Kawasaki shock syndrome complicating a recurrence of Kawasaki disease

• Published in: Pediatrics (Evanston) Vol. 134; no. 6; pp. e1695 - e1699
• Main Authors: Tissandier, Côme, Lang, Matthieu, Lusson, Jean René, Bœuf, Benoit, Merlin, Etienne, Dauphin, Claire
• Format: Journal Article
• Language: English
• Published: United States American Academy of Pediatrics 01-12-2014
• Subjects: Analysis; Aspirin - administration & dosage; Boys; Cardiovascular disease; Care and treatment; Case studies; Child; Children; Combination drug therapy; Combined Modality Therapy; Corticosteroid drugs; Corticosteroids; Diagnosis; Diagnosis, Differential; Diseases; Dosage and administration; Dose-Response Relationship, Drug; Drug Administration Schedule; Drug therapy, Combination; Echocardiography; Health aspects; Heart Failure - diagnosis; Heart Failure - therapy; Humans; Hypotension - diagnosis; Hypotension - therapy; Immunization, Passive; Inflammation Mediators - blood; Intensive Care Units, Pediatric; Kawasaki disease; Kawasaki syndrome; Lymphatic system; Male; Medical diagnosis; Methylprednisolone - administration & dosage; Mucocutaneous Lymph Node Syndrome - diagnosis; Mucocutaneous Lymph Node Syndrome - therapy; Pediatrics; Recurrence; Recurrence (Disease); Relapse; Shock - diagnosis; Shock - therapy; Ultrasound imaging; mucocutaneous lymph node syndrome/diagnosis; recurrence; glucocorticoids/therapeutic use; mucocutaneous lymph node syndrome/therapy; mucocutaneous lymph node syndrome; drug therapy/combination; case report; shock/diagnosis
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.2014-0004

We describe a case of recurrent Kawasaki disease (KD) in a non-Asian 6-year-old boy who had been diagnosed with typical KD without cardiac involvement at age 3 years. He was admitted to the PICU 3 years later for heart failure, hypotension, and deterioration of his general condition. Ultrasonography revealed left ventricular dysfunction with a 44% ejection fraction and grade I mitral valve failure without coronary artery involvement. Subsequent observation of hyperemic conjunctiva, bilateral cervical adenopathies with erythematous skin (normal neck ultrasound and computed axial tomography findings), peeling of the fingertips at day 8 of the illness, and occurrence of an inflammatory syndrome led to a diagnosis of incomplete recurrent KD with a clinical picture of Kawasaki shock syndrome (KSS). Clinical improvement was rapidly obtained after intravenous immunoglobulin and intravenous corticosteroid therapy (30 mg/kg per day for 3 subsequent days). Left ventricular function gradually improved, with ultrasound returning to normal after 3 months. Diagnosis was difficult to establish because of the recurrence of the disease and the incomplete clinical picture, with clinical features of KSS. Physicians need to be aware of these pitfalls in the management of patients with clinical signs of KD.