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Idiopathic Perifoveal Telangiectasia Laura S Gilmore, MD

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

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Title: Idiopathic Perifoveal Telangiectasia Laura S Gilmore, MD


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, Raynauds 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 4DQ quiet corneas clear
  • Fundus Small hyperpigmented areas temporal to
    maculas OU

4
OCT
5
Visual Field
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9
Differential Diagnosis
  • Diabetic retinopathy
  • Venous occlusive disease
  • Coats disease
  • Idiopathic Perifoveal Telangiectasia
  • CME 2o uveitis
  • Lamellar macular hole
  • Eales disease
  • CSR

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11
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

12
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, Coats disease

13
Typical Presentation
  • Decreased VA to 20/30 or better
  • Area of telangiectasia lt1DD, confined to temporal
    macula
  • Only minimal intraretinal serous exudation
  • No lipid exudation

14
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

15
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

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18
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

19
Complications
  • Only occur in 5 of pts
  • Subretinal neovascularization
  • Subretinal hemorrhage
  • VA worse than 20/50
  • Disciform scar formation

20
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





 
21
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

22
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