Pediatr Emerg Med J.  2022 Jun;9(1):48-51. 10.22470/pemj.2022.00479.

Kawasaki disease shock syndrome rescued by a combination of extracorporeal membrane oxygenation, steroids, and intravenous immunoglobulin

• Chung C
• Ko H
• Byun JH
• Kim TH
• Lee HD

• KMID: 2531530
• DOI: http://doi.org/10.22470/pemj.2022.00479

Abstract

Kawasaki disease shock syndrome (KDSS) is defined as a sustained decrease in systolic blood pressure or signs of poor perfusion. Some children with KDSS are refractory to conventional therapy, such as intravenous immunoglobulin (IVIG). A 4-year-old boy with Kawasaki disease was referred to the emergency department. Despite the conventional therapy, his vital signs and cardiac function worsened. The boy promptly underwent extracorporeal membrane oxygenation (ECMO), which did not relieve the symptoms. Subsequently, he underwent methylprednisolone pulse therapy and the second cycle of IVIG therapy. Two days after the start of a combination of ECMO, steroids, and IVIG started, his clinical condition was stabilized, and finally, ECMO was removed. This case highlights the combination of ECMO, steroids, and IVIG as a treatment option for children with IVIG-resistant KDSS.

Keyword

Extracorporeal Membrane Oxygenation; Immunoglobulins, Intravenous; Mucocutaneous Lymph Node Syndrome; Shock, Cardiogenic; Steroids

Figure

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  Fig. 1. The chest radiograph obtained just after the commencement of extracorporeal membrane oxygenation on day 2. The radiograph shows cardiomegaly, increased pulmonary vascularity, and multiple subsegmental atelectases. Note the cannulae inserted in the right internal jugular vein (15-French, white arrow) and carotid artery (12-French, black arrow) with the endotracheal tube inserted (arrowhead).

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  Fig. 2. Echocardiogram on day 24. It shows a fusiform teardrop-shaped aneurysm of the distal right coronary artery (dotted caliper; diameter, 6.7 mm; Z-score = 13.5).

Reference

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