Ophthalmologic Manifestations of Kawasaki Disease 

Kawasaki disease is a systemic vasculitis of unknown etiology that affects the small- and medium-sized blood vessels of the body, particularly the coronary arteries.

Since its original description, in Japan in 1967, Kawasaki disease has been reported worldwide in children of all ethnic origins. The yearly incidence of endemic Kawasaki disease in the United States is 67 cases per 100,000 children who are younger than age 5 years. Approximately 3,000 hospitalizations occur annually in the United States as a result of Kawasaki disease.

The incidence in Europe is comparable to that of the United States. In Japan, where Kawasaki disease has the highest incidence, an estimated 5000-6000 cases occur annually.

Go to Kawasaki Disease and Dermatologic Manifestations of Kawasaki Disease for complete information on these topics.

The most common ophthalmologic finding in Kawasaki disease is bilateral conjunctival injection. Iridocyclitis, superficial punctate keratitis, vitreous opacities, papilledema, and subconjunctival hemorrhage also may occur.

In a study by Ohno et al, the incidence of superficial punctate keratitis, vitreous opacities, papilledema, and subconjunctival hemorrhage was, respectively, 22%, 11%, 11%, and 6%.^[1]

Bilateral conjunctival erythema is one of the classic diagnostic criteria for Kawasaki disease, being present in more than 90% of children with the disorder. It is mostly confined to the bulbar region and is nonexudative. Chemosis, follicles, and papillae are characteristically absent.

Burns et al found evidence of anterior uveitis in 83% of children who were diagnosed with Kawasaki disease in the first week of their illness.^[2] The anterior uveitis, which is usually mild and bilateral, is sometimes associated with keratic precipitates. Anterior uveitis peaks about a week after fever onset. It generally resolves within 2-8 weeks after disease onset without any of the common sequelae that often are seen with many other infectious causes of uveitis.

Ryan and Walton described a case of a 10-month-old male infant in whom bilateral scarring of the superior and inferior fornices developed in association with Kawasaki disease.^[3]

Less common posterior segment manifestations usually are limited to case reports. One such report, by Font et al, described bilateral inner retinal ischemia due to thrombotic occlusion as a result of systemic vasculitis.^[4]

One case documented periorbital vasculitis in an 8-month-old infant with Kawasaki disease, a previously unreported manifestation of the disorder.

Kawasaki disease is uncommon, while bilateral conjunctivitis is extremely common. The astute primary care physician, emergency room specialist, urgent care physician, or ophthalmologist should scrutinize cases of bilateral ocular redness associated with persistent fever, rash, and lymphadenopathy, particularly in the pediatric, Asian demographic groups most at risk. Referral to the appropriate pediatric subspecialist will ascertain community standard care in selected cases.^[5]

Consultation with a pediatrician, a pediatric intensivist, or a pediatric cardiologist is essential to the delivery of community standard care.

Treat Kawasaki disease uveitis with topical corticosteroids, such as prednisolone acetate, loteprednol etabonate, or dexamethasone, as well as with topical cycloplegics. Although it generally resolves within days of intravenous immunoglobulin therapy, conjunctival inflammation also may be treated with topical corticosteroids.

Keratitis is treated with supportive care using topical tear replacement, preservative-free tears, and ophthalmic ointments, and through the avoidance of irritants, tap water, rubbing behavior, and excessive use of topical preserved drops.