Abstract：
Bilateral renal agenesis (BRA) is a fetal anomaly which leads to anhydramnios and resultant pulmonary hypoplasia. Historically, this anomaly was universally fatal early in the neonatal period due to the severity of the associated lung disease. Over the last 30 years, innovations in fetal therapies-specifically, serial amnioinfusions-have led to instances of infant pulmonary survival and initiation of postnatal dialysis, raising the possibility that early neonatal death may not be inevitable. Amnioinfusions are not without risk, and maternal complications can include prelabor rupture of membranes, preterm labor, infection, and bleeding. The data detailing neonatal outcomes are still limited and actively being collected. Two case series and one non-randomized clinical trial have supplied most of the known outcome data for infants with BRA after prenatal amnioinfusion. Although there are survivors reported in the literature, mortality remains high, with many deaths in infancy due to dialysis-associated sepsis. In addition, previously unknown morbidities have been documented in these infants, including neurologic injury. These challenges, in addition to the mechanical difficulties of providing dialysis to extremely small infants, can result in significant burdens for patients and their caregivers and moral distress for the health care team. The present review aims to explain the pathophysiology of BRA, detail the historical context and rationale for serial amnioinfusions to treat the pulmonary insufficiency associated with BRA, describe the available data regarding outcomes of infants born following prenatal amnioinfusions, discuss ethical issues surrounding this fetal intervention, and describe critical aspects of prenatal counseling for patients considering the intervention.
摘要：
双侧肾发育不全（BRA）是一种胎儿异常，可导致羊水过多和肺发育不全。历史上,由于相关肺部疾病的严重程度,这种异常在新生儿期早期普遍致命.在过去的30年里,胎儿疗法的创新-特别是，连续羊膜输注-导致婴儿肺部存活和出生后透析的开始，提高早期新生儿死亡可能不是不可避免的可能性。羊膜输注并非没有风险，产妇并发症可能包括胎膜破裂，早产,感染,和出血。详细说明新生儿结局的数据仍然有限，并且正在积极收集。两个病例系列和一个非随机临床试验提供了产前羊膜输注后BRA婴儿的大多数已知结果数据。尽管文献中报道了幸存者，死亡率仍然很高，许多婴儿因透析相关脓毒症而死亡。此外,以前未知的发病率已被记录在这些婴儿中，包括神经损伤.这些挑战,除了为极小的婴儿提供透析的机械困难之外，可能会给患者及其护理人员带来沉重负担，并给医疗保健团队带来道德困扰。本综述旨在解释BRA的病理生理学，详细介绍了连续羊膜输注治疗与BRA相关的肺功能不全的历史背景和基本原理，描述产前羊膜输注后出生的婴儿结局的可用数据，讨论围绕这个胎儿干预的伦理问题，并描述考虑干预措施的患者产前咨询的关键方面。
