Deep Trouble. Thomas Stone, MD Retina Associates of Kentucky River City Retina Conference May 15, 2014

Transcription

1 Deep Trouble Thomas Stone, MD Retina Associates of Kentucky River City Retina Conference May 15, 2014

2 History 20 yo WM Decreased vision OU, OD>OS Sudden onset blurred central vision 12 days prior 4 days after onset V A recorded as 20/15 OU 2 months prior had febrile viral illness PMH: Appendectomy, Left meniscus repair No medications, NKDA FH: 2 healthy siblings

3 Exam V A (distance, sc) OD 20/125, OS 20/70 1+ anterior chamber cell both eyes Trace vitreous cell right eye, quiet vitreous left eye Imaging

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12 Block early, stain late

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14 Block early, stain late

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16 H V

17 H V

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19 Diagnosis Multiple deep white lesions centrally located Flu like prodrome Angiogram block early, fade late OCT showing destruction of ellipsoid zone Acute Multifocal Placoid Posterior Epitheliopathy (AMPPE)

20 Treatment Started on Prednisone 60 mg QD

21 Workup MRI (Brain & Orbits, with/without), MRV wnl Lab Tests CBC wnl CMP wnl Lyme negative HIV negative B12, Folate wnl RPR negative ANA negative

22 One Week Later VA (sc, distance) OD 20/100, OS 20/40 Anterior chamber quiet OU Vitreous quiet OU Increased pigmentation of lesions in the macula Resolution of peripheral choroidal filling delays apparent on initial FA, no new lesions FAF increased hyperautofluorescence OCT disruption of ellipsoid zone in placoid lesions Seen at VRMNY

23 Presentation 20/125 20/100 Image courtesy KBF at VRMNY

24 Presentation 20/70 20/40 Image courtesy KBF at VRMNY

25 Treatment Continue Prednisone Eventual slow taper

26 Two weeks later Presentation 20/125 One week later 20/100 20/40

27 Presentation Two weeks later 20/70 One week later 20/40 20/25

28 Acute Multifocal Placoid Pigment M/F in 20s 50s Epitheliopathy Rapid visual loss, central or paracentral scotomas, photopsias Usually bilateral, second eye can be delayed by several weeks 1/3 with flu-like prodrome, may have HLA-B7 or DR2 Vitritis/disc edema/vasculitis often present during acute phase Lesions usually less than 1DD, posterior pole, level of RPE/choriocapillaris Within days of onset RPE atrophy, hyperpigmentation of active lesions New lesions may develop Visual prognosis varies - near normal to severe loss, persistent scotomas, 25% worse than 20/50 (Fiore T, Retina, 2009) OCT may have retinoschisis, intraretinal cysts, disruption of ellipsoid zone Angiogram shows early hypofluorescence, late stain and leak

29 AMPPE Issues Pathogenesis Associated Findings CNS Involvement Treatment with Steroids

30 Pathogenesis RPE Disease? Pathology - 1 patient who died of cerebral vasculitis Choroidal granuloma under RPE, focal RPE disruption Choriocapillaris spared OCT suggestive of RPE process, or is it secondary? FA early blockage due to RPE opacification? Choriocapillaris Disease? Segmental choroidal vasculitis seen in the periphery ICG hypofluorescence delineates visual field changes Sensitive follow-up parameter for response to treatment Type IV Hypersensitivity suspected

31 Associated Findings Can have serous detachments, transient Harada s overlap? Variation in findings may be associated with timing and extent of inflammation/disease

32 CNS Involvement CNS Vasculitis can occur Headache (50%) Hearing symptoms reported (e.g. tinnitus) Paresthesias, vertigo, psychosis, stroke Stroke can present late after steroid taper

33 Treatment 1997 study Roberts and Mitchell 9/15 patients treated with prednisone with little clinical effect 16/28 eyes 20/20 or better at most recent follow up Some centers treat with Prednisone if central vision involved, or if systemic symptoms/findings are present

34 Thank You!

35 From VRMNY Additional Images

36 Early FA/ICG Image courtesy KBF

37 Early FA/ICG Image courtesy KBF

38 Dilated choroidal vessels Image courtesy KBF

39 ICG 20 min Image courtesy KBF

40 ICG and OCT Image courtesy KBF

41 ICG and OCT Image courtesy KBF

42 Microperimetry Image courtesy KBF

43 EDI Image courtesy KBF

44 EDI Image courtesy KBF