Patient Presentation: A 23-year-old obese female was diagnosed with idiopathic intracranial hypertension (IIH) and referred to neurosurgery for ventriculoperitoneal shunt. A baseline ocular examination was performed prior to the procedure.
On examination, vision was 20/200 in the right eye, and 20/40 in the left eye. There was a right relative afferent pupillary defect. Slit lamp examination was normal.
A dilated fundus examination was performed demonstrating the following:
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Retina
Case 76
Contributors: Korolos Sawires MD (C) and Dr. R. Rishi Gupta MD, FRCSC, FASRS
Patient presentation: A 62-year-old man was referred to a tertiary retina clinic for presumed cystoid macular edema (CME) in the left eye. The patient noted progressive painless blurring of his vision OS over the last few years. His past medical, family, and ocular history were unremarkable. He was emmetropic and not taking any medications.
Best corrected distance visual acuity was 20/25 OD and 20/100 OS (pinhole 20/40) with an IOP of 16 mmHg in both eyes. Slit lamp examination revealed 1+ nuclear sclerotic cataract OD and a 2+ nuclear sclerotic cataract with posterior subcapsular component OS. There was no intraocular inflammation.
Fundus, fundus autofluorescence and near infrared photographs were taken and are shown below:
Question 1: What is the main finding of the photos above?
Next, a spectral-domain OCT (SD-OCT) of the macula OU and a peripheral SD-OCT OS were taken and are shown below:
Left eye temporal to macula:
Question 2: What is the most notable abnormality in the above OCT scans?
Question 3: Which layer(s) of the neurosensory retina are affected in the OCT of the left eye?
Ultrawide field fluorescein angiography was performed and is shown below:
Question 4: Which of the following pathologies do not leak on fluorescein angiography?
Question 5: What is the most likely diagnosis?
References:
1. Gaudric, Alain et al. “Non-vasogenic cystoid maculopathies.” Progress in retinal and eye research vol. 91 (2022): 101092. doi:10.1016/j.preteyeres.2022.101092
2. Feldman, Brad H et al. "Cystoid Macular Edema." Eyewiki, American Academy of Ophthalmology, 21 Jan. 2024, https://eyewiki.aao.org/Cystoid_Macular_Edema.
3. Ober, Michael D et al. “Stellate nonhereditary idiopathic foveomacular retinoschisis.” Ophthalmology vol. 121,7 (2014): 1406-13. doi:10.1016/j.ophtha.2014.02.002
4. Light, Jacob G et al. “Stellate nonhereditary idiopathic foveomacular retinoschisis and an approach to the differential diagnosis of macular star.” Current opinion in ophthalmology vol. 33,3 (2022): 157-166. doi:10.1097/ICU.0000000000000844
6. Mandviwala, Murtaza Mustafa. "Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis (SNIFR)." Eyewiki, American Academy of Ophthalmology, 4 Nov. 2022, https://eyewiki.org/Stellate_Nonhereditary_Idiopathic_Foveomacular_Retinoschisis_(SNIFR).
Learning Objectives:
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To identify OCT features of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR)
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Review layers of the retina and which are affected in SNIFR
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Appreciate the role of fluorescein angiography in differentiating CME and retinoschisis