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.
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.
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: email@example.com).
Verm AM, Scott IU, Davis JL. Necrotizing Herpetic Retinopathy Associated With Ramsay Hunt Syndrome. Arch Ophthalmol. 2002;120(7):989-990. doi: