Posner-Schlossman Syndrome (Glaucomatocyclitic Crisis)

Updated: Jul 07, 2017
  • Author: Leonard K Seibold, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Overview

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. 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. [1] 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. [2]

Since this original description, other cases attributed to glaucomatocyclitic crisis have been found to deviate from these criteria. [3]

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. [4, 5]
  • 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. [6]
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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. [7]

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Epidemiology

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. [8]

In Japan, among 2556 cases of uveitis, 1.8% had PSS. [9]

Mortality/Morbidity

Prolonged IOP elevation results in damage to the optic nerve head and visual field defects typical of glaucomatous atrophy. [10, 11]

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. [12]

Sex

Most studies report men to be at a higher risk of developing PSS. [6]

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