History of present illness
We present a case of a 64-year-old woman with an unremarkable medical history referred for an epiretinal membrane of her right eye. She denies blurry vision, floaters, photopsias or flashes, metamorphopsia, or nyctalopia. She denies any history of ocular conditions or ocular surgeries.
Ocular examination findings
Visual acuity with correction was 20/20 in each eye. Intraocular pressure were normal. Anterior segment examination showed mild nuclear sclerotic cataracts but otherwise was unremarkable. Dilated fundus examination showed radial spoke-like striae of the fovea with a central cyst in the right eye and macular edema of the left eye ( Fig. 52.1 ).
Imaging
Optical coherence tomography (OCT) showed cyst-like changes in the outer retina in the foveal and parafoveal regions and in the inner retina in the temporal macula in the right eye. OCT of the left eye showed cyst-like changes in the outer retina in the inferior macula ( Fig. 52.2 ).
Questions to ask
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What is the patient’s refractive error? Myopic foveoschisis or myopic traction maculopathy should be considered in the differential diagnosis for retinoschisis.
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Plano + 0.25 × 153 in the right eye and −0.50 + 0.25 × 009 in the left eye
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Does the patient have a family history of ocular conditions or consanguinity? A family history would be important to ascertain etiologies for retinoschisis such as congenital juvenile X-linked retinoschisis (XLRS), retinitis pigmentosa, or familial internal limiting membrane dystrophy.
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No
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History of trauma? Trauma has been associated with macular retinoschisis.
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No
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What medications does the patient take? Medications such as niacin and taxanes can cause cystic macular edema that appears similar to retinoschisis.
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None
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Assessment
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This is a case of a 64-year-old woman with no ocular or surgical history and a negative family history demonstrating bilateral macular schisis of mainly the outer retina on OCT.
Differential diagnosis
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Congenital juvenile X-linked retinoschisis (XLRS)
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Myopic retinoschisis
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Optic pit maculopathy
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Retinitis pigmentosa
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Glaucoma
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Vitreomacular traction
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Degenerative retinoschisis
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Enhanced S-cone syndrome
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Medication associated retinoschisis (niacin, taxanes)
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Familial internal limited membrane dystrophy
Working diagnosis
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Stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR)
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Has been described in many studies and can have bilateral or unilateral findings ,
Multimodal testing and results
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Fundus photographs
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On fundus examination, a stellate pattern in the macula is typically visualized, but this may not be present in all patients.
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OCT
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As mentioned, our patient’s OCT showed cyst-like changes in the outer retina in the foveal and parafoveal regions and in the inner retina in the temporal macula in the right eye. OCT of the left eye showed cyst-like changes in the outer retina in the inferior macula.
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In SNIFR, splitting more commonly involves the outer plexiform layer but can involve the outer nuclear layer as well.
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Electroretinogram (ERG)
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ERG was obtained, which was normal in both eyes and did not show the characteristic electronegative pattern seen in XLRS ( Fig. 52.3 ).
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