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Volume 47, Number 2, April 2014

Unusual manifestations of Kawasaki disease with retropharyngeal edema and shock syndrome in a Taiwanese child 

Li-Ching Fang, Shyh-Dar Shyur*, Chun-Chih Peng, Wai-Tim Jim, Szu-Hung Chu, Yu-Hsuan Kao, Chen-Kuan Chen, Ling-Chun Liu

Received: April 1, 2014    Revised: April 1, 2014    Accepted: April 1, 2014   


Corresponding author:

Corresponding author. Department of Pediatrics, Mackay Memorial Hospital, 92 Chungshan North Road, Section 2, Taipei 104, Taiwan. 


Background and purpose: 

We report a 3-year-old girl with Kawasaki disease who presented with retropharyngeal edema and shock syndrome. This is the first reported case in Taiwan. The patient initially presented with fever, cough, and pyuria followed by rapidly progressive enlarged bilateral cervical lymphadenopathy. On the third day of the fever, computed tomography for airway compression sign found widening of the retropharyngeal space mimicking a retropharyngeal abscess. Later, an endotracheal tube was inserted for respiratory distress. A skin rash over her trunk was also noted. On the fifth day of the fever, the clinical course progressed to hypotension and shock syndrome. Because of more swelling of bilateral neck lymph nodes, computed tomography was arranged again and revealed partial resolution of the edematous changes in the retropharyngeal space. Edema of the hands and feet, bilateral bulbar conjunctivitis, and fissured lips were subsequently found. The diagnosis of Kawasaki disease was confirmed on the eighth day of fever. There was no evidence of bacterial infection. She was administered intravenous immunoglobulin (2 mg/kg) and high dose aspirin (100 mg/kg/day). One day later, the fever subsided, and her blood pressure gradually became stable. Heart echocardiography on the Day 13 revealed dilated left coronary artery and mitral regurgitation. Follow-up echocardiography six months later showed normal coronary arteries. To date, the patient has not experienced any complications. This case illustrates that retropharyngeal edema and shock syndrome can be present in the same clinical course of Kawasaki disease. Clinicians and those who work in intensive care units should be aware of unusual presentations of Kawasaki disease to decrease rates of cardiovascular complications. 


Key words:

Kawasaki disease, Retropharyngeal edema, Shock