山田 (平岡) 希生

<title>Abstract</title><sec> <title>Background</title> It is unclear whether perioperative Bispectral Index™ (BIS) monitoring in pediatric cases with acute liver failure (ALF) is effective for evaluation of neurological function. We describe a pediatric patient with hepatic encephalopathy (HE) in whom the BIS value increased from low levels to the normal range during liver transplantation (LT). </sec><sec> <title>Case presentation</title> Electroencephalography in a 6-year-old comatose girl diagnosed with ALF and HE who was unresponsive to pain and auditory stimuli revealed continuous slow waves, and hence, emergency LT was performed. Intraoperatively, BIS values remained low until reperfusion. However, BIS value variability increased after reperfusion. She was subsequently discharged without any neurological sequelae. </sec><sec> <title>Conclusions</title> Low BIS values were considered to reflect the severity of HE. It is possible that improvement of the BIS value and waveform was a reflection of graft function. BIS monitoring might be a good indicator of neurological recovery after LT. </sec>

43歳女。出生時から顔貌異常を認めていたが、Saethre-Chotzen症候群(SCS)の確定診断には至らず、気道閉塞や頭蓋内圧亢進といった症状はなく成長した。42歳時に両手第二三指間合指や短頭、眼瞼下垂、中顔面低形成などの症状と、頭部3DCTでの冠状縫合の消失から臨床的にSCSと診断された。顔面低形成と眼瞼下垂に対して過去2年間に3度の全身麻酔下手術を行ったが、いずれも気道確保は容易であった。本例の経験から、SCS例は骨変形が軽度であれば年齢を重ねても気道管理のリスクが高くならない可能性が示唆された。