Ophthalmic Surgery, Lasers and Imaging Retina

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Case Report 

Unilateral Acute Retinal Necrosis 2 Months After Herpes Simplex Encephalitis

Kazuhiko Sugitani, MD; Yoshio Hirano, MD; Tsutomu Yasukawa, MD, PhD; Munenori Yoshida, MD, PhD; Yuichiro Ogura, MD, PhD

Abstract

A case with unilateral acute retinal necrosis (ARN) following herpes simplex virus (HSV) encephalitis was reported. A 40-year-old man presented with unilateral visual loss. He had a history of HSV encephalitis 2 months previously, and had been successfully treated with intravenously acyclovir. The ophthalmologic examination in his left eye suggested ARN syndrome. The best-corrected visual acuity was 20/222. Magnetic resonance imaging (MRI) showed high density signals from the optic chiasm to optic disc. The patient underwent encircling scleral buckling, lensectomy, and vitrectomy with endolaser photocoagulation and silicone oil tamponade. Postoperatively, the high density signals on MRI decreased, the retina maintained attached, and the visual acuity at the final visit improved to 20/20. Unilateral ARN can develop after HSV encephalitis. Acyclovir should be administered over the long term to control the virus.

Abstract

A case with unilateral acute retinal necrosis (ARN) following herpes simplex virus (HSV) encephalitis was reported. A 40-year-old man presented with unilateral visual loss. He had a history of HSV encephalitis 2 months previously, and had been successfully treated with intravenously acyclovir. The ophthalmologic examination in his left eye suggested ARN syndrome. The best-corrected visual acuity was 20/222. Magnetic resonance imaging (MRI) showed high density signals from the optic chiasm to optic disc. The patient underwent encircling scleral buckling, lensectomy, and vitrectomy with endolaser photocoagulation and silicone oil tamponade. Postoperatively, the high density signals on MRI decreased, the retina maintained attached, and the visual acuity at the final visit improved to 20/20. Unilateral ARN can develop after HSV encephalitis. Acyclovir should be administered over the long term to control the virus.

Unilateral Acute Retinal Necrosis 2 Months After Herpes Simplex Encephalitis

From the Department of Ophthalmology, Nagoya City University Medical School, Nagoya, Japan.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Yoshio Hirano, Department of Ophthalmology & Visual Science, Nagoya City University Graduate School of Medical Sciences, 1-Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan.

Accepted: April 27, 2008
Posted Online: March 09, 2010

Introduction

Acute retinal necrosis (ARN) syndrome, one of the most serious intraocular diseases, is diagnosed based on a clinical triad of vitreous inflammation, occlusive vasculopathy, and progressive retinal necrosis.1 The visual outcomes in eyes with ARN syndrome have not been satisfactory because of retinal detachment, optic nerve dysfunction, or macular abnormalities.2 Vitreoretinal surgery is effective for repairing rhegmatogenous retinal detachment. More recent studies3,4 have reported encouraging results of prophylactic vitrectomy before rhegmatogenous retinal detachment occurs.

ARN syndrome may be associated with herpes simplex virus (HSV) or varicella zoster virus (VZV). The herpes virus, once it infects the retinal neurons, may cause ARN syndrome in healthy individuals and immunocompromised patients.5–7 The syndrome may occur in association with herpetic dermatitis,8 keratitis,9 and encephalitis.10 Maertdorf et al. identified the same viral strains in the aqueous and the cerebrospinal fluid (CSF) samples using nucleotide sequence analyses, suggesting transneuronal spread from the brain to the eye.11

We report a case of ARN syndrome that occurred 2 months after the onset of HSV encephalitis in which prophylactic vitrectomy with lensectomy, endolaser photocoagulation, and silicone oil tamponade preserved good vision.

Case Report

A 40-year-old man presented to another hospital with headache and subsequent confusion on August 27, 2007. Analysis of the CSF showed a white blood cell count of 104/mm3 (monocytes, 96%), a glucose level of 55 mg/dl, and a protein level of 66 mg/dl. Magnetic resonance imaging (MRI) showed nonspecific enhancement of the right temporal area (Fig. 1). Although polymerase chain reaction (PCR) studies did not detect HSV-1 or 2 in the CSF, the patient was diagnosed with HSV encephalitis on the basis of behavioral and psychological symptoms. He was treated with 500 mg of intravenous acyclovir every 8 hours for 3 weeks. He gradually became alert and oriented with normal motor and sensory functions within 24 hours of treatment. He was discharged on October 5, 2007.

An MRI Scan Shows Nonspecific Enhancement of the Right Temporal Area (arrow).

Figure 1. An MRI Scan Shows Nonspecific Enhancement of the Right Temporal Area (arrow).

Ten days later, the patient presented to the Department of Ophthalmology in our hospital with a 5-day history of decreased vision in the left eye. The best-corrected visual acuity (BCVA) was 20/222 in his left eye. Figure 2 shows the clinical findings. The slit-lamp examination showed a ciliary injection with episcleritis, aqueous cells, keratic precipitates, and notable vitreitis, which caused blurred vision. The fundus was affected diffusely by peripheral necrotizing retinopathy, retinal hemorrhage, and occlusive vasculopathy. Fundus fluorescein angiography showed hyperfluorescence associated with vasculopathy and a necrotic retina. The right eye was normal, MRI scans showed abnormal enhancement of the optic nerve and the optic chiasm. Based on this clinical appearance, the patient was diagnosed with unilateral ARN syndrome. He was admitted to our hospital the same day. An aqueous humor specimen was obtained and subjected to PCR amplification to detect cytomegalovirus, Epstein-Barr virus, HSV types 1 and 2, VZV, and toxoplasmosis. The sample was positive for HSV type 1. Beginning on October 16, the patient was treated with 600 mg of intravenous acyclovir every 8 hours, 30 mg/day of oral prednisolone, and 100 mg/day of oral aspirin. On posttreatment day 16, because the fundus lesion was still progressing, the patient underwent prophylactic vitrectomy with encircling sclera buckling, lensectomy, endolaser photocoagulation, and silicone oil tamponade. A vitreous specimen collected intraoperatively was positive for HSV-1 on PCR amplification. The patient was treated postoperatively with 500 mg of oral acyclovir every 8 hours for 4 weeks. He was discharged after 34 days of hospitalization.

(A) A Slit-Lamp Photograph of the Anterior Segment of the Left Eye Shows Ciliary Injection with Episcleritis. (B) A Slit-Lama Photograph of the Anterior Segment of the Left Eye Shows Keratic Precipitates. (C) A Fundus Photograph of the Left Eye Shows Occlusive Vasculopathy (arrow) and Retinal Necrosis (arrowhead). (D) Fundus Fluorescein Angiography Shows Staining Associated with the Vasculopathy (arrow) and a Necrotic Retina (arrowhead). (E) An Enhanced T1-Weighted MRI Scan Shows Abnormal Enhancement of the Optic Nerve (arrows). (F) A Post-Contrast T1-Weighted MRI Scan Shows Abnormal Enhancement of the Optic Chiasm (arrow).

Figure 2. (A) A Slit-Lamp Photograph of the Anterior Segment of the Left Eye Shows Ciliary Injection with Episcleritis. (B) A Slit-Lama Photograph of the Anterior Segment of the Left Eye Shows Keratic Precipitates. (C) A Fundus Photograph of the Left Eye Shows Occlusive Vasculopathy (arrow) and Retinal Necrosis (arrowhead). (D) Fundus Fluorescein Angiography Shows Staining Associated with the Vasculopathy (arrow) and a Necrotic Retina (arrowhead). (E) An Enhanced T1-Weighted MRI Scan Shows Abnormal Enhancement of the Optic Nerve (arrows). (F) A Post-Contrast T1-Weighted MRI Scan Shows Abnormal Enhancement of the Optic Chiasm (arrow).

Figure 3 shows the postoperative findings. Three months after treatment, the high-density signals on MRI decreased. Six months after the primary surgery, the silicone oil was removed and an intraocular lens was implanted. Postoperatively, the retina remained attached without active inflammatory and hemorrhagic lesions. Fundus fluorescein angiography showed decreased hyperfluorescence. The BCVA recovered to 20/20 (Fig. 4).

(A) High-Density Signals of the Optic Disc on MRI Are Decreased After Treatment (arrows). (B) High-Density Signals of the Optic Chiasm on MRI Are Decreased After Treatment (arrow). (C) A Fundus Photograph Shows that the Retina in the Left Eye Is Attached Under Silicone Oil. Spots of Laser Photocoagulation in the Posterior Areas of Active Retinitis Are Observed. (D) Fundus Fluorescein Angiography Shows Decreased Staining.

Figure 3. (A) High-Density Signals of the Optic Disc on MRI Are Decreased After Treatment (arrows). (B) High-Density Signals of the Optic Chiasm on MRI Are Decreased After Treatment (arrow). (C) A Fundus Photograph Shows that the Retina in the Left Eye Is Attached Under Silicone Oil. Spots of Laser Photocoagulation in the Posterior Areas of Active Retinitis Are Observed. (D) Fundus Fluorescein Angiography Shows Decreased Staining.

A Fundus Photograph Shows that the Retina in the Left Eye is Attached, the Optic Disc Is Normal, and the Macula is Intact.

Figure 4. A Fundus Photograph Shows that the Retina in the Left Eye is Attached, the Optic Disc Is Normal, and the Macula is Intact.

Discussion

The herpes virus family, especially HSV and VZV, is associated with ARN syndrome11; these viruses are also important causes of encephalitis. Most infections in adults are caused by HSV-1, although HSV-2 also causes encephalitis.12 While most cases of ARN are caused by VZV and only a minority by HSV,13 thus far all cases of ARN associated with herpetic encephalitis have been reported to be caused by HSV, a finding that supports the possibility of brain-to-retina infection by HSV. The time between the diagnosis of encephalitis and the onset of ocular symptoms can be as long as 1 to 5 months,13–16 but an interval of up to 20 years has been reported.17

In cases of ARN syndrome that are concurrent with herpetic encephalitis, brain-to-eye transmission of the herpes virus has been proposed as the pathogenic mechanism.13,14 In an animal model, injection of recombinant HSV into the anterior chamber of one eye results in retinitis in the fellow eye through viral migration along an anatomic pathway involving the hypothalamus and contralateral optic nerve; the virus presumably migrated from the suprachiasmatic nucleus to the retina by retrograde axonal transport through the optic nerve.18 In the current report, the MRI scans showed a lesion of the optic chiasm but no optic disc swelling. The reactivated virus in the inferior frontal lobe and optic chiasm did not migrate to the retina by axonal transport through the optic nerve; the virus might have been reactivated in the retina and the optic chiasm. Three months after treatment, the high-density MRI signals decreased. HSV is not eradicated by acyclovir but suppressed to a level at which the host immunity is in balance with the viral replication; lengthy years between the onset of ARN in one eye and involvement of the second eye have been reported.19 Therefore, some clinicians now recommend year-long acyclovir treatment after diagnosis of ARN. We should administer acyclovir at least until the activated lesions disappear from the MRI scans.

The visual outcomes in patients with ARN are often unsatisfactory because of retinal detachment, optic nerve dysfunction, or macular abnormality.11 Retinal detachment is a frequent complication of the ARN syndrome. The incidence of retinal detachment depends on the extent of the peripheral retinal involvement and the degree of vitritis; the incidence has been reported to be as high as 85% despite the efficacy of acyclovir in resolving the retinitis.20–24 Several studies have reported the benefit of prophylactic laser photocoagulation posterior to areas of active retinitis.25,26 McDonald et al. reported that 3 eyes treated prophylactically with laser developed a posterior retinal detachment.27 In one of these eyes, the break developed outside the area of the demarcated retina, presumably in uninfected retina. Once retinal detachment occurs, vitrectomy is difficult to perform because of the iatrogenic retinal breaks resulting from removal of the attached vitreous from the necrotic detached retina. We performed a prophylactic vitrectomy with encircling scleral buckling, lensectomy, endolaser photocoagulation, and silicone oil tamponade. The retina remained attached, and the patient obtained good VA.

We watched changes over time in the high-density signals on MRI from the optic nerve to the optic chiasm. It is unclear whether the high signals means that the lesion is still active, but administration of acyclovir should continue until the high signals disappear.

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Authors

From the Department of Ophthalmology, Nagoya City University Medical School, Nagoya, Japan.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Yoshio Hirano, Department of Ophthalmology & Visual Science, Nagoya City University Graduate School of Medical Sciences, 1-Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan.

10.3928/15428877-20100215-50

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