糖原病Ⅰa型合併妊娠に対する選択的帝王切開の麻酔経験

Anesthetic Management for a Cesarean Section in a Patient with Glycogen Storage Disease TypeⅠa

要旨

　糖原病Ⅰa型（glycogen storage disease type Ⅰa：GSDⅠa）を合併した妊婦の分娩に際して，脊髄クモ膜下麻酔による選択的帝王切開を施行した。短時間の分娩を計画し，絶食期間中は経静脈的に糖の持続投与を行うことで，母児の周産期の代謝性合併症を予防できた。

　We report the anesthetic management of a pregnant woman with glycogen storage disease typeⅠa. She had lost consciousness due to hypoglycemia while fasting for an intervention. After her pregnancy we enhanced her dietary management and estimated various risks. Throughout the pregnancy the patient’s blood glucose level was well controlled, and the fetal growth was good. For the prevention of hypoglycemia, an elective cesarean section was performed with the patient under spinal anesthesia, together with a continuous intravenous glucose infusion. The patient’s early term infant was delivered successfully, with no hypoglycemia or lactic acidemia in the patient or neonate.