Incomplete presentations in a series of 37 children with Kawasaki disease: The role of the pediatric ophthalmologist

Background: Kawasaki disease (KD) is an acute and sometimes fatal febrile vasculitis of childhood, the presenting signs of which include conjunctival vessel dilatation and iridocyclitis. Consultation with a pediatric ophthalmologist is helpful for early recognition of the disease, especially in identifying 'incomplete' cases, ie, those which lack all of the classical, systemic signs. Methods: After encountering a case of incomplete KD in which diagnosis was delayed, we reviewed the hospital records of 37 children with KD to establish how often the disease manifested 'incompletely,' and how often pediatricians consulted pediatric ophthalmologists to help in its early diagnosis. Results: Forty-five percent of the children eventually diagnosed with KD lacked the complete diagnostic criteria of KD when admitted to the hospital, and diagnosis and treatment therefore were delayed. Coronary artery aneurysms, a complication that might have less serious consequences if treated earlier, developed in 24% of these patients. Although 67% presented to their pediatrician with bilateral conjunctival injection and/or iridocyclitis, pediatricians requested ophthalmologic consultation for only 5% of them. Conclusions: Our review of these cases indicates that the eye findings in KD could play a role in earlier diagnosis and treatment. Pediatric ophthalmologists and pediatricians should be more aware of their combined responsibilities for expedient recognition of KD.