Case Presentation: A 9-year-old male with congenital Horner’s syndrome presented with two weeks of nonspecific, intermittent symptoms beginning with a nonproductive cough, congestion, nausea and vomiting followed by a diffuse maculopapular erythematous rash on his back and chest with RUQ pain. Soon after, he developed a separate warm, erythematous, tender rash of the left leg concerning for cellulitis. CT of the leg showed minimal skin thickening, no abnormal enhancements/abscess, but could not rule out early manifestation of cellulitis. He was given one dose of ceftriaxone then was sent home. But later presented to the ED for worsening symptoms. He developed a temperature of 38 C in the ED. Physical exam demonstrated inguinal lymphadenopathy, pedal edema, RUQ tenderness and rashes as described above. Labs showed elevated LFTs, alkaline phosphatase, total bilirubin and inflammatory markers. Echo showed normal coronary arteries without dilation or aneurysms. A RUQ US demonstrated gallbladder hydrops, common bile duct dilation and possible early cholecystitis. Zosyn was initiated initially with concern for acute cholangitis. Initially, infectious etiologies were considered high on the differential, however, after the development of bilateral conjunctivitis combined with a second documented fever, Kawasaki Disease (KD) was considered the leading diagnosis. IVIG was administered with swift resolution of presenting symptoms.
Discussion: Kawasaki disease affects many children less than five years of age and is the primary cause of acquired heart disease in children in the US. This case describes an uncommon presentation of a relatively known condition, highlighting the importance of considering KD even in uncharacteristic presentations. The wide array of potential presentations makes diagnosis difficult. For example, KD has been diagnosed in cases presenting with: tonsilitis with cervical lymphadenopathy, a lower extremity limp with urinary incontinence, a pleural effusion secondary to pneumonia unresponsive to antibiotic therapy and even more thought-provoking a four year old with anasarca, oliguria & shock. In cases such as these, many times treatment is delayed and life-threatening consequences can occur as a result. Abrupt RUQ abdominal pain, rash, gallbladder hydrops, common bile duct dilation and possible signs of early cholecystitis without the presence of a fever, suggested etiology originating from the bilious system rather than KD. An Echo without dilation/aneurysms also supported this thought. Patients’ leukocytosis, elevated LFTs and age which was older than typical presentation of KD, supported further work-up and evaluation.Infectious etiologies were extensively explored and ruled out. Ultimately, it was decided to move forward with administration of IVIG, as other causes of symptoms had been ruled out. After administration, complete symptomatic resolution occurred with subsequent resolution of abnormal lab values.
Conclusions: There is currently no lab testing for diagnosing KD making it crucial for medical providers to identify and recognize the wide variety of potential clinical presentations. Making a swift diagnosis is crucial to ensure timely administration of IVIG and avoidance of risk of devastating cardiac complications. In this case, the avoidance of anchoring bias with a flexible and easily evolving differential diagnosis was necessary in establishing a definitive diagnosis and should always be considered when symptomatology is unclear.