Echocardiograms in Typical vs Incomplete Kawasaki’s

On my first shift in the Children’s ED, I saw a child with what appeared to be Kawasaki’s Disease.  She was a referral patient who had been seeing her PCP throughout her current illness.  The child’s PCP had done appropriate lab work on her, and the results combined with the child’s clinical picture warranted a referral to our ED for further evaluation and management.

When she presented to us, her symptoms seemed to be resolving.  She had bilateral conjunctivitis that her mom stated was improving, she had a mild rash and swelling of her tongue and she had a dry and peeling rash in her genital region.  Her mother stated that her limbs had been swollen but were now returning to normal size and that her fever had been high and unrelenting for several days, but was normal (36.7 and 37.0) in our ED.  To me, this child was healing and did not require treatment at this time.  But the question was whether or not an echocardiogram was indicated for this patient.

According to the UpToDate website, an echocardiogram is indicated as soon as Kawasaki’s is even suspected as a diagnosis.  The test is highly sensitive and specific for detecting coronary arterial dilation in the acute stage of the disease.  The initial echo will help establish a baseline for follow-up echos that should take place two and six weeks after the original if Kawasaki’s Disease is the true diagnosis.  But there is a blurred line between Typical Kawasaki’s Disease and Incomplete (Atypical) Kawasaki’s Disease.  The latter consists of children who do not fulfill diagnostic criteria but have many of the same signs and symptoms.  These patients have the high fever but with less than four signs of mucocutaneous inflammation and may also have an unrelated complaint to confuse the clinical picture.  UpToDate gives indications for echocardiography in these patients as well:  an echocardiogram is necessary either when the CRP or ESR lab values are elevated along with other labs including WBC, ALT, albumin or a UA.  Another indication is when periungual desquamation is seen after resolution of the fever.  In both of these situations, a normal echocardiogram would likely rule out the disease, as long as the fever resolves.

In my patient, I witnessed the conjunctivitis, tongue edema and the genital rash.  Her EMR that was sent from her PCP was reviewed, and I saw documentation of the high fever for over five days and elevated ESR, CRP and WBC.  Even though this information would only give my patient the diagnosis of Incomplete Kawasaki’s Disease, the clinical picture plus the lab values earned her the echo.  A flowchart in the Pediatrics in Review journal led us to the same course of management.  Thus, I now recognize the importance in evaluating patients suspicious for Kawasaki’s Disease with an echocardiogram early and have learned how the results can help distinguish between Typical and Incomplete Kawasaki’s Disease.

References

  1. Incomplete (atypical) Kawasaki disease.  Robert Sundel.  Last updated May 06, 2014.
  2. Cardiovascular sequelae of Kawasaki disease.  Jane W Newburger, Sarah D de Ferranti and David R Fulton.  Last updated Feb 20, 2014.
  3. Kawasaki Disease. Mary Beth F. Son and Jane W. Newburger. Pediatrics in Review Journal.
  4. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease.  Jane W. Newburger, et al. American Heart Association Circulation Journal.

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