Kawasaki Disease

Anita Oza, MD

Cynthia K. Rigsby, MD

Children's Memorial Hospital

Abstract

Three different patients with a history of fever for at least five days, and differing manifestations of injected/fissured oropharynx, rash, erythema of the palms and soles, conjunctivitis, and cervical lymphadenopathy.

Keywords

1556 Kawasaki Disease coronary aneurysms


Publication Date: 2009-11-04

History

Image 1: 13 yo m

Image 2&3: 12 yo female

Image 4-7: 4 yo female

All 3 patients have a history of fever for at least five days, and differing manifestations of injected/fissured oropharynx, rash, erythema of the palms and soles, conjunctivitis, and cervical lymphadenopathy.

Findings

Patient #1 Image 1: MIP image of the heart demonstrates a giant aneurysm of the proximal RCA, followed by mild dilation and ectsia of the mid RCA.

Patient #2 Image 2&3: MIP images demnstrate a giant fusiform aneurysm of the proximal to mid RCA with poor opcaification consistent with thrombosis

Patient #3: Images 4,5,& 6: Extensive extra-cardiac aneurysm formation in a 4 yo with Kawasaki disease. Image 5: Fusiform aneurysm involving the common iliac arteries. Image 6: Left brachial artery demonstrates a saccular aneurysm. Image 7: Fusiform aneurysm of the right brachial/axillary artery junction. Just superior and peripheral to the aneurysm is extensive thrombus formation. Peripheral vascular calcification is also seen.

Diagnosis

Coronary, axillary, and iliac artery aneurysms secondary to Kawasaki Disease.

DDx

Coronary aneurysm secondary to trauma, procedure (angioplasty, stent, atherectomy) mycotic emboli, connective tissue disease (SLE, Marfan, Behcet).

Atypical or incomplete Kawasaki Disease should be considered in those patients who do not meet the diagnostic criteria but have a similiar clinical course with development of coronary artery aneurysms.

Discussion

Kawasaki disease is the most common systemic vasculitis in childhood after Henoch-Schonlein Purpura, and the most comon cause of acquired heart disease among children living in the Western countries. Kawasaki Disease, also known as acute febrile mucocutaneous disease, is a systemic vasculitis of medium and small arteries and occasionally veins. It is of unknown etiology, suspected to be a abnormal immune response to a toxin or an infectious agent. In the United States 80% of Kawaski Disease presents in patients less than 5 years of age. The peak inicidence is in 1-2 year-olds. The disease is usually self-limited, with the greatest morbidity and mortality arising from coronary artery and myocardial involvement.

Kawaski disease has been traditionally diagnosed based on clinical criteria. This includes Fever of 5 days duration and having four out of the five following: 1) Bilateral conjunctival injection 2) Oral changes 3) Rash 4) Changes of peripheral extremities 5) Cervical lymphadenopathy

Coronary aneurysms are the most well known complication of Kawasaki Disease, and occur in 20-25% of children with Kawasaki disease not treated with intravenous gamma globulin or aspirin. The left coronary artery is more commonly affected than the right. The proximal artery is more commonly involved than the distal. Coronary artery aneurysms have been classified as small if they are less than 5mm, medium if they are 5-8 mm, and giant if larger than 8mm. Giant aneurysm have the worst prognosis and are at greatest risk of developing thrombosis, stenosis, and myocardial infarction.

Evaluation of coronary aneurysms typically begins with echocardiogram. Magnetic resonance imaging, coronary angiogram, and CT angiogram are also used in evaluation.

Small aneurysms likely regress, medium and giant aneurysms can progress. Ring calcifications can be seen in 36% of adults with a history of Kawasaki Disease.

Other cardiac complications include myocarditis, pericarditis, valvulitis, papillary muscle dysfunction, acute mitral regurgitation, arrhythmias, cardiac function abnormalities, and noncoronary vascular abnormalities as seen with the last patient who demonstrated aneurysm formation of the axillary and iliac arteries.

References

  1. Chung JC, Stein LS. Kawaski Disease: A review. Radiology 1998 208:25-33
  2. Cimaz R, Sundel R. Atypical and incomplete Kawasaki Disease Best Practice and Research Clinical Rheumatology 2009 23:689-697
  3. statdx.com

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