Retinal Review, Issue 41
CASE NUMBER 41
A 23 year old woman was referred for reduced vision in her right eye. She had been told of scars in her eye in the past but had relatively intact vision. She noted the onset of a central scotoma in the right eye 6 months before presentation which persisted. Past medical history is unremarkable. Past ocular history is also significant for moderate myopia. She was born in New Jersey and had family in Puerto Rico. On examination, VA was OD: 20/100 and OS: 20/20. IOP’s and the slit lamp examination were normal. Dilated examination of the left eye was normal. Please see the color photos and fluorescein angiograms of both eyes below:
Although the left eye was completely normal, the right eye had multiple round chorioretinal scars, peripapillary scarring, and a fibrotic membrane in the macula. Fluorescein angiogram confirmed that the macular scar was fibrotic and inactive, as were the other chorioretinal scars. There are three conditions that can have the retinal appearance seen above. The first is the presumed ocular histoplasmosis syndrome (POHS). Patients with this condition have the triad of peripapillary scarring, “histo spots”, and choroidal neovascular membranes. There is an absence of inflammation. Visual acuity can be normal unless there are either histo spots or choroidal neovascular membranes in the fovea. Two things argued against this diagnosis. First, the patient never traveled to endemic areas. Although POHS can be found anywhere in the country, it is most prevalent in the Midwest, as the histoplasmosis organism is more common in farm animals and birds. Second, POHS is always a bilateral condition. If the patient had similar findings in both eyes, POHS would have been the diagnosis. She had a normal left eye. The second diagnosis is multifocal choroiditis with panuveitis (MFC). This condition is typically found in young female myopes. Peripapillary scarring, chorioretinal scars, and choroidal neovascular membranes can all be present. However, this is an inflammatory condition. Patients have vitritis, retinal vasculitis, and often have optic nerve swelling, cystoids macular edema, and chorioretinal scars in varying stages and degrees of activity. Our patient had no sign of active or previous inflammation, thus making this diagnosis unlikely. The third condition is punctuate inner choroidopathy (PIC). This condition is also found in young myopic females. It can be unilateral or bilateral. It can be associated with the same triad as the POHS patients. Inflammation is minimal or nonexistent. Because our patient had findings in one eye, PIC was her diagnosis. Unfortunately she presented too late in her course for treatment. If she had been evaluated when her choroidal neovascular membrane first developed, she could have been treated with anti VEGF agents or possibly steroids in order to reduce scarring and improve vision. She will be monitored and was advised to report any new changes in her vision immediately.