Case of the Month

Edited by Robert N. Johnson, MD

Case History

A 55-year-old Caucasian male presented with a 4-day history of worsening vision in his left eye.  He had undergone uncomplicated cataract extraction in his right eye 18 days prior to presentation and in his left eye 7 days prior to presentation.  His past medical history is notable for systemic hypertension and myasthenia gravis, for which he is currently taking mycophenolate mofetil and pyridostigmine.  His past ocular history, family history and review of systems were otherwise noncontributory.

On examination, his best-corrected visual acuity was 20/40 in the right eye and no light perception in the left eye.  There was a brisk afferent pupillary defect present in the left eye.  Ocular motility, intraocular pressure, gonioscopy, and anterior segment examinations were normal in both eyes, with the exception of rare cell in the anterior chamber of both eyes.  The posterior segment examination of the right eye revealed multiple dot-blot hemorrhages in the mid and far periphery of all four quadrants as well as several areas of retinal vascular sheathing (Figure 1).  Fundus examination of the left eye showed widespread intraretinal hemorrhages with whitening of the macula consistent with a combined central retinal artery and central retinal vein occlusion (Figure 2).  Fluorescein angiography revealed widespread nonperfusion of the mid and far periphery of the right eye (Figure 3) and complete nonperfusion of the fundus in the left (Figure 4).

Optical coherence tomography (OCT) scans of the right eye showed a preserved foveal contour with associated moderate medial and temporal inner retinal hyperreflectivity (Figure 5).  OCT scans of the left eye shows severe full thickness retinal edema with near-total obliteration of retinal layer segmentation (Figure 6).

Differential Diagnosis

Acute post-operative endophthalmitis, Acute retinal necrosis/viral retinitis, Medication toxicity, Central retinal vein occlusion, Combined central retinal artery and central retinal vein occlusion, hemorrhagic occlusive retinal vasculitis (HORV)

Additional Case History

Discussion with the patient’s cataract surgeon revealed the use of intracameral vancomycin at the time of uncomplicated cataract surgery in each eye.  Due to a presumed diagnosis of HORV, the patient was started on prednisone 80mg and clopidogrel 75mg PO daily, with little effect.  He also underwent two bevacizumab injections in the right eye and a single bevacizumab injection in the left eye, as well as panretinal photocoagulation in both eyes.  The patient subsequently developed a superotemporal midperipheral retinal tear and an associated retinal detachment, which was repaired with pars plana vitrectomy.  At final followup 1.5 years later, the patient’s best corrected visual acuity was 20/50 in the right eye and no light perception in the left eye.

Discussion

Hemorrhagic occlusive retinal vasculitis (HORV) was first described in 2014 by Nicholson et al,¹ when two patients developed severe bilateral ischemic retinal vasculitis following uncomplicated cataract surgery.  Since then, two further case series, the largest of which included 36 eyes of 23 patients, have been reported.^2,3 The presentation varies widely, from asymptomatic to sudden, painless, profound loss of vision from 1 to 21 days following surgery, with an average of 8 days.  On examination, affected patients have mild to moderate anterior chamber and vitreous inflammation and multiple intraretinal hemorrhages within areas of nonperfusion, commonly around venules.  Fluorescein angiography reveals retinal vasculitis with vascular occlusion.

Since all patients in the published series of HORV received vancomycin,^1-4 it is widely believed to be the most likely cause of HORV.  The mechanism of action is most likely related to a type III hypersensitivity reaction, where a delayed immune response is caused by the deposition of antibody-antigen complexes in the vessel walls, resulting in complement activation, recruitment of macrophages, and inflammatory cytokine cascades.³ In cases where bilateral sequential cataract surgery with intracameral vancomycin was performed, the second eye was always involved and had a faster onset with more severe disease, suggesting a role for sensitization of the immune system with the first surgery.^1-3

Interestingly, recent papers have shown that some patients develop a milder presentation with a few scattered peripheral retinal hemorrhages, peripheral nonperfusion, and little to no vision loss.⁴ These cases may represent a forme fruste variant of HORV and may be underdetected or underreported due to their mild presentation.  Dilated fundus examination with careful peripheral retinal examination or wide-field imaging may be particularly useful in detecting these cases.

Due to the unpredictable and devastating effects of HORV, the ASRS and ASCRS joint task force³ have recommended that routine use of intracameral vancomycin in cataract surgery should be reconsidered.  In cases where vancomycin is used, a dilated retinal examination should be performed at least 3 weeks post-operatively, especially in cases where second eye surgery with use of vancomycin is planned.  Prophylaxis of endophthalmitis with intracameral moxifloxacin or cefuroxime have been shown to be effective without the risk of HORV.⁵

No curative treatment has been found for HORV.  High dose systemic, topical, periocular, and intraocular corticosteroids have been used with limited success to combat the inflammatory component of the disease.  Anti-vascular endothelial growth factor (VEGF) injections and panretinal photocoagulation can be effective in treating the severe ischemia caused by HORV, preventing retinal neovascularization or neovascular glaucoma.

References

1. Nicholson LB, Kim BT, Jardon J, et al. Severe bilateral ischemic retinal vasculitis following cataract surgery. Ophthal Surg Lasers and Imag Ret. 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; 124:583-595.
4. Goldberg RA, Crawford C, Heier JS. Postoperative hemorrhagic occlusive retinal vasculitis: a forme fruste variant? Ret Case Brief Rep. 2016; 0:1-2.
5. Haripriya A, Chang DF, Ravindran RD. Endophthalmitis reduction with intracameral moxifloxacin prophylaxis: analysis of 600,000 surgeries. Ophthalmol. 2017; 124:768-775.