进行性骨化性纤维发育不良与妊娠：病例系列及文献综述
Fibrodysplasia Ossificans Progressiva and Pregnancy: A Case Series and Review of the Literature.
作者信息
Forrest Alexandra D, Vuncannon Danielle M, Ellis Jane E, Grunwald Zvi, Kaplan Frederick S
机构信息
Department of Gynecology and Obstetrics, Emory University School of Medicine, 80 Jesse Hill Jr. Drive SE, Atlanta, GA 30303, USA.
Department of Anesthesiology, Sidney Kimmel Medical College at Thomas Jefferson University Hospital, Suite 515, 1020 Walnut Street, Philadelphia, PA 19107, USA.
出版信息
Case Rep Obstet Gynecol. 2022 Sep 16;2022:9857766. doi: 10.1155/2022/9857766. eCollection 2022.
OBJECTIVE
To evaluate maternal and fetal outcomes in pregnant patients with fibrodysplasia ossificans progressiva (FOP; OMIM#135100), an ultrarare genetic disorder characterized by progressive heterotopic ossification of soft tissues and cumulative disability.
METHODS
This is a retrospective case series of three patients with FOP who were admitted to Grady Memorial Hospital in Atlanta, Georgia, from to February 2011 to July 2021.
RESULTS
Three women delivered preterm infants at our institution. These cases posed unique anesthetic and obstetric technical challenges, particularly when securing the airway and performing cesarean delivery. Importantly, each patient received perioperative glucocorticoids for prevention of further heterotopic ossification.
CONCLUSION
FOP is a unique clinical diagnosis encountered by obstetricians and requires multidisciplinary management for optimal outcomes.
目的
评估进行性骨化性纤维发育不良（FOP；OMIM#135100）孕妇的母婴结局，FOP是一种极为罕见的遗传性疾病，其特征为软组织进行性异位骨化和累积性残疾。
方法
这是一项回顾性病例系列研究，研究对象为2011年2月至2021年7月期间入住佐治亚州亚特兰大格雷迪纪念医院的3例FOP患者。
结果
3名女性在我们机构分娩了早产儿。这些病例带来了独特的麻醉和产科技术挑战，尤其是在确保气道安全和进行剖宫产时。重要的是，每位患者均接受了围手术期糖皮质激素治疗以预防进一步的异位骨化。
结论
FOP是产科医生遇到的一种独特临床诊断，需要多学科管理以实现最佳结局。
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