XI型成骨不全症孕妇的麻醉管理：一份综合病例报告。
Anesthetic management in pregnancy with osteogenesis imperfecta type XI: A comprehensive case report.
作者信息
Punnaniti Korakod, Sangkum Lisa, Termpornlert Sivaporn, Komonhirun Rojnarin, Dusitkasem Sasima
机构信息
Department of Anesthesiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Department of Anesthesiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
出版信息
Int J Surg Case Rep. 2025 Feb;127:110971. doi: 10.1016/j.ijscr.2025.110971. Epub 2025 Feb 1.
INTRODUCTION
Osteogenesis imperfecta (OI) type XI or Bruck syndrome is an extremely rare genetic disorder characterized by congenital joint contractures and bone fragility. OI presents unique and considerable challenges in the perioperative and anesthetic management of affected patients.
PRESENTATION OF CASE
A 29-year-old primigravida with OI type XI (100 cm, 26 kg) and severe kyphoscoliosis underwent urgent Caesarean delivery at 32 weeks under general anesthesia (sevoflurane/nitrous oxide). A female infant (1470 g) required resuscitation. Postoperative recovery was uneventful.
DISCUSSION
The rarity of this syndrome, along with the physiological changes associated with pregnancy, creates an unprecedented clinical scenario that demands a thorough and cautious approach to patient care.
CONCLUSION
Osteogenesis imperfecta (OI) type XI necessitates careful anesthetic management in pregnancy. This case highlights the anesthetic management challenges and the use of a multidisciplinary approach to enhance clinical understanding and improve patient outcomes.
引言
XI型成骨不全症（OI）或布鲁克综合征是一种极为罕见的遗传性疾病，其特征为先天性关节挛缩和骨质脆弱。OI在受影响患者的围手术期和麻醉管理方面带来了独特且巨大的挑战。
病例介绍
一名29岁的初产妇，患有XI型OI（身高100厘米，体重26千克）及严重脊柱后凸侧弯，在全身麻醉（七氟烷/氧化亚氮）下于32周时接受了紧急剖宫产。一名女婴（体重1470克）需要复苏。术后恢复顺利。
讨论
该综合征的罕见性，以及与妊娠相关的生理变化，创造了一种前所未有的临床情况，这需要对患者护理采取全面且谨慎的方法。
结论
XI型成骨不全症（OI）在妊娠期间需要仔细的麻醉管理。本病例突出了麻醉管理挑战以及采用多学科方法来增强临床理解并改善患者预后。
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