Brees C K, Gall S A
Department of Obstetrics and Gynecology, University of Louisville, KY, USA.
J Ky Med Assoc. 1995 Dec;93(12):553-5.
Ehlers-Danlos Syndrome is a group of connective tissue disorders involving defects in collagen synthesis. Ten different types have been described. The most lethal of these is type IV, with up to 25% maternal mortality described. The defect in type III collagen can lead to rupture of arteries, bowel, or uterus. We present a maternal mortality that occurred in a patient not previously known to have Ehlers-Danlos syndrome. She presented at 29 weeks gestational age with a ruptured external iliac artery. Further complications, including jejunal rupture and aortic disruption ultimately led to her demise. Postmortem examination ultimately confirmed the diagnosis of type IV Ehlers-Danlos syndrome. Although type IV Ehlers-Danlos syndrome is rare, it carries a high risk for maternal morbidity and mortality. Therefore, it is important to be aware of the diagnosis prior to pregnancy and to have a high index of suspicion for arterial or viscus perforation in the face of acute abdominal findings.
埃勒斯-当洛综合征是一组涉及胶原蛋白合成缺陷的结缔组织疾病。已描述了十种不同类型。其中最致命的是IV型，据描述产妇死亡率高达25%。III型胶原蛋白缺陷可导致动脉、肠道或子宫破裂。我们报告一例产妇死亡病例，该患者此前未知患有埃勒斯-当洛综合征。她在孕29周时出现髂外动脉破裂。包括空肠破裂和主动脉破裂在内的进一步并发症最终导致了她的死亡。尸检最终确诊为IV型埃勒斯-当洛综合征。尽管IV型埃勒斯-当洛综合征罕见，但它具有较高的孕产妇发病和死亡风险。因此，在怀孕前了解该诊断并在面对急性腹部症状时对动脉或脏器穿孔保持高度怀疑指数非常重要。
