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无心畸胎

[Acardiac fetus].

作者信息

Faguer C, Bonan J, Mulliez N, Migne G

机构信息

Unité de Foeto-Pathologie, Hôpital Saint-Antoine, Paris.

出版信息

Presse Med. 1996 Sep 14;25(26):1191-4.

PMID:8949622
Abstract

Acardiac fetus is a rare lethal fetopathy usually encountered in monozygous pregnancies. Ultrasound prenatal diagnosis has enabled an increasing number of observations and raised the need for an adequate therapeutic approach since the spontaneous prognosis for the healthy twin is unfavorable in half of the cases. An acardiac fetus was identified at 12 weeks gestation in a 36-year-old woman. Growth of the healthy fetus was carefully monitored and progressed normally to delivery by cesarean section of a 2.900 kg boy at 36 weeks. At delivery, the acardiac fetus was found to be totally free of any attachment, floating in the remaining fluids. Pathology examination showed a 16 g macerated fetus with a cephalic extremity, a ventral pedicle and a syrenomelic caudal extremity. The caryotype was not significative. Acardiac fetus occurs in less than 1% of multiple pregnancies and can develop in single pregnancies. Twin reversed arterial perfusion has been recognized as necessary for development of the perfused fetus. Genetic and immunologic theories have been proposed to explain the pathogenesis which remains unknown. Clinical management depends on the spontaneous development of the acardiac fetus and the deleterious consequences for the healthy twin resulting from heart failure, anasarca or prematurity. Medical management with digoxin, or selective extraction by hysterotomy may improve prognosis but results have been variable. Echoguided umbilical cord ligation has also been proposed to improve maternal mortality. Therapeutic abstention is no longer indicated at prenatal diagnosis of an acardiac fetus and a healthy twin despite the risk of invasive treatment.

摘要

无心胎儿是一种罕见的致死性胎儿病变,通常见于单卵双胎妊娠。超声产前诊断使越来越多的病例得以发现,并且由于在半数病例中健康双胎的自然预后不佳,因此需要采取适当的治疗方法。一名36岁女性在妊娠12周时被诊断出无心胎儿。对健康胎儿的生长情况进行了仔细监测,其生长正常,至36周时通过剖宫产娩出一名体重2.900千克的男婴。分娩时,发现无心胎儿完全没有任何附着,漂浮在剩余羊水中。病理检查显示一个16克的浸软胎儿,有头部、腹侧蒂和尾侧肢体发育不全。染色体核型无明显异常。无心胎儿在多胎妊娠中的发生率不到1%,也可发生于单胎妊娠。双胎反向动脉灌注被认为是灌注胎儿发育所必需的。已经提出了遗传和免疫理论来解释其发病机制,但发病机制仍不清楚。临床处理取决于无心胎儿的自然发展情况以及心力衰竭、全身水肿或早产对健康双胎造成的有害后果。使用地高辛进行药物治疗或通过子宫切开术进行选择性取出可能会改善预后,但结果不一。也有人提出超声引导下脐带结扎可降低产妇死亡率。尽管存在侵入性治疗的风险,但在产前诊断出无心胎儿和健康双胎时,不再建议采取保守治疗。

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