Idiopathic Perifoveal Telangiectasia

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Idiopathic Perifoveal Telangiectasia

• Laura S Gilmore, MD
• Department of Ophthalmology
• TTUHSC
• March 12, 2004
• Discussant Kelly T Mitchell, MD

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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

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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

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OCT
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Visual Field
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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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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

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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

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Typical Presentation

• Decreased VA to 20/30 or better
• Area of telangiectasia macula
• Only minimal intraretinal serous exudation
• No lipid exudation

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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

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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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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

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Complications

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

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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

 
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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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