An atypical case of Kawasaki disease

An 8-month-old male developed a fever of 40°C, cough and rhinorrhea. He was diagnosed to have a right otitis media and was treated with amoxicillin but continued to have fevers. A sepsis workup was negative, the patient was given one dose of IM ceftriaxone and continued to be febrile and had increased somnolence and decreased oral intake. On examination, the child was found to be alert but appeared moderately ill. Temperature was 39.7°C. The lips were dry, cracked and erythematous. There was no lymphadenopathy. There were no other positive findings. Investigations showed WBC 14.6 x 10³/cmm, hemoglobin 10.4 g/dl, platelets 177 x 10³/cmm, serum sodium 130 mEq/L. On day three of the hospitalization, the child had increased swelling of the extremities, nonpurulent conjunctivitis, red lips and a total of five days of fever. An echocardiogram showed a left main coronary artery aneurysm and an aneurysm of the right coronary artery and involvement of the left anterior descending coronary artery. The patient was considered to have Kawasaki disease even though the absence of cutaneous rash and lymph node enlargement made it atypical. He was given IVIG and high doses of aspirin. Three more doses of IVIG and also steroids and dipyridamole were given because of worsening of the fever and the appearance of a rash. Patients with Kawasaki disease normally respond well to IVIG. The use of corticosteroids in Kawasaki was thought to be contraindicated but is being reconsidered in patients who have a poor prognosis or are doing poorly. The patient was given intravenous pulse steroids and then high dose oral corticosteroid therapy, and over the course of a week or so he improved clinically. It is likely that he will be left with fusiform and perhaps saccular aneurysms of the coronary arteries.