Benvenuto! Retina Grand Rounds Carlo Pelino, OD [email protected] Joe Pizzimenti, OD [email protected] Willkommen! Salute Our Financial Disclosure ! Honoraria " Alcon " Notal Vision Sponsors! " Reichert ! Scientific Advisory Boards " Zeavision " Carl Zeiss Meditec ! Proprietary Interests " None ! CEO/Founder " Optometryboardcertified.com Course Goals ! To apply principles of: The eye is the only part of the " Functional anatomy " Examination body where neurological and " Imaging and other diagnostic workup vascular tissues can be viewed " Management/Co-mgt ! Clinical cases directly. ! Evidence based treatment ! Interactive 1 Case History • A 32 year-old black female presented with gradual, bilateral reduced vision, photophobia, and poor color vision. • Ocular History was positive for previous episodes of pars Hereditary Dystrophic Dx planitis and anterior uveitis OU. • Health history was positive for mitral valve prolapse of 10 years duration and occasional migraines. • The patient was taking no medications at the time of the visit. • Her moderate myopia was corrected with soft daily wear disposable contact lenses. Case Report • Best-corrected acuities were 20/25 OD, 20/30 OS. • Amsler revealed a central ring-shaped defect OD and inferotemporal distortion OS. • The patient was unable to identify shapes and numbers on Ishihara color testing. • Dilated fundoscopy showed a bilateral, irregular, atrophic, “bull’s eye” maculopathy. • Automated threshold perimetry revealed paracentral defects OD and OS. Imaging CD with OCT ! SD-OCT revealed disruption of the photoreceptor inner-segment/outer-segment junction, external limiting membrane. 2 OD OS OD 10/2005 OD 4/2006: Note increased flattening and more “blue” Mf-ERG revealed a “ring-depression” surrounding a small central peak in each eye. Cone Dystrophy ! Probably part of the retinitis pigmentosa spectrum in which cones are affected more than rods At right, another " Cone-Rod Dystrophy patient with full ! Inheritance presentation of cone " A/R - earlier onset A/D - generally later; commonest, first 2 decades dystrophy (20/40 VA) X/L - Rare; tapetal sheen, Mizuo-Nakamura FX Cone Dystrophy Imaging CD with FAF ! Presentation ! Fundus (FAF) " Childhood (4-8) or young adulthood (teens-30) autofluorescence may demonstrate increased ! Symptoms autofluorescence ring " Photophobia and central spots in ! Signs the foveola. " Bull’s eye macula common " Diffuse macular pigment changes 3 Cone Dystrophy Cone Dystrophy ! Signs ! No treatment " Acquired nystagmus ! Some animal studies have shown that antioxidant vitamins can slow further vision loss. " Defective color vision ! Some mice with cone dystrophy have been ! ERG - abnormal photopic response effectively treated with gene therapy, but it is EOG – subnormal unknown if the approach will work in humans. ! Low Vision Rehabiliattion ! Variable VA ! Genetic counseling Outcome and Conclusions ! Patient is currently being followed with periodic mfERG, central threshold perimetry, contrast sensitivity, OCT, color vision, and dilated QUESTIONS AND fundoscopy. COMMENTS? ! She continues to enjoy successful contact lens wear. ! This case underscores the importance of electrodiagnostic testing when photoreceptor disease is suspected. ! MfERG is of significant value in the detection of CD in patients that develop symptoms later in life. Coup/contrecoup Injury Blunt Trauma Compression, Decompression, Overshoot, Oscillation 4 L floor fracture w/blood in maxillary sinus. Medial wall of L orbit also fractured. Traumatic Choroidal Rupture 5 Surgical Video-IOFB Sx Courtesy: Steven Sinclair, MD Neoplastic Disease QUESTIONS AND COMMENTS? 6 To Find Small Ocular Melanoma To Find Small Ocular Melanoma T= thickness No risk factors (<4%) F= subretinal fluid 1 risk factor (36%) S= symptoms 3 risk factors (50%) O= orange pigment 5 risk factors (70%) M= margin touches disc DOCUMENTED GROWTH - MEANS EVERYTHING Using Helpful Hints = Ultrasound hollow, halo NEVOMA FAF in Choroidal Melanoma Echography Acoustic hollowness on B-scan of small melanoma. Echography of large melanoma Choroidal Metastasis 7 Choroidal Metastasis Capillary Hemangioblastoma Cavernous Hemangioma Astocytic Hamartoma Retinoblastoma Choroidal Osteoma 8 Retinoblastoma Leukemia Infection QUESTIONS AND and COMMENTS? Inflammation Cytomegalovirus Retinitis (CMV) Cytomegalovirus Retinitis HIV encephalopathy, Toxoplasmosis 9 Acute Retinal Necrosis Toxoplasmosis Initial Presentation Toxoplasmosis Toxoplasmosis Day 13 Day 3 Day 6 Ocular Histoplasmosis Syndrome (OHS) Toxoplasmosis • From exposure to fungus Histoplasma capsulatum via respiratory tract • Endemic to Ohio/Mississippi River Valley where more than 60% test positive 10 Case Hx: A 16 year old Caucasian male presented with a chief complaint of blurred vision and floaters in both eyes for a one month duration. Floaters began immediately following a viral prodrome with mild headache. He then noticed progressive blur OS > OD. Visual field defects were also noted in both eyes. No other ocular complaints. Medical history: negative Medications: none Allergies: None to environment/medications Inflammatory “White Dot Syndrome” - APMPPE • Occurs in healthy men or women between 20 – 50 years old • Acute bilateral vision loss • Prodromal viral illness in about 30 % of patients Symptoms: • Central or paracentral scotomas/hotopsia/floaters • Reduced visual acuity Signs: • Mild vitreous cells are usually present • Yellow/white placoid lesions located in the posterior pole/midperiphery • Located at the level of the retinal pigment epithelium/choriocapillaris • Lesions start to fade within 2 weeks • Lesions are replaced with atrophy and hyperpigmentation 11 White Dot Syndromes • Multifocal Choroiditis/Panuveitis (MCP) The White Dot Syndromes are • Acute retinal pigment epitheliitis (ARPE) characterized by bilateral choroidal • APMPPE (Acute posterior multifocal pigment placoid epitheliopathy) infiltrates and retinal vasculitis. • Vitiliginous choroiditis (Birdshot) • MEWDS (Multiple Evanescent White Dot Syndrome) • DUSN (Diffuse Unilateral Subacute Neuroretinitis) • Punctate Inner Choroidopathy (PIC) • Sarcoid Choroidopathy • Intraocular lymphoma (Retinal/Vitreal or Uveal ) Birdshot Retinochoroidopathy White Dot Syndromes Non-infectious Choroidopathies RPED, ERM in Birdshot Cellular debris in vitreous 12 APMPPE Birdshot in another patient: FA Pathology: • Immune response from a virus ? • Infarction of the choroid secondary to a vasculitis • Described with Mumps, Strep infections, TB, Hep B vaccine, Lyme, Sarcoidosis, Thyroiditis Treatment: • No systemic or ocular treatment needed • VA 20/40 or better within 8 weeks in 80% of untreated patients • Systemic steroids if central nervous system or foveal involvement Systemic Evaluation: • Venous stage hyperfluorescence • No systemic evaluation needed unless CNS involvement • Extensive late intraretinal and disc leakage • Headaches or neuro signs – get MRI • Associations with adenovirus type 5, cerebral vasculitis – must undergo a systemic and neurologic evaluation Importance of Dilated Post Birdshot Retinochoroidopathy Seg Exam in All Uveitis Cases • Vitreous Exam ! Treatment: " Immunosuppressive therapy reduces the risk of • Technique vision loss from CME. (Cyclosporine) • Anterior vitreous • Posterior vitreous " Chronic low dose oral corticosteroids (10mg or less), does not seem to prevent the occurrence of CME. • Inflammatory cells and protein " Immunosuppressive therapy may reduce the risk of progressive diffuse retinal dysfunction. • Comes from the choroid, retina and ciliary body JE Thorne, DA Jabs, et al. AJO 2005;140:45-51. • “Vitritis”?? Immune Privilege • The eye enjoys a special relationship We do encounter situations in with the immune system. – Ability to quench unwanted immune-mediated clinical practice when the eye’s inflammation. immune privilege is overcome. – This ability is known as immune privilege. Uveitis is an example. – Immune privilege enables ocular tissues to remain clear. 13 The Optic Nerve in Posterior Uveitis • Disc hyperemia • Papillitis • Disc edema may occur with toxoplasmosis, viral retinitis, lymphoma, or sarcoidosis. Sarcoid Sarcoid OCT • Cystoid Macular Edema in a Sarcoid patient with chronic uveitis. Common Etiologies of Posterior Uveitis Toxocariasis • Toxoplasmosis (photo below) • HSV, HZV • Granulomatous disease (e.g. tuberculosis, sarcoidosis, Lyme disease, syphilis) • Histoplasmosis • Birdshot or Serpiginous • Inflammatory choroidopathies 14 Common Etiologies of Multifocal Choroiditis and Panuveitis Panuveitis • Bilateral, chronic uveitis – Punched-out chorioretinal lesions • Infections, such as similar to POHS. – Infantile toxocariasis • CNV is the most worrisome – Post-op bacterial endophthalmitis potential complication. – Toxoplasmosis • Conservative monitoring • Granulomatous Dx. and timely steroid • Multifocal treatment essential to care. Choroiditis and • Immunosuppressive drug Panuveitis (right) therapy appears to limit the – MCP in a 27 y/o Asian Female number of recurrences. Beyond Anterior Uveitis Slide 58 Iluvien 8 Emerging Treatments for Posterior Uveitis • Iluvien (formerly known as • Dexamethasone Drug Delivery System Medidure) – OZURDEX (intravitreal implant) – Tube 3.5mm x 0.37mm containing 0.7mg (Allergan) fluocinolone – Implanted into vitreous w/ 25g – Intraocular, biodegradable implant for the (~0.5mm) inserter treatment of persistent ME • Sutureless – Clinical trial validated Ozurdex – Designed to provide sustained effect for ME in CRVO up to 24 months – FAME