Summary: This 12-week-old female
infant born at term after an uncomplicated pregnancy presented to the emergency
room with a fever, rash, acrocyanosis of the fingers and toes, and lethargy. The white count was 34,000 (normal 6,000-17,500);
hematocrit was 20.9 (normal 28-42) and hemoglobin was 6.2 g/dl (normal 9-14
g/dl). Thrombocytosis was present with measured at 916,000 /µL. Erythrocyte
sedimentation rate (ESR) was 50 mm/hr (normal 0-20 mm/hr), and the C-reactive
protein (CRP) was normal. Echocardiography, performed because of suspected
congestive heart failure, demonstrated abnormal coronary arteries with
aneurysms, measuring up to 5 mm in size. A computed tomography (CT) scan of the
chest, abdomen, and pelvis was performed on a Somatom Definition AS 64 row
scanner (Siemens, Forchheim Germany). Images were obtained following the administration
of 13 mL of Optiray-350 nonionic contrast material (Covidien, St. Louis, MO)
(Figure 1). The acquisition parameters were 0.6 mm collimation, 65 auto mAs, 80
auto kV, pitch of 1, and a gantry rotaion time of 0.33 sec. CT dose index (CTDI) volume
was 1.07 mGy and dose length product (DLP) was 47 mGy·cm. Using the conversion
factor (0.044 mSv/(mGy·cm) for trunk CT examinations, effective dose (E) is
estimated to be 2.1 mSv (47 mGy·cm x 0.039). Over the next few days, progressive
ischemia and gangrene of the fingers and toes occurred.
Infantile Kawasaki disease with peripheral gangrene
dilatation of the right coronary artery and focal aneurysms of the common iliac
arteries, external iliac arteries, and proximal brachial arteries
Kawasaki disease is a
febrile, inflammatory disease of childhood of unknown etiology, usually seen in
children between the ages of 1 and 5 years.1 It is diagnosed
clinically since there are no specific laboratory tests for diagnosis, although
thrombocytosis and an elevated erythrocyte sedimentation rate and C-reactive
protein may be noted. Classically,
4 of 5 clinical criteria must be present in order to establish the diagnosis: 1) bilateral nonsuppurative conjunctivitis
(i.e., conjunctival injection,
2) erythema of the lips or the oropharynx mucosa including the tongue (i.e., “strawberry
tongue”), 3) edema of the hands and/or feet with periungual desquamation, 4) a nonvesicular
truncal rash, and 5) cervical lymphadenopathy. Children under 3 months of age account
for less than 2% of patients.2 Younger patients, especially infants,
often have only 2 or 3 of the classic diagnostic features of Kawasaki disease.
Because there are incomplete criteria, the condition is often called incomplete
or atypical Kawasaki disease.
In classic Kawasaki disease, coronary artery aneurysms occur
in 20% to 25% of children.3 Incomplete Kawasaki disease in young
infants is more likely to be associated with coronary aneurysms and peripheral
gangrene.2 The frequency of coronary aneurysm is 79% in infants
under 6 months of age compared with 44% in infants 6-12 months of age. The left
coronary artery is involved more often than the right coronary artery. Small
coronary aneurysms (< 5 mm) may regress, while larger ones may remain
unchanged or progress to stenosis.1 A low-dose CT scan on a fast
helical scanner, such as the Somatom Definition AS 64, is able to confirm the
diagnosis of coronary artery and systemic artery aneurysms in Kawasaki disease.
Although coronary artery
aneurysms are the best-known vascular feature of Kawasaki disease, systemic
vasculitis of the medium sized muscular arteries and veins also occurs.3
Systemic artery aneurysms develop in < 2% of patients.3-5 The
axillobrachial arteries and iliofemoral arteries are the most common involved,
as in this patient.4 Other cardiac manifestations of Kawasaki
disease include pericarditis with pericardial effusion, wall-motion abnormalities,
valvulitis, papillary muscle dysfunction, and acute mitral regurgitation due to
chordate tendineae rupture.1
Treatment includes intravenous
immunoglobulins (IVIG), high dose steroids, prostaglandins, Infliximab
(Remicade), which works by binding tumor necrosis factor alpha, and aspirin. Infantile Kawasaki disease
is often resistant to therapy and carries a high mortality.2 The
peripheral ischemia and gangrene progressed in our patient despite therapy.
We present a patient with infantile Kawasaki disease with
coronary and systemic arterial aneurysms. Fast helical CT scanning was able to provide
excellent delineation of anatomy with very low radiation dose, allowing for
- Chung CJ, Stein L. Kawasaki disease: A review.
Radiology. 1998; 208:25-33.
- O’Connor MJ, Saulsbury FT. Incomplete and
atypical Kawasaki disease in a young infant: Severe, recalcitrant disease
responsive to infliximab. Clin Pediatr
(Phila). 2007;46: 345-348.
- Yacoe ME, Dake MD. Development and
resolution of systemic and coronary artery aneurysms in Kawasaki disease. AJR Am J Roentgenol. 1992:159:708-710.
- Cura MA, Haska ZJ, Weintraub J,
Benvenisty A;SIR 2004 film
panel case: Systemic artery aneurysms in
Kawasaki disease. J Vasc Interv Radiol. 2004:15:1009-1011.
- Durall AL, Phillips JR, Weisse ME, Mullett
CJ;Infantile Kawasaki disease and
peripheral gangrene. J Pediatr. 2006;149:131-133.