Overview
Kawasaki disease is an acute febrile illness associated with multiorgan vasculitis of unknown etiology that primarily affects infants and children.[1, 2, 3, 4] The disease probably has existed for a long time, but it was not recognized as a separate entity until Dr Tomisaku Kawasaki first described it in 1967.[5] Kawasaki disease was later reported in the English-language literature in 1974.[6]
Kawasaki disease is a multiorgan disease. During the acute phase, children may develop aseptic meningitis, hyperemic tympanic membrane, or uveitis. Neurologic complications, which include facial nerve palsy, seizures, and ataxia cerebral infarctions, are rare. Other common features include diarrhea, vomiting, abdominal pain, and pneumonitis. Gallbladder hydrops (acute acalculous distention of the gallbladder; see image below) may occur in the first 2 weeks of illness, it may be the result of the extension of periportal inflammation to the cystic duct, and it is typically self-limited. Arthritis and arthralgia are common in the acute phase. Findings that include erythema and induration at the site of recent Bacille Calmette-Guérin vaccination, testicular swelling, and peripheral gangrene also have been reported in patients with this disease.
Kawasaki disease. Sonogram of the right upper quadrant shows hydrops of the gallbladder. Note the size of the gallbladder compared with that of the inferior vena cava. Courtesy of Dr S. Methratta, UMDNJ-New Jersey Medical School. The ECG is usually normal.
Radiography
Chest radiography is not routinely performed to evaluate for Kawasaki disease, and the results are often normal.[7]
If coronary aneurysm and calcification of the coronary artery aneurysm wall are present, they may be detected as cystic calcification in the region of the coronary vessels, overlying the heart shadow.
Computed Tomography
CT is not routinely performed for the evaluation of Kawasaki disease; however, CT is more sensitive than chest radiography in detecting coronary calcifications. Contrast-enhanced CT may demonstrate enhancement of the vessel walls in the coronaries, particularly in the acute phase.[8]
Although no published data describe the use of CT in detecting Kawasaki-related coronary aneurysms, recent advances in multihelical technologies, as well as electron-beam CT, may be useful in detecting and sizing coronary artery aneurysms. CT is also useful in evaluating the size of the gallbladder and in detecting gallstones.
Magnetic Resonance Imaging
MRI is not performed routinely to investigate Kawasaki disease; however, MRI may depict coronary aneurysms. MRI with gadopentetate dimeglumine can be use to noninvasively and simultaneously evaluate myocardial thinning and the presence of circulation. In addition, MRI is considered useful for the long-term follow-up of patients with Kawasaki disease.[9] Recent advances in cardiac MRI may enable the detection of early changes in Kawasaki disease. Acute-phase disease may be detected on MRI with or without Gd enhancement as an area of inflammation characterized by a high signal intensity on T2-weighted images; this area may be enhancing.[8, 10, 11]
Gadolinium-based contrast agents have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness.
Ultrasonography
When performed by skilled imagers, 2-dimensional echocardiography has 100% sensitivity and approximately 90% specificity for detecting aneurysms of the proximal coronary arteries. (See the image below.)
Kawasaki disease. Sonogram of the right upper quadrant shows hydrops of the gallbladder. Note the size of the gallbladder compared with that of the inferior vena cava. Courtesy of Dr S. Methratta, UMDNJ-New Jersey Medical School. Angiography
Angiography may be used to evaluate for coronary aneurysms (see the image below).[12] Although it is considered to be the criterion standard for evaluating coronary aneurysms, ultrasonography provides an alternative method of detecting coronary aneurysms, particularly proximal ones.
Kawasaki disease. Angiogram of the ascending aorta and coronary vessels shows aneurysmal dilatation of the coronary vessels. Courtesy of Dr Chong Hyun Yoon, Professor of Radiology, University of Ulsan, Seoul, Korea. AHA. Diagnostic guidelines for Kawasaki disease. American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. Am J Dis Child. Nov 1990;144(11):1218-9. [Medline].
Japan Kawasaki Disease Research Committee. Diagnostic guideline of Kawasaki disease. Tokyo:. Japan Kawasaki Disease Research Committee;1984.
Leung DY. Kawasaki disease. Curr Opin Rheumatol. Jan 1993;5(1):41-50. [Medline].
Shulman ST, De Inocencio J, Hirsch R. Kawasaki disease. Pediatr Clin North Am. Oct 1995;42(5):1205-22. [Medline].
Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi. Mar 1967;16(3):178-222. [Medline].
Kawasaki T, Kosaki F, Okawa S. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics. Sep 1974;54(3):271-6. [Medline].
Lapierre C, Bitsch A, Guérin R, Garel L, Miró J, Dahdah N. Follow-up chest X-ray in patients with Kawasaki disease: the significance and clinical application of coronary artery macro-calcification. Pediatr Cardiol. Jan 2010;31(1):56-61. [Medline].
Cantin L, Chartrand-Lefebvre C, Marcotte F, Pressacco J, Ducharme A, Lapierre C. Coronary artery noninvasive imaging in adult Kawasaki disease. Clin Imaging. May-Jun 2009;33(3):181-7. [Medline].
Fujiwara M, Yamada TN, Ono Y. Magnetic resonance imaging of old myocardial infarction in young patients with a history of Kawasaki disease. Clin Cardiol. 2001;24:247-52. [Medline].
Greil GF, Seeger A, Miller S, Claussen CD, Hofbeck M, Botnar RM, et al. Coronary magnetic resonance angiography and vessel wall imaging in children with Kawasaki disease. Pediatr Radiol. Jul 2007;37(7):666-73. [Medline].
Mavrogeni S, Papadopoulos G, Karanasios E, Cokkinos DV. How to image Kawasaki disease: a validation of different imaging techniques. Int J Cardiol. Feb 20 2008;124(1):27-31. [Medline].
Kato H, Ichinose E, Yoshioka F. Fate of coronary aneurysms in Kawasaki disease: serial coronary angiography and long-term follow-up study. Am J Cardiol. May 1982;49(7):1758-66. [Medline].

