En Face Optical Coherence Tomography of Macular Microcysts Due to Optic Neuropathy From Neuromyelitis Optica

Chen, Xuejing; Kuehlewein, Laura; Pineles, Stacy L.; Tandon, Anika; Bose, Shelley X.; Klufas, Michael A.; Sadda, Srinivas R.; Sarraf, David

doi:10.1097/icb.0000000000000197

Cited by 15 publications

(9 citation statements)

References 10 publications

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“…Van Buren first described the histopathology of inner nuclear layer microcysts, from experimental optic nerve injury, and highlighted the extensive loss of the ganglion cells and cystic degeneration of the inner nuclear layer on the medial side of the fovea [ 7 ]. Since that time, microcystic inner nuclear layer abnormalities have been described in a variety of optic neuropathies including multiple sclerosis [ 8 ], neuromyelitis optica [ 5 ], hereditary optic neuropathies [ 9 ] and compressive optic pathway diseases [ 10 ]. Different theories have been put forth to explain these changes, including trans-synaptic retrograde degeneration, glial cell activation and mitochondrial dysfunction [ 7 , 9 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…The former has a high degree of uniformity, characteristic shape and specific location within the INL whereas the latter tend to be larger, more irregular, and can localize to the outer plexiform layer as well. Further, INL microcysts are bilateral and the retina is not thickened [ 5 ]. Multimodal imaging of macular microcysts demonstrating unremarkable optical coherence tomography angiography of the superficial and deep retinal capillary plexuses and fluorescein angiography, suggest a nonvascular etiology such as Muller cell degeneration [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

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Optical coherence tomography findings in methanol toxicity

et al. 2017

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Background Methanol toxicity poses a significant public health problem in developing countries, and in Southeast Asia, where the most common source of poisoning is via adulterated liquor in local drinks. Methanol toxicity can have devastating visual consequences and retinal specialists should be aware of the features of this toxic optic neuropathy. The authors report a case of severe systemic methanol toxicity and relatively mild optic neuropathy demonstrating unique retinal changes on optical coherence tomography (OCT).Case presentation A previously healthy student developed ataxia, difficulty breathing and loss of consciousness hours after drinking homemade alcohol while traveling in Indonesia. She was found to have a serum pH of 6.79 and elevated methanol levels. She was treated with intravenous ethanol, methylprednisolone and sodium bicarbonate. When she awoke she had bilateral central scotomas. At presentation, she had central depression on visual field testing. OCT of the retinal nerve fiber layer (RNFL) was normal but ganglion cell layer analysis (GCL) showed highly selective loss of the nasal fibers in both eyes. Further, OCT of the macula demonstrated inner nuclear layer (INL) microcysts in the corresponding area of selective GCL loss in both eyes.ConclusionsThe selective involvement of the papillomacular bundle fibers is common in toxic optic neuropathies and represents damage to the small caliber axons rich in mitochondria. Despite severe systemic toxicity, the relative sparing of the optic nerve in this case enabled characterization of the evolution of methanol toxicity with segmental GCL involvement and preservation of the RNFL, corresponding to the papillomacular bundle. This is the first reported case of INL microcysts in methanol optic neuropathy and supports that they are a non-specific finding, and may represent preferential damage to the papillomacular bundle.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Optical coherence tomography findings in methanol toxicity

et al. 2017

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“…Brandt 2014 20 Nine patients Retrospective cohort study Follow-up on patients with MME to look for association with vitreous traction which was not found. Brazerol Chen 2015 21 One patient Case report MME in a child with NMO, unremarkable angio-OCT. Chen 2016 22 One patient Case report MME in a woman with Schwartz-Matsuo syndrome. Gelfand 2013 23 25 consecutive patients with NMO Case series MME was found in five of 25 patients with NMO and seven of 29 NMO optic neuritis eyes.…”

Section: Oct Methodologymentioning

confidence: 99%

Microcystic macular oedema in optic neuropathy: case series and literature review

Kessel

Hamann

Wegener

et al. 2018

Clinical Exper Ophthalmology

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Cavitations in the inner nuclear layer associated with severe optic atrophy and loss of retinal ganglion cells have clinically been termed microcystic macular oedema (MME). We describe a case series of MME in patients of all ages but predominantly younger patients with a wide range of optic atrophies ranging from acute onset optic disc drusen associated ischemic optic neuropathy to slowly progressive disease as glaucoma. There were no physical distinctions between MME in different causes of optic atrophy suggesting a common causative mechanism. We reviewed the literature on MME and it appears that MME is associated with more severe visual loss, and is more common in hereditary optic neuropathies and neuromyelitis optica spectrum disease rather than in patients with optic atrophy secondary to multiple sclerosis and glaucoma. Three main causative mechanisms have been proposed, including increased vitreal traction on the macular as the ganglion cells are lost. Others have suggested that trans-synaptic loss of cells in the inner nuclear layer causes formation of empty spaces or cavities. Finally, some have hypothesized a disturbance in the fluid homeostasis of the inner retina as Müller cells are lost or their function is impaired. There are no known treatments of MME. In conclusion, MME seems to be a marker of severe optic nerve atrophy irrespective of the underlying cause.

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“…The role of the Müller cell may be critical in these pathoanatomical considerations and clinicopathological correlations would be very supportive. A similar pattern of INL cavitation called "microcystic macular edema" occurs in a range of optic neuropathies including those associated with multiple sclerosis, neuromyelitis optica, and glaucoma [11]. While the underlying cause of these structural changes may vary with each associated disease, the numerous regularly spaced vessels bridging the INL between the ICP and DCP are likely to influence the anatomic pattern seen with all.…”

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confidence: 99%

“…Cystic lesions in the macula can have diverse etiologies including exudative cystoid macular edema (CME), macular schisis, and microcystic degeneration [6]. Differentiation by OCT or OCTA can be very challenging although the cysts of microcystic degeneration tend to present as narrow, vertically oriented, slit-like spaces which can be very characteristic [11]. The role of the Müller cell may be critical in these pathoanatomical considerations and clinicopathological correlations would be very supportive.…”

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confidence: 99%