FIGURE 1
Bilateral non-exudative conjunctivitis, consistent with Kawasaki disease.
FIGURE 2
Oral mucosal changes, including erythematous cracked lips with oral ulceration, were key findings supporting the diagnosis of Kawasaki disease.
DISCUSSION
KD is an uncommon pediatric vasculitis that primarily affects young children; North American incidence is approximately 10-20 per 100,000 children under 5 years of age. 1 Atypical presentations, which do not meet all clinical criteria, account for roughly 15- 40 % of cases, highlighting the importance of supportive laboratory and imaging data in establishing the diagnosis. 2 Detection of viruses and other pathogens can complicate the diagnostic evaluation of febrile children, but should not exclude the diagnosis of KD. PCR tests for respiratory viruses may be positive in approximately 40-42 % of children diagnosed with KD, and identification of a respiratory virus does not reliably distinguish viral infection from KD. 3
KD remains a clinical diagnosis, often aided by laboratory and imaging findings. The patient must have fevers for five or more days, with at least four of the following: non-exudative bilateral conjunctival injection, erythematous mouth, and pharynx, strawberry tongue with cracked lips, polymorphous exanthem, edema in hands and feet, and cervical lymphadenopathy. 1 Children who fall short of the full criteria but have cardiac abnormalities on echocardiogram meet the diagnosis of KD. 1 As this case evolved, the patient manifested each of the clinical criteria for a diagnosis of classic KD. Leukocytosis, transaminitis, and elevated acute phase reactants further supported the diagnosis.
Features that made KD less likely in our patient included viral test positivity and the absence of several classic findings, such as thrombocytosis, sterile pyuria, and anemia for their age. Normal echocardiogram, while encouraging, does not exclude the diagnosis of KD, as CAA can still develop later in the course. 4 An echocardiogram should be repeated especially in patients with highrisk clinical features or IVIG resistance, which is defined as a persistent fever 36 hours after the completion of the initial IVIG infusion. 4 Our IVIG-resistant patient had normal follow up echocardiograms, which may be reflective of the timely escalation of immunomodulatory therapy.
Atypical KD is used to describe clinical manifestations not part of the standard criteria, such as hepatobiliary or other organ-system involvement, and may include features like gallbladder hydrops. 5 In a prospective study, gallbladder enlargement occurred in 35 % of KD patients, suggesting that its presence can support the diagnosis in atypical cases. 6 Studies have shown that KD patients with acute gallbladder findings often exhibit higher levels of inflammatory markers, such as CRP, ALT, and neutrophils, indicating a more intense inflammatory response. 7 While gallbladder hydrops can be seen in other conditions, such as viral infections, systemic illnesses, or biliary tract anomalies, KD is a relatively common cause in pediatric patients. 6 In this case, the presence of gallbladder hydrops, despite IVIG resistance and diagnostic uncertainty with viral positivity, supported the diagnosis of KD and contributed to the decision to escalate immunosuppressive treatment. 7
Once KD is suspected, prompt administration of IVIG at 2 g / kg and high-dose aspirin( 30-50 mg / kg / day) reduces systemic inflammation and the risk of coronary
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