AFFECTS THE RETINA, OPTIC NERVE, AND MAKES THE PATIENT SEE DOUBLE? - - PowerPoint PPT Presentation

WHAT TO DO WHEN VZV AFFECTS THE RETINA, OPTIC NERVE, AND MAKES THE PATIENT SEE DOUBLE?

Sachin Kedar MD Department of Neurological Sciences and Stanley M Truhlsen Eye Institute

Disclosures: No conflicts of interest

Learning objectives

• List neuro-ophthalmic presentations

secondary to varicella zoster infection

• Discuss pertinent diagnostic studies for

confirming ocular zoster infections.

• Describe anti-viral agents commonly used

in ophthalmic varicella zoster.

• Manage the neuro-ophthalmological

manifestations of varicella-zoster infection

Case presentation

• 76y developed a “fixed and

swollen” right eye 1 week after discharge from IM.

• 3wk previously- he had V1- HZ

and AMS- secondary to hypoNa (116). No ocular involvement

• HypoNa corrected per protocol;

IV ACV for 3 days followed by 14 days oral ACV

• Anterior uveitis; fixed

pupil 3rd nerve palsy; mild 6th; V1 anesthesia

• MRI brain:
• Orbital apex/CS

enhancement

• AIS- right centrum

semiovale

Case presentation

What do we do next?

• Diagnostic considerations
• Therapeutic options
• Long term treatment
• Prophylaxis

Neuro-ophthalmic spectrum of HZ

• 1. Dermatologic involvement- ptosis from rash

and edema

• 2. Ocular inflammation of all layers-

conjunctiva, cornea, sclera, uvea, retina

• 3. Orbital inflammation- soft tissue, muscles
• 4. Neurological- optic nerve, cranial nerves,

meningo-encephalitis, CNS vasculitis

Epidemiology of HZO

• VZV reactivation in V1; 10-20% of all HZ
• ~200,000 new cases annually
• Frontal branch (supratrochlear/supraorbital)

commonly involved

• Nasociliary branch involvement (Hutchinson

sign) increases risk of inflammatory disease by 3.35 times (Zaal 2003)

• 5 year recurrence rate ~25% (Tran 2016)

Retinal disease

• Retinal vasculitis and

necrosis

• Occlusive vasculopathy-

arteritis and phlebitis of retinal and choroidal vasculature

• Patchy or confluent

areas of white or cream- colored retinal necrosis

Courtesy: Amol Kulkarni MD, University of Wisconsin- Madison

Retinal disease

• Common clinical forms:
• ARN: immuno-competent
• PORN: immuno-deficient
• ARN is a CLINICAL diagnosis and medical

emergency

Diagnosis

American Uveitis Society criteria

• ≥ 1 foci of retinal necrosis with discrete borders in the

peripheral retina

• rapid circumferential progression of necrosis in the

absence of antiviral therapy

• Occlusive vasculitis with arteriolar involvement
• Prominent AC and vitreous inflammation

Microbial confirmation not needed prior to treatment

Microbial confirmation

• PCR to detect DNA has >95% sensitivity and

specificity

• May be combined with serology on paired

serum/intra-ocular fluid to increase sensitivity and specificity.

• No difference between aqueous or vitreous

samples

Treatment

• Standard treatment
• Induction: IV ACV 10 mg/kg every 8 h x 7-10d
• Maintenance: oral ACV 800 mg 5/d x 6w
• Alternate: ARN w/o neurological involvement
• Induction: PO Valacyclovir 8000 mg/d x 7-10d
• Maintenance: PO valacyclovir 1000 dailyX 6m
• Adjunct IVT foscarnet may improve outcome

Outcomes

• Complications: VA worse than 20/200 in ~50%
• Rhegmatogenous RD
• Chronic vitritis,
• ERM and maculopathy
• Neovascularization and vitreous

hemorrhage

• Untreated, bilateral ARN in 70% (Palay 1991)

Diplopia in VZV

• Relatively uncommon or underdiagnosed
• Orbital complications leading to restrictive

strabismus

• Cranial neuropathy leading to paralytic forms.
• Neurological complications- INO, Skew

deviation

Orbital complications

• Acute orbital signs: proptosis, ptosis,

chemosis, ophthalmoplegia and visual loss

• Radiological evidence of orbital inflammation
• n MRI orbit
• Prompt treatment with systemic antiviral

medications

Cranial neuropathy

• Ocular motor cranial neuropathy occurs in 7-

31% HZO

• Multiple mechanisms postulated- contiguous

spread from CS; occlusive vasculitis

• Recommend MRI brain and orbit for

evaluation

• Treatment with systemic antiviral ± steroids
• Prognosis for recovery excellent with

complete recovery >50%

Optic neuropathy

• Extremely uncommon (<0.5% of HZO)
• May be seen in acute, subacute or late stages of

HZO; may accompany anterior/posterior HZO including ARN

• Variable presentation
• Diagnosis established by close temporal

association of optic neuropathy with HZO

• Should be evaluated and treated as neurological

HZO

• Use of steroids is controversial

HZ and GCA

• Several reports of HZ leading to ischemic optic

neuropathy

• Several reports of HZ association with GCA

based on TABx

• It is unclear if HZ causes GCA
• Unclear if all GCA should be evaluated for VZV

serology

• Unclear if GCA treatment should include

antiviral; perhaps case by case basis

Prevention

• Role of vaccination (will fill this section

depending on Dr. Berger’s slide)

Summary

• 20% of HZ can present with HZO
• HZO has myriad presentations of relevance to

neuro-ophthalmologists

• ARN and optic neuropathy is a medical

emergency and should be promptly treated with systemic antivirals.

• PCR ± serology on paired sera and

intraocular/CSF fluid is highly sensitive and specific

• VZV vaccination is recommended to reduce

incidence of HZO