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

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 2 o 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”

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

OCT

Visual Field

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

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

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 years  No sex predilection  No lipid (seen in CSR, choroidal neovascularization, DR, Coat’s disease

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

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

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 2 o to RPE hyperplastic response Stage 5: choroidal neovascularization, peculiar retinochoroidal anastamoses unique to this disease

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

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

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

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…