121 - LONG-TERM MANIFESTATIONS OF PERFUSION ASYMMETRY FOLLOWING SEVERE KAWASAKI DISEASE: A CLINICAL CASE REPORT

Introduction: This report details an unusually severe case of KD with long-term manifestations, specifically bilateral axillary aneurysms leading to perfusion asymmetry, resulting in right-sided unilateral hand hypoplasia and left-sided unilateral digital clubbing.

Case Report: The patient, diagnosed at 1.5 months of age in 2011, exhibited severe KD symptoms with aneurysmal dilation of the RCA, LAD, and circumflex, as well as large bilateral axillary aneurysms (20mm – Fig. 1). Despite prompt treatment, including immunomodulatory therapies, the patient suffered short-term complications, both peripheral and cardiac. The rupture of the right-sided axillary aneurysm created local compression with severe trophic and neurological manifestations. This potentially life-threatening complication prompted rapid endovascular treatment, whereas the contralateral aneurysm was left unstented but closely monitored. Meanwhile, the occlusion of the circumflex coronary artery resulted in myocardial infarction with low-grade left ventricular dysfunction (LVEF 50%).

Despite anticoagulant therapy, complications arose during short- and long-term follow-up. Rapid thrombosis (1-year mark) of the stented right axillary aneurysm resulted in unilateral arterial hypoperfusion and subsequent right-sided upper hand hypoplasia. Contralaterally, sequential angio-MRI studies confirmed continued permeability of the pulsatile aneurysm, with a progressing thrombotic component within the aneurysmal pouch (Fig. 2). This manifested clinically as unilateral left-hand digital clubbing, 12 years after initial disease onset (Fig. 3).

Distinct manifestations of chronic arterial hypoperfusion emerged, affecting limb development and causing digital clubbing. Rapid-onset right-sided complete thrombosis with collaterality resulted in delayed hand growth. Contralaterally, clubbing, known to result from distal hypoxia, inflammation, and abnormal perfusion, could have developed secondary to both disrupted flow pattern hemodynamics (pulsatile aneurysmal pouch) and chronic micro-embolization of the thrombotic pannus. Warfarin and Aspirin therapy, initiated upon diagnosis, may have prevented acute limb ischemia but did not halt subsequent thrombotic manifestations. Identification of peripheral aneurysmal dilations is crucial in severe cases for guiding future management and addressing specific short- and long-term complications.

Conclusion: Thorough vascular screening, especially in high-risk groups with severe Kawasaki disease, is essential for early detection and management of potential complications. The late occurrence of unilateral clubbing during the follow-up of KD should prompt a search for an underlying vascular anomaly.