Giant Coronary Aneurysms with Multiple Large Resistant Thromboses in an 8-Month-Old Boy with IVIg-Resistant Kawasaki Disease: A Case Report

Mojtaba  Gorji; Maryam Taraz; Behdad  Gharib; Vahid  Ziaee

doi:10.18502/jthc.v18i3.14118

Mojtaba Gorji Department of Pediatric Cardiology, Children’s Medical Center, Pediatric Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran.
Maryam Taraz Department of Pediatric Cardiology, Children’s Medical Center, Pediatric Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran.
Behdad Gharib Department of Pediatric Cardiology, Children’s Medical Center, Pediatric Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran.
Vahid Ziaee Department of Pediatric Rheumatology, Children's Medical Center, Pediatric Center of Excellence, Tehran, Iran.

DOI: https://doi.org/10.18502/jthc.v18i3.14118

Keywords: Kawasaki disease; Coronary aneurysms; Thrombosis; Thrombophilia

Abstract

Kawasaki disease is an acute self-limiting systemic vasculitis in childhood, resulting in arterial swelling or inflammation and eventually leading to cardiovascular problems, such as coronary artery aneurysms. Based on previous studies, serum sodium ≤133 mmol/L, albumin ≤3.2 g/dL, alanine transaminase ≥80 U/L, and neutrophil percentage ≥80% at diagnosis are risk factors for intravenous immunoglobulin (IVIg). However, the prevalence of resistance to Ig among children with Kawasaki disease varies among different countries due to diversity in evaluation, treatment, and diagnosis.

Approximately, 10% to 20% of patients have IVIg-resistant Kawasaki disease. As the probability of coronary artery damage associated with IVIg-resistant Kawasaki disease is higher than that with IVIg-sensitive Kawasaki disease, the early detection and appropriate treatment of IVIg-resistant Kawasaki disease can decrease the probability of damage to coronary arteries and hospital lengths of stay and cost.

Kawasaki disease in early infancy is uncommon, and sometimes it occurs with thrombosis and peripheral gangrene. A positive genetic background may play a role in susceptibility to thrombosis.

We herein describe a patient suffering from an IVIg-resistant Kawasaki disease with severe coronary artery thrombosis and positive genetic mutation. Medical treatment resolved the thrombosis, but the coronary arteries remained dilated.