[P3-82] Two cases of late-onset postpump chorea
[Introduction] Postpump chorea is a rare but serious neurologic complication of childhood cardiac surgery. Chorea begins within 2 weeks postoperatively. Some children have only mild transient chorea within 2 months and other children have severe permanent chorea. The mechanisms that underlie the pathogenesis of postpump chorea remain unclear but deep hypothermia and circulatory arrest are often associated with basal ganglia injury. Herein we report two cases of late-onset postpump chorea. [Case reports] The first case was an 8-year-old boy with atrioventricular septal defect (AVSD). At the age of 8, he underwent a second cardiac surgery with normothermia because of worsened mitral valve regurgitation. One month after surgery, he underwent debridement and resuture for postoperative wound infection. He developed left dominant chorea at the same period. Various investigations including biochemical analysis, autoimmune screening, cerebrospinal fluid analysis, brain MRI, ECD Spect were normal. Diagnosis of postpump chorea was made, and pimozide was started showing partial response. Prednisolone was effective and chorea disappeared. The second case was an 11-year-old girl with AVSD. At the age of 10, she underwent cardiac surgery with normothermia. Two months after surgery, she developed left dominant chorea. Various laboratory investigations showed no abnormal results. She was diagnosed with postpump chorea and started with pimozide. Treatment was effective and chorea disappeared three months later. [Conclusions] In both cases, late-onset chorea was observed after a period of more than 2 weeks post-surgically. Since the first case was effective to steroid, postpump chorea might be associated with an autoimmune mechanism.