OSLI Retina

July 2020

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July 2020 ยท Vol. 51, No. 7 419 ated foveal avascular zone with macular-foveal capil- laries, and areas of capillary dropout (Figures 1D and 1E). The patient was diagnosed with bilateral OND and AVM-related impending vein occlusion OS. Vein occlusion remains the most common com- plication in AVMs: increased intravascular pressure and high, turbulent flow lead to endothelial damage, and fibrosis of vessel walls. 2,3 As per Virchow's tri- ade, the coagulative cascade may be triggered. OND are also a known risk factor for retinal vascular oc- clusion, as they cause mechanical compression at the lamina cribrosa, elevating central retinal venous pressure. Enlargement of drusen volume, which typi- cally occurs in teenagers, might have precipitated the impaired perfusion state, leading to clinically signifi- cant flow obstruction. 4 The co-existence of AVM and OND in a patient with suspected Wyburn-Mason syndrome is a novel description. Such association has been described in Klippel-Trenaunay syndrome. 5 The presence of both AVM and OND might have an additive effect on the likelihood of vascular events to occur. REFERENCES 1. Mansour AM, Walsh JB, Henkind P. Arteriovenous anastomoses of the retina. Ophthalmology. 1987;94(1):35-40. https://doi.org/10.1016/ S0161-6420(87)33505-5 PMID:3561955 2. Callahan AB, Skondra D, Krzystolik M, Yonekawa Y, Eliott D. Wyburn-Mason Syndrome Associated with Cutaneous Reac- tive Angiomatosis and Central Retinal Vein Occlusion. Ophthal- mic Surg Lasers Imaging Retina. 2015;46(7):760-762. https://doi. org/10.3928/23258160-20150730-12 PMID: 26247458 3. Salati C, Ferrari E, Basile R, Virgili G, Menchini U. Retinal vein occlusion: late complication of a congenital arteriovenous anoma- ly. Ophthalmologica. 2002;216(2):151-152. https://doi.org/10.1159/ 000048316 PMID:11919443 4. Malmqvist L, Lund-Andersen H, Hamann S. Long-term evolution of superficial optic disc drusen. Acta Ophthalmol. 2017;95(4):352- 356. https://doi.org/10.1111/aos.13315 PMID:27996202 5. Bothun ED, Kao T, Guo Y, Christiansen SP. Bilateral optic nerve drusen and gliomas in Klippel-Trenaunay syndrome. J AAPOS. 2011;15(1):77-79. https://doi.org/10.1016/j.jaa- pos.2010.10.009 PMID:21315629 Figure 1. Multimodal imaging of arteriovenous malformation and optic nerve drusen. (A) Ultra-widefield (UWF) retinography showing dilated and tortuous vessels in the inferotemporal branch and rare hemorrhages (arrow); optic nerve head contours are blurred. (B) Fun- dus autofluorescence showing optic disc multiple, rounded hyperautofluorescent lesions (asterisk). (C) Optical coherence tomography highlights subfoveal irregularity in the outer retinal layers (arrowhead). A dilated vessel is seen nasally with slight tenting of the internal limiting membrane (arrow). (D) UWF fluorescein angiography reveals vascular loops, arteriovenous connections, and inferotemporal capillary drop-out. In the macular area, some macular-foveal capillaries are noticeable (detail). No late-phase leakage or epiretinal neo- vascularization is seen. (E) Optical coherence tomography angiography showing the abnormal capillary bed and focal areas of nonperfu- sion in the inferotemporal retina.

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