Polymerase chain reaction (PCR) for direct detection of viral DNA is highly sensitive with 80% to 96% positivity in both immunocompetent and immunocompromised hosts.
Indirect detection of antibodies directed against virus proteins has 50% to 70% positivity in immunocompetent patients.
TABLE 9-1. Necrotizing Retinopathies
The goal of systemic antiviral therapy is to control viral replication and to reduce the risk of bilateral involvement. Intravitreal antiviral injections can be used in cases that are imminently threatening the macula or optic nerve, as a combined approach.
The combination of systemic and intravitreal therapy increasingly has been reported as a treatment paradigm in cases of ARN; however, further studies are necessary to define the exact role of intravitreal therapy in the management of ARN.
TABLE 9-2. Medical Treatment of ARN
Anti-inflammatory therapy is used to minimize the deleterious effects associated with inflammation. Viral replication must be under control with antiviral medications before administration of systemic corticosteroids.
Whenever an infectious viral uveitis is a diagnostic consideration, systemic and local corticosteroids should be used with extreme care (not only because of exacerbation of ARN but also because of the development of de novo ARN related to corticosteroid use).
Antithrombotic therapy should be considered in order to reduce vascular complications; however, strong evidence supporting its use does not currently exist.
TABLE 9-3. Clinical Characteristics of ARN and PORN Syndromes
TABLE 9-4. Medical Treatment of PORN
FIGURE 9-1. Acute retinal necrosis. Granulomatous anterior uveitis in a patient with acute retinal necrosis syndrome.
FIGURE 9-2. Acute retinal necrosis. There are multiple white to yellow-white foci of peripheral retinal necrosis in a case of acute retinal necrosis syndrome. These occur in a circumferential manner.
FIGURE 9-3. Acute retinal necrosis. This 48-year-old patient presented with sudden onset of blurred vision and floaters. A. There are numerous focal white areas of retinitis and vasculitis in the periphery along with optic disc edema.
FIGURE 9-4. Acute retinal necrosis. This patient with extensive acute retinal necrosis developed a retinal detachment.
FIGURE 9-5. Toxoplasma retinochoroiditis. This person had extensive toxoplasma retinochoroiditis masquerading as acute retinal necrosis.
FIGURE 9-6. Primnary intraocular lymphoma. Pseudo-acute retinal necrosis syndrome with dense vitritis, retinal necrosis, and hemorrhages in a case of primary intraocular lymphoma.
FIGURE 9-7. Progressive outer retinal necrosis. Varicella zoster virus-associated progressive outer retinal necrosis with retinal necrosis and retinal hemorrhages in an AIDS patient.
FIGURE 9-8. Progressive outer retinal necrosis. Multifocal areas of outer retinal necrosis in a patient with progressive outer retinal necrosis.
Patent ductus arteriosus
Pulmonary artery stenosis
Atrial and ventricular septal defects
The most common finding is progressive sensorineural deafness, which occurs in 44% of cases.
Vestibular function is rarely impaired.
Moderate-to-severe mental retardation
Schizophrenia-like clinical picture
Intrauterine growth retardation
Failure to thrive
Insulin-dependent diabetes mellitus
Cataract (usually bilateral, occasionally unilateral) occurs in the majority of patients.
Salt and pepper retinopathy occurs in approximately 20% of patients. The appearance ranges from a fine stippling of the retinal pigment epithelium (RPE) to dark, patchy areas, and are most prominent in the posterior pole.
Congenital glaucoma (in 10% of cases)
Microphthalmos (occurs in 10% of cases)
Corneal edema in the absence of a raised IOP
FIGURE 9-11. Congenital rubella syndrome. A. This child had bilateral cornea clouding, and is small for gestational age. B. A higher magnification of another child with cloudy corneas.
FIGURE 9-13. Congenital rubella syndrome. This child has corneal scarring and buphthalmos as a result of congenital glaucoma.
FIGURE 9-14. Congenital rubella syndrome. There is a mature cataract in the right eye and an early cataract in the left eye.
lesions will show “target-shaped” focal areas of central hypofluorescence surrounded by hyperfluorescence.
Acute fever with headache, fatigue, myalgia, and diffuse maculopapular rash
Polyarticular and migratory joint pains
Photophobia, red eye, blurred vision, floaters, and retro-orbital pain
Loss of vision, color vision defect, central or centrocecal scotoma, and peripheral field defects
Treatment is mainly supportive, including rest, nonsteroidal anti-inflammatory agents, and corticosteroids for refractory arthritis.
Topical steroid eye drops for anterior uveitis
Treating confluent retinitis with IV/oral acyclovir and oral prednisone may be tried. However, efficacy of acyclovir against this virus is not yet established.
FIGURE 9-22. There are multiple, diffuse, pigmented keratic precipitates in chikungunya anterior uveitis.
Most commonly results in either an asymptomatic infection or self-limited symptoms (fever, rash, arthralgias, myalgias, and headache)
Thought to be associated with Guillain-Barré syndrome
Conjunctivitis (common symptom, often part of mild systemic syndrome described earlier)
Reports of association with both anterior and posterior uveitis (blurred vision, eye pain, photopsias, and red eye)
Intracranial calcifications (present between cortex and subcortical white matter)
Congenital brain abnormalities
Arthrogryposis (joint contractures)
Ophthalmic (Fig. 9-25)
▶ Most common
Chorioretinal atrophy (excavated appearance is typical)
RPE pigment mottling
Optic nerve abnormalities (severe optic disc cupping and hypoplasia)
▶ Other congenital ophthalmic findings that have been described
Mild anterior uveitis
Mild vitreous inflammation
Chorioretinal lesions (Fig. 9-25)
Other TORCH infections
▶ T. gondii
▶ Others (syphilis, HIV, West Nile virus, VZV, Epstein-Barr virus)
Maternal exposure to teratogens (alcohol, tobacco, teratogenic medications, and illicit drugs)
West Nile virus
Consider anterior chamber tap and submitting aqueous sample to the U.S. Centers for Disease Control (CDC) or similar organization for ZIKV RT-PCR testing.
RNA nucleic acid testing (NAT) on serum and urine within first 14 days following the onset of symptoms
If testing is performed after 14 days or RNA NAT testing is negative, obtain ZIKV serology (IgM is positive from day 4 to 12 weeks postinfection).