Ueda Y, Shimomura T, Kurehara K, Iwasaka T, Tatsumi K, Fukushima T
Department of Anesthesiology, Nara Prefectural Hospital.
Masui. 1994 Dec;43(12):1876-80.
21-hydroxylase deficiency was described as a fatal disease in infancy manifested by severe adrenal insufficiency with lack of differentiation of the external genitalia in males. Patients are in adrenal insufficiency or receiving long term corticosteroid therapy and are unable to respond normally to surgery or other forms of stress. Therefore, supplementation with corticosteroids and various regimens are required for surgery and anesthetic management. A 3 year 10 months correction of 6] old phenotypic female [correction of male] with 21-hydroxylase deficiency and congenital adrenal hyperplasia was scheduled for critroplasty. Anesthesia was induced with sevoflurane, nitrous oxide and oxygen. After endotracheal intubation, a caudal epidural catheter was placed. At the induction of anesthesia, the patient received a bolus of hydrocortisone 30 mg i.v. followed by a continuous infusion of hydrocortisone. The patient's general condition was good during surgery without any major complications. Caudal epidural anesthesia is considered to be effective for anesthetic management of these patients.
21-羟化酶缺乏症在婴儿期被描述为一种致命疾病，表现为严重肾上腺皮质功能不全，男性外生殖器分化缺失。患者存在肾上腺皮质功能不全或正在接受长期皮质类固醇治疗，对手术或其他形式的应激无法正常反应。因此，手术和麻醉管理需要补充皮质类固醇及采用各种方案。一名3岁10个月大、表型为女性[实际为男性]、患有21-羟化酶缺乏症和先天性肾上腺皮质增生症的患儿计划进行尿道下裂修复术。使用七氟醚、氧化亚氮和氧气诱导麻醉。气管插管后，置入骶管硬膜外导管。麻醉诱导时，患者静脉注射30mg氢化可的松推注量，随后持续输注氢化可的松。手术期间患者一般情况良好，无任何重大并发症。骶管硬膜外麻醉被认为对这些患者的麻醉管理有效。
