Atypical Presentation of Kawasaki Disease in Pediatric Patient: Why Clinical Judgement is Key.

1Tolia S, 2Rivers K
1Nova Southeastern University, Fort Lauderdale, FL, United states; 2Broward Health Medical Center, Fort Lauderdale, FL, USA

Kawasaki disease is a medium vessel vasculitis that most commonly affects the pediatric population. The typical presentation consists of a high-grade fever for greater than five days along with symptoms of mucocutaneous inflammation and cervical adenopathy. The AHA and AAP have created an algorithm to aid in the diagnosis of patients who do not meet the diagnostic criteria, but may be at risk of developing CAA.
Case Presentation: We present a case of a 4-year-old previously healthy male who presented to the ED with a 2-day history of a subjective fever, abdominal discomfort, left sided cervical adenopathy, significant cracking and swelling of the lips, but denied any rashes. Ultrasound of the abdomen revealed a slightly distended gallbladder. Patient was being evaluated for possible hepatitis with stomatitis and cervical adenopathy and discharged within 24 hours. Patient was transferred from a local medical facility three days later. Patient's chief complaint is a 5-day history of a constant, high grade fever, T-max of 104.7 F, and intermittent abdominal discomfort. Upon physical examination, patient was noted to have swollen dorsum of feet bilaterally, cracking and swelling of lips, strawberry tongue, bilateral lymphadenopathy, mild swelling and erythema of penis and scrotum, and mild perianal rashes. Lab values showed elevated LFT's, and elevated inflammatory markers. Imaging studies showed slight hydrops of the gallbladder, chest x-ray was within normal limits, and echocardiogram demonstrated normal function. Patient was admitted for evaluation of Kawasaki disease and treatment with IVIG and high-dose aspirin was started. During IVIG infusion, patient became hypotensive and tachypneic, after which the patient's IVIG infusion was stopped. He was placed on blow-by oxygen and given a normal saline bolus. However, patient had persistent tachypnea and tachycardia and was noted to have hepatomegaly and icterus. Repeat chest x-ray showed edema and enlarged cardiac size, which prompted a stat echocardiogram. Echo showed worsened cardiac function, ectasia of left coronary artery, dilated left ventricle and decreased systolic function LVEF 47-50%. Patient received a dose of Lasix and was then transferred to another local medical facility for further cardiac care.
Discussion: The diagnosis of atypical Kawasaki disease rests upon clinical judgement, laboratory values and imaging studies. A case such as this one demonstrates the importance of identifying children at risk of developing CAA using clinical judgement.
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