van Lier Monique G J T B, Lopriore Enrico, Vandenbussche Frank P H A, Streekstra Geert J, Siebes Maria, Nikkels Peter G J, Oepkes Dick, van Gemert Martin J C, van den Wijngaard Jeroen P H M
Department of Biomedical Engineering and Physics, Academic Medical Center-University of Amsterdam, Amsterdam, The Netherlands.
Division of Neonatology, Department of Pediatrics, Leiden University Medical Center, Leiden.
Birth Defects Res A Clin Mol Teratol. 2016 Mar;106(3):213-7. doi: 10.1002/bdra.23477. Epub 2015 Dec 21.
Acardiac twinning is a rare anomaly of monochorionic twin pregnancies. Acardiac fetuses lack a functional heart but are passively perfused by arterial blood from their pump co-twin. Although four acardiac morphological types have been classified, the various paths of anatomical and circulatory acardiac twin development, and the potential influence of acardiac size and perfusion flow as possible predictors of pump twin morbidity and mortality are poorly understood. This report presents the first high resolution three-dimensional reconstruction of the vasculature of an acardiac twin by cryomicrotome imaging.
A small, approximately 7.5-cm-diameter ball-shaped acardius amorphous of 30 5/7 weeks had caused pump twin cardiac decompensation that necessitated an emergency cesarian section. The pump twin survived well. The acardiac body had a partially intact vascular system with large diameter arteries and veins and multiple zones that appeared devoid of perfusion. The three-dimensional reconstruction showed neither recognizable organ structures nor identifiable blood vessels except for the umbilical artery and vein.
Our case showed a small acardiac mass with large diameter vessels and consequential low outflow resistance that caused pump twin complications. This indicates that the development of a method that allows pump twin prognosis is likely more successful if based on the use of acardiac versus pump twin perfusion flows than on body volume ratios.
无心畸形是单绒毛膜双胎妊娠的一种罕见异常。无心胎儿缺乏功能性心脏,但由其供血双胎的动脉血被动灌注。尽管已将无心胎儿分为四种形态类型,但对无心双胎解剖和循环发育的各种路径,以及无心胎儿大小和灌注流量作为供血双胎发病和死亡潜在预测指标的可能影响了解甚少。本报告展示了通过冷冻切片成像对无心双胎血管系统进行的首次高分辨率三维重建。
一名直径约7.5厘米的小球形无心胎儿,孕30又5/7周,导致供血双胎心脏失代偿,需紧急剖宫产。供血双胎存活良好。无心胎儿身体有部分完整的血管系统,有大直径动静脉,还有多个无灌注区域。三维重建显示除脐动静脉外,既无可识别的器官结构,也无可识别的血管。
我们的病例显示一个小的无心肿物,有大直径血管,导致流出阻力低,引起供血双胎并发症。这表明,如果基于无心胎儿与供血双胎的灌注流量而非体积比来开发一种能预测供血双胎预后的方法,可能会更成功。