Background

Posner-Schlossman syndrome, also known as glaucomatocyclitic crisis, is an ocular condition with self-limited recurrent episodes of markedly elevated intraocular pressure (IOP) and nongranulomatous anterior chamber inflammation.^[1]It is most often classified as secondary inflammatory glaucoma.^[2]

In 1948, Posner and Schlossman first recognized glaucomatocyclitic crisis and described the features of this syndrome.^[3]For this reason, the entity is often termed Posner-Schlossman syndrome (PSS). Their original paper identified key features of the condition, including recurrent episodes of mild cyclitis and uniocular involvement. Attack duration varies from a few hours to several weeks. Glaucomatocyclitic crisis is characterized by a slight decrease in vision, elevated IOP, and open anterior chamber angles, with normal visual fields and optic nerve appearance. In addition, IOP and outflow facility are normal between episodes.^[4]

Since this original description, other cases attributed to glaucomatocyclitic crisis have been found to deviate from these criteria.^[5]

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 simultaneously. ^{[6, 7]}
The rise of IOP is out of proportion to the severity of the uveitis, and this rise in IOP precedes the identifiable inflammatory reaction, at times by several days.
Current literature supports cytomegalovirus (CMV) infection as the likely inflammatory precursor to the anterior uveitis and elevated IOP. ^[8]

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.^[9]

Frequency

Glaucomatocyclitic crisis is a rare condition in the United States. It is more common in Asia and northern Europe.

In Finland, the incidence is 0.4 and the prevalence is 1.9 per 100,000 population.^[10]

In Japan, among 2556 cases of uveitis, 1.8% had PSS.^[11]

Mortality/Morbidity

Prolonged IOP elevation results in damage to the optic nerve head and visual field defects typical of glaucomatous atrophy.^{[12, 13]}

Age

PSS almost exclusively affects individuals aged 20-50 years. However, there are reports of rare episodes in individuals older than 60 years, as well as in adolescence.^[14]

Sex

Most studies report men to be at a higher risk of developing PSS.^[8]

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.

Clinical Presentation

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Author

Leonard K Seibold, MD Assistant Professor, Co-Director, Glaucoma Fellowship, Department of Ophthalmology, University of Colorado School of Medicine

Leonard K Seibold, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, Association for Research in Vision and Ophthalmology, Chandler Grant Glaucoma Society, Colorado Society of Eye Physicians and Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Erin Gwen Sieck, MD Assistant Professor, Department of Ophthalmology and Visual Sciences, Washington University School of Medicine

Erin Gwen Sieck, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, American Society of Cataract and Refractive Surgery, ASCRS Foundation, Interventional Glaucoma Consortium

Disclosure: Received income in an amount equal to or greater than $250 from: Abbvie; Alcon.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Martin B Wax, MD Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Research and Development, Head, Ophthalmology Discovery Research and Preclinical Sciences, Alcon Laboratories, Inc

Martin B Wax, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, Society for Neuroscience

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD † Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew I Rabinowitz, MD Director of Glaucoma Service, Barnet Dulaney Perkins Eye Center

Andrew I Rabinowitz, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Ophthalmology, American Society for Laser Medicine and Surgery, American Academy of Ophthalmology, American Medical Association

Disclosure: Nothing to disclose.

James H Oakman, Jr, MD Partner, Southern Eye Center, Augusta, Georgia

James H Oakman, Jr, MD is a member of the following medical societies: Association of American Physicians and Surgeons, Georgia Medical Society, Georgia Society of Ophthalmology, Georgia Society of the American College of Surgeons, American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.