AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Glaucomatocyclitic crisis is a condition with self-limited recurrent episodes of markedly elevated intraocular pressure (IOP) with mild idiopathic anterior chamber inflammation. It is most often classified as secondary inflammatory glaucoma. In 1948, Posner and Schlossman first recognized glaucomatocyclitic crisis and described the features of this syndrome. For this reason, the entity is often termed Posner-Schlossman syndrome (PSS). They identified the following features:

• Recurrent episodes of mild cyclitis
• Uniocular involvement
• Duration of attack varying from a few hours to several weeks
• Signs of a slight decrease in vision, elevated IOP with open angles, corneal edema with a few keratic precipitates, heterochromia with anisocoria, and a large pupil in the affected eye
• Normal visual fields
• Normal optic disc
• Normal IOP, outflow facility, and all provocative tests between episodes

Since this original description, other cases attributed to glaucomatocyclitic crisis have been found to deviate from these criteria. For instance, some patients with glaucomatocyclitic crisis have abnormal aqueous humor dynamics and may have an underlying primary open-angle glaucoma (POAG). Additional features that are now recognized are as follows:

• Almost exclusively, this condition affects individuals aged 20-50 years.
• Both eyes may be involved at different times but very rarely contemporaneously.
• The rise of IOP is out of proportion to the severity of the uveitis, and this rise in IOP precedes the identifiable inflammatory reaction, often by several days.

Pathophysiology

Episodic changes in the trabecular meshwork lead to impairment of outflow facility and result in an elevation of IOP. These changes are accompanied by mild intraocular inflammation. In the acute phase of PSS, optic nerve head parameters and retinal flow rates were altered; however, all returned to normal without any permanent damage after resolution of the elevated IOP. Electroretinogram studies in the acute phase demonstrate a selective reduction in the S-cone b-wave.

Frequency

United States

Glaucomatocyclitic crisis is a rare condition.

International

In Finland, the incidence is 0.4 and the prevalence is 1.9 per 100,000 population.

Mortality/Morbidity

Prolonged IOP elevation results in damage to the optic nerve head and visual field defects.

Age

This condition almost exclusively affects individuals aged 20-50 years. Rarely, episodes occur in individuals older than 60 years. Also, rarely, episodes have been reported in adolescence.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

• In general, the symptomatology is vague.
• Most commonly, a crisis presents with slight discomfort. The patient may be pain-free even though the IOP is quite elevated.
• The patient may report blurred vision or halo vision if the IOP is high and induces corneal edema.
• A history of past attacks of blurred vision lasting several days, which recurs monthly or yearly, is usual.
• Each crisis may last several hours to a few weeks.
• Patients follow a variable clinical course; some experience 1 or 2 episodes in their lives, while others have recurrences over many years.
• Typically, these episodes decrease in frequency with advancing age.

Physical

• On examination, the eye is quiet with either no injection or a mild ciliary flush.
• The pupil often is dilated slightly or sluggishly reactive; the anterior chamber is deep and has an open angle.
• This condition should be differentiated from closed-angle glaucoma with the help of gonioscopy.
• IOP usually is elevated in the range of 40-60 mm Hg.
• IOP is related to the duration of uveitis but not to the degree of uveitis.
• Eyes with active inflammatory disease often have wide swings in IOP, leading to glaucomatous damage.
• The elevated IOP can last for several hours to a few weeks; therefore, it can be missed on initial examination.
• If the elevated IOP is of significant duration and elevation, corneal epithelial edema develops.
• Signs of anterior inflammation are characteristically minimal with faint flare, rare cells, and only a few keratic precipitates that are stellate, flat, nonpigmented, and concentrated over the inferior half of the endothelium.
• Fine keratic precipitates appear after 2-3 days of elevated IOP and resolve rapidly.
• The inflammation never leads to the development of posterior synechiae or peripheral anterior synechiae.
• Fresh precipitates may appear with each episode of increased IOP.
• Heterochromia, described originally, is no longer considered a characteristic of this syndrome.
• Typical of inflammatory conditions, early segmental iris ischemia and associated late iris-vessel congestion have been observed. These vessels leak on iris fluorescein angiography.

Causes

• The etiology of glaucomatocyclitic crisis has remained elusive. Several factors have been postulated as contributors to the development of glaucomatocyclitic crisis, to include the following:
• • Abnormal vascular process
  • Autonomic defect
  • Allergic condition
  • Variation of developmental glaucoma
  • Cytomegalovirus (CMV)
  • Herpes simplex virus
• Description of a final common pathway usually includes a reference to changes in the trabecular meshwork leading to a reduction of outflow facility. However, some authors describe an increase in aqueous production.
• Transfer coefficients of fluorescein in aqueous in the anterior chamber, by flow and by diffusion, are elevated during attacks of glaucomatocyclitic crisis. Between attacks, both coefficients return to normal.
• Elevations in IOP are postulated to be secondary to inflammation of the trabecular meshwork, which may be mediated by prostaglandins.
• Prostaglandins, especially prostaglandin E, have been found in higher concentration in the aqueous humor of patients during acute attacks. These levels return to normal between episodes.
• • In a study using rabbit eyes, prostaglandin E was shown to contribute to a breakdown of the blood-aqueous barrier. This increase in the transfer coefficient of fluorescein is consistent with a similar response in animal eyes to prostaglandin E.
  • The vascular effects of prostaglandins may contribute to the tortuosity seen in iris vessels and the leakage demonstrated with fluorescein angiography of the iris.
  • To confuse matters, in another animal study, elevated prostaglandins increased outflow facility, which would contribute to a lower IOP and, thus, not be consistent with the reduced outflow facility seen in patients with glaucomatocyclitic crisis during an acute episode.
  • Another theory purports an increased aqueous production resulting from elevated levels of aqueous prostaglandins.
  • In summary, the exact mechanism by which prostaglandins regulate IOP has not been described, but a direct correlation between elevated levels of prostaglandins in the aqueous humor and the level of IOP has been found during acute attacks of glaucomatocyclitic crisis.
• Evidence exists that glaucomatocyclitic crisis may be associated with POAG. Patients with a 10-year or longer history of PSS are 3 times more likely to develop visual field changes and optic disc changes. These patients may have a higher than normal incidence of corticosteroid responsiveness, leading to an elevated IOP. This must be kept in mind during the treatment of this disorder with corticosteroids.
• Associations with immunogenetic factors also exist; in one study, the presence of human leukocyte antigen Bw54 (HLA-Bw54) was found in 41% of patients.
• Associations with certain allergic conditions and gastrointestinal diseases, most notably peptic ulcer disease, have been described.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Nongranulomatous iritis
Nonarteritic anterior ischemic optic neuropathy

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Procedures

• Perform gonioscopy to rule out closed-angle glaucoma.

Histologic Findings

Microscopic examination of trabeculectomy specimens taken at the time of an acute attack reveals inflammation with an abundant mononuclear cell infiltration of the trabecular meshwork. The keratic precipitates associated with PSS have been shown by specular microscopy to differ in morphology from other nongranulomatous fresh keratic precipitates in that they cluster with pseudopods extending between them.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

Complete medical care for patients presenting with glaucomatocyclitic crisis includes a reasonably thorough history of present illness, a review of drug allergies and sensitivities, a targeted past medical history and review of systems, a complete eye examination, a careful explanation of the disorder in accordance with the patient's level of understanding, and a commitment to long-term follow-up care of the patient.

• Medical therapy should be individualized to meet the patient's needs. Currently, the favored initial treatment is a combined regimen of a topical nonsteroidal anti-inflammatory drug (NSAID) and an antiglaucoma drug.
• Treatment recommendations include the following:
• • Topical steroids - Prednisolone acetate 1% 1 gtt qid, followed by taper
  • Topical antiglaucoma drops - Timolol 0.25-0.5% 1 gtt bid or equivalent, or dorzolamide 2% 1 gtt bid/tid or equivalent (Beta-blockers should be avoided in patients with asthma.)
  • Systemic carbonic anhydrase inhibitors - Acetazolamide 250 mg PO qid
  • Topical NSAIDs - Diclofenac 0.1% 1 gtt tid/qid or equivalent
  • Oral NSAIDs - Indomethacin 75-150 mg/d PO
• Miotics and mydriatic agents seldom are used because they may have further deleterious effects on the blood-aqueous barrier, and long-acting periocular steroids are frowned upon because of lingering IOP effects.
• NSAIDs reduce the inflammatory component by inhibiting the production of prostaglandins, and antiglaucoma medications reduce the influx of new aqueous; both these effects rapidly control the IOP. This combination also avoids potential IOP elevations caused by steroids in steroid-responsive patients.
• Well-informed and educated patients often can sense an impending attack based on ocular symptomatology, and they can institute appropriate self-therapy using an aqueous suppressant and a topical NSAID to blunt IOP elevations associated with treatment delays.
• Carefully observe patients periodically for recurrences of attacks and for development of POAG.
• In the absence of underlying chronic glaucoma, antiglaucoma agents do not prevent recurrences of glaucomatocyclitic crisis; therefore, they are not necessary between episodes.

Surgical Care

An occasional patient may require a filtering procedure, which is not effective in preventing recurrences of the episodes of iritis but may be useful in the management of high IOP seen with these episodes. For example, a patient with excessively high pressures threatening vascular perfusion would be a candidate for a filtering procedure. No benefit is gained from laser trabeculoplasty.

Consultations

• An ophthalmologist should be consulted to treat the elevated IOP and to provide long-term follow-up care for patients with POAG.
• Gastroenterology consultation should be sought if the patient has gastric symptoms.

Activity

No restrictions on activity are required.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Currently, a combined regimen of a topical NSAID and an antiglaucoma drug is favored. NSAIDs reduce the inflammatory component by inhibiting the production of prostaglandins, and antiglaucoma medications reduce the influx of new aqueous; both these effects rapidly control the IOP. This combination also avoids potential IOP elevations caused by steroids in steroid-responsive patients.

Drug Category: Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Drug Category: Beta-adrenergic blockers

Reduce elevated and normal IOP.

Drug Category: Alpha-adrenergic agonists

Reduce IOP by reducing the formation of aqueous humor.

Drug Category: Carbonic anhydrase inhibitors

Carbonic anhydrase is an enzyme found in many tissues of the body, including the eye. Catalyzes a reversible reaction where carbon dioxide becomes hydrated and carbonic acid dehydrated. By slowing the formation of bicarbonate ions with subsequent reduction in sodium and fluid transport, it may inhibit carbonic anhydrase in the ciliary processes of the eye. This effect decreases aqueous humor secretion, reducing IOP.

Drug Category: Nonsteroidal anti-inflammatory drugs (NSAIDs)

Have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known but may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms also may exist, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell membrane functions.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Outpatient Care

• Patients with elevated IOP should be monitored carefully and frequently until the IOP normalizes and the medications are tapered off appropriately.
• Annual visual field assessment is indicated in patients with ongoing optic nerve compromise or pressure irregularities. This assessment also helps in identifying those patients who are at risk of developing POAG.
• Long-term follow-up care of patients by an ophthalmologist is important.

In/Out Patient Meds

• See Medication.

Complications

• Complications are related to prolonged IOP elevations secondary to aqueous outflow impairment, resulting in damage to the optic nerve head and visual field compromise.
• Three mechanisms are identified with these prolonged elevations, as follows:
• • Underlying POAG
  • Prolonged steroid administration
  • Increased frequency of recurrent glaucomatocyclitic attacks

Prognosis

• An uncomplicated course is usual for most patients.
• In properly diagnosed and treated patients, vision remains uncompromised. However, glaucomatous optic nerve atrophy is irreversible.

Patient Education

• Appropriate discussion with patients and their families must include an emphasis on the recurrent nature of this disorder and its association with POAG.
• Patients should be educated about the medications, their limitations, and their adverse effects.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Early detection to prevent permanent damage from glaucoma and iritis
• May be overlooked if presenting in an emergency room setting

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. Vol 3. Philadelphia: Saunders;1994:1426-35.
• Bloch-Michel E, Dussaix E, Cerqueti P, Patarin D. Possible role of cytomegalovirus in the etiology of the Posner-Schlossman syndrome. Int Ophthalmol. 1987;11:95. [Medline].
• Burnstein Y, Shelton K, Higginbotham EJ. Glaucomatocyclitic crisis in a child. Am J Ophthalmol. Jul 1998;126(1):136-7. [Medline].
• Darchuk V, Sampaolesi J, Mato L. Optic nerve head behavior in Posner-Schlossman syndrome. Int Ophthalmol. 2001;23(4-6):373-9. [Medline].
• DeRoeth A Jr. Glaucomatocyclitic crisis. Am J Ophthalmol. 1970;69:370. [Medline].
• Dinakaran S, Kayarkar V. Trabeculectomy in the management of Posner-Schlossman syndrome. Ophthalmic Surg Lasers. Jul-Aug 2002;33(4):321-2. [Medline].
• Eakins KE. Increased intraocular pressure produced by prostaglandins E1 and E2 in the cat eye. Exp Eye Res. Jul 1970;10(1):87-92. [Medline].
• Fiore PM. Inflammatory glaucoma. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. Vol 3. 1994;1426-35.
• Grant WM. Clinical measurements of aqueous outflow. Arch Ophthalmol. 1951;46:113.
• Hahn IH, Stillman MC. A case of glaucomatocyclitic crisis in the emergency department. Ann Emerg Med. Feb 2006;47(2):167-9. [Medline].
• Harstad HK, Ringvold A. Glaucomatocyclitic crises (Posner-Schlossman syndrome). A case report. Acta Ophthalmol (Copenh). Apr 1986;64(2):146-51. [Medline].
• Harstad HK, Ringvold A. Glaucomatocyclitic crises (Posner-Schlossman syndrome). A case report. Acta Ophthalmol (Copenh). Apr 1986;64(2):146-51. [Medline].
• Hart CT, Weatherill JR. Gonioscopy and tonography in glaucomatocyclitic crises. Br J Ophthalmol. Sep 1968;52(9):682-7. [Medline].
• Hirose S, Ohno S, Matsuda H. HLA-Bw54 and glaucomatocyclitic crisis. Arch Ophthalmol. Dec 1985;103(12):1837-9. [Medline].
• Hollowich F. Clinical aspects and therapy of the Posner-Schlossman syndrome. Klin Monatsbl Augenheilkd. 1978;172:336.
• Hoskins HD, Kass MA. Secondary open-angle glaucoma. Diagnosis and therapy of the glaucomas. 1989;332-4. [Medline].
• Hung PT, Chang JM. Treatment of glaucomatocyclitic crisis. Am J Ophthalmol. Feb 1974;77(2):169-72. [Medline].
• Jap A, Sivakumar M, Chee SP. Is Posner Schlossman syndrome benign?. Ophthalmology. May 2001;108(5):913-8. [Medline].
• Kass MA, Becker B, Kolker AE. Glaucomatocyclitic crisis and primary open-angle glaucoma. Am J Ophthalmol. Apr 1973;75(4):668-73. [Medline].
• Kim R, Van Stavern G, Juzych M. Nonarteritic anterior ischemic optic neuropathy associated with acute glaucoma secondary to Posner-Schlossman syndrome. Arch Ophthalmol. Jan 2003;121(1):127-8. [Medline].
• Knox DL. Glaucomatocyclitic crises and systemic disease: peptic ulcer, other gastrointestinal disorders, allergy and stress. Trans Am Ophthalmol Soc. 1988;86:473-95. [Medline].
• Krupin T, Feitl ME. Glaucoma associated with uveitis. In: The Glaucomas. 1989;1205-23.
• Levatin P. Glaucomatocyclitic crisis occurring in both eyes. Am J Ophthalmol. 1956;41:1056.
• Maeda H, Nakamura M, Negi A. Selective reduction of the S-cone component of the electroretinogram in Posner-Schlossman syndrome. Eye. Apr 2001;15(Pt 2):163-7. [Medline].
• Masuda K, Izawa Y, Mishima S. Prostaglandins and glaucomatocyclitic crisis. Jpn J Ophthalmol. 1975;19:368.
• Nagataki S, Mishima J. Aqueous humor dynamics in glaucomatocyclitic crisis. Invest Ophthalmol. 1976;15:365.
• Neufeld AH, Sears ML. Prostaglandin and eye. Prostaglandins. Aug 1973;4(2):157-75. [Medline].
• Paivonsalo-Hietanen T, Tuominen J, Vaahtoranta-Lehtonen H. Incidence and prevalence of different uveitis entities in Finland. Acta Ophthalmol Scand. Feb 1997;75(1):76-81. [Medline].
• Perdviel G, Raynaud G, Gayard M. Syndrome de Posner-Schlossman et glaucoma. Bull Soc Ophthalmol Fr. 1962;62:611.
• Pillai CT, Dua HS, Azuara-Blanco A. Evaluation of corneal endothelium and keratic precipitates by specular microscopy in anterior uveitis. Br J Ophthalmol. Dec 2000;84(12):1367-71. [Medline].
• Posner A, Schlossman A. Syndrome of unilateral recurrent attacks of glaucoma with cyclitic symptoms. Arch Ophthalmol. 1948;39:517. [Medline].
• Posner A, Schlossman A. Further observations on the syndrome of glaucomatocyclitic crisis. Trans Am Acad Ophthalmol Otolaryngol. 1953;57:531.
• Raitta C, Klemetti A. Steroidbelastung bei Posner-Schlossmanschem Syndrom. V Graefes Arch Klin Exp Ophthalmol. 1967;174:66. [Medline].
• Rhee DJ, Rapuano CJ, Belzer TL, Fraunfelder FT, eds. Physician Desk Reference for Ophthalmic Medicines. 2006.
• Riatta C, Vannas A. Glaucomatocyclitic crisis. Arch Ophthalmol. 1977;95:608. [Medline].
• Richardson K. Acute glaucoma after trauma. In: Freeman HM, ed. Ocular Trauma. New York: Appleton-Century-Crofts;1979.
• Ritch R, Shields MB, Krupin T. The Glaucomas. St. Louis: Mosby;1989:1205-23.
• Shields MB. Glaucomas associated with ocular inflammation. In: Textbook of Glaucoma. 1992;356-61.
• Spivey BE, Armaly MF. Tonographic findings in glaucomatocyclitic crisis. Am J Ophthalmol. 1963;55:47. [Medline].
• Teoh SB, Thean L, Koay E. Cytomegalovirus in aetiology of Posner-Schlossman syndrome: evidence from quantitative polymerase chain reaction. Eye. Dec 2005;19(12):1338-40. [Medline].
• Theodore FH. Observations on glaucomatocyclitic crisis. Br J Ophthalmol. 1952;36:207. [Medline].
• Varma R, Katz LJ, Spaeth GL. Surgical treatment of acute glaucomatocyclitic crisis in a patient with primary open-angle glaucoma. Am J Ophthalmol. Jan 15 1988;105(1):99-100. [Medline].
• Wong PC, Ruderman JM, Krupin T, et al. 5-Fluorouracil after primary combined filtration surgery. Am J Ophthalmol. Feb 15 1994;117(2):149-54. [Medline].
• Yamamoto S, Pavan-Langston D, Tada R. Possible role of herpes simplex virus in the origin of Posner-Schlossman syndrome. Am J Ophthalmol. Jun 1995;119(6):796-8. [Medline].

Article Last Updated: Jun 18, 2006