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Optic Chiasm, Optic Nerve, and Retinal Involvement Secondary to Varicella-zoster Virus
Craig M. Greven, MD;
Tina Singh, MD;
Constance A. Stanton, MD;
Timothy J. Martin, MD
Arch Ophthalmol. 2001;119:608-610.
ABSTRACT
Immunocompromised patients are known to be at risk for varicella-zoster
virus reactivation, often in atypical manners. We describe a 30-year-old man
with simultaneous involvement of the retina, optic chiasm, and optic nerve
with varicella-zoster virus who had a bitemporal visual field defect.
INTRODUCTION
A 30-year-old man with chronic myelogenous leukemia who had undergone
bone marrow transplantation developed blurred vision in both eyes, dizziness,
and mild headaches. He had a bitemporal hemianopia that rapidly progressed
to complete blindness. In his right eye a single focus of deep retinochoroiditis
developed, which over time progressed and became more confluent. Three weeks
later the patient died secondary to massive subarachnoid hemorrhage. Findings
from the postmortem evaluation revealed viral intranuclear inclusions in the
retina and optic nerve, and immunostaining of the optic nerve was positive
for varicella-zoster virus (VZV).
REPORT OF A CASE
In April 1996, a 30-year-old white man received a diagnosis of chronic
myelogenous leukemia that was Philadelphia chromosome positive. In September
1998 he had dizziness, bilateral blurred vision, and slight headaches of 5
days' duration. He had undergone allogenic bone marrow transplantation in
March 1998. His clinical course posttransplantation had been complicated by
graft-vs-host disease, and he was being treated with cyclosporine and prednisone.
On evaluation September 20, 1998, his visual acuity was 20/400 OD and
20/200 OS. No afferent pupillary defect was present, and visual field testing
showed a bitemporal defect (Figure 1). The slitlamp examination findings were normal, and funduscopic examination
of the right eye revealed a gray, deep retinochoroidal infiltrate nasal to
the optic nerve (Figure 2 and Figure 3). The left retina and optic nerve
were normal. Magnetic resonance imaging of the brain revealed an increased
signal in the area of the posterior optic nerve and chiasm (Figure 4).
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Figure 1. Humphrey 30-2 visual field showing
bitemporal visual field loss.
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Figure 2. Fundus photograph of infiltrate
nasal to the optic nerve of the retina of the right eye showing patchy area
of deep retinochoroiditis.
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Figure 3. Late-frame fluorescein angiogram
of the same area as in Figure 2 showing late hyperfluorescence of a deep retinal
lesion.
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Figure 4. Magnetic resonance imaging (1.5
T); flair sequences.) Coronal image with increased signal in the chiasm (arrows),
also seen in the optic nerves.
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Within 1 week the patient had progressive deep and full-thickness areas
of retinitis in the right eye, and he developed 2 small foci of retinitis
in the left eye. In 2 days the patient had no light perception OU. Two weeks
later he experienced a large subarachnoid hemorrhage secondary to pancytopenia
and coagulopathy and died.
RESULTS
Findings from postmortem examination of the brain revealed diffuse multifocal
subarachnoid hemorrhage covering most of the brain, brainstem, and cerebellum.
Sections of the globes showed areas of full-thickness retinal necrosis with
lymphocytic infiltration of the choroid (Figure 5A) and in some areas superficial retinitis with relative
sparing of the deep retina (Figure 5B).
Sections of the optic nerve and retina showed intranuclear inclusions suggestive
of infection with a herpesvirus (Figure 5C). Focal areas of demyelination were present in the nerve with
infiltration of the tissue by macrophages. Immunostains for VZV were strongly
positive (Figure 5D), while immunostains
for herpes simplex virus and cytomegalovirus were negative. Polymerase chain
reaction of the tissue was positive for VZV but negative for the other herpesviruses.
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Figure 5. A, Area of left retina exhibiting
full-thickness retinal necrosis with lymphocytic infiltration of the choroid
(hematoxylin-eosin, original magnification x200). B, A low-power view
of the left retina shows necrosis of the inner layers of the retina with relative
sparing of the photoreceptors (hematoxylin-eosin, original magnification x100).
C, A high-power view of the same area of the left retina in part B shows numerous
viral intranuclear inclusions (arrows) (hematoxylin-eosin, original magnification
x400). D, Immunohistochemical stain for varicella-zoster virus shows
staining of the proximal left optic nerve (original magnification x20).
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COMMENT
The ocular manifestations of VZV infections are protean and include
dermatitis, keratitis, uveitis, retinitis, and optic nerve involvement.1 Patients who are immunocompromised are particularly
susceptible to these complications, and they frequently occur without typical
cutaneous dermatomal eruption. Simultaneous involvement of the retina and
optic nerve from VZV in acute retinal necrosis syndrome is well documented.2 Optic disc edema with associated optic neuropathy
and retrobulbar optic neuritis have been described as preceding the acute
retinal necrosis syndrome in patients with the acquired immunodeficiency syndrome.3-4 Progressive outer retinal necrosis
is another retinal manifestation of VZV that often occurs in patients with
acquired immunodeficiency syndrome, presenting with multiple discrete areas
of deep retinal opacification and progressing to confluent retinal involvement.5
The most common significant viral infections in bone marrow transplant
recipients are due to members of the herpesvirus family.6
Infection with VZV is almost always due to a reactivation of a previous VZV
infection. At 5 years, more than 50% of bone marrow transplant recipients
have clinical infection with VZV.7 The greatest
risk period for development of serious life-threatening VZV infections in
patients undergoing bone marrow transplantation is 2 to 10 months posttransplantation,
with a median occurrence at 5 to 7 months.7-8
The presence of graft-vs-host disease is a major risk factor for VZV reactivation.7
Our patient was severely immunocompromised by his underlying leukemia
and graft-vs-host disease that required therapy with prednisone and cyclosporine.
The atypical chiasmal involvement he had as evidenced by the bitemporal hemianopia
appears to be unique. The diagnosis of VZV infection became more clinically
apparent with the onset and progression of the retinitis in the right eye
and was confirmed by autopsy findings. Varicella-zoster infection should be
considered in immunocompromised patients with retrobulbar optic nerve or chiasmal
lesions, even in the absence of cutaneous or retinal lesions.
AUTHOR INFORMATION
Accepted for publication October 18, 2000.
Corresponding author and reprints: Craig M. Greven, MD, Wake Forest
University Eye Center, Medical Center Boulevard, Winston-Salem, NC 27157-1033
(e-mail: cgreven{at}wfubmc.edu).
From the Wake Forest University Eye Center (Drs Greven, Singh, and
Martin) and the Department of Pathology, Wake Forest University (Dr Stanton),
Winston-Salem, NC.
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ABSTRACT
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8. Locksley RM, Flournoy N, Sullivan KM, Myers JD. Infection with varicella-zoster virus after marrow transplantation. J Infect Dis. 1985;152:1172-1181.
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SECTION EDITOR: W. RICHARD GREEN, MD
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