus
  • sarcoidosis
  • spondyloarthropathies
  • Vogt-Koyanagi-Harada disease
• acute or chronic angle-closure glaucoma⁽¹⁾
• primary open-angle glaucoma and ocular hypertension due to other causes
•  Fuchs heterochromic iridocyclitis (Fuchs heterochromic uveitis)⁽¹⁾
• uveitic glaucoma⁽¹⁾
• anisocoria (asymmetric pupil size)⁽¹⁾
•  recent eye trauma (Ann Emerg Med 2006 Feb;47(2):167)

Testing Overview

• perform diagnostic testing including^(1,2)
  • imaging of optic nerve head and retinal nerve fiber layer with optical coherence tomography or confocal microscopy
  • visual field evaluation
  • serology or aqueous biopsy with polymerase chain reaction (PCR) if suspicion of viral etiology (primarily cytomegalovirus)

Imaging Studies

• perform imaging of optic nerve head and endothelial cell layer on anterior segment to assess for indications of cytomegalovirus endotheliitis; findings may include⁽²⁾
  • on optical coherence tomography
    • coin-shaped lesions of irregular thickness
    • highly reflective endothelial cell layer on anterior segment
    • immune ring formation, similar to herpes simplex keratitis
    • nodular endothelial lesion surrounded by translucent halo with or without associated brown pigment
  • on confocal microscopy of corneal endothelium, large cells containing nuclei with high reflection area but surrounded by halo of low reflection (resembling the eye of an owl)

Visual Field Evaluation

• use standard automated perimetry with white-on-white stimuli to evaluate visual fields (Ophthalmology 2021 Jan;128(1):P71, correction can be found in Ophthalmology 2021 May;128(5):P805)
•  visual fields usually appear normal between episodes of glaucomatocyclitic crisis⁽²⁾

Biopsy and Pathology

• if viral etiology suspected⁽²⁾
  • perform biopsy and evaluate with polymerase chain reaction (PCR)
  • strong PCR suggests cytomegalovirus

Management

Management Overview

• primary goal of treatment is to control intraocular inflammation and intraocular pressure (IOP) during acute episodes^(1,2)
• offer topical corticosteroid therapy to reduce intraocular inflammation; first-line options include
  •  topical corticosteroid such as prednisolone acetate 1% suspension in the eye 4 times daily, followed by rapid taper
  • if IOP elevation from topical corticosteroid, offer topical nonsteroidal anti-inflammatory drugs (NSAIDs) such as diclofenac sodium 0.1% 3-4 times daily or equivalent
• offer IOP-lowering medications to control IOP
  • consider antiglaucoma medications such as
    • topical or oral carbonic anhydrase inhibitors reported to be effective for managing IOP in patients with severe trabeculitis
    • beta-adrenergic antagonists (beta-blockers)
    • alpha-adrenergic agonists
  • in patients with cytomegalovirus infection-positive glaucomatocyclitic crisis, consider
    • topical 2% ganciclovir gel reported to lower recurrence rate, decrease loss of endothelial cells, and control intraocular pressure
    • oral valganciclovir reported to reduce inflammation and control intraocular pressure in short- and long-term
    • long-term maintenance therapy with topical valganciclovir may reduce relapse following adequate treatment response from systemic valganciclovir
  • other potential indications for ocular antihypertensive medications include
    •  large cup to disc ratio, to reduce risk of glaucomatous damage
    •  very small cup to disc ratio, to reduce risk of nonarteritic anterior ischemic optic neuropathy
    •  Reference – J Neuroophthalmol 2003 Dec;23(4):264
• benefit of surgery for management of glaucomatocyclitic crisis controversial
  • may be indicated in patients with
    • intraocular pressure uncontrolled by medical therapy
    • glaucomatous optic nerve damage
    • visual field loss
  • surgical options include
    • glaucoma filtration surgery
    • trabeculectomy augmented with mitomycin C
    • nonpenetrating trabecular meshwork-based techniques (such as trabectome surgery)
• offer appropriate follow-up
  • provide routine monitoring of optic disc appearance and visual fields in patients with glaucomatocyclitic crisis to assess for glaucoma progression
  • consider prophylactic medications to lower IOP in patients with glaucomatocyclitic crisis who are at increased risk for ischemic optic neuropathy (including very small cup to disk ratios)
  • inform patients about self-monitoring and self-care of ocular symptoms and IOP

Medications

Overview of Medical Therapies

• medical therapy to reduce intraocular inflammation typically includes⁽¹⁾
  •  topical corticosteroid such as prednisolone acetate 1% suspension in the eye 4 times daily, followed by rapid taper
  • if intraocular pressure (IOP) elevation from topical corticosteroid, offer topical nonsteroidal anti-inflammatory drugs (NSAIDs) such as diclofenac sodium 0.1% 3-4 times daily or equivalent
• antiglaucoma medications commonly used to lower IOP include^(1,2)
  • topical or oral carbonic anhydrase inhibitors reported to be effective for managing IOP in patients with severe trabeculitis
  • beta-adrenergic antagonists (beta-blockers)
  • alpha-adrenergic agonists
• in patients with cytomegalovirus (CMV) infection-positive glaucomatocyclitic crisis, medication options include
  • topical 2% ganciclovir gel
    • reported to lower recurrence rate, decrease loss of endothelial cells, and control intraocular pressure
    • recurrence reported in 37% of CMV-positive eyes with chronic inflammation in trabecular meshwork
    • long-term use reported to reduce flare-ups from 85% to 57% in CMV-positive eyes
  • valganciclovir
    • oral valganciclovir
      • reported to reduce aqueous CMV, decrease inflammation, and control intraocular pressure in short- and long-term
      • may increase risk of leukopenia
      • about 39% recurrence reported following treatment cessation
    • long-term maintenance therapy with topical valganciclovir may reduce relapse following adequate treatment response from systemic valganciclovir
    • intravitreal valganciclovir reported to reduce aqueous CMV, decrease inflammation, and control intraocular pressure; however, this formulation may have limited role in treatment of anterior chamber disease
    • 75% recurrence reported following treatment cessation of any valganciclovir formulation
• evidence for efficacy of prostaglandin analogs in glaucomatocyclitic crisis is lacking⁽¹⁾
• cholinergic miotics (pilocarpine) contraindicated due to potential to cause ciliary spasm, worsening inflammation, and synechiae; may consider weaker cycloplegics such as
  • scopolamine 0.25% twice daily
  • homatropine 5% twice daily
  • Reference – Ann Emerg Med 2006 Feb;47(2):167

Antiviral Medications in Cytomegalovirus-positive Patients

•  antiviral medications have been used to control ocular inflammation in patients with cytomegalovirus-positive glaucomatocyclitic crisis⁽²⁾
• topical 0.15% ganciclovir gel reported to reduce cytomegalovirus titers in aqueous humor of adults with cytomegalovirus anterior segment infection (level 3 [lacking direct] evidence)
  •  based on uncontrolled trial without clinical outcomes
  • 29 adults (mean age 60 years, 69% male) with CMV anterior segment infection affecting 29 eyes had 0.15% ganciclovir gel topically applied 1 mL 5 times daily for 6 weeks
    •  diagnosis based on clinical manifestations and positive real time polymerase chain reaction of aqueous humor
    •  23 eyes had CMV anterior uveitis, 6 eyes had CMV endotheliitis
  • after 6 weeks, 26 patients (89.7%) had undetectable CMV titers in aqueous humor and no anterior chamber activity
    •  2 patients (determined to be nonadherent with treatment protocol) had increased CMV titers in aqueous humor with raised intraocular pressure and increased anterior chamber activity
    •  1 patient had reduced CMV titer in aqueous humor with normal intraocular pressure and minimal anterior chamber inflammation
  •  no side effects or adverse events reported
  •  Reference – PLoS One 2018;13(1):e0191850full-text
• topical 0.15% ganciclovir gel reported to improve symptoms of corneal endotheliitis due to cytomegalovirus infection (level 3 [lacking direct] evidence)
  •  based on uncontrolled trial
  •  7 male adults (mean age 63 years) with immunocompetence and CMV corneal endotheliitis affecting 7 eyes had 0.15% ganciclovir gel topically applied 6 times daily in combination with 0.1% fluorometholone and antibiotic eye drops 4 times daily for 12 weeks
  •  clinical improvement in 6 eyes (85.7%), persistent corneal endothelial dysfunction due to irreversible damage in 1 eye
  •  significant decrease in CMV DNA copy numbers measured in aqueous humor of all eyes at 12 weeks (p < 0.0001)
  •  Reference – Br J Ophthalmol 2017 Feb;101(2):114
• topical 2% ganciclovir eyedrops may be associated with improved intraocular pressure and cup to disc ratio (CDR) in adults with glaucomatocyclitic crisis and reactivation of cytomegalovirus (level 2 [mid-level] evidence)
  •  based on prospective cohort study
  • 101 adults (mean age 38-41 years) with relapse of glaucomatocyclitic crisis (Posner-Schlossman syndrome) with or without CMV reactivation were evaluated
  • all patients received topical corticosteroids (primarily 1% prednisolone, 0.1% dexamethasone, or 0.1% fluorometholone) and medications to control intraocular pressure (primarily beta-blockers, alpha-2 adrenergic agonists, or carbonic anhydrase inhibitors), with tapering or termination of corticosteroids based on treatment response
  • paired aqueous humor and serum samples were assayed with enzyme-linked immunosorbent assay (ELISA) to measure CMV antibodies and calculate aqueous humor/serum correction ratio
    • mean aqueous humor/serum correction ratio 1.78 in 46 patients (CMV reactivation group)
    • mean aqueous humor/serum correction ratio 0.17 in 55 patients (CMV latency group)
  • patients with aqueous humor/serum correction ratio > 0 received 2% ganciclovir eyedrops 4 times daily for up to 8 weeks
  • comparing CMV reactivation group vs. CMV latency group at 6-week follow-up
    • IOP 19.83 vs. 16.17 (p < 0.05)
    • mean CDR 0.61 vs. 0.47 (p < 0.001)
  • cumulative maximum dosing of 2% ganciclovir drops for complete relapse control estimated to be 560 drops in patients with CMV reactivation and 448 drops in patients with CMV latency
  • Reference – Front Med (Lausanne) 2022;9:848820full-text
• topical 0.15% ganciclovir gel reported to reduce recurrence in patients with anterior uveitis due to cytomegalovirus infection (level 3 [lacking direct] evidence)
  •  based on case series
  • 31 adults (mean age 57 years, 74% male) with anterior uveitis due to cytomegalovirus infection affecting 33 eyes had 0.15% ganciclovir gel topically applied 6 times daily (tapered based on severity of inflammation in anterior chamber) in combination with topical corticosteroids and antiglaucoma medications (at discretion of clinician) 4 times daily for 12 weeks
  •  decreased recurrence of uveitis recurrence (total number of episodes)
  •  Reference – J Ophthalmic Inflamm Infect 2016 Dec;6(1):10full-text
• intravitreal ganciclovir reported to resolve symptoms of ocular cytomegalovirus infection in case reports
  •  improved visual acuity and reduced CMV viral load in aqueous humor after 2 courses of intravitreal ganciclovir 0.4 mg in 0.05 mL once weekly for 8 weeks followed by once monthly injections for 4 months reported in 46-year-old man with 7-year history of recurrent anterior chamber inflammation and elevated intraocular pressure
  •  improved vascular leakage (by fluorescein angiography) after 1 dose intravitreal ganciclovir 1 mg in 0.1 mL followed by ganciclovir 5 mg/kg IV twice daily for 2 weeks then valganciclovir 900 mg orally once daily reported in 63-year-old woman with 18-year history of recurrent anterior uveitis and elevated intraocular pressure
  • Reference – Am J Ophthalmol Case Rep 2018 Jun;10:189full-text

Surgery and Procedures

Overview

• benefit of surgery for management of glaucomatocyclitic crisis controversial⁽²⁾
• may consider surgery in patients with^(1,2)
  • intraocular pressure (IOP) uncontrolled by medical therapy
  • glaucomatous optic nerve damage
  • visual field loss
• surgical options include
  • filtration surgery^(1,2)
    • procedure involves draining of inflammatory cells from filtering bleb in anterior chamber
    • reported to decrease trabeculitis and elevations in intraocular pressure during recurrent episodes
    • disadvantages include increased risk of surgical failure due to scarring from uveitic conjunctiva
  • trabeculectomy (another type of filtration surgery) that may be augmented with mitomycin C
    • procedure involves provision of alternative path for escape of aqueous humor into subconjunctival space
    • reported to reduce intraocular pressure and need for medical therapy
    • Reference – J Glaucoma 2014 Feb;23(2):88
  • nonpenetrating trabecular meshwork-based techniques (such as trabectome surgery)
    • procedure involves targeted ablation to remove selected arc of trabecular meshwork and inner wall of Schlemm canal in combination with oral valganciclovir for 3 months and antiglaucoma medication as needed
    • reported to decrease trabeculitis and elevations in intraocular pressure during recurrent episodes
    • Reference – Eye (Lond) 2015 Oct;29(10):1335full-text

Efficacy of Filtration Surgery

• filtration surgery (including trabectome, trabeculectomy, and drainage devices) have been used to treat intraocular pressure refractory to medication management^(1,2)
• trabectome surgery reported to improve intraocular pressure in patients with glaucomatocyclitic crisis due to cytomegalovirus infection (level 3 [lacking direct] evidence)
  •  based on a nonclinical outcome in uncontrolled trial
  •  7 adults (mean age 44 years) with glaucomatocyclitic crisis (Posner-Schlossman syndrome) due to cytomegalovirus infection and uncontrolled IOP had trabectome surgery in combination with oral valganciclovir twice daily, topical corticosteroids and local antiglaucoma medication
  • all patients had phakic lens
  • reduction in mean IOP from 40 mm Hg to 13 mm Hg at 12-month follow-up (no p value reported)
  • blood reflux reported in all patients
  • Reference – Eye (Lond) 2015 Oct;29(10):1335full-text
• trabeculectomy reported to control IOP and prevent further attacks in case report (Ophthalmic Surg Lasers 2002 Jul;33(4):321)
• trabeculectomy with peripheral iridectomy reported to control IOP and prevent further visual loss and changes to optic disc in patient with glaucomatocyclitic crisis (Posner-Schlossman syndrome) and primary open-angle glaucoma in case report (Am J Ophthalmol 1988 Jan 15;105(1):99)

Follow-up

• provide routine monitoring of optic disc appearance and visual fields in patients with glaucomatocyclitic crisis to assess for glaucoma progression⁽²⁾
• in patients with glaucomatocyclitic crisis who are at increased risk for ischemic optic neuropathy (including very small cup to disk ratios), consider prophylactic medications to lower intraocular pressure (IOP)⁽²⁾
• inform patients about self-monitoring and self-care of ocular symptoms and IOP
  • pressure phosphene tonometry device to self-monitor IOP at home described in case report (Clin Experiment Ophthalmol 2006 Aug;34(6):513)
  • self-regulated therapy with aqueous suppressant and topical nonsteroidal anti-inflammatory drugs (NSAIDs) may help patients to suppress IOP elevations associated with treatment delays ⁽¹⁾

Complications

• nearly 3-fold increase in glaucoma progression reported in patients with glaucomatocyclitic crisis for ≥ 10 years⁽²⁾
• glaucoma reported in up to 45% of cases⁽²⁾
• older age and longer disease duration may be associated with increased risk of glaucomatous optic nerve damage among patients with glaucomatocyclitic crisis
  •  based on systematic review limited by significant heterogeneity
  • systematic review of 13 studies evaluating association between glaucomatocyclitic crisis (Posner-Schlossman syndrome [PSS]) and glaucomatous optic nerve damage in 811 eyes of patients with PSS
  • overall meta-analysis had incidence ratio 0.251 (95% CI 0.175-0.327) for optic nerve damage, results limited by significant heterogeneity
  • in meta-analysis of 3 trials with 358 eyes
    • factors associated with increased risk of optic nerve damage (p = 0 for each), results limited by significant heterogeneity
      • older age
      • longer disease duration
    • no significant differences in risk of optic nerve damage associated with gender, intraocular pressure at onset, or ocularity (monocular/binocular vision)
  • Reference – Int Ophthalmol 2020 Nov;40(11):3145, correction can be found in Int Ophthalmol 2022 Jul;42(7):2311

Prognosis

•  generally considered self-limiting, with recurring flare-ups followed by recovery^(1,2)
• permanent damage may develop, including ⁽¹⁾
  •  optic nerve head damage
  •  visual field defects
  •  vision loss
• factors associated with worse prognosis include
  • longer duration of glaucomatocyclitic crisis⁽²⁾
  • optic neuropathy⁽²⁾
  • glaucoma⁽²⁾
  • iris involvement (Graefes Arch Clin Exp Ophthalmol 2021 Dec;259(12):3757)
• incidence of glaucoma about 67% over 5 years and iris involvement associated with increased need for glaucoma surgery in patients with glaucomatocyclitic crisis
  •  based on retrospective cohort study
  • 98 patients/eyes (mean age 53 years, 65% male) diagnosed with glaucomatocyclitic crisis (Posner-Schlossman syndrome) at 1 hospital in Korea were evaluated
  • 47% had iris involvement (sectoral iris changes in 26 eyes and diffuse iris changes in 20 eyes)
  • 67% developed glaucoma and 21% had glaucoma surgery during mean follow-up 50.1 months
  • iris involvement associated with increased need for glaucoma surgery compared to no iris involvement (adjusted hazard ratio 5.22, 95% CI 1.39-19.61)
  • complete surgical success (defined as intraocular pressure > 5 mm Hg and ≤ 21 mm Hg at 2 consecutive visits without antiglaucoma medication) at 2 years in 35% (95% CI 18.5%-67%)
  • Reference – Graefes Arch Clin Exp Ophthalmol 2021 Dec;259(12):3757

Prevention and Screening

•  not applicable

Guidelines and Resources

Guidelines

•  no relevant guideline for “glaucomatocyclitic crisis” or “Posner-Schlossman syndrome” found 2023 Feb 20 on MEDLINE using guidelines limiter

Review Articles

• review can be found in Surv Ophthalmol 2017 May-Jun;62(3):277
• review can be found in Semin Ophthalmol 2011 Jul;26(4-5):282
• review of viral etiologies of Posner-Schlossman Syndrome and anterior uveitis can be found in Indian J Ophthalmol 2020 Sep;68(9):1764full-text
• case report of retinal nerve fiber layer loss detected by optical coherence tomography can be found in Taiwan J Ophthalmol 2015 Apr;5(2):90full-text
•  case report can be found in Clin Exp Optom 2007 Jan;90(1):53PDF
•  case report of nonarteritic anterior ischemic optic neuropathy associated with acute glaucoma secondary to glaucomatocyclitic crisis can be found in Arch Ophthalmol 2003 Jan;121(1):127
•  case report of trabeculectomy in management of glaucomatocyclitic crisis can be found in Ophthalmic Surg Lasers 2002 Jul-Aug;33(4):321
• case reports of glaucoma progression can be found in Ophthalmology 2001 May;108(5):913, commentary can be found in Ophthalmology 2002 Mar;109(3):409

MEDLINE Search

•  to search MEDLINE for (Glaucomatocyclitic Crisis [Posner-Schlossman syndrome]) with targeted search (Clinical Queries), click therapy, diagnosis, or prognosis

Patient Information

• handout on uveitis from Patient UK PDF
• information on uveitis from American Optometric Association

References

General References Used

The references listed below are used in this DynaMed topic primarily to support background information and for guidance where evidence summaries are not felt to be necessary. Most references are incorporated within the text along with the evidence summaries.

1. Shazly TA, Aljajeh M, Latina MA. Posner-Schlossman glaucomatocyclitic crisis. Semin Ophthalmol. 2011 Jul;26(4-5):282-4.
2. Megaw R, Agarwal PK. Posner-Schlossman syndrome. Surv Ophthalmol. 2017 May;62(3):277-285.