Advertisement

Combined anterior ischemic optic neuropathy and hemiretinal artery occlusion during hemodialysis

      Nonarteritic anterior ischemic optic neuropathy (NAION) and retinal artery occlusion cause acute, painless vision loss. Both are ischemic disorders, with NAION involving posterior ciliary arteries supplying the optic nerve head, and retinal artery occlusion implicating the central retinal artery or its downstream branches. Although sharing numerous cardiovascular risk factors,
      • Beatty S.
      • Au Eong K.G.
      Acute occlusion of the retinal arteries: current concepts and recent advances in diagnosis and management.
      • Kerr N.M.
      • Chew S.S.
      • Danesh-Meyer H.V.
      Non-arteritic anterior ischaemic optic neuropathy: a review and update.
      it is rare for these conditions to present concurrently, because insult to 2 distinct vascular territories is required. In this article, we report the simultaneous development of NAION and hemiretinal artery occlusion (HRAO) in the setting of hemodialysis. We ascribe this unique presentation to a collection of risk factors predisposing the patient to the requisite vascular insults.
      A 35-year-old white male with history of migraine, hyperlipidemia, and chronic kidney disease experienced vision loss in the right eye during home hemodialysis. The vision loss was sudden, painless, and limited to the inferior visual field. The hemodialysis was routine with no overt complications. There was no improvement 1 week after onset, at which time the patient presented to clinic.
      On examination, blood pressure was 112/81 mm Hg. The right eye demonstrated 20/25 acuity. There was a right relative afferent pupillary defect. An inferior altitudinal defect was detected with confrontation fields and threshold perimetry (Fig. 1A). Funduscopy revealed a 0.1 cup/disc ratio, peripapillary splinter hemorrhages, segmental disc swelling, and a whitened superior retina (Fig. 1B). Arterial filling of the superior retina was delayed on fluorescein angiography (Fig. 1C). The left eye was completely normal with an optic disc that was healthy but crowded, with cup/disc ratio of 0.1.
      Figure thumbnail gr1
      Fig. 1Right eye on initial presentation. A, Threshold perimetry. B, Fundus photography (arrowhead demonstrates segmental disc swelling). C, Fluorescein angiography.
      Notably, the patient had a history of Hodgkin’s lymphoma 10 years before presentation, successfully treated with chemoradiation. Unfortunate sequelae of that treatment included premature coronary artery disease and kidney failure. The patient did receive a kidney transplant 4 years before presentation, although this was complicated by post-transplant lymphoproliferative disorder. Despite successful treatment, the transplant ultimately failed, with the patient requiring hemodialysis ever since.
      Given this medical history, magnetic resonance imaging and angiography were performed, albeit without gadolinium because of the patient’s kidney failure. No optic nerve compressive or infiltrative lesions were apparent. Vein arterialization was noted at the skull base, although this was deemed incidental as subsequent cerebral angiography was normal, with no dural fistula and no stenotic lesions. Transesophageal echocardiogram showed no masses or thrombi. A hypercoagulability panel performed 2 years prior as work-up for premature coronary artery disease was unremarkable. Hemoglobin obtained 1 month prior was 12.6 gm/dL. This was repeated 2 weeks after presentation and was still normal at 13.8 gm/dL. Optical coherence tomography was not performed.
      On follow-up 7 weeks later, visual acuity of the right eye improved to 20/20. The right relative afferent pupillary defect and inferior altitudinal defect remained present and stable (Fig. 2A). Funduscopy showed resolution of peripapillary hemorrhages, segmental disc swelling, and superior retinal whitening (Fig. 2B). Improved arterial filling of the superior retina was demonstrated on fluorescein angiography (Fig. 2C). The left eye remained completely normal.
      Figure thumbnail gr2
      Fig. 2Threshold perimetry (A), fundus photography (B), and fluorescein angiography (C) of the right eye on 7-week follow-up.
      To our knowledge, this is the first report of simultaneous NAION and HRAO developing during dialysis. NAION is thought to be due to reduced flow in the posterior ciliary artery circulation, which supplies the optic nerve head.
      • Hayreh S.S.
      Ischemic optic neuropathy.
      It typically occurs in patients 60 to 70 years of age.
      • Boghen D.R.
      • Glaser J.S.
      Ischaemic optic neuropathy. The clinical profile and history.
      A wide variety of risk factors and associations have been reported for NAION,
      • Wray S.H.
      The management of acute visual failure.
      some supported by stronger evidence than others. These include hypertension, hyperlipidemia, diabetes mellitus, nocturnal hypotension, hypotension from the use of erectile dysfunction medication, obstructive sleep apnea, and small, crowded optic discs. Notably, 23% of patients with NAION are younger than 50 years.
      • Preechawat P.
      • Bruce B.B.
      • Newman N.J.
      • Biousse V.
      Anterior ischemic optic neuropathy in patients younger than 50 years.
      In these cases, other, less common precipitants of NAION may predominate, including systemic vasculitis,
      • Duran E.
      • Merkel P.A.
      • Sweet S.
      • Swan N.
      • Babikian V.L.
      ANCA-associated small vessel vasculitis presenting with ischemic optic neuropathy.
      hypercoagulability,
      • Acheson J.F.
      • Sanders M.D.
      Coagulation abnormalities in ischaemic optic neuropathy.
      migraine-induced vasospasm,
      • Weinstein J.M.
      • Feman S.S.
      Ischemic optic neuropathy in migraine.
      and perioperative anemia.
      • Stevens W.R.
      • Glazer P.A.
      • Kelley S.D.
      • Lietman T.M.
      • Bradford D.S.
      Ophthalmic complications after spinal surgery.
      Retinal artery occlusion shares many common risk factors with NAION,
      • Wray S.H.
      The management of acute visual failure.
      including cardiovascular disease, iatrogenic hypotension, vasculitis, hypercoagulability, and vasospasm. Embolic disease is an additional important risk factor for retinal artery occlusion.
      • Babikian V.
      • Wijman C.A.
      • Koleini B.
      • Malik S.N.
      • Goyal N.
      • Matjucha I.C.
      Retinal ischemia and embolism. Etiologies and outcomes based on a prospective study.
      Despite shared risk factors, NAION and retinal artery occlusion rarely present concurrently because 2 vascular territories require insult: the posterior ciliary arteries and central retinal artery (or distal branches). The few instances of co-occurrence have transpired mainly in the setting of systemic vasculitis.
      • Emad Y.
      • Basaffar S.
      • Ragab Y.
      • Zeinhom F.
      • Gheita T.
      A case of polyarteritis nodosa complicated by left central retinal artery occlusion, ischemic optic neuropathy, and retinal vasculitis.
      Other cases have occurred in association with cavernous sinus thrombosis
      • Gupta A.
      • Jalali S.
      • Bansal R.K.
      • Grewal S.P.
      Anterior ischemic optic neuropathy and branch retinal artery occlusion in cavernous sinus thrombosis.
      and prostatectomy.
      • Williams G.C.
      • Lee A.G.
      • Adler H.L.
      • et al.
      Bilateral anterior ischemic optic neuropathy and branch retinal artery occlusion after radical prostatectomy.
      The combined NAION and HRAO in our patient were likely multifactorial, a “perfect storm” of risk factors converging to produce the requisite vascular insults. The patient had small, crowded optic discs and a history of hyperlipidemia. He likely possessed a migraine-related predisposition to vasospasm, as well as borderline hypercoagulability from his history of chronic disease and previous malignancy. Hemodialysis almost assuredly had a role despite the patient’s failure to recall any overt complications. Hypotensive episodes in the setting of hemodialysis are associated with NAION,
      • Mullaem G.
      • Rosner M.H.
      Ocular problems in the patient with end-stage renal disease.
      and it is possible that this occurred despite lack of symptoms. Intraocular pressure fluctuations common to hemodialysis
      • Mullaem G.
      • Rosner M.H.
      Ocular problems in the patient with end-stage renal disease.
      may also have occurred, further predisposing the patient to vascular insult.
      Other causes of the patient’s combined NAION and HRAO seem less likely. An infiltrative lesion of the optic nerve or sheath was effectively excluded by the normal magnetic resonance imaging, as well as the stable visual field defect and resolved fundus pathology on follow-up, the latter reflecting the natural history of NAION and retinal artery occlusion, not neoplasm. An underlying clotting disorder is unlikely given no personal or family history of thrombosis, as well as the previously negative hypercoagulability work-up. The cerebral angiogram showed no evidence of ipsilateral carotid or ophthalmic artery disease.
      Overall, a confluence of risk factors provoked insult to 2 vascular territories, resulting in a rare simultaneous presentation of NAION and HRAO.

      Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

      References

        • Beatty S.
        • Au Eong K.G.
        Acute occlusion of the retinal arteries: current concepts and recent advances in diagnosis and management.
        J Accid Emerg Med. 2000; 17: 324-329
        • Kerr N.M.
        • Chew S.S.
        • Danesh-Meyer H.V.
        Non-arteritic anterior ischaemic optic neuropathy: a review and update.
        J Clin Neurosci. 2009; 16: 994-1000
        • Hayreh S.S.
        Ischemic optic neuropathy.
        Prog Retin Eye Res. 2009; 28: 34-62
        • Boghen D.R.
        • Glaser J.S.
        Ischaemic optic neuropathy. The clinical profile and history.
        Brain. 1975; 98: 689-708
        • Wray S.H.
        The management of acute visual failure.
        J Neurol Neurosurg Psychiatry. 1993; 56: 234-240
        • 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
        • Duran E.
        • Merkel P.A.
        • Sweet S.
        • Swan N.
        • Babikian V.L.
        ANCA-associated small vessel vasculitis presenting with ischemic optic neuropathy.
        Neurology. 2004; 62: 152-153
        • Acheson J.F.
        • Sanders M.D.
        Coagulation abnormalities in ischaemic optic neuropathy.
        Eye (Lond). 1994; 8: 89-92
        • Weinstein J.M.
        • Feman S.S.
        Ischemic optic neuropathy in migraine.
        Arch Ophthalmol. 1982; 100: 1097-1100
        • Stevens W.R.
        • Glazer P.A.
        • Kelley S.D.
        • Lietman T.M.
        • Bradford D.S.
        Ophthalmic complications after spinal surgery.
        Spine (Phila Pa 1976). 1997; 22: 1319-1324
        • Babikian V.
        • Wijman C.A.
        • Koleini B.
        • Malik S.N.
        • Goyal N.
        • Matjucha I.C.
        Retinal ischemia and embolism. Etiologies and outcomes based on a prospective study.
        Cerebrovasc Dis. 2001; 12: 108-113
        • Emad Y.
        • Basaffar S.
        • Ragab Y.
        • Zeinhom F.
        • Gheita T.
        A case of polyarteritis nodosa complicated by left central retinal artery occlusion, ischemic optic neuropathy, and retinal vasculitis.
        Clin Rheumatol. 2007; 26: 814-816
        • Gupta A.
        • Jalali S.
        • Bansal R.K.
        • Grewal S.P.
        Anterior ischemic optic neuropathy and branch retinal artery occlusion in cavernous sinus thrombosis.
        J Clin Neuroophthalmol. 1990; 10: 193-196
        • Williams G.C.
        • Lee A.G.
        • Adler H.L.
        • et al.
        Bilateral anterior ischemic optic neuropathy and branch retinal artery occlusion after radical prostatectomy.
        J Urol. 1999; 162: 1384-1385
        • Mullaem G.
        • Rosner M.H.
        Ocular problems in the patient with end-stage renal disease.
        Semin Dial. 2012; 25: 403-407