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Evidence-Based Management of Kawasaki Disease in the Emergency Department

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Evidence-Based Management of Kawasaki Disease in the Emergency Department January 2015 Evidence-Based Management Volume 12, Number 1 Authors Of Kawasaki Disease In The Kara K. Seaton, MD Fellow, Pediatric Emergency Medicine, Children’s Hospital and Clinics of Minnesota, Minneapolis, MN Emergency Department Anupam Kharbanda, MD Director of Research, Emergency and Trauma Services, Abstract Children’s Hospital and Clinics of Minnesota, Minneapolis, MN Peer Reviewers Kawasaki disease, also known as mucocutaneous lymph node syn- Catherine Sellinger, MD, FAAP Associate Director, Pediatric Emergency Services, Children’s drome, was first described in Japan in 1967. It is currently the leading Hospital at Montefiore; Assistant Professor of Pediatrics, Albert cause of acquired heart disease in children in the United States. Un- Einstein College of Medicine, Bronx, NY treated Kawasaki disease may lead to the formation of coronary artery Michael J. Stoner, MD Assistant Professor of Pediatrics, The Ohio State University aneurysms and sudden cardiac death in children. This vasculitis pres- College of Medicine; Attending Physician, Pediatric Emergency ents with fever for ≥ 5 days, plus a combination of key criteria. Because Medicine, Nationwide Children’s Hospital, Columbus, OH each of the symptoms commonly occurs in other childhood illnesses, CME Objectives the disease can be difficult to diagnose, especially in children who Upon completion of this article, you should be able to: present with an incomplete form of the disease. At this time, the etiol- 1. Describe the clinical features and risks associated with ogy of the disease remains unknown, and there is no single diagnostic Kawasaki disease. 2. Identify epidemiologic trends in Kawasaki disease in high- test to confirm the diagnosis. This issue reviews the presentation, diag- risk populations. nostic criteria, and management of Kawasaki disease in the emergency 3. Assess the differences between complete and incomplete presentations of Kawasaki disease. department. Emergency clinicians should consider Kawasaki disease 4. Discuss the initial treatment of Kawasaki disease and as a diagnosis in pediatric patients presenting with prolonged fever, as possible options for treating resistant disease. prompt evaluation and management can significantly decrease the risk Prior to beginning this activity, see “Physician CME of serious cardiac sequelae. Information” on the back page. Editor-in-Chief Ilene Claudius, MD Therapeutics; Research Director, Melissa Langhan, MD, MHS Christopher Strother, MD Associate Professor of Emergency Pediatric Emergency Medicine, BC Associate Professor of Pediatrics, Assistant Professor, Director, Adam E. Vella, MD, FAAP Medicine, Keck School of Medicine Children's Hospital, Vancouver, BC, Fellowship Director, Pediatric Undergraduate and Emergency Associate Professor of Emergency of the University of Southern Canada Emergency Medicine, Director of Simulation, Icahn School of Medicine, Pediatrics, and Medical California, Los Angeles, CA Education, Pediatric Emergency Medicine at Mount Sinai, New Alson S. Inaba, MD, FAAP Education, Director Of Pediatric Medicine, Yale School of Medicine, York, NY Ari Cohen, MD Associate Professor of Pediatrics, Emergency Medicine, Icahn New Haven, CT School of Medicine at Mount Sinai, Chief of Pediatric Emergency University of Hawaii at Mãnoa AAP Sponsor New York, NY Medicine Services, Massachusetts John A. Burns School of Medicine, Robert Luten, MD General Hospital; Instructor in Division Head of Pediatric Professor, Pediatrics and Martin I. Herman, MD, FAAP, FACEP Associate Editor-in-Chief Pediatrics, Harvard Medical Emergency Medicine, Kapiolani Emergency Medicine, University of Professor of Pediatrics, Attending Physician, Emergency Medicine Vincent J. Wang, MD, MHA School, Boston, MA Medical Center for Women and Florida, Jacksonville, FL Children, Honolulu, HI Department, Sacred Heart Associate Professor of Pediatrics, Marianne Gausche-Hill, MD, Garth Meckler, MD, MSHS Children’s Hospital, Pensacola, FL Keck School of Medicine of the FACEP, FAAP Madeline Matar Joseph, MD, FAAP, Associate Professor of Pediatrics, University of Southern California; Professor of Clinical Medicine, FACEP University of British Columbia; International Editor Associate Division Head, David Geffen School of Medicine Professor of Emergency Medicine Division Head, Pediatric Lara Zibners, MD, FAAP Division of Emergency Medicine, at the University of California at and Pediatrics, Chief and Medical Emergency Medicine, BC Honorary Consultant, Paediatric Children's Hospital Los Angeles, Los Angeles; Vice Chair and Chief, Director, Pediatric Emergency Children's Hospital, Vancouver, Emergency Medicine, St Mary's Los Angeles, CA Division of Pediatric Emergency Medicine Division, University BC, Canada Hospital, Imperial College Trust; Medicine, Harbor-UCLA Medical of Florida Medical School- Editorial Board Joshua Nagler, MD EM representative, Steering Group Center, Los Angeles, CA Jacksonville, Jacksonville, FL Assistant Professor of Pediatrics, ATLS ®-UK, Royal College of Jeffrey R. Avner, MD, FAAP Michael J. Gerardi, MD, FAAP, Stephanie Kennebeck, MD Harvard Medical School; Surgeons, London, England Professor of Clinical Pediatrics FACEP, President-Elect Associate Professor, University Fellowship Director, Division of and Chief of Pediatric Emergency Associate Professor of Emergency of Cincinnati Department of Emergency Medicine, Boston Pharmacology Editor Medicine, Albert Einstein College Medicine, Icahn School of Pediatrics, Cincinnati, OH Children’s Hospital, Boston, MA James Damilini, PharmD, MS, of Medicine, Children’s Hospital at Medicine at Mount Sinai; Director, BCPS Montefiore, Bronx, NY Anupam Kharbanda, MD, MS James Naprawa, MD Pediatric Emergency Medicine, Research Director, Associate Associate Clinical Professor Clinical Pharmacy Specialist, Steven Bin, MD Goryeb Children's Hospital, Fellowship Director, Department of Pediatrics, The Ohio State Emergency Medicine, St. Associate Clinical Professor, Morristown Medical Center, of Pediatric Emergency Medicine, University College of Medicine; Joseph's Hospital and Medical Division of Pediatric Emergency Morristown, NJ Children's Hospitals and Clinics of Attending Physician, Emergency Center, Phoenix, AZ Medicine, UCSF Benioff Children’s Sandip Godambe, MD, PhD Minnesota, Minneapolis, MN Department, Nationwide Children’s Quality Editor Hospital, University of California, Vice President, Quality & Patient Hospital, Columbus, OH Tommy Y. Kim, MD, FAAP, FACEP San Francisco, CA Safety, Professor of Pediatrics and Steven Choi, MD Assistant Professor of Emergency Steven Rogers, MD Richard M. Cantor, MD, FAAP, Emergency Medicine, Attending Medical Director of Quality, Medicine and Pediatrics, Loma Assistant Professor, University of Director of Pediatric Cardiac FACEP Physician, Children's Hospital Linda University Medical Center and Connecticut School of Medicine, Inpatient Services, The Children’s Professor of Emergency Medicine of the King's Daughters Health Children’s Hospital, Loma Linda, CA; Attending Emergency Medicine and Pediatrics, Director, Pediatric System, Norfolk, VA Hospital at Montefiore; Assistant California Emergency Physicians, Physician, Connecticut Children's Professor of Pediatrics, Albert Emergency Department, Medical Ran D. Goldman, MD Riverside, CA Medical Center, Hartford, CT Director, Central New York Einstein College of Medicine, Professor, Department of Pediatrics, Bronx, NY Poison Control Center, Golisano University of British Columbia; Children's Hospital, Syracuse, NY Co-Lead, Division of Translational Case Presentation Since it was first described, Kawasaki disease has been well characterized. Affected children generally A 3-year-old girl presents to the emergency department have fever for at least 5 days, plus 4 of 5 key criteria. for evaluation of fever. Her mother reports that the child These criteria include conjunctival injection, oral has had a fever for the past 5 days, with temperatures mucosal changes, polymorphous rash, distal extremity 5,6 ranging from 38.3°C (101°F) to 40°C (104°F). The child changes, and cervical lymphadenopathy. Nonspe- has some rhinorrhea, but no significant cough. She has cific symptoms, such as vomiting, joint pain, cough, been complaining of abdominal pain and had 2 episodes of decreased oral intake, rhinorrhea, and abdominal pain, nonbilious vomiting in the last 2 days. She was evalu- may also be present, and may make the diagnosis ated at her pediatrician’s office 2 days ago, and she was more challenging. To add to the diagnostic challenge, diagnosed with a viral illness. Her mother reports the some patients may develop an incomplete form of the subsequent development of a red rash that started on disease. These children will not meet all of the clinical the child’s face and chest, and it is now present on her criteria of Kawasaki disease, but they may still develop trunk, back, and extremities. Her lips are cracked, and her the cardiovascular complications. Incomplete Kawasaki mother attributes this to poor oral intake over the past few disease accounts for 15% to 20% of Kawasaki disease days. Today, the child was noted to have red eyes, without diagnoses, and it is more common in children aged < 6 5 discharge, and redness in the genital area. The mother has months and in children aged > 5 years. not noticed swelling of the extremities or peeling skin. The No single laboratory test can be used to establish remainder of the review of systems is negative. The child the diagnosis of Kawasaki disease, although labora-
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