British Journal of Radiology 75 (2002),289-290 © 2002 The British Institute of Radiology
A nipper from Nippon?
I Vlahos, MBBS, MRCP, FRCR
and
K McHugh, FRCPI, DCH, FRCR
Department of Radiology, Great Ormond Street Hospital for Sick Children, Great Ormond Street, London WC1N 3JH, UK
Correspondence: I Vlahos, Academic Department of Radiology, St Bartholomew's Hospital, 59 Bartholomew Close, London EC1A 7ED, UK
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Introduction
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A 2-year-old Caucasian boy presented with a 3-week history of malaise, pyrexia, poor appetite and sore throat. Past medical history was unremarkable. An initial diagnosis of acute tonsillitis was made and the child was treated with penicillin. However, he remained pyrexial and unwell, developing a maculopapular rash on the lower extremities as well as arthralgias accompanied by ankle swelling. Examination at that time demonstrated small oral ulcers and cervical lymphadenopathy. Laboratory investigations showed leucocytosis, an elevated erythrocyte sedimentation rate (ESR) and negative blood cultures. An initial chest radiograph was normal. Over the next 10 days the fever and rash subsided and the leucocyte count returned to normal levels, but a gradual thrombocytosis was noted. A presumptive new diagnosis was made and the child was treated appropriately.
A chest radiograph from 3 years later is provided (Figure 1
), with an accompanying CT examination (Figure 2). What do the radiological investigations show? What is the underlying diagnosis and what complication has arisen?
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Answer
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The abnormal convexity below the pulmonary artery (third mogul) on the chest radiograph was shown on contrast enhanced CT to be due to a large fusiform aneurysm of the left anterior descending (LAD) coronary artery. The findings were confirmed on echocardiography and selective coronary angiography (Figure 3). The preceding acute febrile illness confirms the diagnosis of Kawasaki's disease (mucocutaneous lymph node syndrome). The child was treated with aspirin, prostacyclin and gamma globulin and he made a good recovery.
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Figure 3. Echocardiogram. AO, aortic root; RVOT, right ventricular outflow tract; LAD, left anterior descending coronary artery.
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Kawasaki's disease is an illness of early childhood of unknown aetiology, initially described in Japan but now recognized worldwide. An initial, usually self-limiting, febrile episode is characterized by hyperaemia of the oropharynx, conjuctival injection, a strawberry tongue and cervical lymphadenopathy. Peripheral oedema and rashes occur, with occasional palmar or plantar desquamation [1].
Generalized vasculitis develops in 5–10%, with a predilection for coronary arterial involvement leading to aneurysm formation. Subsequent thrombosis or narrowing may result in acute infarction or chronic ischaemia with left ventricular malfunction. Pancarditis with a variety of conduction abnormalities can occur.
Coronary aneurysms are seen on selective angiography in 40% of cases at 10–12 days from disease onset, diminishing to 20% at 30 days with only 10% at 60 days. The left coronary artery, and in particular the LAD coronary artery, is involved in approximately two-thirds of affected cases. 70% of all aneurysms are proximally located [2]. Aneurysms less than 4 mm almost always regress over 6 months to 2 years without sequelae [3]. Regression is unlikely in aneurysms over 8 mm, and stenosis and thrombosis occur in approximately 8%. Saccular-shaped aneurysms, onset of disease after 2 years and a fever exceeding 21 days are poor prognostic factors [4]. Overall mortality is 1–2% due to myocarditis, myocardial infarction or acute mitral insufficiency due to papillary muscle dysfunction.
Echocardiography of the coronary arteries is the mainstay of non-invasive assessment in Kawasaki's disease, with a 92% sensitivity and no false positives [3]. Selective angiography should be performed in lesions over 4 mm at 1–3 months to assess morphology and size of aneurysms.
CT is complementary to echocardiography in assessing aneurysms [5]. Ultrafast CT and isotope studies may be utilized to quantify myocardial perfusion [6]. ECG-gated MRI demonstrates aneurysms well but discriminates poorly between slow flow and thrombus [7]. Recent evidence suggests significant progressive stenoses may occur even after regression of aneurysms. This may predispose to premature atherosclerotic disease [8].
Early treatment with aspirin, other anti-platelet agents and gamma globulin reduces the incidence of aneurysms and later sequelae. Warfarin prophylaxis is used in larger aneurysms, and thrombolytic therapy is used for acute thrombosis. Coronary artery bypass grafting is used selectively for multiple segmental stenoses. Steroids are contraindicated owing to associated aneurysm rupture.
Received for publication January 2, 2001.
Accepted for publication January 24, 2001.
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References
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Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Jpn J Allerg 1967;16:178–222.
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Onouchi Z, Shimazu S, Kiyosawa N, Takamatsu T, Hamaoka T. Aneurysms of the coronary arteries in Kawasaki disease. An angiographic study of 30 cases. Circulation 1982;66:6–13.[Abstract/Free Full Text]
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Kato H, Koike S, Yokoyama T. Kawasaki disease: effect of treatment on coronary artery involvement. Pediatrics 1979;63:175–9.[Abstract/Free Full Text]
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Kato H, Akagi T. Ischaemic heart disease in Kawasaki disease. Prog Pediatr Cardiol 1997;6:219–26.
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Frey EE, Matherne GP, Mahoney LT, Sato Y, Stanford W, Smith WL. Coronary artery aneurysms due to Kawasaki disease: diagnosis with ultrafast CT. Radiology 1988;167:725–6.[Abstract/Free Full Text]
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Rumberger JA, Feiring AJ, Lipton MJ, Higgins CB, Marcus ML. Use of ultrafast CT to quantitate regional myocardial perfusion: a preliminary report. J Am Coll Cardiol 1987;9:59–69.[Abstract]
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Bisset III GS, Strife JL, McCloskey J. MR imaging of coronary artery aneurysms in a child with Kawasaki disease. AJR 1989;152:805–7.[Free Full Text]
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Suzuki A, Kamiya T. Visualisation of the coronary arterial lesions in Kawasaki disease by coronary angiography. Cardiol Young 1991;1:225–33.
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