Discussion
Atypical initial presentation in KD is very important, because it can
lead to serious complications, especially cardiovascular adverse events.
Atypical or incomplete KD diagnosis is challenging. Uncommon
manifestations of KD in children have been reported in previous case
reports including pyuria, meningitis, ventricular arrhythmia, or shock
(9, 10). Introducing such atypical presentations of KD will be
beneficial in increasing physicians’ attention in suspicious to KD and
prevent delayed diagnosis. In the case, we mentioned here, a
seven-year-old girl presented with initial isolated torticollis that
subsequently other classic symptoms were added and finally giant
aneurysm was diagnosed.
Torticollis in this seemed to be due to painful cervical lymphadenitis
(11). The rate of cervical lymphadenopathy occurring as the initial
presenting symptoms is only approximately 12% (12). Despite of uncommon
manifestation, some cases are reported previously KD presented by
torticollis. Dyer et al. reported a 6-year-old male who presented with
torticollis. In their case, a series of investigations for elevated
inflammatory markers revealed dilated coronary artery aneurysms on
echocardiogram and treated with high-dose IVIG and low-dose aspirin
(11). Runel-Belliard et al. reported a case of KD with arthritis in a
4-year-old girl whose initial presentation was a febrile torticollis
(13). In another case, a nine-year-old boy presented with fever,
lymphadenopathy, parotitis, and torticollis with initial diagnosis of
infective parotitis and finally KD was diagnosed (14). In our case, she
was underwent CABG because of LV dysfunction and after that, she was
followed and was healthy until 2 years later.
Dilatation of the coronary arteries can occur in approximately 10-20%
of KD patients. In half of them, the aneurysms can regress within 1 or 2
years, and no abnormalities are found by angiography in the
coronary-arterial system. In the remaining, in contrast, the aneurysms
can persist with obviously irregular lumens of the coronary arteries. In
3% of the patients initially having aneurysms, coronary-arterial
obstruction progresses. The time-span between the onset of the disease
and development of the coronary-arterial stenosis leading to CABG varies
from several months to 20 years. The indications for CABG are to be
determined not only by findings derived from angiography, but also on
the basis of other clinical factors, such as severity of myocardial
ischemia, history of myocardial infarction and ventricular performance
(15-19). It is very difficult to determine the indications for catheter
intervention and CABG in children, based on the diagnosis and
localization of the affected coronary artery, using standard methods
(20).