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Infective Uveitis, Retinitis, and Chorioretinitis
Uveitis refers to any intraocular inflammation. Anterior uveitis is characterized by inflammation of the iris and ciliary body (iridocyclitis). Intermediate uveitis includes inflammation of the pars plana and vitreous (pars planitis, vitritis). Posterior uveitis features inflammation of the retina and choroid (retinitis, choroiditis). Uveitis is an uncommon disorder in childhood; among affected children, the anterior form is more common than the posterior form.
Table 82.1 summarizes the important pathogens and disorders responsible for anterior uveitis, with distinguishing features of each. Kawasaki disease is the most common acute pediatric illness associated with anterior uveitis. Other chronic pediatric diseases frequently associated with anterior uveitis are juvenile idiopathic arthritis and human leukocyte antigen (HLA)-B27-related spondyloarthropathy. ,
TABLE 82.1
Common Causes of Anterior Uveitis
| Organism or Condition | Onset/Duration | Associated Ocular Findings | Associated Systemic Findings |
|---|---|---|---|
| Varicella | Acute | Iris atrophy (rare) | Vesicular skin rash |
| Herpes simplex | Acute | Keratitis | None |
| Mumps | Acute | NS | Fever, headache, parotitis |
| Influenza | Acute | NS | Febrile respiratory illness |
| Epstein-Barr virus mononucleosis | Acute | NS | Fever, pharyngitis, lymphadenopathy |
| Measles | Acute | NS | Fever, coryza, Koplik spots |
| Kawasaki disease | Acute | NS | Fever, mucocutaneous findings, peripheral edema, unilateral lymphadenopathy |
| Herpes zoster | Chronic | Iris atrophy (common), KP | Fever, vesicular skin rash |
| Syphilis | Chronic | Iris nodules, chorioretinitis, optic neuritis | Maculopapular skin rash (palms and soles) |
| Tuberculosis | Chronic | Iris nodules, granulomatous KP, choroidal granulomas | Pulmonary lesions |
| Lyme disease | Chronic | Keratitis, optic neuritis | Erythema migrans, arthritis |
| Leprosy | Chronic | “Iris pearls” of iris atrophy | Peripheral neuropathy, skin lesions |
KP, keratitic precipitates that represent aggregates of inflammatory cells on the corneal endothelium; NS, nonspecific.
Patients with anterior uveitis come to attention because of ocular pain, photophobia, and blurred vision. The whole eye looks red, but closer inspection reveals that the predominant area of hyperemia is at the corneal limbus owing to the vascular engorgement of the underlying ciliary body. Slit-lamp examination allows visualization of (1) cells floating in the anterior chamber or adherent to the corneal endothelium (keratic precipitates), and (2) turbidity of the normally optically clear aqueous humor resulting from the spillover of protein (flare). Distortion of the pupil is related to formation of inflammatory scars between the iris and lens surface (synechiae). Dystrophic calcification of the epithelial basement membrane (band keratopathy), iris nodules, lens opacities, and phthisis bulbi can occur. Patients with posterior uveitis will often present with decreased vision and floaters and pain, and photophobia is often not present unless there is coexistent inflammation of the sclera or anterior segment of the eye (iris and ciliary body).
Etiologic Agents of Anterior Uveitis
Viruses
Many viruses are associated with an acute nongranulomatous uveitis that is mild and self-limited. Varicella zoster virus (VZV) demonstrates the spectrum of disease involvement. Anterior uveitis resulting in mild inflammation occurs in up to 25% of patients with chickenpox. It tends to be self-limited, resolving within 10–14 days. By contrast, uveitis associated with herpes zoster ophthalmicus is more severe and usually requires aggressive treatment with a topical corticosteroid, cycloplegic agents, and systemic acyclovir. Devastating visual complications can occur when there is concurrent involvement of structures of the posterior segment. Uveitis associated with herpes simplex virus (HSV) infection usually is a complication of a deep keratitis but can occur without antecedent keratitis.
Mycobacterium tuberculosis
Chronic granulomatous uveitis, sometimes in association with iris nodules, once was caused commonly by Mycobacterium tuberculosis . It is now uncommon, except in developing countries and in patients with AIDS.
Treponema pallidum
The incidence of congenital syphilis parallels the incidence of syphilis among heterosexual persons in the United States. Congenital syphilis also remains a problem in developing countries. Chorioretinitis is a classic sign of early congenital syphilis, “salt-and-pepper” pigmentary changes of the peripheral retina being the most common finding. Although anterior uveitis is a common presentation, some patients predominantly have intense posterior uveitis with necrotizing retinitis, retinal vasculitis, or optic neuritis.
Kawasaki Disease
Anterior uveitis is common in Kawasaki disease. In one prospective study, anterior uveitis was found in 36% of patients with Kawasaki disease in the first week of the disease. Iridocyclitis and periorbital vasculitis also have been described. Conjunctival hyperemia is present in >90% of cases.
Etiologic Agents of Posterior Uveitis (Retinitis)
Table 82.2 summarizes the predominant ocular manifestations and associated findings for the infectious agents most commonly implicated in retinitis.
TABLE 82.2
Common Infectious Causes of Retinitis
| Organism | Predominant Manifestation | Associated Ocular Findings |
|---|---|---|
| Toxoplasma gondii | Necrotizing retinitis | Vitritis, optic neuritis |
| Rubella virus | Pigmentary retinopathy | Cataract, microphthalmia |
| Cytomegalovirus | Hemorrhagic retinitis | Cotton-wool spots |
| Herpes simplex virus | Necrotizing retinitis | Vitritis, vasculitis, optic neuritis |
| Varicella zoster virus | Necrotizing retinitis | Occlusive vasculitis, vitritis |
| Toxocara canis | Retinal granuloma | Vitritis, endophthalmitis |
Bacteria and Fungi
Before the advent of effective antibacterial and antifungal therapies, the prevalence of septic emboli and secondary retinitis from bloodstream infection (BSI) was high. The presence of Roth spots (white-centered intraretinal hemorrhages, once believed to be pathognomonic for septic emboli) was a sign of endocarditis. In fact, white-centered hemorrhages are nonspecific and can be seen with septic emboli, clotting abnormalities, trauma, vascular occlusive disease, and vasculitis. The white center can be composed of a fibrin aggregate or can be a mixture of inflammatory cells, bacteria, or fungal organisms. In patients with BSI from infective endocarditis or catheter-related infection, the appearance and resolution of septic emboli can be used to monitor antibacterial therapy.
The incidence of positive retinal findings in patients with fungal BSI ranges from 3%–27%, with a highest incidence in neutropenic patients receiving chemotherapy or after organ or bone marrow transplantation. The incidence in children is lower than in adults. In premature infants, fungal emboli to the crystalline tunica vasculosa lentis can cause inflammatory ocular disease that mimics retinopathy of prematurity, congenital cataracts, or conjunctivitis. Systemic treatment of Candida and other fungi with amphotericin, an echinocandin, or voriconazole usually results in resolution of chorioretinal infection; the need for intravitreal injections and surgical vitrectomy has decreased in recent years. For patients with BSI whose initial examination is negative but whose BSI persists, serial examinations should be performed to make certain that there are not new lesions.
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