Uveitis (Iritis, Cyclitis, Chorioretinitis)

The uveal tract (the inner vascular coat of the eye, consisting of the iris, ciliary body, and choroid) is subject to inflammatory involvement in a number of systemic diseases, both infectious and noninfectious, and in response to exogenous factors, including trauma and toxic agents (Table 621-1). Inflammation may affect any 1 portion of the uveal tract preferentially or all parts together.

Iritis may occur alone or in conjunction with inflammation of the ciliary body as iridocyclitis or in association with pars planitis. Pain, photophobia, and lacrimation are the characteristic symptoms of acute anterior uveitis, but the inflammation may develop insidiously without disturbing symptoms. Signs of anterior uveitis include conjunctival hyperemia, particularly in the perilimbal region (ciliary flush), and cells and protein (“flare”) in the aqueous humor (Fig. 621-1). Inflammatory deposits on the posterior surface of the cornea (keratic precipitates) and congestion of the iris may also be seen. More chronic cases may show degenerative changes of the cornea (band keratopathy), lenticular opacities (cataract), development of glaucoma, and impairment of vision. The cause of anterior uveitis is often obscure; primary considerations in children are rheumatoid disease, particularly pauciarticular rheumatoid arthritis, Kawasaki disease, reactive arthritis (postinfectious), and sarcoidosis. Iritis may be secondary to corneal disease, such as herpetic keratitis or a bacterial or fungal corneal ulcer, or to a corneal abrasion or foreign body. Traumatic iritis and iridocyclitis are especially common in children.

Figure 621-1 Cell and flare in the anterior chamber. The flare represents protein leakage.

(Courtesy of Peter Buch, CRA.)

Iridocyclitis that occurs in children with arthritis deserves special mention. Unlike most forms of anterior uveitis, it rarely creates pain, photophobia, or conjunctival hyperemia. Loss of vision may not be noticed until severe and irreversible damage has occurred. Because of the lack of symptoms and the high incidence of uveitis in these children, routine periodic screening is necessary. Ophthalmic screening guidelines are based on 3 factors that predispose children with arthritis to uveitis:

Table 621-2 has been developed by the American Academy of Pediatrics for children with juvenile rheumatoid arthritis without known iridocyclitis.

Table 621-2 EXAMINATION SCHEDULE FOR CHILDREN WITH JRA WITHOUT KNOWN IRIDOCYCLITIS

ANA, antinuclear antibody; JRA, juvenile rheumatoid arthritis.

Choroiditis, inflammation of the posterior portion of the uveal tract, invariably also involves the retina; when both are obviously affected, the condition is termed chorioretinitis. The causes of posterior uveitis are numerous; the more common are toxoplasmosis, histoplasmosis, cytomegalic inclusion disease, sarcoidosis, syphilis, tuberculosis, and toxocariasis (Fig. 621-2). Depending on the etiology, the inflammatory signs may be diffuse or focal. Vitreous reaction often occurs as well. With many types, the result is atrophic chorioretinal scarring demarcated by pigmentation, often with visual impairment. Secondary complications include retinal detachment, glaucoma, and phthisis.

Figure 621-2 Focal atrophic and pigmented scars of chorioretinitis.

Panophthalmitis is inflammation involving all parts of the eye. It is frequently suppurative, most often as a result of a perforating injury or of septicemia. It produces severe pain, marked congestion of the eye, inflammation of the adjacent orbital tissues and eyelids, and loss of vision. In many cases, the eye is lost despite intensive treatment of the infection and inflammation. Enucleation of the eye or evisceration of the orbit may be necessary.

Sympathetic ophthalmia is a rare type of inflammatory response that affects the uninjured eye after a perforating injury. It may occur weeks, months, or even years after the injury. A hypersensitivity phenomenon is the most probable cause. Loss of vision in the uninjured (sympathizing) eye may result. Removal of the injured eye prevents the development of sympathetic ophthalmia but does not stop the progression of the disease once it has occurred. Therefore, early enucleation should be considered if there is no hope of visual recovery after a severe injury.