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GRAND ROUNDS

GRAND ROUNDS. September 1, 2006 Denise A. John St. Thomas Hospital. Case. HPI : 60 y/o ♂ presents for an eye exam. ø ocular c/o’s ROS : (+) L temporal headache All other systems unremarkable FHX : Diabetes; HTN, stroke SHX : Quit ETOH ‘04; ø tobacco/IVDA Allergies : NKDA.

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GRAND ROUNDS

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  1. GRAND ROUNDS September 1, 2006 Denise A. John St. Thomas Hospital

  2. Case • HPI: 60 y/o ♂ presents for an eye exam. • ø ocular c/o’s • ROS: (+) L temporal headache • All other systems unremarkable • FHX: Diabetes; HTN, stroke • SHX: Quit ETOH ‘04; ø tobacco/IVDA • Allergies: NKDA

  3. Ocular Exam NLP • VA SC CF ‘4ft  20/200 • Motility: Full OU 4 • IOP 8 • Pupils: (+) RAPD OD

  4. NO VIEW B-Scan: Funnel-shaped retinal detachment

  5. Infectious Indolent CMV retinitis Toxoplasma retinitis Acute retinal necrosis (ARN) Progressive outer retinal necrosis (PORN) Choroidal pneumocystosis Cryptococcal choroiditis Tuberculosis Candidiasis Syphilis Neoplastic Ocular lymphoma Metastasis Inflammatory Sarcoidosis Vasculitides Differential Diagnosis

  6. PMHX: HIV/AIDS (Dx ’04) CD4: 155 cells/mm3 (5/05) HIV nephropathy Hepatitis C Chronic anemia HTN Chickenpox POHX: S/p steel injury OD HZO OS (5/05) Conjunctivitis Keratitis Uveitis Post-herpetic Neuralgia MEDS: Acyclovir 400mg QID; HAART; lisinopril; atenolol; bactrim; Refresh PM External Exam: Left hypo-pigmented scar: CNV1distribution LUL Entropion & trichiasis SLE: OD: Shallow AC; 360° posterior synechiae; white cataract OS: PEE; posterior synechiae; ø AC rxn; 2-3+ NSC/3-4+ PSC; ø vitritis More Info…

  7. Epidemiology: HIV • 40 million individuals infected worldwide • ~ 900,000 in the U.S. • 70-80% treated for a HIV-related eye disorder • CD4 count may be used to predict the occurrence of specific ocular infections

  8. < 500 cells/mm3 Kaposi sarcoma Tuberculosis Lymphoma < 250 cells/mm3 Pneumocystosis Toxoplasmosis < 100 cells/mm3 Conjunctival/retinal microvasculopathy CMV retinitis Most common VZV retinitis 2nd most common Cryptococcosis Microsporidiosis CD4 Count & Ocular Infections

  9. CMV Retinitis • Occurs in immunocompromised • Slow progression • Starts in periphery • Spreads along retinal vasculature towards posterior pole • Dense white/granular opacification (full-retinal thickness) • Hemorrhage • Mild vitritis

  10. Acute Retinal Necrosis • Occurs in immunocompetent • > 1 foci of full-thickness retinal necrosis with discrete borders • Spreads 360° circumferentially in peripheral retina • Posterior pole involvement is spared until late • Vasculitis • Prominent inflammatory reaction (AC & vitreous)

  11. Progressive Outer Retinal Necrosis

  12. PORN • Rare form of necrotizing herpetic retinopathy • First described by Forster et al. (1990) • 2 pts: Fulminant outer retinal necrosis sparing the inner retina & vasculature • Occurs in the immunocompromised: • Immunosuppressed organ-transplant recipients • Immune-deficient individuals: • Cancer • Advanced AIDS

  13. Pathogenesis • Varicella-Zoster Virus • Virus remains latent in sensory ganglia • Reactivated during times of loss of T-cell regulatory control • Difficult to isolate/grow in-vitro • Only organism isolated in the retina via culture, PCR & direct fluorescent antibody assay

  14. PORN • Engstrom, et al. PORN: A variant of necrotizing herpetic retinopathy in patients with AIDS. Ophth 1994. 38 ♂ pts (65 eyes): CD4 count: 21 cells/mm3 (0-130 cells/mm3) • Largest study on PORN • Retrospective chart review • Median f/u 3 months (0-10 months) • Objective: Characterize the clinical features & course

  15. PORN • History of cutaneous zoster : 67% (22/33 pts) • 41%: involved CNV1 • 12 of 15 pts: PORN occurred after a median of 2 months (2 months – 2 years) • 3 of 15 pts: PORN occurred concurrently • 12 of 38 pts: Taking oral acyclovir at the time PORN was diagnosed • 50%: Acyclovir 800mg 5x/day

  16. Clinical Features • Ocular complaints •  vision: Most common (54% eyes) • Constriction of visual fields (28% eyes) • Floaters (11% eyes) • Pain (6% eyes) • 7 pts with unilateral symptoms had asymptomatic disease in the fellow eye

  17. Clinical Features • Usually bilateral disease • 28 of 38 pts: Unilateral disease at diagnosis • 2nd eye became affected in 17 pts  median of 10 days after diagnosis (3 days – 4 weeks) in 6 pts • Intraocular inflammation is minimal to absent • 23 of 60 eyes: Anterior segment inflammation • 61% mild AC reaction • 11% keratic precipitates (fine, white deposits) • 6% posterior synechiae • 15 of 61 eyes: Vitreous inflammation • 80% mild vitritis

  18. Clinical Features • Multifocal, discrete lesions of the outer retina  rapidly progress to confluence & full-thickness retinal involvement • Perivenular lucency

  19. Clinical Features • Characteristic macular lesion: • Parafoveal opacification with a “cherry-red spot” • Ø contiguous with peripheral lesions • Peripheral lesions + posterior pole • Zone 1: 32% eyes • ø lesions only in zone 1 • Zone 2: 72% eyes • Zone 3: 86% eyes • 28% eyes all 3 zones

  20. Clinical Features • Disease quiescence • Dense white plaques: “cracked mud” appearance • Atrophic areas + holes

  21. Clinical Features • Other manifestations: • 11 of 65 eyes: Optic nerve abnormalities • Disc swelling • Hyperemia • Atrophy • 11 of 29 pts: Afferent pupillary defect • 13 of 61 eyes: Retinal vasculopathy • Vascular sheathing/occlusion • Areas within or near retinal necrosis

  22. FA: PORN • Walton et al. FA in PORN. Retina 16: 1996 • Early: Microvascular changes  equatorial & peripheral retina • Confluent retinal disease:  retinal vasculature & loss of capillaries; RPE damage; choriocapillaris leakage • Reactivation: Brush-fire pattern of choroidal leakage at lesion border • PORN = Retinochoroiditis

  23. Management: PORN • Exact combination of antivirals & duration of treatment not known • Guided by anecdotal information • Herpes-zoster traditionally treated with acyclovir; however, may not be effective in pts treated long-term with the oral form 2° to resistance

  24. Management • Moorthy et al. Management of VZVR in AIDS. Br J Ophth, 1997. 20 pts (39 eyes); 11 pts using oral acyclovir at time of diagnosis • Retrospective chart review • median f/u 6 months (1-26 months) • Objective: Investigate visual outcome • 2 week IV treatment: • Acyclovir (10mg/kg Q8h) • Ganciclovir (5mg/kg Q12h x 2 weeks; then 5mg/kg/day) • Foscarnet (180mg/kg/day in 2 or 3 divided doses) • Ganciclovir + foscarnet

  25. Management • Results: • Rates of NLP : acyclovir (9 of 10 eyes) & foscarnet (3 of 5 eyes) • Ø VA > 20/200 • Rates of NLP : combination therapy (5 of 18 eyes) & ganciclovir (2 of 6 eyes) • VA > 20/200 • Combination therapy: 3 of 18 eyes • Ganciclovir: 1 of 6 eyes • Conclusion: Treatment with IV combination therapy or ganciclovir associated with a better final vA VS acyclovir or foscarnet alone

  26. Management • Ciulla, et al. PORN: Successful treatment with combination antiviral therapy. Ophth Surgery & Lasers. 1998. 6 pts with AIDS • Retrospective chart review • Median f/u 29 weeks (27 -38 weeks) • Objective: Assess 2-drug combination therapy • IV Treatment duration: median 29 weeks (27-38 weeks) • Ganciclovir (5mg/kg Q12h) + acyclovir (500mg/m2 Q8h) • Foscarnet (60mg/kg Q8-12h) + ganciclovir • Foscarnet + acyclovir

  27. Management • Results: • All 6 pts had resolution of disease • 1 of 6 pts had recurrence • At diagnosis 3 of 12 eyes without disease  remained uninvolved • 10 of 12 eyes developed RD • Conclusion: Prolonged combination therapy arrested progression of retinitis; maintained remission & prevented fellow eye involvement; does not prevent retinal detachment • Role of ganciclovir intraocular implant & oral agents in combination therapy is unclear

  28. Management • Intravitreal injection (ganciclovir & foscarnet); intravitreal ganciclovir implant • No protection for fellow eye

  29. Complications • Retinal necrosis • Retinal tears/holes • Rhegmatogenous retinal detachment

  30. Management • Prophylactic laser retinopexy • Engstrom et al. 1994 • 14 of 54 eyes: Laser ~ 1 week after diagnosis • 93% developed a RD • Median interval ~ 3 weeks • Ø significant difference: laser VS no laser: • Zone of involvement of RD • Extent of RD • Interval from diagnosis to RD

  31. Management • Rhegmatogenous retinal detachment • Engstrom et al. 1994 • 43 of 65 eyes: Median interval 4 weeks • No relationship to extent of disease or disease activity • Vitrectomy/endolaser/silicone oil • 16 of 43 eyes: Retinas successfully attached in all eyes • Re-detached in 4 eyes • NLP in 56% (laser) VS 63% (no laser)

  32. Prognosis • Visual prognosis is poor • Macular involvement • Ineffectiveness of antiviral agents • Recurrence • Engstrom, et al. 1994 • At least 10 pts despite being on maintenance therapy • Characterized by development of new disease foci • In 6 pts associated with discontinuation/reduction in maintenance dose; median time to recurrence was 2 weeks (1-6 weeks) • ~ 50% of individuals  deceased 5 months after diagnosis

  33. Back To Our Patient… • Patient admitted to medicine • Infectious disease consulted • Received IV foscarnet (40mg/kg Q12 x 14 days) • ø Progression of lesions • VA improved to 20/60 (+1.75) • Discharged on oral acyclovir 800mg 5x day

  34. Take Home Points… • Progressive outer retinal necrosis is a rapidly progressive necrotizing retinitis occurring in immunocompromised individuals, esp. AIDS pts • Management is anecdotal: use of high-dose IV anti-virals may be beneficial • Poor visual prognosis

  35. References • E-Medicine: Ocular manifestations of HIV • Forster et al. Rapidly PORN in AIDS. Am J Ophth 110: 341. 1990 • Moorthy et al. Management of VZV retinitis in AIDS. Br J Ophth, 1997. • Walton et al. Fluorescein angiography in PORN. Retina 16: 1996 • Ciulla, et al. The PORN: Successfully treatment with combination antiviral therapy. Ophth Surgery & Lasers. 1998 • BCSC. Retina & Vitreous. AAO. 2004-05 • BCSC. Uveitis & Intraocular Inflammation. AAO. 2004-05 • Yanoff. Ophthalmology, 2nd Ed. Mosby. 1121-22 • Kanski. Clinical Ophthalmology, 5th Ed. Butterworth Heinemann. 288-93. 2003

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