Hemorrhagic Occlusive Retinal Vasculitis

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 by Jennifer I Lim MD on May 13, 2020.


Disease Entity

Hemorrhagic occlusive retinal vasculitis (HORV) has been described as a very rare but potentially devastating complication occurring after cataract surgery in which vancomycin is used. It has a delayed onset and is associated with retinal hemorrhages, vascular nonperfusion, and venous sheathing. The severity appears to be worse when the second eye has undergone cataract surgery in which vancomycin is used.

Disease

Visual acuity is typically severely decreased. In the original descriptions by Nicholson et al and Witkin et al[1] [2], presenting visual acuities were very poor. Patients typically presented 1 to 14 days after successfully cataract surgery. However, the patients can present from 1-21 postoperative day (mean 8 days).[3] Visual acuity outcomes were poor: 4 of 11 eyes were NLP and the others were less than 20/ 100. A larger series of 23 patients and 36 eyes[3] noted the following characteristic features of HORV-

  • Typically postoperative day 1 undilated examination is unremarkable
  • 'Delayed onset vision loss
  • mild anterior chamber and vitreous inflammation,
  • sectoral retinal hemorrhages in areas of ischemia, and
  • predilection for venules and peripheral involvement.'

Etiology

Presumed reaction to vancomycin

Risk Factors

Prior intracameral vancomycin during cataract surgery. In the study by Witkin and colleagues[3] 'All eyes received intraocular vancomycin via intracameral bolus (33/36), via intravitreal injection (1/36), or through the irrigation bottle (2/36).' Specifically, visual outcome was poor in patients who received additional intravitreal vancomycin for presumed endophthalmitis after surgery (5 of 7 such eyes lost perception of light, one each had 20/800 and 20/400).[3] The fellow eye has higher risk of HORV and more aggressive disease if intracameral vancomycin is used again as was done in the operated eye. Five of the 23 patients reported by Witkin et al had allergy to penicillin.[3] However, skin testing for hypersensitivity to vancomycin, lidocaine, and viscoelastic was negative in the patients in whom it was performed.[3]

General Pathology

Pathology in 1 patient:

  • Chronic nongranulomatous choroiditis (T cells)
  • Unusual glomeruloid proliferation of endothelial cells in the choroid and elsewhere in the eye
  • No leukocytoclastic vasculitis

Pathophysiology

Necrotizing retinal vasculopathy, presumed type IV hypersensivitiy reaction (Todorich et al).[4] T-cell mediated process on pathology with intravascular thrombosis.

Primary prevention

Avoid sequencial cataract surgery with intracameral vancomycin if the fellow eye had HORV

Diagnosis

Diagnostic criteria were created by the ASRS & ASCRS Task Force and published by Witkin et al.[5] The anterior segment exam is usually unremarkable on postoperative day 1. Patients complain typiclly of delayed-onset painless vision loss. At this stage, there is mild anterior chamber and vitreous inflammation, sectoral retinal hemorrhages in areas of ischemia, and a predilection for involvement of venules. A case of bilateral mild HORV has been reported, suggesting that the incidence may be higher than previously thought. [6]

The diagnostic criteria[3] given by Witkin and colleagues are as follows

"Characteristic findings

1. Occurs after intraocular procedure with normal undilated examination on postoperative day 1.

2. Delayed onset of sudden painless decreased vision (may be asymptomatic in mild cases).

3. Visual acuity often poor at presentation (may be normal in mild cases).

4. Mild to moderate anterior chamber and vitreous inflammation, with no hypopyon.

5. Sectoral intraretinal hemorrhage in areas of nonperfusion (often along venules).

6. Peripheral retinal involvement in all cases, with macular ischemia in advanced disease.

7. Sectoral retinal vasculitis and vascular occlusion on fluorescein angiography, corresponding to areas of hemorrhage.

Other common associations

1. Use of intraocular vancomycin during procedure.

2. Rapid progression to neovascular glaucoma.

3. History of similar reaction in fellow eye.

4. When both eyes involved, second eye often has faster onset and more severe course.

5. Minimal to no corneal edema.

6. Retinal hemorrhages are often large, confluent, or both.

7. Propensity for retinal venule involvement.

8. No significant increase in venous dilation or tortuosity.

9. Optical coherence tomography findings: hyperreflectivity and thickening of the inner retinal layers.

10. Therapy with additional intravitreal vancomycin associated with especially poor outcomes."

History

Prior cataract procedure with normal examination at post-operative day 1. This is followed by sudden, painless decreased vision. Mild inflammation is seen with no hypopyon. Peripheral sectoral hemorrhages with non-perfusion. There may be macular non-perfusion but always peripheral non-perfusion is present.

Physical examination

Mild inflammation is seen with no hypopyon.

Peripheral sectoral hemorrhages with non-perfusion.

There may be macular non-perfusion but always peripheral non-perfusion is present.

Symptoms

Decreased painless loss of vision, variable degree.

Diagnostic procedures

Fundus photo shows vasculitis, hemorrhage, and retinal whitening in some cases

Fundus fluorescein angiogram shows severe capillary nonperfusion

Optical coherence tomography may show inner retinal hyperreflectivity, increased thickness of inner retina, and cysoid macular edema (rare)

Ultrasonogram is needed in case of media haze due to vitreous hemorrhage

Differential diagnosis

  • Endophthalmitis- but HORV eye is quiet, has no pain and has only mild posterior uveitis at presentation.
  • Viral retinitis- but HORV has mild vitritis and no progression of retinitis
  • CRVO/central retinal venous occlusion- but HORV does not have dilated vessels.

Management and Treatment

  • Systemic and topical corticosteroids may help. Early anti-VEGF intravitreous injection and PRP to prevent neovascular glaucoma.
  • Avoid vancomycin for suspected endophthalmitis.
  • Avoid vancomycin use in second eye.

The recommendations for HORV (by Witkin and colleagues[3]) are

"Considerations for intraocular vancomycin use

1. Because HORV seems to be extremely rare, each surgeon should weigh the potential risk of HORV associated with vancomycin against the risk of endophthalmitis.

2. Reconsider using vancomycin with close sequential bilateral cataract surgery, especially if immediate sequential same-day bilateral surgery is performed.

3. Surgeons using intraocular vancomycin with sequential cataract surgery should be aware that in addition to delayed onset, HORV may not cause symptoms in the first eye, and a dilated retinal examination may be the only way to detect it.

4. Surgeons desiring an alternative to vancomycin for intracameral prophylaxis may consider cefuroxime or moxifloxacin.

Recommendations for management of HORV

1. Avoid intravitreal vancomycin if HORV is suspected.

2. Consider an ocular or systemic workup, or both, for other syndromes (e.g., viral retinitis) if the diagnosis is unclear.

3. Aggressive systemic and topical corticosteroids; consider periocular or intraocular steroids.

4. Early anti-VEGF treatment.

5. Early panretinal photocoagulation.

6. If you identify a patient with HORV, please submit the clinical data to the HORV registry: (links from https://www.surveymonkey.com/r/HORV). Patient and surgeon names will be kept confidential."

Prognosis

Poor visual acuity and rapid progression to neovascular glaucoma which can occur in 56% patients.[3]

ASRS and ASCRS HORV register

The new cases of HORV can be registered at https://www.surveymonkey.com/r/HORV

References

  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. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 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. doi:10.1016/j.ophtha.2016.11.042
  4. Todorich B. et al. Vancomycin-Associated Hemorrhagic Occlusive Retinal Vasculitis: A Clinical-Pathophysiological Analysis. Am J Ophthalmol 2018;188:131–140.
  5. Andre J. Witkin, MD, David F. Chang, MD, J. Michael Jumper, MD, Steve Charles, MD, FACS, Dean Eliott, MD, Richard S. Hoffman, MD, Nick Mamalis, MD, Kevin M. Miller, MD, Charles C. Wykoff, MD, PhD. Vancomycin-Associated Hemorrhagic Occlusive Retinal Vasculitis Ophthalmology  2017;124:583-595
  6. Arepalli S, Modi YS, Deasy R, Srivastava SK. Mild Bilateral Hemorrhagic Occlusive Retinal Vasculitis Following Intracameral Vancomycin Administration in Cataract Surgery. Ophthalmic Surg Lasers Imaging Retina. 2018 May1;49(5):369-373.