Vascular imaging findings with high-pitch low-dose dual-source CT in atypical Kawasaki disease

Abstract

Kawasaki disease (KD) is an acute, febrile, self-limited vasculitis that affects small-and medium-sized arteries, with a predilection for the coronary arteries (1). The disease mainly affects infants and children younger than 5 years of age. The etiology of KD is unknown, and there are no specific diagnostic tests (2). The diagnosis of this disease is ideally made by clinical criteria according to the American Heart Association. Typical or classical Kawasaki (TKD) disease can be diagnosed if a fever lasts longer than 5 days and if a patient has 4 of the 5 clinical features. However, according to the Japanese guidelines, atypical or incomplete Kawasaki disease (AKD) is defined as the presence of 4 or fewer of the main findings of KD regardless of the presence or absence of coronary artery aneurysm (CAA).In some cases with unexplained prolonged fever, the clinical features may be insufficient for the diagnosis of TKD. In this instance, AKD may be considered. Determining the presence of aneurysms in small-and medium-sized arteries is quite important in the diagnosis of AKD (3). CAAs are seen in up to 25% of cases, and systemic artery aneurysms (SAA) are seen in 2% (4). Especially in atypical cases, cardiac and other vascular complications can be more frequently seen because the diagnosis can be delayed. Therefore, early diagnosis and treatment is very important to prevent complications (5).There are several imaging modalities for scanning vascular structures, including echocardiography, magnetic resonance imaging (MRI), computed tomography (CT) and ultrasonography (US). In the diagnosis of AKD, each of these imaging techniques has specific handicaps. High-pitch low-dose CT angiography can be very useful in screening for CAA and associated SAA in patients with AKD. PURPOSEDetermining the presence of aneurysms, thrombosis, and stenosis is very important for the diagnosis of atypical Kawasaki disease (AKD) and in the follow-up of AKD patients with aneurysms. We aimed to demonstrate high-pitch low-dose dual-source computed tomography (CT) angiography findings in pediatric patients with AKD. METHODSOver a 5-year period, high-pitch low-dose CT angiography was performed to determine vascular aneurysms or occlusions in 17 patients who had suspected AKD. The patients ranged from 2 months of age to 11.3 years, with a mean age of 3 years. The American Heart Association's criteria were used to diagnose AKD. RESULTSWe did not detect any vascular problems in 6 of the patients, and they were not included in our study. Arterial aneurysms were present in 11 patients (aged 2 months to 11.3 years; mean age, 4.2 years; 7 males). In one patient, there was also a thrombus at an arterial aneurysm. Coronary artery aneurysms were detected in 7 patients and systemic artery aneurysms were detected in 7 patients. Three patients had both systemic and coronary aneurysms. CONCLUSIONOur results suggest that high-pitch low-dose dual-source CT can detect all types of aneurysms, stenosis and occlusions of vessels in patients with AKD who were not pre...

An Update on Reports of Atypical Presentations of Kawasaki Disease and the Recognition of IVIG Non-Responder Children

“…All results have been tabulated and analyzed in Table 1 and Table 2 . The first one reports on the general data and canonical KD manifestations found in such case reports with atypical KD; the second one details the non-canonical manifestations which were observed in these same patients, challenging the proper diagnosis of KD [ 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73…”

“…Coronary arteries were the most common location for aneurysms in patients with atypical KD, but also the involvement of systemic vessels had been disclosed. As reported by Kantaci et al, several patients with atypical KD presented vascular lesions which were misdiagnosed by ultrasound assessment or magnetic resonance angiography, but were detected by high-pitch low-dose CT angiography [ 28 ].…”

Pitfall for systemic artery aneurysms evaluation using electrocardiogram-gated subtracted three-dimensional fast spin echo sequence of magnetic resonance imaging in patients with Kawasaki disease

“…Therefore, patients with KD should be evaluated for both coronary and systemic arterial aneurysms. The evaluation of SAA is mainly performed via angiography (AG) [1] , computed tomography angiography [6] , and contrast-enhanced magnetic resonance angiography (CE-MRA) [2] . However, since those examination are intended for pediatric patients, there are concerns regarding radiation exposure and the use of contrast media [7] .…”