A 27 year-old African American woman with high myopia presented to Illinois Retina Associates complaining of decreased vision in her right eye over the past four (4) months with more recent decreased vision over the past week in her left eye. Four months earlier, she was seen by an outside ophthalmologist who diagnosed choroidal neovascularization (CNV) in her right eye. A sub-tenons injection of Kenalog was administered. She noticed mild improvement after the one treatment. Of note, she has a family history of migraines which she takes Imitrex, Topomax and Excedrin pm. She has a family history of rheumatoid arthritis. She is a current smoker but denies other toxic habits.
Visual acuity with was count fingers at 2 feet in her right eye and count fingers at 1 foot in her left eye. The prescription that she had been wearing was -8.75 in her right eye and -10.75 in her left. No APD was noted. Her extraocular movements were within normal limits and her visual fields were grossly full. The anterior examination was within normal limits. The posterior segment exam was abnormal in both eyes with macular scarring with scattered whitish-yellowish lesions (Figures 1 & 2). Fluorescein angiography revealed extensive staining and window defects with an area of active CNV in the left eye (Figures 3 & 4)
White Dot Syndromes:
- Punctate Inner Choroidopathy (PIC)
- Multifocal Choroiditis & Panuveitis (MFC)
- Ocular Histoplasmosis Syndrome (OHS)
- Acute Idiopathic Blind Spot Elargement
Clinical Course and Follow-Up
The patient was treated with multiple Avastin injections for CNV in her left eye. The decision was made to avoid treatment in her right eye due to the extensive macular scarring. The patient’s vision improved markedly to 20/50 in the left eye by the second injection, which was done one month after the first. The patient returned in one month and fluorescein angiography was repeated which revealed quiescent disease state. No further injections were given. Her manifest refraction remained at 20/50 in her left eye. Her right eye was still count fingers secondary to a large central macular scar.
Puntate inner choroidopathy is a white dot syndrome which tends to affect young patients with myopia. More than 90% are women. It often manifests with bilateral loss of visual acuity, with prominent photopsia and scotomata. During acute phase, there are 100-300um round yellow-white lesions of RPE or inner choroid that may coalesce and form a serious RD. Mild optic disc edema has been reported. There is characteristically no vitritis or iritis. Lesions fill and stain during late phase of FA, especially if a serious RD is present. Lesions later become atrophic yellow-white scars, which may become pigmented or enlarge over time, which may appear similar to histoplasmosis or multifocal choroiditis. Spontaneous improvement can occur without treatment. Prognosis for vision tends to be good and recurrence is uncommon. Photopsias may persist for years.
One third of eyes develop CNV within a site of old scarring, some of which involute spontaneously. Treatment options for CNV include anti-VEGF injections, systemic corticosteroids, immunosuppresive agents, photodynamic therapy.
Chan, W., et al, “intravitreal Bevacizumab (Avastin) for Choroidal Neovascularization Secondary to Central Serous Chorioretinopathy, Secondary to Punctate Inner Choroidopathy, or of Idiopathic Origin” American Journal of Ophthalmology. 2007, June 143(6): 977-983
Cirino, A. et al, “Resolution of Activity (Choroiditis and choroidal Neovascularization) or Chronic Recurrent Punctate Inner Choroidopahty After treatment with Interferon b-1A” Retina. 2006, Nov-Dec; 26(9): 1091-2
Flaxel, C, et al., “The Use of Corticosteroids for Choroidal Neovascularisation in Young Patients” Eye. 1998, 12: 266-272
Fong, KCS, et al. “Photodynamic Therapy Combined with Systemic Corticosteroids for Choroidal Neovascularization Secondary to Punctate Inner Choroidopathy” Eye. 2007
Gerstenblith, A, et al, “Punctate Inner Choriopathy: A Survey Analysis of 77 Persons” Ophthalmology. 2007: advance online publication
Postelmans, L., et al. “Photodynamic Therapy for Subfoveal Classic Choroidal Neovascularization Related to Punctate Inner Choroidopathy or Presumed Ocular Histoplasmosis-Like Syndrome” Ocular Immunology and Inflammation. 2005, 13: 61-366
Watzke, R., et al. “Punctate Inner Choroidopathy” American Journal of Ophthalmology. 1984, Nov 98(5): 572-84