The authors report extensive peripheral retinoschisis in a patient with stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) detected by widefield optical coherence tomography (OCT). A 64-year-old woman diagnosed with foveomacular retinoschisis 3 years prior presented for evaluation after being seen by multiple other retina specialists. Standard macular spectral-domain OCT (6 mm) revealed typical foveomacular schisis involving only the outer retina. However, widefield OCT (12 mm) revealed diffuse bilateral retinoschisis involving both inner and outer retinal layers in the macula and midperiphery. Widefield imaging is important to evaluate and monitor complex peripheral retinoschisis that may be otherwise undetectable using conventional techniques. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:774-777.].

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http://dx.doi.org/10.3928/23258160-20160808-12DOI Listing

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Article Synopsis
  • A 70-year-old woman was diagnosed with stellate nonhereditary idiopathic foveomacular retinoschisis, showing schisis cavities in the outer plexiform layer of her left eye through optical coherence tomography.
  • No leakage was observed in fluorescein angiography, and other potential causes for her condition were ruled out.
  • After five years, her visual acuity remained stable, the retinoschisis in her left eye resolved, but new cystoid spaces developed in the right eye, highlighting the need for multimodal imaging for monitoring such conditions.
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Stellate nonheritable idiopathic foveomacular retinoschisis in juveniles: case report.

BMC Ophthalmol

September 2023

Department of Ophthalmology, Hebei Medical University, Shijiazhuag, 050017, Hebei, China.

Background: Stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) is a rare type of retinoschisis with a spoke-like splitting that occurs in the outer plexus layer. We present a case of stellate nonhereditary idiopathic foveomacular retinoschisis in a juvenile, in which two eyes show different development trends and macular retinoschisis could be associated with mechanical force in the Henle fibre layer. The removal of mechanical force can partially restore vision.

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Stellate foveomacular retinoschisis is commonly associated with congenital X-linked retinoschisis, which is almost exclusively seen bilaterally in males. In the absence of a family history of retinoschisis and other related conditions, the term stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) is used. SNIFR constitutes a rather rare diagnosis and is usually observed unilaterally in myopic females.

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We describe the macular morpho-functional assessment of a 65-year-old man affected by stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR), studied by visual field, SD-OCT, autofluorescence, full-field electroretinogram (ffERG), multifocal electroretinogram (mfERG) and multifocal Photopic Negative Response (mfPhNR) recordings. The typical presentation consists of the foveal appearance of radial cartwheel pattern for the splitting of the retinal layers at the level of the Henle fiber layer (HFL) and the outer plexiform layer (OPL), perfectly seen by Spectral Domain-Optical Coherence Tomography (SD-OCT). Despite a normal function of the outer retina of the peripheral and central retina evaluated by ffERG and mfERG respectively, we observed a reduced function of the retinal elements involved in the retinoschisis by recording mfPhNR that assesses mainly inner retina function (retinal ganglion cells and their axons).

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