A healthy 18-year-old man presented with a 2-week history of chickenpox, and was treated with oral acyclovir for 5 days. Ten days after onset of the cutaneous vesicular eruptions, he experienced blurring of vision in his right eye that descended to the level of hand motion (HM) in 6 hours. At presentation, 4 days after blurred vision, visual acuity in his right eye was hand movement. Examination of the anterior segments was unremarkable except for an obvious relative afferent pupillary defect. His extraocular muscle examination was normal. Fundus examinations revealed an edematous disc, retinal whitening and edema along the posterior pole, and the presence of a cherry red spot; no retinal infiltration or vitreous opacity was observed (). His left eye was normal with 20/20 vision. Optical coherence tomography showed increased thickness and hyperreflectivity in the inner retina layer, consistent with inner retina infarction. The early phase of fundus fluorescein angiography demonstrated delayed filling of the optic disc with minimal leakage from the small retinal arteriole, which was more prominent in the macular and optic disc regions. Minimal capillary dropout in the macular area was also noted. Staining of small vessels was observed in the late phase of the angiogram (–). A multifocal electroretinogram (ERG) showed diminished waveform in the entire macular area (). A full field ERG showed decreased b-wave amplitude of both rod and cone response and oscillatory potential wave. Electronegative ERG pattern was evidenced in maximal combined ERG that represented a generalized ischemic process, which involved mainly the inner retina layer (). The patient demonstrated decreased amplitude and increased latency in flash visual evoked potential (VEP), which is consistent with generalized involvement of the entire retina, not limited only to the posterior pole ().\nMinimal increased signal in T2-weighted magnetic resonance imaging and enhancing with contrast limited to the anterior part of the optic nerve could represent an inflammatory response to the ischemic process of the optic nerve head (). Serologic tests were positive for both immunoglobulin (Ig)M and IgG anti-VZV antibodies, confirming recent primary VZV infection. The diagnosis of ischemic vasculitis of the small retinal vessels associated with chickenpox was made. Since this process could either be caused by infection or an immunological response to VZV infection, and there is no consensus on the optimal treatment regimen for this VZV-associated vasculopathy, either treated with antiviral alone or combined with corticosteroid, we decided to treat the patient with antiviral agent according to the acute retinal necrosis treatment guideline published by the American Uveitis Society, ie, with intravenous Acyclovir 10 mg/kg every 8 hours for 2 weeks in conjunction with intravenous methylprednisolone 1 g daily for 3 days, then with oral prednisolone tapering over 1 month and oral Acyclovir 800 mg 5 times daily for 1 month. Despite the treatment, a pale optic disc and attenuated retinal artery resulted. His final best-corrected visual acuity was counting fingers at 1 foot at 6 months’ follow-up.
A 28-year-old caucasian woman presented to the emergency room with a history of a pressure-type frontal headache during the previous week; there was no exacerbation when lying down nor with Valsalva maneuvers and no accompanying features, namely, visual symptoms. Medical history was unremarkable except for venous thrombosis on her left leg 5 years before. She was not taking any drugs other than oral contraceptives. She had a son who was diagnosed with chickenpox two weeks before. She was febrile (temperature of 38°C), and there was a rash suggestive of chickenpox. Uncorrected visual acuity was 20/20 in both eyes, and the optic discs were swollen and hyperemic, with ophthalmological and neurological examinations otherwise normal. Two days later, she complained of ocular pain on eye movement and bilateral blurred vision. By that time, examination revealed reduced visual acuity of 20/32 in her right eye (OD) and 20/60 in her left eye (OS) and relative afferent pupillary defect on her left eye. She reported a difference in brightness of the red color, with less saturation in the left eye.\nRoutine blood testing and immunological workup (ANA, ANCA, anticardiolipin antibodies, and complement levels) were normal. HIV serology was negative as well as VDRL, Toxoplasma, Bartonella, and Borrelia antibodies.\nCerebral computed tomography (CT) scan and magnetic resonance imaging (MRI) were normal.\nCerebrospinal fluid (CSF) examination was unremarkable: clear fluid with a normal opening pressure of 130–140 mm H2O with no block when pressing the jugular vein, normal protein and glucose levels, less than 5 leukocytes, and no oligoclonal bands. Antibodies for the following microorganisms were negative in the CSF: Cytomegalovirus, Epstein Barr virus, Parvovirus, Herpes simplex virus, Varicella zoster virus, Mycoplasma pneumoniae, Borrelia burgdorferi, and Leptospira species. VDRL was also negative in the CSF.\nGoldmann perimetry showed an enlarged blind spot and constriction of the nasal field, especially in her left eye (). Farnsworth Munsell 100 color vision test was abnormal in both eyes (in the red axis), with more pronounced defect in her left eye.\nShe was started on acyclovir 4 g/day per os, as is indicated to treat adult chickenpox.\nTwo days after the initial visual symptoms, visual acuity began to increase, and one week later, the optic disc edema had almost resolved (). Visual acuity continued to improve, and two months later, she had a visual acuity of 20/20 bilaterally. Retinal fluorescein angiography one month after presentation was normal. She has been free of further attacks of optic neuritis for three years after the admission.