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1 2,3 AESERIES CASE Spontaneous SR Rathinam and ET Cunningham Jr hyphaema and acute ocular hypertension associated with severe -induced

Abstract including one patient each with idiopathic anterior uveitis, Reiter’s syndrome, juvenile Purpose To report the occurrence of rheumatoid arthritis, ankylosing spondylitis, spontaneous hyphaema and acute ocular and herpes simplex virus-associated hypertension in four patients with severe keratouveitis. Three of these five patients lens-induced anterior uveitis. had current or prior rubeosis irides. Similarly, Methods Retrospective case series. Klemperer et al described two patients with Results Four patients with mature anterior uveitis associated with spontaneous developed severe acute, unilateral, anterior 1Aravind Eye Hospital and hyphaema, both of whom had ankylosing uveitis associated with spontaneous Postgraduate Institute of spondylitis and rubeosis irides,3 Hayasaka et al4 , Madurai, hyphaema formation and acutely elevated reported on hyphaema formation in two Tamil Nadu, India . None of the patients had patients with herpes zoster ophthalmicus in a history of trauma or evidence of angle, , 2 the absence of rubeosis irides, and Mamo Department of or retinal neovascularization. Prompt cataract and Baghdassarian5 described hyphaema Ophthalmology, California removal led to resolution of the inflammation, Pacific Medical Center, San in a patient with Behcet’s syndrome, anterior clearing of the anterior chamber haemorrhage, Francisco, CA, USA uveitis, and no obvious iris neovascularization. and normalization of intraocular pressure Most recently, Gan and Teoh6 reported the 3Department of in all four affected eyes. occurrence of haemorrhagic, hypertensive, Ophthalmology, Stanford Conclusions Spontaneous hyphaema and uveitis in a HIV-positive patient University School of acute ocular hypertension can occur in patients with a history of cytomegalovirus uveitis and Medicine, Stanford, CA, with severe lens-induced intraocular USA immune reactivation uveitis. No mention was inflammation in the absence of trauma or made regarding the presence or absence of ocular neovascularization. Correspondence: neovascularization of the angle, iris, or . ET Cunningham Jr, Eye (2010) 24, 1822–1824; doi:10.1038/eye.2010.105; West Coast Retina Medical published online 30 July 2010 Group, Inc., 185 Berry Street, Keywords: haemorrhage; inflammation; iritis; Case reports Lobby 2, Suite 130, phacolytic San Francisco, We describe four patients who developed CA 94107-1739, spontaneous hyphaema and acute ocular USA hypertension in the setting of severe lens- Tel: þ 1 415 972 4600; Introduction Fax: þ 1 415 975 0999. induced anterior uveitis in the absence of E-mail: Although spontaneous hyphaema has been trauma or ocular neovascularization. Patient emmett_cunningham@ reported to occur in approximately 5% of characteristics are summarized in Table 1 and yahoo.com patients with Fuchs’ uveitis syndrome (FUS),1 their appearance at presentation is illustrated in the occurrence of anterior chamber bleeding in Figure 1. Intraocular pressure was controlled Received: 17 February 2010 other forms of uveitis is distinctly unusual. before surgery with topical pressure-lowering Accepted in revised form: 2 17 May 2010 Fong and Raizman reported on the largest agents. There was no history of trauma in any of Published online: 30 July series of patients with severe anterior uveitis the patients and none had newly formed angle, 2010 and spontaneous hyphaema formation, iris, or retinal vessels on detailed examination Spontaneous hyphaema and acute ocular hypertension in lens-induced uveitis SR Rathinam and ET Cunningham Jr 1823

Table 1 Clinical characteristics of patients at presentation

Case Age (years) Sex Affected eye Presenting vision Presenting IOP (mm Hg) Final vision

1 77 W OD LP 21 (7 OS) 6/9 2 59 M OS LP 34 (12 OD) 6/12 3 62 M OS LP 32 (18 OD) 6/6 4 65 M OD LP 56 (18 OS) 6/9

Abbreviations: W, woman; M, man; LP, light perception; IOP, intraocular pressure; OD, right eye; OS, left eye. All patients had a mature cataract, severe anterior uveitis, haemorrhagic hypopyon formation, and acutely elevated intraocular pressure in the affected eye.

Figure 1 Slit-lamp photographs showing mature cataract, severe anterior uveitis, and spontaneous hyphaema formation in cases 1 (a), 2 (b), 3 (c), and 4 (d). before or after removal of the cataract. All four patients chamber paracentesis (Amsler’s sign).1,5,7 Inflammatory underwent emergent lens removal followed by occlusion of iris vessels, as can occur in Behcet’s intraocular lens placement in the primary posterior disease, or in herpetic infection,2,4 can directly chamber. Intraocular pressure normalized and the damage the iris vessels, resulting in anterior chamber anterior chamber inflammation and haemorrhage haemorrhage. Some patients with anterior uveitis,2,3,5 resolved following removal of the cataract in all four including a small percentage of patients with FUS,1,7 patients. have abnormal vessels in either the angle or the iris, which may bleed during periods of inflammation. Finally, patients with severe anterior chamber Discussion inflammation and no evidence of iris neovascularization There are a number of possible causes of spontaneous can have such severe vascular compromise that hyphaema formation in patients with uveitis. Trauma, both white and red blood cells extravagate, when severe, can cause both uveitis and vascular resulting in both a hypopyon and spontaneous damage, producing haemorrhagic anterior chamber hyphaema formation, as we presume occurred in inflammation.2 Patients with FUS have abnormally our four patients. friable iris and anterior chamber angle vessels, which Anterior chamber bleeding resolves spontaneously, may bleed in response to trauma, or following anterior as the inflammation is controlled in most patients with

Eye Spontaneous hyphaema and acute ocular hypertension in lens-induced uveitis SR Rathinam and ET Cunningham Jr 1824

uveitis and spontaneous hyphaema formation.1–7 Surgical Acknowledgements removal of blood from the anterior chamber This study was supported in part by the Aravind may be required when the bleeding fails to clear Eye Hospital and by the Pacific Vision Foundation, spontaneously.2 Posterior segment examination and San Francisco. fluorescein angiography should be performed whenever possible to exclude retinal ischaemia or neovascularization. The primary treatment for lens-induced uveitis is References prompt and complete removal of all lens material. 1 Jones NP. Spontaneous hyphaema associated with anterior uveitis. Br J Ophthalmol 1994; 78: 420. Summary 2 Fong DS, Raizman MB. Spontaneous associated What was known before with anterior uveitis. Br J Ophthalmol 1993; 77: 635–638. K Uveitis can be associated with haemorrhagic hypopyon 3 Klemperer I, Yassur Y, David R. Spontaneous hyphema: an formation. unusual complication of uveitis associated with ankylosing spondylitis. Ann Ophthalmol 1992; 24: 177–179. What this study adds 4 Hayasaka S, Watanabe M, Yamamoto Y, Noda S, Sekimoto M, K First to describe spontaneous hyphema formation Setogawa T. Herpes zoster ophthalmicus complicated by and ocular hypertension in patients with lens-induced hyphema and hemorrhagic . Ophthalmologica 1988; uveitis. 196: 185–187. 5 Mamo JG, Baghdassarian A. Behcet’s disease. Arch Ophthalmol 1971; 71: 38–48. 6 Gan NY, Teoh SC. Haemorrhagic, hypertensive hypopyon Conflict of interest uveitis in early immune reactivation uveitis. Eye 2010; 24: 383. 7 Jones NP. Fuchs’ heterochromic uveitis: an update. The authors declare no conflict of interest. Surv Ophthalmol 1993; 37: 253–272.

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