心・血管疾患を合併したSeckel症候群患者の麻酔経験 A Case Report of Seckel Syndrome with Cardiovascular Dysfunction

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Seckel症候群は,子宮内発育障害に起因する低体重かつ低身長で,均整はとれているが小人症である.特徴ある小顔面を呈するまれな症候群であり,外観の奇形に関する報告が多い.本症候群は外観の奇形のみならず,心・血管系に重篤な合併症を有している可能性もあり,慎重な麻酔前評価と麻酔管理が必要である.<br>今回,心不全,血栓性動脈閉塞症,深部静脈血栓症,肝腫大,腎機能低下を呈した本症候群患者の麻酔を経験した.

We described a first case report of Seckel syndrome (bird-headed dwarfism) with cardiovascular dysfunction. A 20-year-old man affected with Seckel syndrome had congestive heart failure, thromboembolism in the femoral artery, thrombis in deep veins, hepatomegaly and renal dysfunction. He underwent a thrombectomy of the right femoral artery on an emergency basis under epidural anesthesia. An epidural catheter was inserted through the L3/4 intervertebral space, and a total dose of 1.5% mepivacaine 7 ml was injected into the epidural space. After 15 min, analgesia was obtained until the Th 8 level. When fentanyl 25, μg as a sedative was injected intravenously, severe bradycardia and hypotension occurred suddenly. After cardiac massage for a few seconds and injection of atropine, HR and BP were recovered immediately. After these treatments, hemodynamics were stable. It was considered that these events were due to the sudden pain relief and venous pooling produced by epidural blockade using local anesthetic, parasympathetic nerve activation caused by the use of fentanyl, and so on. Since Seckel syndrome may be associated with not only surface anomaly but also cardiovascular dysfunction, a close preoperative examination of cardiovascular system and strict anesthetic management are necessary.

収録刊行物

  • 日本臨床麻酔学会誌 = The Journal of Japan Society for Clinical Anesthesia

    日本臨床麻酔学会誌 = The Journal of Japan Society for Clinical Anesthesia 19(2), 149-151, 1999-03-15

    THE JAPAN SOCIETY FOR CLINICAL ANESTHESIA

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各種コード

  • NII論文ID(NAID)
    10010152891
  • NII書誌ID(NCID)
    AN00330159
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    02854945
  • データ提供元
    CJP書誌  J-STAGE 
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