Young Adults With Anterior Ischemic Optic Neuropathy: A Multicenter Optic Disc Drusen Study

Published:April 13, 2020DOI:


      Optic disc drusen (ODD), present in 2% of the general population, have occasionally been reported in patients with nonarteritic anterior ischemic optic neuropathy (NA-AION). The purpose of this study was to examine the prevalence of ODD in young patients with NA-AION.


      Retrospective, cross-sectional multicenter study.


      All patients with NA-AION 50 years old or younger, seen in neuro-ophthalmology clinics of the international ODDS (Optic Disc Drusen Studies) Consortium between April 1, 2017, and March 31, 2019, were identified. Patients were included if ODD were diagnosed by any method, or if ODD were excluded by enhanced-depth imaging optical coherence tomography (EDI-OCT) using ODDS Consortium guidelines. NA-AION eyes with ODD were termed “ODD-AION”; those without were termed “NODD-AION”.


      A total of 65 patients (127 eyes) with NA-AION were included (mean 41 years old). Of the 74 eyes with NA-AION, 51% had ODD-AION, whereas 43% of fellow eyes without NA-AION had ODD (P = .36). No significant differences were found between ODD-AION and NODD-AION eyes in terms of Snellen best-corrected VA or perimetric mean deviation. According to EDI-OCT results, 28% of eyes with NODD-AION had peripapillary hyperreflective ovoid mass-like structures (PHOMS); 7% had hyperreflective lines, whereas 54% with ODD-AION had PHOMS; and 66% had hyperreflective lines (P = .006 and P < .001, respectively).


      Most of these young NA-AION patients had ODD. This indicates that ODD may be an independent risk factor for the development of NA-AION, at least in younger patients. This study suggests ODD-AION be recognized as a novel diagnosis.
      To read this article in full you will need to make a payment


      Subscribe to American Journal of Ophthalmology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Biousse V.
        • Newman N.J.
        Ischemic optic neuropathies.
        N Engl J Med. 2015; 372: 2428-2436
        • Arnold A.C.
        Pathogenesis of nonarteritic anterior ischemic optic neuropathy.
        J Neuroophthalmol. 2003; 23: 157-163
        • Hayreh S.S.
        Ischemic optic neuropathies—where are we now?.
        Graefes Arch Clin Exp Ophthalmol. 2013; 251: 1873-1884
      1. Characteristics of patients with nonarteritic anterior ischemic optic neuropathy eligible for the Ischemic Optic Neuropathy Decompression Trial.
        Arch Ophthalmol. 1996; 114: 1366-1374
        • Preechawat P.
        • Bruce B.B.
        • Newman N.J.
        • Biousse V.
        Anterior ischemic optic neuropathy in patients younger than 50 years.
        Am J Ophthalmol. 2007; 144: 953-960
        • Hattenhauer M.G.
        • Leavitt J.A.
        • Hodge D.O.
        • Grill R.
        • Gray D.T.
        Incidence of nonarteritic anterior ischemic optic neuropathy.
        Am J Ophthalmol. 1997; 123: 103-107
        • Arnold A.C.
        • Costa R.M.
        • Dumitrascu O.M.
        The spectrum of optic disc ischemia in patients younger than 50 years [thesis].
        Trans Am Ophthalmol Soc. 2013; 111: 93-118
        • Miller N.R.
        • Arnold A.C.
        Current concepts in the diagnosis, pathogenesis and management of nonarteritic anterior ischaemic optic neuropathy.
        Eye (Lond). 2015; 29: 65-79
        • Knox D.L.
        • Kerrison J.B.
        • Green W.R.
        Histopathologic studies of ischemic optic neuropathy [thesis].
        Trans Am Ophthalmol Soc. 2000; 98 (discussion 221–222): 203-220
        • Hamann S.
        • Malmqvist L.
        • Costello F.
        Optic disc drusen: understanding an old problem from a new perspective.
        Acta Ophthalmol. 2018; 96: 673-684
        • You Q.S.
        • Xu L.
        • Wang Y.X.
        • Jonas J.B.
        Prevalence of optic disc drusen in an adult Chinese population: the Beijing Eye Study.
        Acta Ophthalmol. 2009; 87: 227-228
        • Lorentzen S.E.
        Drusen of the optic disk. A clinical and genetic study.
        Acta Ophthalmol (Copenh). 1966; : 1-180
        • Friedman A.H.
        • Gartner S.
        • Modi S.S.
        Drusen of the optic disc. A retrospective study in cadaver eyes.
        Br J Ophthalmol. 1975; 59: 413-421
        • Skougaard M.
        • Heegaard S.
        • Malmqvist L.
        • Hamann S.
        Prevalence and histopathological signatures of optic disc drusen based on microscopy of 1713 enucleated eyes.
        Acta Ophthalmol. 2020; 98: 195-200
        • Auw-Haedrich C.
        • Staubach F.
        • Witschel H.
        Optic disk drusen.
        Surv Ophthalmol. 2002; 47: 515-532
        • Wilkins J.M.
        • Pomeranz H.D.
        Visual manifestations of visible and buried optic disc drusen.
        J Neuroophthalmol. 2004; 24: 125-129
        • Vahlgren J.
        • Malmqvist L.
        • Ruelokke L.L.
        • Karlesand I.
        • Lindberg A.W.
        • Hamann S.
        The angioarchitecture of the optic nerve head in patients with optic disc drusen.
        Neuroophthalmology. 2020; 44: 5-10
        • Borruat F.X.
        • Sanders M.D.
        [Vascular anomalies and complications of optic nerve drusen].
        Klin Monbl Augenheilkd. 1996; 208 ([article in French]): 294-296
        • Purvin V.
        • King R.
        • Kawasaki A.
        • Yee R.
        Anterior ischemic optic neuropathy in eyes with optic disc drusen.
        Arch Ophthalmol. 2004; 122: 48-53
        • Nanji A.A.
        • Klein K.S.
        • Pelak V.S.
        • Repka M.X.
        Nonarteritic anterior ischemic optic neuropathy in a child with optic disk drusen.
        J AAPOS. 2012; 16: 207-209
        • Newman W.D.
        • Dorrell E.D.
        Anterior ischemic optic neuropathy associated with disc drusen.
        J Neuroophthalmol. 1996; 16: 7-8
        • Liew S.C.
        • Mitchell P.
        Anterior ischaemic optic neuropathy in a patient with optic disc drusen.
        Aust N Z J Ophthalmol. 1999; 27: 157-160
        • Pineles S.L.
        • Arnold A.C.
        Fluorescein angiographic identification of optic disc drusen with and without optic disc edema.
        J Neuroophthalmol. 2012; 32: 17-22
        • Loft F.C.
        • Malmqvist L.
        • Wessel Lindberg A.S.
        • Hamann S.
        The influence of volume and anatomic location of optic disc drusen on the sensitivity of autofluorescence.
        J Neuroophthalmol. 2019; 39: 23-27
        • Kurz-Levin M.M.
        • Landau K.
        A comparison of imaging techniques for diagnosing drusen of the optic nerve head.
        Arch Ophthalmol. 1999; 117: 1045-1049
        • Almog Y.
        • Nemet A.
        • Nemet A.Y.
        Optic disc drusen demonstrate a hyperechogenic artifact in B mode ultrasound.
        J Clin Neurosci. 2016; 23: 111-119
        • Lam B.L.
        • Morais Jr., C.G.
        • Pasol J.
        Drusen of the optic disc.
        Curr Neurol Neurosci Rep. 2008; 8: 404-408
        • Merchant K.Y.
        • Su D.
        • Park S.C.
        • et al.
        Enhanced depth imaging optical coherence tomography of optic nerve head drusen.
        Ophthalmology. 2013; 120: 1409-1414
        • Sim P.Y.
        • Soomro H.
        • Karampelas M.
        • Barampouti F.
        Enhanced depth imaging optical coherence tomography of optic nerve head drusen in children.
        J Neuroophthalmol. 2019; (Epub ahead of print)
        • Malmqvist L.
        • Bursztyn L.
        • Costello F.
        • et al.
        The optic disc drusen studies consortium recommendations for diagnosis of optic disc drusen using optical coherence tomography.
        J Neuroophthalmol. 2018; 38: 299-307
        • Costello F.
        • Malmqvist L.
        • Hamann S.
        The role of optical coherence tomography in differentiating optic disc drusen from optic disc edema.
        Asia Pac J Ophthalmol. 2018; 7: 271-279
        • Sato T.
        • Mrejen S.
        • Spaide R.F.
        Multimodal imaging of optic disc drusen.
        Am J Ophthalmol. 2013; 156: 275-282
        • Tso M.O.
        Pathology and pathogenesis of drusen of the optic nervehead.
        Ophthalmology. 1981; 88: 1066-1080
        • Maloca P.M.
        • Tufail A.
        • Egan C.
        • et al.
        Volume rendering of superficial optic disc drusen.
        Spektrum der Augenheilkunde. 2017; 31: 288-293
        • Malmqvist L.
        • Li X.Q.
        • Hansen M.H.
        • et al.
        Progression over 5 years of prelaminar hyperreflective lines to optic disc drusen in the Copenhagen Child Cohort 2000 Eye Study.
        J Neuroophthalmol. 2020;
        • Hayreh S.S.
        • Zimmerman M.B.
        Optic disc edema in non-arteritic anterior ischemic optic neuropathy.
        Graefes Arch Clin Exp Ophthalmol. 2007; 245: 1107-1121
        • Fard M.A.
        • Abdi P.
        • Kasaei A.
        • Soltani Mogaddam R.
        • Afzali M.
        • Moghimi S.
        Peripapillary choroidal thickness in nonarteritic anterior ischemic optic neuropathy.
        Invest Ophthalmol Vis Sci. 2015;
        • Moghimi S.
        • Afzali M.
        • Akbari M.
        • et al.
        Crowded optic nerve head evaluation with optical coherence tomography in anterior ischemic optic neuropathy.
        Eye (Lond). 2017; 31: 1191-1198
        • Nagia L.
        • Huisingh C.
        • Johnstone J.
        • et al.
        Peripapillary pachychoroid in nonarteritic anterior ischemic optic neuropathy.
        Invest Ophthalmol Vis Sci. 2016; 57: 4679-4685
        • Malmqvist L.
        • Sibony P.A.
        • Fraser C.L.
        • et al.
        Peripapillary ovoid hyperreflectivity in optic disc edema and pseudopapilledema.
        Ophthalmology. 2018; 125: 1662-1664
        • Hayreh S.S.
        • Zimmerman B.
        Visual field abnormalities in nonarteritic anterior ischemic optic neuropathy: their pattern and prevalence at initial examination.
        Arch Ophthalmol. 2005; 123: 1554-1562
        • Chang M.Y.
        • Keltner J.L.
        Risk factors for fellow eye involvement in nonarteritic anterior ischemic optic neuropathy.
        J Neuroophthalmol. 2019; 39: 147-152