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Idiopathic Perifoveal Telangiectasia

Idiopathic Perifoveal Telangiectasia. Laura S Gilmore, MD Department of Ophthalmology TTUHSC March 12, 2004 Discussant: Kelly T Mitchell, MD. Case Presentation. CC: decreased VA

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Idiopathic Perifoveal Telangiectasia

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  1. Idiopathic Perifoveal Telangiectasia Laura S Gilmore, MD Department of Ophthalmology TTUHSC March 12, 2004 Discussant: Kelly T Mitchell, MD

  2. Case Presentation CC: decreased VA HPI: 56yo WF with sudden onset of decreased VA September, 2003, progressively worsening; denies past ocular disease or injury PMH/ROS: wheelchair-bound 3 months 2o disc disease, hearing loss since birth, fibromyalgia, peripheral neuropathy, poor circulation, arthritis, anemia, Raynaud’s Disease, ataxia, skin rashes, arythmia FH: glaucoma, diabetes Meds: Seroquel, Procardia, Diazide, Soma, B12, Levbid, Paxil, Lasix, Darvocet Allergies: “need to mail list”

  3. Physical Exam VA: 20/100 OU 2/5/04; 20/400 OU 2/24/04 IOP: 16 OU VS: 110/78, P 80 Pupils: 5 to 3 OU, 0 APD Motility: Full OU VF: mild, equal constriction OU AC: trace NSC OU; 4+DQ; quiet; corneas clear Fundus: Small hyperpigmented areas temporal to maculas OU

  4. OCT

  5. Visual Field

  6. Differential Diagnosis • Diabetic retinopathy • Venous occlusive disease • Coat’s disease • Idiopathic Perifoveal Telangiectasia • CME 2o uveitis • Lamellar macular hole • Eales’ disease • CSR

  7. Basics of IPT • Dilation and incompetence of retinal capillaries • Solely in perifoveal area • Bilateral • IPT = Group 2A of Gass Classification of perifoveal telangiectasia • Probably acquired • By definition, no associated retinal vascular diseases

  8. Distinguishing Features of IPT • Most important distinguishing feature is the limitation to perifoveal macular region • Small refractile golden crystalline deposits in 50% • Yellow foveal lesion in one or both eyes in 5% • No progression • Bilateral • Usually presents at 50-60 years • No sex predilection • No lipid (seen in CSR, choroidal neovascularization, DR, Coat’s disease

  9. Typical Presentation • Decreased VA to 20/30 or better • Area of telangiectasia <1DD, confined to temporal macula • Only minimal intraretinal serous exudation • No lipid exudation

  10. Gass Classification • Group 1A: unilateral, congenital parafoveolar telangiectasia • Group 1B: unilateral, idiopathic, focal PFT • Group 2A: bilateral, idiopathic, acquired PFT • Group 2B: juvenile occult familial PFT, but no right-angled venules, crystals, or pigmented plaques • Group 3A: occlusive idiopathic PFT • Group 3B: occlusive idiopathic JFT associated with CNS vasculopathy

  11. Staging • Stage 1: mild staining of outer retina of temporal macula on FA • Stage 2: some graying of involved area, mild telangiectasias • Stage 3: severely blunted, dilated, right-angle venules diving deep into outer retinal plexus • Stage 4: stellate RPE plaques along right-angle venules 2o to RPE hyperplastic response • Stage 5: choroidal neovascularization, peculiar retinochoroidal anastamoses unique to this disease

  12. Pathogenesis and Histopathology • Possible genetic component • See thickened capillary endothelial wall of affected vessels • Extracellular fluid • Nutritional deprivation of middle retinal cells causes degeneration of outer retinal cell layers and outer retinal atrophy • RPE hyperplasia and migration along right-angle venules

  13. Complications • Only occur in 5% of pts • Subretinal neovascularization • Subretinal hemorrhage • VA worse than 20/50 • Disciform scar formation

  14. Treatment and Course • Disease is usually self-limited and VA STABLE • Laser is only considered with persistent, advanced disease • Laser is often not effective, because visual loss is due to atrophy, not serous exudation • PDT • Grid laser

  15. In Our Patient • Why the severe vision loss? • + edema • No CNVM • No SRH • No serous RD • Any connection with positive ROS? • How to treat her specifically…

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