PDF(Pubmed)
Abstract：
Complicated pregnancies are nowadays a major public health concern, with possible lethality or sequelae both for the mother and the fetus. Blood coagulation disorders (including antiphospholipid syndrome, factor V Leiden mutation and antithrombin deficiency) and hypertensive gestational disorders are very well-known contributors of complicated pregnancies with poor fetal outcome, such as intrauterine growth retardation (IUGR) and fetal demise. Less commonly, vascular malformations of the placenta can also potentially lead to serious complications such as IUGR and fetal death. These malformations include hypercoiled umbilical cord, umbilical cord knot, umbilical cord varix, umbilical cord arterial or venous aneurysm, and velamentous insertion of the umbilical cord potentially leading to Benckiser\'s hemorrhage. Here, we report the case of a 29-year-old Gravida 2 Para 0 mother with previous history of stillbirth and smoking, admitted to the obstetrics department for the absence of fetal movement at 38 weeks of amenorrhea (WA). First-trimester and second-trimester routine ultrasounds were otherwise normal. Ultrasound performed at 38 WA revealed a 83 × 66 × 54 mm cystic heterogenous mass at the umbilical cord insertion. After delivery, fetal and placental pathology as well as maternal blood testing were performed. Fetal pathology was otherwise normal, except for diffuse congestion and meconial overload suggesting acute fetal distress. Fetal karyotype was normal (46 XX). Placental pathology revealed an umbilical artery aneurysm (UAA) at the base of the insertion of the umbilical cord, lined with a CD34+ CD31+ endothelium. After dissection, the aneurysm was filled with hemorrhagic debris, indicating aneurysm thrombosis. Histopathology revealed associated maternal vascular malperfusion (MVM) and increased peri-villous fibrin (IPF). Maternal blood tests revealed heterozygous factor V Leiden mutation, without other associated auto-immune conditions (such as antiphospholipid syndrome). Umbilical artery aneurysms remain extremely rare findings in the placenta, with <20 reported cases. Umbilical artery aneurysms have tendency to be located at the base of the insertion of the placenta, and lead to fetal demise in more than 60% of cases, mainly due to aneurysmal thrombosis, hematoma, possible vascular compression and/or rupture. Umbilical vessel aneurysms can be associated with trisomy 18 or 13. In our case, the association of factor V Leiden mutation, a hypercoagulable state, with UAA could explain massive thrombosis of the aneurysmal lumen and sudden fetal demise. Further consideration of current guidelines for surveillance and management of UAA would allow appropriate planned delivery in maternal care settings.
摘要：
复杂的怀孕现在是一个主要的公共卫生问题，对母亲和胎儿都有可能致命或后遗症。血液凝固障碍（包括抗磷脂综合征，因子VLeiden突变和抗凝血酶缺乏症）和高血压妊娠疾病是导致胎儿结局不良的复杂妊娠的众所周知的因素，如宫内发育迟缓（IUGR）和胎儿死亡。不太常见，胎盘血管畸形也可能导致IUGR和胎儿死亡等严重并发症.这些畸形包括脐带过度卷曲，脐带结,脐带静脉曲张,脐带动脉或静脉动脉瘤，脐带的绒毛插入可能导致Benckiser出血。这里,我们报告了一例29岁的Gravida2Para0母亲，既往有死产和吸烟史，因闭经（WA）38周时没有胎动而入院产科。妊娠早期和妊娠中期常规超声均正常。在38WA进行的超声检查显示脐带插入处存在83×66×54mm的囊性异质性肿块。交货后,进行了胎儿和胎盘病理以及母体血液检查。胎儿病理正常,除了弥漫性充血和中期超负荷提示急性胎儿窘迫。胎儿核型正常（46XX）。胎盘病理显示脐带插入底部有脐动脉瘤（UAA），衬有CD34+CD31+内皮。解剖后,动脉瘤充满了出血性碎片，提示动脉瘤血栓形成.组织病理学显示相关的母体血管灌注不良（MVM）和绒毛周围纤维蛋白（IPF）增加。母体血液检查显示杂合因子VLeiden突变，没有其他相关的自身免疫性疾病（如抗磷脂综合征）。脐动脉瘤在胎盘中仍然极为罕见，报告病例<20例。脐动脉动脉瘤倾向于位于胎盘插入的底部，导致60%以上的胎儿死亡,主要是由于动脉瘤血栓形成,血肿,可能的血管压缩和/或破裂。脐血管动脉瘤可与18或13三体相关。在我们的案例中,因子V莱顿突变的关联，高凝状态,UAA可以解释动脉瘤腔的大量血栓形成和胎儿突然死亡。进一步考虑当前的UAA监测和管理指南，将允许在孕产妇护理环境中进行适当的计划分娩。
