204 - ATYPICAL KAWASAKI DISEASE PRESENTING AS ANTERIOR MEDIASTINITIS

Case report:                         

   One year old female child presented  with high fever and cough for 5 days. She had received  amoxicillin-clavulanic acid for 4 days at OPD but developed painful swelling and redness of anterior part of neck along with dysphagia to solid food 1 day prior to admission. At admission, child was well and active in between fever spikes. No other focus of fever could be found except for the tenderness and erythema over anterior part of neck and suprasternal notch. Total leucocyte count (TLC) was 12070/cmm and neutrophils constituted 52%, Hb was 9.3gm%, platelet was 4.46lakhs and CRP was high 109.2 ( Normal < 6 mg/L). Considering cellulitis of neck , iv ceftriaxone and flucloxacillin were started. The following morning child was found to have stridor for which single dose of dexamethasone( 0.6 mg/kg) was given  and stridor settled. MRI neck (Fig.1) revealed oedema of anterior mediastinum, enlarged lymph nodes and cellulitis suggestive of anterior mediastinitis. Teicoplanin was added.

Following dexamethasone, fever subsided for 3 days and TLC, CRP decreased to 11000/cmm and 58 respectively but platelets increased from 4.5 to 5.6 lakhs. Blood culture was negative, the cellulitis and tenderness of neck subsided but fever recurred. TLC increased to 18000/ cmm with 60% neutrophils and platelets increased to 6lakhs/ cmm. As platelet count was persistently increasing with relapse of fever, an echocardiography was advised that showed LMCA dilatation (+2.9z). The next day morning BCG site showed signs of inflammation. Repeat echo after 2 days showed aneurysm in LMCA (+6z). With a diagnosis of atypical KD on ?? day of illness the child was initiated on IVIg 2g/kg, aspirin. With a medium sized aneurysm at diagnosis, treatment was intensified with Inj. Infliximab ( 10 mg/ kg) and anticoagulation with LMWH (1mg/kg subcutaneous) was also started. The child became afebrile within 24 hours of completion of IVIg therapy. After 48 hours of IVIg Echo showed some regression of LMCA aneurysm +5.8z, but CRP persisted to be high ( 67.7 mg/l ). Hence, oral prednisolone  2mg/kg was started with a plan to taper it over next 2 weeks. Repeat echo after 2 weeks showed z score of 4.6, so LMWH was stopped.

 

Discussion:

  This case highlights that clinical symptoms of atypical KD are diverse as this child presented with cellulitis of neck and anterior mediastinitis. Elevated TLC,CRP with thrombocytosis in serial blood tests raises the suspicion. Mediastinal lymphadenopathy may occur in KD.