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Figure 1. Color fundus photograph and fluorescein angiography. A, Color fundus photograph of the left eye taken 10 days after the initial visit showing diffuse retinal edema and sheathing of the threadlike retinal arteries and veins. B, Fluorescein angiography of the left eye showing patchy areas of reperfused peripheral retina.

Figure 1. Color fundus photograph and fluorescein angiography. A, Color fundus photograph of the left eye taken 10 days after the initial visit showing diffuse retinal edema and sheathing of the threadlike retinal arteries and veins. B, Fluorescein angiography of the left eye showing patchy areas of reperfused peripheral retina.

Figure 2. Electroretinography and magnetic resonance angiography. A, International Society for Clinical Electrophysiology of Vision standard electroretinography performed with gold foil recording electrodes, mydriasis, and Ganzfeld stimulation of the right and left eyes compared with a normal control. No electroretinogram is detectable in the patient under any recording conditions (note the different scales in the patient's data). Flash visual evoked potentials (VEPs) are not definitely detectable and show only residual background noise. DA indicates dark adapted; LA, light adapted. B, Short T1 inversion recovery magnetic resonance angiography of the cerebral vasculature demonstrating an absence of signal within the ophthalmic arteries (arrows). Asterisks indicate the internal carotid artery.

Figure 2. Electroretinography and magnetic resonance angiography. A, International Society for Clinical Electrophysiology of Vision standard electroretinography performed with gold foil recording electrodes, mydriasis, and Ganzfeld stimulation of the right and left eyes compared with a normal control. No electroretinogram is detectable in the patient under any recording conditions (note the different scales in the patient's data). Flash visual evoked potentials (VEPs) are not definitely detectable and show only residual background noise. DA indicates dark adapted; LA, light adapted. B, Short T1 inversion recovery magnetic resonance angiography of the cerebral vasculature demonstrating an absence of signal within the ophthalmic arteries (arrows). Asterisks indicate the internal carotid artery.

1.
Askalan R, Laughlin S, Mayank S,  et al.  Chickenpox and stroke in childhood: a study of frequency and causation.  Stroke. 2001;32(6):1257-1262PubMedArticle
2.
Hall S, Carlin L, Roach ES, McLean WT Jr. Herpes zoster and central retinal artery occlusion.  Ann Neurol. 1983;13(2):217-218PubMedArticle
3.
Gilden DH, Lipton HL, Wolf JS,  et al.  Two patients with unusual forms of varicella-zoster virus vasculopathy.  N Engl J Med. 2002;347(19):1500-1503PubMedArticle
4.
Gilden D, Cohrs RJ, Mahalingam R, Nagel MA. Varicella zoster virus vasculopathies: diverse clinical manifestations, laboratory features, pathogenesis, and treatment.  Lancet Neurol. 2009;8(8):731-740PubMedArticle
5.
Zamora RL, del Priore LV, Storch GA, Gelb LD, Sharp J. Multiple recurrent branch retinal artery occlusions associated with varicella zoster virus.  Retina. 1996;16(5):399-404PubMedArticle
6.
Nagel MA, Cohrs RJ, Mahalingam R,  et al.  The varicella zoster virus vasculopathies: clinical, CSF, imaging, and virologic features.  Neurology. 2008;70(11):853-860PubMedArticle
7.
Hilt DC, Buchholz D, Krumholz A, Weiss H, Wolinsky JS. Herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: diagnosis and management approaches.  Ann Neurol. 1983;14(5):543-553PubMedArticle
Research Letters
Nov 2012

Bilateral Ophthalmic Artery Occlusions Due to Probable Varicella-Zoster Virus Vasculopathy

Author Affiliations

Author Affiliations: NIHR Biomedical Research Centre for Ophthalmology, UCL Institute of Ophthalmology and Moorfields Eye Hospital (Dr Jayaram) and Department of Electrophysiology, Moorfields Eye Hospital (Dr Holder), and Medical Eye Unit, St Thomas' Hospital (Dr Graham), London, England; and Academisch Ziekenhuis Maastricht, Maastricht, the Netherlands (Dr Stanescu-Segal).

Arch Ophthalmol. 2012;130(11):1492-1494. doi:10.1001/archophthalmol.2012.544

Varicella-zoster virus (VZV) vasculopathies account for almost one-third of arterial ischemic strokes in children.1 Visual complications are rare, with previous reports occurring secondary to unilateral central retinal artery2 or posterior ciliary artery3 occlusion. We describe the first case, to our knowledge, of an immunocompetent child who became blind due to bilateral ophthalmic artery occlusions secondary to probable VZV vasculopathy.

Report of a Case

A 6-year-old boy visited his local emergency department with a history of sudden painless bilateral visual loss. He was otherwise in good health apart from a history of chickenpox 8 weeks previously. His father had been treated for culture-negative mediastinal tuberculosis a year earlier. Initial examination in the emergency department showed visual acuity of counting fingers OU, a moderate bilateral panuveitis, and diffuse bilateral retinal edema with sheathing of both retinal arteries and veins. The optic discs were not swollen. Neurological examination findings were otherwise normal. Initial management aimed to treat a possible tuberculosis optic neuropathy and/or vasculopathy, using oral prednisolone, rifampin, pyrazinamide, and isoniazid. Initial investigations showed no abnormality on hematology and biochemistry tests of peripheral blood. Cytomegalovirus and VZV IgG were both detected on testing of serum, but polymerase chain reaction results for the respective DNA were negative. Evidence of tuberculosis infection was not found, with negative results on both enzyme-linked immunosorbent spot and Heaf tests and normal findings on chest radiography. Magnetic resonance imaging demonstrated no abnormal enhancement in the brain or chiasm, with normal optic nerve appearances. During the next week, visual acuity deteriorated to light perception OU. Panuveitis with retinal vascular sheathing (Figure 1A) persisted, with patchy areas of reperfused retina observed peripherally in the left eye on fluorescein angiography (Figure 1B). Doppler ultrasonography showed no detectable flow in central retinal artery or central retinal vein. Polymerase chain reaction results for a vitreous biopsy specimen were negative for both VZV DNA and tuberculosis. Electrophysiology showed a completely undetectable electroretinogram in both eyes, indicative of loss of outer retinal photoreceptor function and thus not in keeping with dysfunction confined to central retinal artery or vein circulation (Figure 2A). Magnetic resonance angiography showed a mostly normal cerebral vasculature but neither ophthalmic artery could be visualized (Figure 2B), in keeping with the electrophysiological data. Subsequently, VZV IgG was detected in cerebrospinal fluid, confirming VZV as the likely cause of the vasculopathy. Despite a course of systemic acyclovir and intravenous methylprednisolone, he maintains visual acuity of light perception OU.

Comment

Vasculopathy caused by VZV can occur after both reactivation (zoster) and primary VZV infection and may involve both large and small cerebral arteries.4 Visual loss, previously reported to affect only the posterior ciliary artery2 and central retinal artery3 unilaterally, is rare but devastating. Branch retinal arterial occlusions in an immunocompetent adult have been reported in association with posterior uveitis and the presence of VZV DNA detected in the vitreous by polymerase chain reaction.5 However, VZV IgG and VZV DNA were absent in the cerebrospinal fluid in that case and subsequent investigations confirmed isolated ocular involvement.

Detection of VZV IgG in the cerebrospinal fluid has been shown to be a more sensitive and specific marker for the diagnosis of VZV vasculopathy compared with detection of VZV DNA by polymerase chain reaction alone,4 where a negative result does not exclude the diagnosis.4 The diagnosis in the present case was challenging owing to the absence of cerebrospinal fluid pleocytosis (present in one-third of cases6) and the presence of normal brain magnetic resonance imaging findings (unusual in VZV vasculopathy3).

Prompt diagnosis of VZV vasculopathy is crucial, with an untreated mortality rate reported at 25%.7 Although ocular complications may be irreversible after several weeks, treatment with systemic acyclovir may prevent further neurological complications and loss of life.3

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Article Information

Correspondence: Dr Jayaram, NIHR Biomedical Research Centre for Ophthalmology, UCL Institute of Ophthalmology and Moorfields Eye Hospital, 11-43 Bath St, London EC1V 9EL, England (h.jayaram@ucl.ac.uk).

Author Contributions: Dr Graham had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Conflict of Interest Disclosures: None reported.

References
1.
Askalan R, Laughlin S, Mayank S,  et al.  Chickenpox and stroke in childhood: a study of frequency and causation.  Stroke. 2001;32(6):1257-1262PubMedArticle
2.
Hall S, Carlin L, Roach ES, McLean WT Jr. Herpes zoster and central retinal artery occlusion.  Ann Neurol. 1983;13(2):217-218PubMedArticle
3.
Gilden DH, Lipton HL, Wolf JS,  et al.  Two patients with unusual forms of varicella-zoster virus vasculopathy.  N Engl J Med. 2002;347(19):1500-1503PubMedArticle
4.
Gilden D, Cohrs RJ, Mahalingam R, Nagel MA. Varicella zoster virus vasculopathies: diverse clinical manifestations, laboratory features, pathogenesis, and treatment.  Lancet Neurol. 2009;8(8):731-740PubMedArticle
5.
Zamora RL, del Priore LV, Storch GA, Gelb LD, Sharp J. Multiple recurrent branch retinal artery occlusions associated with varicella zoster virus.  Retina. 1996;16(5):399-404PubMedArticle
6.
Nagel MA, Cohrs RJ, Mahalingam R,  et al.  The varicella zoster virus vasculopathies: clinical, CSF, imaging, and virologic features.  Neurology. 2008;70(11):853-860PubMedArticle
7.
Hilt DC, Buchholz D, Krumholz A, Weiss H, Wolinsky JS. Herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: diagnosis and management approaches.  Ann Neurol. 1983;14(5):543-553PubMedArticle
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