Posner-Schlossman Syndrome Clinical Presentation

  • Author: James H Oakman Jr, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Mar 2, 2012
 

History

In general, the symptomatology of glaucomatocyclitic crisis 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 recur 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.

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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.[11] 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.

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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
  • Infection[12] - Cytomegalovirus (CMV),[13, 14, 15, 16] herpes simplex virus,[16, 17] Helicobacter pylori[18]

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.[19]

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.[20, 21]

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.[22] 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.[23] 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.[24]

Associations with certain allergic conditions and gastrointestinal diseases, most notably peptic ulcer disease, have been described.[25]

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Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Andrew I Rabinowitz, MD  Consulting Staff, Department of Ophthalmology, Barnet Dulaney Perkins Eye Center

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

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

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

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

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

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, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

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