Incomplete Kawasaki Disease in an Infant with Cholangitis Post Kasai Surgery for Biliary Atresia.

ABSTRACT

Kawasaki's disease (KD) is a systemic vasculitis often seen with viral and bacterial infections. Cholangitis is a known complication in biliary atresia patients post Kasai Portoenterostomy (KP). However KD, in a biliary atresia patient post KP has not been previously reported. A 1 years old girl who had previously undergone a KP for BA, presented with cholangitis which was presumed to be caused by a previous enterobacter infection that she had 2 months ago. However, on treating the cholangitis, the patient developed fever again after ten days which persisted even after changing the antibiotics. By this time she also displayed three of five characteristic features of KD in form of fever, strawberry tongue and cervical adenopathy. Investigations showed high ESR, high CRP, thrombocythemia and dilated coronary vessels on echocardiography. Treatment with intravenous immunoglobulin (IVIG) and steroids caused the symptoms to subside.