146 - 7-MONTH-OLD INFANT WITH INCOMPLETE KAWASAKI DISEASE, GIANT CORONARY ARTERY ANEURYSM, CORONARY ARTERY THROMBUS: CHALLENGES IN MANAGEMENT DURING THE ACUTE PHASE

Introduction:

Kawasaki disease (KD) is a medium vessel vasculitis of childhood with a predilection for the coronary arteries. Infants with KD often present with incomplete and atypical forms (40%), as compared to older children (10–12%). Delays in diagnosis and treatment, higher incidence of coronary artery abnormalities (CAAs), and greater incidence of intravenous immunoglobulin (IVIG) resistance are commonly encountered in infants with KD. Here we describe a 7-month-old infant who presented with incomplete Kawasaki disease and a giant coronary aneurysm complicated with a thrombus.

Clinical description: A 7-month-old boy presented with fever, generalized maculopapular rash, redness of eyes and swelling around left side of the neck. Investigations showed haemoglobin 60 g/L, total white cell counts 18.2x10⁹/L (N₇₅L₂₁M₃E₁), platelet count 175x10⁹/L, elevated C- reactive protein (67 mg/L; N < 6). He was noted to have congestive cardiac failure, pericardial effusion and mild mitral regurgitation, requiring mechanical ventilation and inotropic support at 4^th week of illness. SARS-CoV-2 antibodies were positive. Clinical possibilities included Multisystem Inflammatory Syndrome in children (MIS-C)  and  Kawasaki disease (KD). He received intravenous immunoglobulin (IVIg; 2g/kg) and oral prednisolone (2mg/kg/day initially and then tapering), digoxin, enalapril, and furosemide. A 2-dimensional(D)-echocardiogram done at 10^th week of illness showed giant aneurysms of left anterior descending artery (LAD) with thrombus. He was thereafter referred to our institute for further evaluation. The 2D-echocardiography confirmed giant aneurysms of LAD (14.9 mm; +47.2 Z) with thrombus, left circumflex (4.39mm; +11.48Z), and right coronary artery (RCA) (4.17 mm; +9.5Z).  The N-terminal pro-B-type natriuretic peptide (NT-proBNP) (3807 pg/mL; N < 125), creatine kinase-MB fraction (42 IU/L; N:5-25) and troponin T (58.6 ng/L; N: 12.7- 24) were still elevated. He was treated with a second dose of IVIg (2g/Kg); infliximab (10 mg/kg); oral prednisolone (2mg/kg/day, tapered and stopped over 6 weeks); cyclosporine (3mg/kg/day); low molecular weight heparin (2mg/kg/day) and aspirin (5mg/kg/day). Nail examination at 2 weeks of follow-up showed nail pitting over multiple nails with Beau’s lines

Conclusion:


Identification of KD in infants can be very challenging as they may not fulfil the diagnostic guidelines. Further, the disease tends to be more aggressive in young infants with a significant risk of developing CAAs despite appropriate treatment.  A high index of suspicion for KD is required in an infant presenting with unexplained fever lasting more than 5 days. Early initiation of IVIG, along with pre-emptive intensification with other adjuvant therapy is often necessary in such situations.