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cardiac complications. Depending on the series, between 10% and 60% of children
who develop coronary aneurysms never meet clinical criteria for KD. The 2017
guidelines of the American Heart Association (AHA) provide a useful framework for
managing children with suspected KD who do not meet criteria for the diagnosis (see
Fig. 101.15 and also section on Incomplete KD below).
Clinical manifestations of KD tend to be most incomplete and atypical in the
youngest patients, the subgroup at highest risk for development of coronary artery
abnormalities. Infants younger than 6 months are at particularly high risk. Thus, KD
should be considered in any infant with prolonged, unexplained fever. In contrast,
alternative explanations for the child’s symptoms must be carefully excluded before
treating empirically with IVIG. Consideration should be given to referring children to a
regional KD center for further evaluation when the diagnosis is unclear.
At the other end of the spectrum, older children and adolescents with KD appear to
be at increased risk for developing coronary aneurysms; however, older age at
presentation is also associated with delayed diagnosis, which is known to incur
significant risk. Unlike infants, in whom the clinical findings of KD are often
incomplete, older children appear to present with fairly typical manifestations.
Diagnosis may be delayed because clinicians are less likely to consider the diagnosis in
older patients because most cases involve young children. Further, children older than 8
years of age frequently exhibit GI and meningeal symptoms, potentially clouding the
diagnostic picture. In any event, whether KD is indeed more aggressive in older
children, or simply because diagnosis is more likely to be delayed, pediatricians must
consider KD as a possible cause of prolonged fever in young people of any age.