Kawasaki: COVID-19-related Illness in Children


by Gail I. Smith, MA, RHIA, CCS-P

As coding professionals, we listen to news stories with a “clinical ear.” COVID-19 has many mysteries but the most recent involves children and what is described as Kawasaki-like inflammatory disease that has a link to COVID-19.

Kawasaki disease is characterized by inflammation of blood vessels that could potentially damage the heart. According to the Centers for Disease Control and Prevention (CDC), Kawasaki syndrome is an acute febrile illness of unknown cause that primarily affects children younger than 5 years of age. The clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat. Kawaski does not have a specific test available however diagnosis involves ruling out other diseases that cause similar signs and symptoms

In ICD-10-CM the syndrome would be reported with code M30.3 Mucocutaneous lymph node syndrome [Kawasaki] in the category described as Polyarteritis nodosa and related conditions.

In a recent edition of the medical journal, The Lancet, researchers are concerned about the number of Kawasaki-like syndrome cases since the outbreak of COVID-19 (see the article here). The Italy-based report states a 30-fold increase in the number of cases between February 18 and April 20, 2020 compared to the previous 5 years. In the United States, this mystery illness has emerged in New York City where 85 reported cases are being investigated. Children’s National Hospital in Washington, DC recently reported two cases of the illness in children with COVID-19. Current reporting declares that the inflammatory condition is reported in at least 14 states.

As we wait for the mystery to unfold, the question becomes ‘How do we code this?’

The ICD-10-CM Official Guidelines for Coding and Reporting, Section II, H, includes a section on Uncertain Diagnosis. In summary, an uncertain condition is coded as if it exists for inpatient admissions only. The Kawasaki-like disease/syndrome is treated the same as the diagnosed Kawasaki; therefore, M30.3 would appropriately classify the illness and more importantly would allow researchers to gather data in this time of uncertainty. Coding symptoms or assigning an unspecified code would cripple the data at a time when determining the extent and manifestation of this illness is critical to public health.

The next step is to refer to the CDC’s supplement to the ICD-10-CM Official Guidelines for coding encounters related to the COVID-19 coronavirus outbreak. The link is here. Sequencing guidelines point us to assigning confirmed cases with the principal diagnosis of U07.1 COVID-19 with associated manifestations as a secondary code (in this case M30.3 Mucocutaneous lymph node syndrome [Kawasaki]).

If the documentation only mentions symptoms such as fever or inflammation of arteries, follow your internal coding guidelines. Personally, I would query the physician. It is that important.

Note: At the time of publishing this blog, more details about this syndrome are emerging. There are some health officials calling this condition “pediatric multisystem inflammatory syndrome.” This diagnosis will present a more difficult coding decision pathway.

There have been no updates to American Hospital Association (AHA) FAQ since April 28, 2020. A representative of the Children’s Hospital Association reported that they are researching the topic and seeking to obtain case studies to present to Coding Clinic.

The author reached out to the CDC for insight and is awaiting a response. Any comments will be shared in a future blog.

A final thought: Is this a perfect example of why we need real time coding advice from authoritative bodies? I realize it is difficult to classify a mystery condition, but we have to decide quickly. The data needs to be accurate, timely and consistent.

CPT: Introduction to Procedural Coding
Let Gail Show You How!  CPT: Introduction to Procedural Coding prepares you for the broad challenges of outpatient coding in Ambulatory Surgery Centers, Hospitals, and multi-Physician Group Practices. Learn more here.

About the Author

Gail Smith, MA, RHIA, CCS-P
Gail Smith, president of Gail I. Smith Consulting, is a nationally recognized coding educator. For most of her career Gail was an associate professor and director of the health information management (HIM) program at the University of Cincinnati and HIM program director at Cincinnati State Technical and Community College. Gail is a currently a consultant with a software company specializing in medical terminologies. Gail has authored several textbooks, including Basic Procedural Terminology and HCPCS Coding published by the American Health Information Management Association (AHIMA).   An advocate for advanced coder training, Gail is the author of several titles for Libman Education including CPT: Introduction to Procedural Coding and two courses on the unique challenge of CPT coding in the pediatric setting: CPT for Pediatrics and CPT for Pediatrics: Advanced Orthopedic Coding. According to Gail: “One cannot possibly teach every coding scenario a coder will experience in their career. That is why coding education needs to focus instead on how to arrive at an accurate and defensible code assignment. It is the difference between learning to fish, and being given a fish. If you learn to code instead of being given answers to memorize, you will be able to arrive at the complete codes more quickly and with greater confidence.”

One thought on “Kawasaki: COVID-19-related Illness in Children

  1. Lori Drodge - May 20, 2020 at 12:00 am

    The multiple updates in the AHA FAQs have been very helpful. It would be great if these could continue with other topics as well for more timely information overall.

    I look forward to the CDC response. Thanks Gail!