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]

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]

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]

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

Posner A, Schlossman A. Syndrome of unilateral recurrent attacks of glaucoma with cyclitic symptoms. Arch Ophthalmol. 1948. 39:517.
Posner A, Schlossman A. Further observations on the syndrome of glaucomatocyclitic crisis. Trans Am Acad Ophthalmol Otolaryngol. 1953. 57:531.
Theodore FH. Observations on glaucomatocyclitic crisis. Br J Ophthalmol. 1952. 36:207.
Levatin P. Glaucomatocyclitic crisis occurring in both eyes. Am J Ophthalmol. 1956. 41:1056.
Dinakaran S, Kayarkar V. Trabeculectomy in the management of Posner-Schlossman syndrome. Ophthalmic Surg Lasers. 2002 Jul-Aug. 33(4):321-2. [QxMD MEDLINE Link].
Chee SP, Jap A. Presumed fuchs heterochromic iridocyclitis and Posner-Schlossman syndrome: comparison of cytomegalovirus-positive and negative eyes. Am J Ophthalmol. 2008 Dec. 146 (6):883-9.e1. [QxMD MEDLINE Link].
Maeda H, Nakamura M, Negi A. Selective reduction of the S-cone component of the electroretinogram in Posner-Schlossman syndrome. Eye. 2001 Apr. 15:163-7. [QxMD MEDLINE Link].
Paivonsalo-Hietanen T, Tuominen J, Vaahtoranta-Lehtonen H, et al. Incidence and prevalence of different uveitis entities in Finland. Acta Ophthalmol Scand. 1997 Feb. 75(1):76-81. [QxMD MEDLINE Link].
Ohguro N, Sonoda KH, Takeuchi M, Matsumura M, Mochizuki M. The 2009 prospective multi-center epidemiologic survey of uveitis in Japan. Jpn J Ophthalmol. 2012 Sep. 56(5):432-5. [QxMD MEDLINE Link].
Darchuk V, Sampaolesi J, Mato L, et al. Optic nerve head behavior in Posner-Schlossman syndrome. Int Ophthalmol. 2001. 23(4-6):373-9. [QxMD MEDLINE Link].
Kim TH, Kim JL, Kee C. Optic disc atrophy in patient with Posner-Schlossman syndrome. Korean J Ophthalmol. 2012 Dec. 26(6):473-7. [QxMD MEDLINE Link].
Burnstein Y, Shelton K, Higginbotham EJ. Glaucomatocyclitic crisis in a child. Am J Ophthalmol. 1998 Jul. 126(1):136-7. [QxMD MEDLINE Link].
Pillai CT, Dua HS, Azuara-Blanco A, et al. Evaluation of corneal endothelium and keratic precipitates by specular microscopy in anterior uveitis. Br J Ophthalmol. 2000 Dec. 84(12):1367-71. [QxMD MEDLINE Link].
Takusagawa HL, Liu Y, Wiggs JL. Infectious theories of Posner-Schlossman syndrome. Int Ophthalmol Clin. 2011 Fall. 51(4):105-15. [QxMD MEDLINE Link].
Kongyai N, Sirirungsi W, Pathanapitoon K, Tananuvat N, Kunavisarut P, Leechanachai P, et al. Viral causes of unexplained anterior uveitis in Thailand. Eye (Lond). 2012 Apr. 26(4):529-34. [QxMD MEDLINE Link]. [Full Text].
Bloch-Michel E, Dussaix E, Cerqueti P, et al. Possible role of cytomegalovirus infection in the etiology of the Posner-Schlossmann syndrome. Int Ophthalmol. 1987 Dec. 11(2):95-6. [QxMD MEDLINE Link].
Teoh SB, Thean L, Koay E. Cytomegalovirus in aetiology of Posner-Schlossman syndrome: evidence from quantitative polymerase chain reaction. Eye. 2005 Dec. 19(12):1338-40. [QxMD MEDLINE Link].
Chee SP, Bacsal K, Jap A, Se-Thoe SY, Cheng CL, Tan BH. Clinical features of cytomegalovirus anterior uveitis in immunocompetent patients. Am J Ophthalmol. 2008 May. 145(5):834-40. [QxMD MEDLINE Link].
Yang SY, Chen MJ, Chen KH, Li AF, Chou CK, Lee SM. Cytomegalovirus and herpes simplex virus as causes of bilateral anterior uveitis in an immunocompetent patient. J Chin Med Assoc. 2011 Jan. 74(1):48-50. [QxMD MEDLINE Link].
Yamamoto S, Pavan-Langston D, Tada R, et al. Possible role of herpes simplex virus in the origin of Posner-Schlossman syndrome. Am J Ophthalmol. 1995 Jun. 119(6):796-8. [QxMD MEDLINE Link].
Choi CY, Kim MS, Kim JM, Park SH, Park KH, Hong C. Association between Helicobacter pylori infection and Posner-Schlossman syndrome. Eye (Lond). 2010 Jan. 24(1):64-9. [QxMD MEDLINE Link].
Nagataki S, Mishima J. Aqueous humor dynamics in glaucomatocyclitic crisis. Invest Ophthalmol. 1976. 15:365.
Li J, Ang M, Cheung CM, Vania M, Chan AS, Waduthantri S. Aqueous cytokine changes associated with Posner-Schlossman syndrome with and without human cytomegalovirus. PLoS One. 2012. 7(9):e44453. [QxMD MEDLINE Link].
Masuda K, Izawa Y, Mishima S. Prostaglandins and glaucomatocyclitic crisis. Jpn J Ophthalmol. 1975. 19:368.
Neufeld AH, Sears ML. Prostaglandin and eye. Prostaglandins. 1973 Aug. 4(2):157-75. [QxMD MEDLINE Link].
Eakins KE. Increased intraocular pressure produced by prostaglandins E1 and E2 in the cat eye. Exp Eye Res. 1970 Jul. 10(1):87-92. [QxMD MEDLINE Link].
Ohira S, Inoue T, Iwao K, Takahashi E, Tanihara H. Factors Influencing Aqueous Proinflammatory Cytokines and Growth Factors in Uveitic Glaucoma. PLoS One. 2016. 11 (1):e0147080. [QxMD MEDLINE Link].
Kandori M, Miyazaki D, Yakura K, Komatsu N, Touge C, Ishikura R, et al. Relationship between the number of cytomegalovirus in anterior chamber and severity of anterior segment inflammation. Jpn J Ophthalmol. 2013 Nov. 57 (6):497-502. [QxMD MEDLINE Link].
Woo JH, Lim WK, Ho SL, Teoh SC. Characteristics of Cytomegalovirus Uveitis in Immunocompetent Patients. Ocul Immunol Inflamm. 2015. 23 (5):378-83. [QxMD MEDLINE Link].
Hedayatfar A, Chee SP. Posner-Schlossman syndrome associated with cytomegalovirus infection: a case series from a non-endemic area. Int Ophthalmol. 2014 Oct. 34 (5):1123-9. [QxMD MEDLINE Link].
Sobolewska B, Deuter C, Doycheva D, Zierhut M. Long-term oral therapy with valganciclovir in patients with Posner-Schlossman syndrome. Graefes Arch Clin Exp Ophthalmol. 2014 Jan. 252(1):117-24. [QxMD MEDLINE Link].
Hedayatfar A, Chee SP. Posner-Schlossman syndrome associated with cytomegalovirus infection: a case series from a non-endemic area. Int Ophthalmol. 2014 Oct. 34(5):1123-9. [QxMD MEDLINE Link].
Su CC, Hu FR, Wang TH, Huang JY, Yeh PT, Lin CP. Clinical Outcomes in Cytomegalovirus-Positive Posner-Schlossman Syndrome Patients Treated With Topical Ganciclovir Therapy. Am J Ophthalmol. 2014 Aug 12. [QxMD MEDLINE Link].
Accorinti M, Gilardi M, Pirraglia MP, Amorelli GM, Nardella C, Abicca I. Cytomegalovirus anterior uveitis: long-term follow-up of immunocompetent patients. Graefes Arch Clin Exp Ophthalmol. 2014 Aug 20. [QxMD MEDLINE Link].
Jap A, Sivakumar M, Chee SP. Is Posner Schlossman syndrome benign?. Ophthalmology. 2001 May. 108(5):913-8. [QxMD MEDLINE Link].
Knox DL. Glaucomatocyclitic crises and systemic disease: peptic ulcer, other gastrointestinal disorders, allergy and stress. Trans Am Ophthalmol Soc. 1988. 86:473-95. [QxMD MEDLINE Link].
Roberts DK, Lukic AS, Yang Y, Moroi SE, Wilensky JT, Wernick MN. Novel observations and potential applications using digital infrared iris imaging. Ophthalmic Surg Lasers Imaging. 2009 Mar-Apr. 40(2):207-16. [QxMD MEDLINE Link].
Shiraishi A, Hara Y, Takahashi M, Oka N, Yamaguchi M, Suzuki T, et al. Demonstration of "owl's eye" morphology by confocal microscopy in a patient with presumed cytomegalovirus corneal endotheliitis. Am J Ophthalmol. 2007 Apr. 143 (4):715-7. [QxMD MEDLINE Link].
Hung PT, Chang JM. Treatment of glaucomatocyclitic crises. Am J Ophthalmol. 1974 Feb. 77(2):169-72. [QxMD MEDLINE Link].
Chee SP, Jap A. Cytomegalovirus anterior uveitis: outcome of treatment. Br J Ophthalmol. 2010 Dec. 94 (12):1648-52. [QxMD MEDLINE Link].
Varma R, Katz LJ, Spaeth GL. Surgical treatment of acute glaucomatocyclitic crisis in a patient with primary open-angle glaucoma. Am J Ophthalmol. 1988 Jan 15. 105(1):99-100. [QxMD MEDLINE Link].
Wong PC, Ruderman JM, Krupin T, et al. 5-Fluorouracil after primary combined filtration surgery. Am J Ophthalmol. 1994 Feb 15. 117(2):149-54. [QxMD MEDLINE Link].
Broadway DC, Bates AK, Lightman SL, Grierson I, Hitchings RA. The importance of cellular changes in the conjunctiva of patients with uveitic glaucoma undergoing trabeculectomy. Eye (Lond). 1993. 7 ( Pt 4):495-501. [QxMD MEDLINE Link].
Kass MA, Becker B, Kolker AE. Glaucomatocyclitic crisis and primary open-angle glaucoma. Am J Ophthalmol. 1973 Apr. 75(4):668-73. [QxMD MEDLINE Link].
Hirose S, Ohno S, Matsuda H. HLA-Bw54 and glaucomatocyclitic crisis. Arch Ophthalmol. 1985 Dec. 103(12):1837-9. [QxMD MEDLINE Link].
Kim R, Van Stavern G, Juzych M. Nonarteritic anterior ischemic optic neuropathy associated with acute glaucoma secondary to Posner-Schlossman syndrome. Arch Ophthalmol. 2003 Jan. 121(1):127-8. [QxMD MEDLINE Link].
Irak I, Katz BJ, Zabriskie NA, et al. Posner-Schlossman syndrome and nonarteritic anterior ischemic optic neuropathy. J Neuroophthalmol. 2003 Dec. 23(4):264-7. [QxMD MEDLINE Link].
Iwao K, Inatani M, Seto T, Takihara Y, Ogata-Iwao M, Okinami S. Long-term outcomes and prognostic factors for trabeculectomy with mitomycin C in eyes with uveitic glaucoma: a retrospective cohort study. J Glaucoma. 2014 Feb. 23(2):88-94. [QxMD MEDLINE Link].
Albert DM, Jakobiec FA. Principles and Practice of Ophthalmology. Philadelphia: Saunders; 1994. Vol 3: 1426-35.
de Roetth A Jr. Glaucomatocyclitic crisis. Am J Ophthalmol. 1970 Mar. 69(3):370-1. [QxMD MEDLINE Link].
Fiore PM. Inflammatory glaucoma. Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. 1994. Vol 3: 1426-35.
Grant WM. Clinical measurements of aqueous outflow. Arch Ophthalmol. 1951. 46:113.
Hahn IH, Stillman MC. A case of glaucomatocyclitic crisis in the emergency department. Ann Emerg Med. 2006 Feb. 47(2):167-9. [QxMD MEDLINE Link].
Harstad HK, Ringvold A. Glaucomatocyclitic crises (Posner-Schlossman syndrome). A case report. Acta Ophthalmol (Copenh). 1986 Apr. 64(2):146-51. [QxMD MEDLINE Link].
Hart CT, Weatherill JR. Gonioscopy and tonography in glaucomatocyclitic crises. Br J Ophthalmol. 1968 Sep. 52(9):682-7. [QxMD MEDLINE Link].
Hollowich F. Clinical aspects and therapy of the Posner-Schlossman syndrome. Klin Monatsbl Augenheilkd. 1978. 172:336.
Hoskins HD, Kass MA. Secondary open-angle glaucoma. Diagnosis and therapy of the glaucomas. 1989. 332-4.
Krupin T, Feitl ME. Glaucoma associated with uveitis. The Glaucomas. 1989. 1205-23.
Perdviel G, Raynaud G, Gayard M. Syndrome de Posner-Schlossman et glaucoma. Bull Soc Ophthalmol Fr. 1962. 62:611.
Raitta C, Klemetti A. Steroidbelastung bei Posner-Schlossmanschem Syndrom. V Graefes Arch Klin Exp Ophthalmol. 1967. 174:66.
Rhee DJ, Rapuano CJ, Belzer TL, et al. Physician Desk Reference for Ophthalmic Medicines. 2006.
Riatta C, Vannas A. Glaucomatocyclitic crisis. Arch Ophthalmol. 1977. 95:608.
Richardson K. Acute glaucoma after trauma. Freeman HM, ed. Ocular Trauma. New York: Appleton-Century-Crofts; 1979.
Ritch R, Shields MB, Krupin T. The Glaucomas. St. Louis: Mosby; 1989. 1205-23.
Shields MB. Glaucomas associated with ocular inflammation. Textbook of Glaucoma. 1992. 356-61.
Spivey BE, Armaly MF. Tonographic findings in glaucomatocyclitic crisis. Am J Ophthalmol. 1963. 55:47.

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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 G Sieck, MD Resident Physician, Department of Ophthalmology, University of Colorado School of Medicine

Erin G Sieck, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

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.