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

Case presentation Anterior uveitis; fixed • pupil 3 rd nerve palsy; mild 6 th ; V1 anesthesia MRI brain: • • Orbital apex/CS enhancement • AIS- right centrum semiovale

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- Courtesy: Amol Kulkarni MD, University of Wisconsin- Madison colored retinal necrosis

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 on 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