Palmquist Maria, Pappas John G, Petrikovsky Boris, Blakemore Karin, Roshan Daniel
Department of OB/GYN, Johns Hopkins, 600 N Wolfe Street, Phipps Bldg, Baltimore, Maryland 21287, USA.
J Matern Fetal Neonatal Med. 2009 Oct;22(10):924-7. doi: 10.1080/14767050902874071.
Ehlers-Danlos syndrome (EDS) is a rare connective tissue disorder characterized by tissue fragility, translucent skin and joint hypermobility. Patients with the vascular type of EDS are prone to spontaneous arterial and visceral rupture. Pregnancy for women with vascular EDS can be life-threatening. Mortality rates are high due to the increased risk for uterine and arterial rupture in the peripartum period.
We describe the counseling, multidisciplinary management, protocol, and successful pregnancy outcome of a 32-year-old woman with vascular EDS.
There is no consensus in the literature on the timing and mode of delivery for pregnant women with vascular EDS. The management undertaken in our patient may assist others in optimizing the perinatal outcome in other women who elect to continue their pregnancy despite the risks of this severe medical condition.
埃勒斯-当洛综合征（EDS）是一种罕见的结缔组织疾病，其特征为组织脆弱、皮肤半透明和关节活动过度。血管型EDS患者容易发生自发性动脉和内脏破裂。患有血管型EDS的女性怀孕可能危及生命。由于围产期子宫和动脉破裂风险增加，死亡率很高。
我们描述了一名32岁血管型EDS女性的咨询、多学科管理、方案及成功的妊娠结局。
关于血管型EDS孕妇的分娩时间和方式，文献中尚无共识。我们对该患者采取的管理措施可能有助于其他患有这种严重疾病且选择继续妊娠的女性优化围产期结局。
