Gazing Into the Crystal Ball Photo Essay: Response

Letters to the Editor 2. Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med. 2018;378:1704–1714. 3. Chang YS, Weng SF, Chang C, Wang JJ, Su SB, Huang CC, Wang JY, Jan RL. Risk of nonarteritic anterior ischemic optic neuropathy following end-stage renal disease. Medicine (Baltimore). 2016;95:e3174. 4. Goldenberg-Cohen N, Naftaliev E, Millmond SH, Segev Y, Reider-Groswasser I. Ophthalmic artery calcification in a patient with renal failure. Neuroradiology. 2001;43:1005–1006. 5. Patel DV, Snead MP, Satchi K. Retinal arteriolar calcification in a patient with chronic renal failure. Br J Ophthalmol. 2002;86:1063. 6. Naysan J, Dansingani KK, Balaratnasingam C, Mrejen S, Levasseur S, Merkur A, Yannuzzi LA. Crystalline retinopathy and retinal vasculopathy in calcific uremic arteriolopathy (calciphylaxis). Retin Cases Brief Rep. 2018;12:331–335. 7. Mishra C, Kannan N, Chakrabarti A, Kumar M, Ramasamy K. Retinal arteriolar calcification and ischemic retinopathy in a patient with chronic kidney disease. Indian J Ophthalmol Case Rep. 2021;1:337. 8. Huerva V, Sánchez MC, Ascaso FJ, Craver L, Fernández E. Calciphylaxis and bilateral optic neuropathy. J Fr Ophtalmol. 2011;34:651–654. 9. Awwad ST, Ghosn S, Hogan RN. Calciphylaxis of the temporal artery masquerading as temporal arteritis. Clin Exp Ophthalmol. 2010;38:511–513. 10. Hepschke JL, Masselos K, Sandbach J, Fraser CL. Seeing the difference-Painless progressive vision loss in a vasculopath. Surv Ophthalmol. 2019;64:123–131. 11. Sivertsen MS, Strøm EH, Endre KMA, Jørstad ØK. Anterior ischemic optic neuropathy due to calciphylaxis. J Neuroophthalmol. 2018;38:54–56. 12. Komurcu HF, Basar E, Kucuksahin O, Uz E, Orhun Yavuz HS, Anlar O. Bilateral optic neuropathy, acral gangrene and visceral ischaemia as a rare presentation of calciphylaxis: a case report. J Pak Med Assoc. 2016;66:1324–1326. 13. Sekulic M, Truskinovsky AM. Metaplastic ossification of the temporal artery with osteoclast-like giant cells: a mimicker of giant cell (temporal) arteritis. Eur J Ophthalmol. 2017;27:e99–103. 14. Chi M, Kim HJ, Basham R, Yoon MK, Vagefi R, Kersten RC. Temporal artery calciphylaxis presenting as temporal arteritis in a case of rhinoorbitocerebral mucormycosis. Ophthalmic Plast Reconstr Surg. 2015;31:e132–e135. 15. Gershoni A, Dotan G, Gal-Or O. Multimodal imaging findings in primary hyperoxaluria. Ophthalmol Retina. 2018;2:1169. 16. Korzets A, Marashek I, Schwartz A, Rosenblatt I, Herman M, Ori Y. Ischemic optic neuropathy in dialyzed patients: a previously unrecognized manifestation of calcific uremic arteriolopathy. Am J Kidney Dis. 2004;44:e93–e97. 17. Roverano S, Ortiz A, Henares E, Eletti M, Paira S. Calciphylaxis of the temporal artery masquerading as temporal arteritis: a case presentation and review of the literature. Clin Rheumatol. 2015;34:1985–1988. 18. Farooqui S, Chan A, Cullen JF, Milea D. Too young to undergo temporal artery biopsy? Calciphylaxis-related anterior ischemic optic neuropathy. Neuroophthalmology. 2019;43:252–255. Gazing Into the Crystal Ball Photo Essay: Response tial involvement of the contralateral eye, are unique and would be unusual for ESRD alone. We speculate that cataract surgery likely served as an insult for progressive calcium deposition leading to patient's abrupt clinical presentation much as skin trauma is believed to be a potential trigger for the development of cutaneous calcifications in patients with ESRD with repeated insulin injections (3,4) and in a single animal model (5). Usually, however, no trigger is identified in those with cutaneous calcification or calciphylaxis in setting of ESRD. The readers point out several reported cases of histologically confirmed cases of calciphylaxis with ophthalmic involvement that did not have retinal vascular calcification (6–13), and conversely, retinal arteriolar calcification reported without systemic calciphylaxis (14,15). We believe appearance of retinal vascular calcification/crystalline retinopathy and bilateral optic neuropathy in conjunction with profound vision loss is particularly unique in our case. Several cases of biopsy-proven calciphylaxis report ischemic optic neuropathy mimicking GCA (7,8,10,11). There has also been a single case report of isolated crystalline and ischemic retinopathy in a patient with a history of confirmed cutaneous calciphylaxis (6). We did consider retinal oxalosis on the differential diagnosis of crystalline retinopathy, noting that oxalosis has a unique deposition pattern in the RPE, Bruch membrane, and choriocapillaris (6,16). Notably, intravascular crystalline deposition is a less common manifestation of oxalosis (6). Unfortunately, high-quality optical coherence tomography was unable to be performed because of poor fixation and poor view of the fundus in setting of corneal opacity. W e thank readers Dr. Patel and Dr. Sinha for their informative response to our case report. Before cataract extraction, our patient had a visual acuity of 20/400 pinhole to 20/70 in the right eye and 20/60 pinhole to 20/25 in the left eye. She was seen twice preoperatively by a comprehensive ophthalmologist with documentation of a normal fundus examination. After right-eye cataract extraction, there was precipitous right-eye vision loss to hand motion with sequential left-eye vision loss in the following weeks. The readers suggest perioperative nonarteritic anterior ischemic optic neuropathy (NAION) as a potential cause of this case presentation; NAION is classically characterized by painless segmental optic nerve head swelling often with flame-shaped disc hemorrhage and typically with altitudinal field loss. Nearly half of patients with NAION present with vision better than 20/70 at presentation (1,2). Our patient had severe bilateral vision loss with pallid swelling of the optic nerves at presentation more characteristic of arteritic ION rather than NAION. End-stage renal disease does put our patient at risk of ischemic complications and certainly contributes to her ocular condition. We agree that our patient did have perioperative ocular ischemia as mentioned in the title of the report; however, precipitous vision loss and the appearance of extensive widespread retinal vascular calcifications, cotton wool spots, crystalline retinopathy, and diffuse corneal opacity, as well as sequenLetters to the Editor: J Neuro-Ophthalmol 2022; 42: e533-e538 e537 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Letters to the Editor We agree that the histopathologic findings on skin biopsy in our report are those of calcinosis cutis, which is not biologically related to calciphylaxis. The readers' point of difficulty disentangling ophthalmic calcific uremic arteriolopathy as an entity separate from the calciumphosphate dyscrasias because of ESRD is well taken; however, appearances of these changes with profound vision loss are unique in this case and on the spectrum of dystrophic calcinosis separate from ESRD alone. We believe the ophthalmic presentation reported in our case perhaps represents a separate ocular clinical syndrome on the same spectrum as cutaneous calciphylaxis with potential commonalities in pathogenesis. Neena R. Cherayil, MD Madhura A. Tamhankar, MD Departments of Neurology and Ophthalmology, Northwestern University, Chicago, Illinois Department of Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania The authors report no conflicts of interest. REFERENCES 1. Repka MX, Savino PJ, Schatz NJ, Sergott RC. Clinical profile And long-term implications of anterior ischemic optic neuropathy. Am J Ophthalmol. 1983;96:478–483. 2. Characteristics of patients with nonarteritic anterior ischemic optic neuropathy eligible for the ischemic optic neuropathy decompression trial. Arch Ophthalmol. 1996;114:1366. 3. Handa SP, Sohi PS. Proximal calciphylaxis in four insulinrequiring diabetic hemodialysis patients. Am J Kidney Dis. 1997;29:812. e538 4. Nigwekar SU, Zhao S, Wenger J, Hymes JL, Maddux FW, Thadhani RI, Chan KE. A nationally representative study of calcific uremic arteriolopathy risk factors. J Am Soc Nephrol. 2016;27:3421–3429. 5. Selye H, Gentile G, Prioreschi P. Cutaneous molt induced by calciphylaxis in the rat. Science. 1961;134:1876–1877. 6. Naysan J, Dansingani KK, Balaratnasingam C, Mrejen S, Levasseur S, Merkur A, Yannuzzi LA. Crystalline retinopathy and retinal vasculopathy in calcific uremic arteriolopathy (calciphylaxis). Retin Cases Brief Rep. 2018;12:331–335. 7. Huerva V, Sánchez MC, Ascaso FJ, Craver L, Fernández E. Calciphylaxis and bilateral optic neuropathy. J Fr Ophtalmol. 2011;34:651–654. 8. Awwad ST, Ghosn S, Hogan RN. Calciphylaxis of the temporal artery masquerading as temporal arteritis. Clin Exp Ophthalmol. 2010;38:511–513. 9. Hepschke JL, Masselos K, Sandbach J, Fraser CL. Seeing the difference-Painless progressive vision loss in a vasculopath. Surv Ophthalmol. 2019;64:123–131. 10. Sivertsen MS, Strøm EH, Endre KMA, Jørstad ØK. Anterior ischemic optic neuropathy due to calciphylaxis. J Neuroophthalmol. 2018;38:54–56. 11. Komurcu HF, Basar E, Kucuksahin O, Uz E, Orhun Yavuz HS, Anlar O. Bilateral optic neuropathy, acral gangrene and visceral ischaemia as a rare presentation of calciphylaxis: a case report. J Pak Med Assoc. 2016;66:1324–1326. 12. Sekulic M, Truskinovsky AM. Metaplastic ossification of the temporal artery with osteoclast-like giant cells: a mimicker of giant cell (temporal) arteritis. Eur J Ophthalmol. 2017;27:e99– e103. 13. Chi M, Kim HJ, Basham R, Yoon MK, Vagefi R, Kersten RC. Temporal artery calciphylaxis presenting as temporal arteritis in a case of rhinoorbitocerebral mucormycosis. Ophthalmic Plast Reconstr Surg. 2015;31:e132–e135. 14. Patel DV, Snead MP, Satchi K. Retinal arteriolar calcification in a patient with chronic renal failure. Br J Ophthalmol. 2002;86:1063. 15. Mishra C, Kannan N, Chakrabarti A, Ramaswamy K. Retinal arteriolar calcification and ischemic retinopathy in a patient with chronic kidney disease. Indian J Ophthalmol Case Rep. 2021;1:337. 16. Yuan A, Ehlers JP. Crystalline retinopathy from primary hyperoxaluria. Retina. 2012;32:1994–1995. Letters to the Editor: J Neuro-Ophthalmol 2022; 42: e533-e538 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.