Refractory Kawasaki Disease with Severe Coronary Complications in an Infant
Case at a Glance
A 5-month-old male infant presented with severe, treatment-resistant Kawasaki disease complicated by giant coronary artery aneurysms (z-score 11) and coronary thrombosis despite anticoagulation therapy.
Patient's Story
A previously healthy 5-month-old male infant (weight 7kg) was brought to the emergency department 5 weeks prior with fever, rash, and irritability. Initial presentation included classic features of Kawasaki disease including prolonged fever, polymorphous rash, conjunctival injection, and cervical lymphadenopathy. Despite prompt recognition and treatment, the patient developed a severe, refractory course of the disease.
Initial Assessment
The infant met criteria for Kawasaki disease and was immediately started on standard first-line therapy including intravenous immunoglobulin (IVIG) and aspirin. Initial echocardiography showed mild coronary artery dilation. Laboratory findings revealed elevated inflammatory markers consistent with acute Kawasaki disease.
The Diagnostic Journey
The patient demonstrated treatment resistance to initial IVIG therapy, prompting escalation to second-line treatments including additional IVIG doses, corticosteroids, and infliximab. Serial echocardiograms revealed progressive coronary artery dilation evolving to giant aneurysms with z-scores reaching 11. Despite anticoagulation with heparin and aspirin therapy, the patient developed coronary thrombosis requiring emergent intervention.
Final Diagnosis
Severe refractory Kawasaki disease with giant coronary artery aneurysms (z-score 11) complicated by coronary artery thrombosis
Treatment Plan
The patient received multiple treatment modalities including: 1) Multiple courses of IVIG, 2) Systemic corticosteroids, 3) Infliximab therapy, 4) Anticoagulation with heparin and aspirin, 5) Emergency thrombectomy for coronary thrombosis. The multidisciplinary team is consulting with international Kawasaki disease experts for additional treatment recommendations given the severity and treatment-resistant nature of this case.
Outcome and Follow-up
The coronary thrombosis was successfully removed via emergency intervention with preservation of cardiac function. However, inflammatory markers remain elevated indicating ongoing active disease process. The patient continues to require intensive monitoring and is being managed at a tertiary pediatric center with ongoing international consultation for this exceptionally severe case of Kawasaki disease.