• Posner–Schlossman syndrome, also known as glaucomatocyclitic crisis, is a form of open-angle glaucoma characterized by self-limited recurrent episodes of marked elevation in intraocular pressure (IOP) associated with nongranulomatous anterior uveitis. It is typically unilateral, with attacks lasting a few hours to several weeks. Mild to no discomfort. Optic nerve damage and visual field damage may occur from repeated attacks (1)[C].
Typically affects patients between 20 and 50 years of age. Rare cases have been reported in adolescence and patients older than 60 years.
• No study in the US
• 0.4 (per 100,000) in Finland
• No study in the US
• 1.9 (per 100,000) in Finland
Unclear, however significantly elevated levels of several entities have been found in the aqueous humor of patients during recurring attacks of Posner–Schlossman syndrome and normalize between episodes:
• Cytomegalovirus (CMV)
• Herpes simplex virus (HSV)
• Associations with HLA-Bw54
• Associations with peptic ulcer disease
COMMONLY ASSOCIATED CONDITIONS
Unclear. See “Etiology”
The patient presents complaining of mild discomfort and blurring of vision or colored halos in 1 eye. However, there may be no pain despite high IOP. The eye is not red and typically looks normal to the patient or the casual observer. Recurrent attacks lasting several hours to 1 month, but rarely over 2 weeks, may occur. Patients have a variable clinical course; some have many recurrences over many years, whereas others have 1 or 2 episodes in their lives.
• On external examination, the eye is quiet and typically looks normal.
• The conjunctiva is white.
• Elevated IOP develops 1–2 days prior to anterior inflammation and keratic precipitates.
• The corneal endothelium develops fine stellate keratic precipitates, nonpigmented, typically distributed over the inferior part of the cornea, ranging from 1 to 20 in number.
• The anterior chamber shows very mild aqueous cell and flare not in proportion to the degree of IOP elevation.
• The IOP elevation is greater than 30, usually in the range of 40–60 mm Hg, much greater than would be expected given the mild degree of inflammation.
• Microcystic corneal edema may or may not be present.
• The iris may be slightly dilated, but peripheral anterior synechiae and posterior synechiae do not develop.
• Rarely, iris heterochromia may occur, with the affected eye being lighter in color, due to stromal atrophy from repeated inflammation.
• Gonioscopy reveals an open angle that may show keratic precipitates on the trabecular meshwork.
• Examination of the lens and fundus are normal.
DIAGNOSTIC TESTS & INTERPRETATION
Gonioscopy to rule out acute angle closure glaucoma.
Posner–Schlossman syndrome is a clinical diagnosis, there are no laboratory tests.
Optic nerve imaging with either stereo disc photographs, Heidelberg retinal tomography (HRT), or optical coherence tomography (OCT) should be obtained to document the current optic nerve status, and to have a baseline to look for future damage to the optic nerve.
Humphrey visual field (HVF) testing to determine loss of a patient’s visual field.
Repeated episodes may lead to optic nerve damage.
• Acute angle-closure glaucoma
• Chronic angle-closure glaucoma
• Fuchs heterochromic iridocyclitis
• Nongranulomatous anterior uveitis
• Inflammatory open-angle glaucoma
• Pigmentary glaucoma
• Neovascular glaucoma
• Herpes simplex and herpes zoster keratouveitis
Topical steroids to control the inflammation and topical glaucoma drops to control the elevated IOP are usually used together to control the acute attack. The steroids are tapered off and glaucoma medications stopped as the inflammation resolves and IOP normalizes (2)[C].
• Topical steroids: Prednisolone acetate 1% q.i.d.
• Topical glaucoma drops: Beta-blockers (timolol 0.5% b.i.d.), and/or alpha-agonists (brimonidine 0.2%, 0.15%, 0.1% b.i.d./t.i.d.), and/or carbonic anhydrase inhibitor (dorzolamide 2% b.i.d./t.i.d.)
• Topical NSAIDs (diclofenac 0.1% q.i.d. or equivalent),
• Oral NSAIDs—indomethacin 75–150 mg PO q.d. (3)
• Oral carbonic anhydrase inhibitors—acetazolamide 250 mg PO b.i.d. to q.i.d. as tolerated
• Note: all of the above can be used as first-line treatment as well, depending on how the patient responds.
Issues for Referral
Ophthalmologist for proper monitoring and treatment
Glaucoma filtering procedures such as trabeculectomy and tube shunts are rarely needed (4)[C].
• Every few days until the IOP improves then weekly until the episode resolves and the patient is tapered off the medications appropriately.
• Monitor with a complete ophthalmic examination and appropriate optic nerve imaging and visual field assessment as circumstances dictate, every 6 months initially to yearly if stable.
• Stress the recurrent nature of the disorder.
• The importance of proper monitoring of this condition as it can silently lead to optic nerve damage with possible visual impairment. Loss of vision can be permanent.
• Monitoring is important because the patient may not know that they are sustaining damage to the optic nerve. The patient experiences no or mild symptoms, and will not experience changes in their vision until a significant portion of the optic nerve is damaged.
• Good, if properly diagnosed, treated and followed.
• Vision should be uncompromised throughout the patient’s lifetime.
• However, it can be easily overlooked in an emergency department or primary care giver setting. Optic nerve damage and visual field changes can be significant and are usually permanent.
1. Posner A, Schlossman A. Syndrome of unilateral recurrent attacks of glaucoma with cyclitic symptoms. Arch Ophthal 1948;39(4):517–535.
2. Moorthy RS, Mermoud A, Baerveldt G, et al. Glaucoma associated with uveitis. Surv Ophthalmol 1997;41:361–394.
3. Masuda K, Izawa Y, Mishima SS. Prostaglandins and glaucomato-cyclitis crisis. Jpn J Ophthalmol 1975;19:368.
4. Dinakaran S, Kayarkar V. Trabeculectomy in the management of Posner-Schlossman syndrome. Ophthalmic Surg Lasers 2002;33(4):321–322.