kawasaki disease , first described by tomisaku kawasaki in japan in 1967 , is an acute systemic vasculitis of unknown etiology involving the small and medium - sized vessels with predilection for coronary artery involvement .
the disease affects infants and children under the age of 5 years with no racial predilection and a sex ratio of 1.5:1 in favor of male children . typically , the disease is a multisystem febrile vasculitis with predominant cardiovascular manifestations .
if untreated , it can lead to development of coronary artery aneurysm in nearly 25% of patients and systemic vascular aneurysms in less than 2% cases .
a 6-month - old male infant was brought to the emergency department with complaints of fever for 1 month , passage of dark stools for 1 week , and discoloration of upper extremity for 2 days .
the child was referred for doppler imaging ( philips hd 11 xe ultrasound machine , philips usa ) of the upper extremity and sonography of the abdomen on emergent basis .
doppler usg showed partially thrombosed fusiform aneurysm of right subclavian artery , axillary artery , brachial artery , and non - thrombosed aneurysm of the right subclavian artery [ figure 1a and b ] .
color doppler image shows non - thrombosed aneurysm of left subclavian artery ( a ) with partially thrombosed fusiform aneurysm of right subclavian artery ( b ) subsequently , ultrafast low - dose ct angiography ( philips brilliance 40 slice ct scanner ; philips usa ) was done in emergency settings which confirmed the color doppler findings and additionally showed aneurysms of bilateral common carotid , extracranial internal and external carotid , and bilateral vertebral arteries [ figure 2 ] .
there were aneurysms involving multiple coronary arteries , with giant fusiform aneurysm of left anterior descending artery measuring 13 mm [ figures 3 and 4 ] .
ct angiography of thoraco - abdominal aorta revealed small saccular aneurysm at the origin of celiac artery measuring 29  25 mm , with fusiform dilatation of superior mesenteric artery [ figure 5a and b ] .
there were non - thrombosed fusiform aneurysms of bilateral common iliac artery and common femoral artery without the involvement of the main aortic trunk [ figure 6 ] .
ct angiography ( coronal mip image ) shows dilated bilateral subclavian and axillary arteries with hypodense non - enhancing thrombus within the right subclavian 
also , fusiform aneurysms of bilateral extracranial carotid and vertebral arteries are noted ( short arrows ) axial ct angiography image at the level of aortic sinus shows giant aneurysm of left anterior descending artery ( short arrow ) with aneurysm of right coronary artery ( long arrow ) volume - rendered image ( left anterior oblique view ) shows giant fusiform aneurysm of left anterior descending artery aneurysms of branches of abdominal aorta . ct angiography sagittal mip image ( a ) shows aneurysm of superior mesenteric artery ( arrow ) . volume - rendered image ( b ) shows saccular aneurysm measuring 29  25 mm at the origin of celiac artery ( arrow ) ct angiography coronal mip image shows fusiform aneurysms of bilateral common iliac and femoral arteries based on the imaging features , a possibility of infantile panvasculitis was considered .
review of the clinical parameters revealed normochromic - normocytic anemia , leukocytosis , and elevated erythrocyte sedimentation rate ( esr ; 43 mm in first hour ) and c - reactive protein ( 34 mg / l ) .
the blood cultures and peripheral smears were negative for microbes and had no malarial parasites [ table 1 ] .
based on the clinical history of conjunctivitis , fleeting rash , swelling of extremities , and multiple aneurysms of medium to small - sized vessels , a diagnosis of infantile atypical kawasaki disease was made .
apart from the supportive management , the infant was started on intravenous immunoglobulin , but succumbed to death on the third day of admission .
kawasaki disease or mucocutaneous lymph node syndrome is a multisystem idiopathic vasculitis affecting medium - sized vessels .
the diagnosis is made based on the amalgam of clinical criteria which occur in a sequential and fleeting progression , thus proving a diagnostic challenge .
the defining criteria for the diagnosis of kawasaki disease include fever for at least 5 days and four of the following five principal features : conjunctivitis , mucositis , cervical lymphadenopathy , truncal rash , and edema of the extremities .
atypical forms of the disease are not rare and are defined as the disease with three principal features or coronary artery involvement with less than three principle diagnostic criteria .
the higher incidence of the atypical form of the disease in infantile age group and the unusual presentations lead to a delay in diagnosis and management , consequently increasing the complication rates .
coronary artery aneurysms , the most serious complication of the disease , occur in up to 25% , whereas other systemic aneurysms occur in less than 2% cases . majority of the mortality in kawasaki disease is attributed to cardiovascular events secondary to these aneurysms .
aneurysm formation is a major cause of morbidity and mortality in children with kawasaki disease with increased risk in patients with delayed diagnosis , age less than 6 months or more than 9 years , male gender , prolonged ( > 14 days ) or persistent fever despite treatment , leukocytosis , elevated esr , low albumin , hyponatremia ( < 135
thus , the diagnosis of kawasaki disease is suspected on the basis of clinical features and an exclusion of other possibilities .
the possible differential diagnoses in the given case of infantile vasculitis consisted of takayasu disease , fibromuscular dysplasia , infective vasculitis , and kawasaki disease .
takayasu 's arteritis is a panarteritis of young adults with involvement of aorta and its main branches with the possible involvement of coronary or pulmonary vasculature .
aneurysms and dilatation in aorto - arteritis commonly involve the ascending aorta and aortic arch .
fibromuscular dysplasia is a congenital non - inflammatory angiogenic dysplasia with predominant involvement of the renal vasculature in young females and classically shows string of beads appearance of angiography .
to conclude , this report emphasizes the role of a radiologist in emergency settings in suspecting the diagnosis and the role of imaging in diagnosis of clinically silent aneurysms in infantile atypical kawasaki disease .
 kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age . 
 there are no specific diagnostic tests , and thus , the diagnosis of the disease is primarily made on the basis of clinical criteria . 
 unusual presentations of kawasaki disease have been variably reported from different parts of the world . 
 however , presentation of the disease in the form of peripheral thromboembolism and florid non - coronary aneurysms has rarely been described this report describes the imaging findings in infantile atypical kawasaki disease with aneurysms of multiple medium - sized arteries , including coronary arteries , emphasizing the detection of clinically silent aneurysms in the disease . 