Posner-Schlossman Syndrome

Updated: Sep 10, 2014
  • Author: James H Oakman, Jr, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Overview

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. [1] For this reason, the entity is often termed Posner-Schlossman syndrome (PSS).

Posner and Schlossman identified the following features [2] :

  • 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 and outflow facility, and all provocative tests normal between episodes

Since this original description, other cases attributed to glaucomatocyclitic crisis have been found to deviate from these criteria. [3] For instance, some patients with glaucomatocyclitic crisis have abnormal aqueous humor dynamics and may have an underlying primary open-angle glaucoma (POAG). [4]

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. [5, 6]
  • 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.
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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

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. [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

This condition 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]

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