Department of Obstetrics and Gynecology, Ente Ospedaliere Cantonale, University of the Italian Switzerland Lugano, Via Tesserete 46, 6900, Lugano, Switzerland.
Institute of Pathology, Locarno, Switzerland.
Arch Gynecol Obstet. 2021 Nov;304(5):1115-1125. doi: 10.1007/s00404-021-06130-7.
Identified by the eponym "Edwards' Syndrome," trisomy 18 (T18) represents the second most common autosomal trisomy after T21. The pathophysiology underlying the extra chromosome 18 is a nondisjunction error, mainly linked with the advanced maternal age. More frequent in female fetuses, the syndrome portends high mortality, reaching a rate of 80% of miscarriages or stillbirths. The three-step evaluation includes first trimester screening for fetal aneuploidy using a combination of maternal age, fetal nuchal translucency thickness, fetal heart rate and maternal serum free ß-hCG and PAPP-A; followed by the research for fragments of fetal DNA in maternal blood; and, finally, invasive techniques leave to the established diagnosis. Starting with the first trimester scan, selected ultrasound findings should be investigated to define not only the impact of the genetic problem on the fetus, but also to address the prenatal counseling. Previous series underline that T18 is not uniformly lethal. An active dialogue on the choices in the management of infants with T18 has emerged, sustained by the transition from the comfort care to the intervention attitude. Survival rates for individuals with supposedly fatal conditions have increased. In this novel scenario, an ad hoc counseling is pivotal. To support it, a comparative analysis by pictorial assays between ultrasound and autopsy findings could be beneficial. We provide an illustrative tool from a clinical case managed in early second trimester, with the purpose to strive a balanced approach in the hard choice faced by couples of fetuses with T18.
爱德华兹综合征(Edwards' Syndrome)命名的 18 三体(T18)是继 21 三体(T21)之后第二常见的常染色体三体。额外的 18 号染色体的病理生理学是不分离错误,主要与高龄产妇有关。女性胎儿更为常见,该综合征预示着高死亡率,流产或死产率达到 80%。三步评估包括使用母亲年龄、胎儿颈项透明层厚度、胎儿心率和母亲血清游离β-hCG 和 PAPP-A 组合对胎儿非整倍体进行早期筛查;然后研究母亲血液中的胎儿 DNA 片段;最后,侵入性技术可确诊。从早期筛查开始,应调查选定的超声发现,不仅要确定遗传问题对胎儿的影响,还要进行产前咨询。以前的系列研究强调 T18 并非一律致命。积极开展了有关 T18 婴儿管理选择的对话,这一态度的转变源于从安慰性护理到干预性护理的转变。具有所谓致命条件的个体的存活率有所提高。在这种新情况下,专门的咨询至关重要。为了支持这一点,超声和尸检结果的图像分析比较分析可能是有益的。我们提供了一个来自早期妊娠中期管理的临床病例的说明性工具,目的是在面对 T18 胎儿夫妇所面临的艰难选择时,努力采取平衡的方法。