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Clinicopathologic Reports, Case Reports, and Small Case Series
July 2002

Necrotizing Herpetic Retinopathy Associated With Ramsay Hunt Syndrome

Arch Ophthalmol. 2002;120(7):989-990. doi:

Ramsay Hunt syndrome is a herpes zoster infection of cranial nerve VII, causing facial paralysis, and may also involve cranial nerves VIII, IX, V, X, and VI, in order of decreasing frequency.1 Necrotizing herpetic retinopathy refers to the spectrum of disease encompassing acute retinal necrosis and progressive outer retinal necrosis, and consists of peripheral necrotizing retinitis, vitritis, and retinal arteritis caused by a herpes virus.2 To our knowledge, we describe the first case of bilateral necrotizing herpetic retinopathy in an immunosuppressed patient with Ramsay Hunt syndrome.

Report of a Case

A 38-year-old woman with acquired immunodeficiency syndrome (CD4 cell count 2 months prior was 214/µL) had vesicular lesions in the distribution of left cranial nerve V3, left-sided hearing loss, left facial weakness, and decreased vision in the left eye. Visual acuity with pinhole was 20/25 OD and 20/30 OS. Slitlamp examination revealed anterior vitreous cells in the left eye. Results of dilated fundus examination of the right eye were unremarkable and in the left eye revealed focal and confluent retinitis inferiorly and temporally. Necrotizing herpetic retinopathy in the left eye was diagnosed, the retinitis was demarcated with laser treatment, and intravenous ganciclovir and foscarnet were initiated. Intravitreal ganciclovir sodium (2000 µg/0.05 mL) and foscarnet sodium (1200 µg/0.05 mL) were administered in the left eye 3 times per week for a total of 7 injections. Audiography demonstrated left-sided neuronal hearing loss.

Five days later, the patient reported decreased vision in the right eye and right-sided hearing loss. Visual acuity was 20/40 OD and 20/50 OS. Dilated fundus examination revealed focal areas of peripheral retinitis in the right eye and stable retinitis in the left eye. Intravitreal ganciclovir sodium (2000 µg/0.05 mL) and foscarnet sodium (1200 µg/0.05 mL) were administered in the right eye 3 times per week for a total of 5 injections. Audiography demonstrated bilateral hearing loss, and magnetic resonance imaging revealed neuritis in bilateral cranial nerve VIII. On serial dilated fundus examinations, the confluent retinitis did not break through the laser demarcation in the right eye, and the focal lesions did not become confluent in the left eye. After 2 weeks of intravenous and intravitreal therapy, the retinitis was healed in both eyes and visual acuity stabilized at 20/50 OD and 20/100 OS. Two months later, after discontinuing systemic medication against medical advice, the patient developed recurrent necrotizing herpetic retinopathy with retinal detachments in both eyes. After intravenous and intravitreal antiviral therapy and retinal detachment repair with vitrectomy and silicone oil tamponade in both eyes, visual acuity was 20/70 OD and 20/40 OS. Nine months after initial examination, visual acuity was stable at 20/300 OD and 20/80 OS, with bilateral posterior subcapsular cataracts.

Comment

In 1907, Ramsay Hunt described facial nerve palsy associated with ipsilateral hearing loss and vesicular lesions on the pinna or in the auditory canal.1 Facial weakness with associated vesicles establishes the diagnosis of Ramsay Hunt syndrome.3 The benefit of systemic steroids is equivocal; intravenous acyclovir sodium is indicated in patients with immunosuppression or encephalitis.3 Most patients regain function of cranial nerves VII and VIII.3 To our knowledge, 1 case of necrotizing herpetic retinopathy associated with Ramsay Hunt syndrome has been reported.4 In contrast to our case, the previously described patient was immunocompetent and developed unilateral necrotizing herpetic retinopa thy. Systemic ganciclovir and foscarnet were administered to our patient because it has been reported that, among patients with acquired immunodeficiency syndrome who develop necrotizing herpetic retinopathy, combination therapy with intravenous ganciclovir and foscarnet is associated with better visual outcomes than treatment with intravenous acyclovir or intravenous foscarnet alone.5 Ophthalmologists should monitor the fundus for retinitis in any patient with Ramsay Hunt syndrome and decreased vision.

This research was supported in part by Research to Prevent Blindness Inc, New York, NY.

Corresponding author: Janet L. Davis, MD, Bascom Palmer Eye Institute, 900 NW 17th St, Miami, FL 33136 (e-mail: jdavis@bpei.med.miami.edu).

References
1.
Aviel  AMarshak  G Ramsay Hunt syndrome: a cranial polyneuropathy. Am J Otolaryngol. 1982;361- 66Article
2.
Guex-Crosier  YRochat  CHerbort  CP Necrotizing herpetic retinopathies: a spectrum of herpes-virus induced diseases determined by the immune state of the host. Ocul Immunol Inflamm. 1997;5259- 265Article
3.
Birinyi  F Facial weakness and rash. Acad Emerg Med. 1996;31144- 11451153- 1155Article
4.
Yeo  JHPepose  JSStewart  JASternberg  PLiss  RA Acute retinal necrosis syndrome following herpes zoster dermatitis. Ophthalmology. 1986;931418- 1422Article
5.
Moorthy  RSWeinberg  DVTeich  SA  et al.  Management of varicella zoster virus retinitis in AIDS. Br J Ophthalmol. 1997;81189- 194Article
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