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QUESTION

WHAT IS HEMORRHAGIC OCCLUSIVE RETINAL VASCULITIS, AND WHY DO I NEED TO KNOW ABOUT IT?

James A. Stefater MD, PhD
Dean Eliott, MD

Hemorrhagic occlusive retinal vasculitis (HORV) is a rare but devastating complication that has been described following uncomplicated cataract surgery. The first report describes 2 patients who had seemingly uneventful cataract surgery in the first eye but developed bilateral, severe, ischemic retinal vasculitis in both eyes within days following cataract surgery performed on the second eye.¹ Both patients had bilateral, severe, irreversible visual loss. Despite an extensive ocular and systemic work-up, a definitive causative factor was not identified; however, the authors note that both patients had received intraoperative intracameral vancomycin for endophthalmitis prophylaxis and proposed that intraoperative vancomycin exposure was the cause of the subsequent reaction. As word spread of the clinical findings and potential association, 4 additional patients were reported (3 bilateral, 1 unilateral) and these patients also had intraoperative exposure to vancomycin.²

The differential diagnosis of HORV includes acute postoperative endophthalmitis, viral retinitis, medication toxicity, and central retinal vein occlusion or combined central retinal artery and vein occlusions Most of these conditions can be ruled out after obtaining a thorough history and performing a careful examination.

A joint task force composed of a group of members from the American Society of Retina Specialists and the American Society of Cataract and Refractive Surgeons was formed to collect additional cases and to investigate causality. The task force ultimately identified a total of 36 eyes from 23 patients who likely had vancomycin-induced HORV.³ All patients in this report had received vancomycin, either as an intracameral injection, in the irrigation solution during cataract surgery, or as intravitreal injection for the treatment of presumed endophthalmitis. Most patients had undergone uneventful first-eye cataract surgery but presented within a few days after second-eye cataract surgery with acute onset of painless visual loss in both eyes. In some patients, the condition developed in one eye after the first-eye cataract surgery, and then also developed in the second eye after second-eye cataract surgery, even when the second surgery was performed years later. In these cases, vancomycin was used in both cataract surgeries.

The clinical findings of HORV are variable but include minimal anterior segment inflammation and severe retinal findings. There is typically a normal cornea or mild corneal edema, minimal anterior chamber reaction, and no hypopyon. The vitreous is clear or has mild inflammation. The retinal findings are usually profound and demonstrate diffuse retinal ischemia with severe, widespread intraretinal hemorrhages associated with retinal vasculitis. A few cases, however, had minimal symptoms and mild retinal findings. The outcome for most of these patients is devastating. Of the 36 eyes in the joint task force series, 8 had visual acuity of no light perception and 23 eyes (66%) were worse than 20/200.3 Over 50% of eyes went on to develop neovascular glaucoma.³

It is worth noting that the association with vancomycin is only correlative; however, all reported cases to date have included vancomycin administration in some form. All cataract surgeries were performed by experienced surgeons and no additional cases from the same surgeon or the same surgery center were reported. The potential mechanism remains unknown, but the authors proposed the most likely mechanism to be a Type III hypersensitivity reaction where the patient receives intraocular vancomycin once without clinical sequelae, followed by a robust bilateral hypersensitivity response upon sequential administration. The time course is not consistent with direct toxicity because there is a delay (on average of 8 days) between the second-eye cataract surgery and presentation. In the cases that occurred after unilateral surgery, some patients reported systemic exposure to vancomycin in the past. Of note, a systemic leukocytoclastic vasculitis (where antigen-antibody complexes deposit in vessel walls) has been reported to occur with systemic administration of vancomycin,⁴ and HORV is presumed to occur via a similar mechanism. This proposed etiology raises the issue of whether aggressive management with immunosuppressive agents could play a therapeutic role. Importantly, in cases where early steroids were implemented, visual prognosis was generally better. According to the joint task force, the 3 eyes that received intravitreal corticosteroids had final visual outcomes of 20/40, 20/70, and hand motion. Early treatment with anti-vascular endothelial growth factor agents and early panretinal photocoagulation also appeared to result in better outcomes.

While causality cannot be definitively determined, and while the response is admittedly extremely rare (the vast majority of patients receive vancomycin without a similar response), the devastating nature of this condition should prompt careful consideration as to the necessity of prophylactic vancomycin during cataract surgery. The seminal study on antibiotic use during cataract surgery to prevent endophthalmitis had a profound impact on the practice patterns of surgeons in Europe.⁵ In this study, the endophthalmitis rate decreased from 0.34% to 0.07% with the use of intracameral cefuroxime. Many US surgeons were skeptical of the results because of the high rate of endophthalmitis in the control group (0.34%). However, over the last 10 years, the trend among US cataract surgeons has begun to shift. In 2007, only 15% of cataract surgeons were using intraoperative prophylactic antibiotics, but in 2014, 36% of surgeons were using such therapy.⁶ Of those surgeons, 15% were using vancomycin in the infusion solution and 22% were performing a direct intracameral injection of vancomycin. In total, 13% of all cataract surgeons in the United States in 2014 were using intraoperative vancomycin.⁶ Out of the patients receiving antibiotic prophylaxis but not receiving vancomycin, the most common agent was moxifloxacin (33%) followed by a cephalosporin (26%).⁶ Interestingly, there are no reports of HORV in patients who received moxifloxacin or cefuroxime.³

If cataract surgeons nonetheless choose to use vancomycin, and a patient presents to a retina specialist, making the correct diagnosis and distinguishing the clinical presentation of HORV from endophthalmitis is of critical importance. Some patients who presented with visual loss after cataract surgery were presumed to have mild endophthalmitis (no hypopyon) when they likely had HORV instead. These eyes received intravitreal vancomycin and had exceptionally poor outcomes. In some of these eyes, fluorescein angiography documented retinal ischemia after the cataract surgery which worsened after the intravitreal vancomycin injection.

1.     Nicholson LB, Kim BT, Jardon J, et al. Severe bilateral ischemic retinal vasculitis following cataract surgery. Ophthalmic Surg Lasers Imaging Retina. 2014;45(4):338-342.

2.     Witkin AJ, Shah AR, Engstrom RE, et al. Postoperative hemorrhagic occlusive retinal vasculitis: expanding the clinical spectrum and possible association with vancomycin. Ophthalmology. 2015;122(7):1438-1451.

3.     Witkin AJ, Chang DF, Jumper JM, et al. Vancomycin-associated hemorrhagic occlusive retinal vasculitis: clinical characteristics of 36 eyes. Ophthalmology. 2017;124(5):583-595.

4.     Pongruangporn M, Ritchie DJ, Lu D, Marschall J. Vancomycin-associated leukocytoclastic vasculitis. Case Rep Infect Dis. 2011;2011:356370.

5.     Endophthalmitis Study Group, European Society of Cataract & Refractive Surgeons. Prophylaxis of postoperative endophthalmitis following cataract surgery: results of the ESCRS multicenter study and identification of risk factors. J Cataract Refract Surg. 2007;33(6):978-988.

6.     Chang DF, Braga-Mele R, Henderson BA, Mamalis N, Vasavada A; ASCRS Cataract Clinical Committee. Antibiotic prophylaxis of postoperative endophthalmitis after cataract surgery: results of the 2014 ASCRS member survey. J Cataract Refract Surg. 2015;41(6):1300-1305.

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