Contemporary Outcomes of Patients with Isolated Bilateral Renal Agenesis with and without Fetal Intervention

Abstract

Introduction: Bilateral renal agenesis (BRA) is a lethal diagnosis, specifically meaning that natural survival beyond birth is not expected secondary to pulmonary hypoplasia. Limited contemporary data are available about intervention and the impact of restoring amniotic fluid volume in relation to the risk for lethal pulmonary hypoplasia and other factors that might influence survival in cases of fetal BRA. Objective: We report the largest series of patients undergoing fetal intervention and postnatal care for BRA at a single comprehensive fetal center. Methods: All patients with fetal BRA were reviewed from January 2004 to November 2017. Maternal and neonatal data were collected in an institutional review board-approved retrospective review. Results: From 2014 to 2017, 20 singleton pregnancies with isolated fetal BRA were evaluated and 14 had amnioinfusion. Eight had serial infusions. Of those, there were 6 neonatal deaths. There were 2 neonatal survivors beyond 30 days; however, both died of sepsis on dialysis. One of these survivors received amnioinfu-sions by percutaneous approach and one via amnioport. There were no survivors to transplantation. Conclusion: Fetal intervention via amnioinfusion may promote pulmonary survivorship after birth, but postnatal survival remains poor. Future studies must place an emphasis on standardizing the postnatal approach to this patient population.

Whole Exome Sequencing in a Population With Severe Congenital Anomalies of Kidney and Urinary Tract

“…This study is limited by exclusion of patients with BRA. In a study by Riddle et al completed at our institution, all 8 patients who underwent serial amnioinfusions for BRA died either in utero ( n = 6) or within 30 days of delivery ( n = 2) ( 15 ). As a result of this study, fetal interventions and infant dialysis are no longer offered to mothers of infants with BRA at our institution.…”

“…Infants with minor or moderate CAKUT not requiring fetal interventions as well as infants with bilateral renal agenesis (BRA) were excluded from this study. BRA was excluded as our center does not currently offer intervention to this population ( 15 ). All study methods were IRB approved at our center and the delivery hospital centers.…”

Precarious hope: Ethical considerations for offering experimental fetal therapies outside of research after initial studies in humans

“…[1][2][3] Several cases of amnioinfusions for BRA resulted in sufficient pulmonary development to sustain survival in neonates, although morbidity and mortality were high. 1,2,4,5 At least two children survived beyond kidney transplantation and are attending school at age 3. 6 In 2018, the first clinical trial started (Renal Anhydramnios Fetal Therapy [RAFT]), after a multidisciplinary collaborative to assess ethical challenges.…”

“…Serial amnioinfusions were hypothesized to ameliorate pulmonary hypoplasia in BRA, although surviving children would need a transplant or lifelong dialysis 1–3 . Several cases of amnioinfusions for BRA resulted in sufficient pulmonary development to sustain survival in neonates, although morbidity and mortality were high 1,2,4,5 . At least two children survived beyond kidney transplantation and are attending school at age 3 6 …”

Response to “Contemporary Outcomes of Patients with Isolated Bilateral Renal Agenesis with and without Fetal Intervention” by RAFT Investigators

“…We read with great interest the recently published article entitled “Contemporary Outcomes of Patients with Isolated Bilateral Renal Agenesis with and without Fetal Intervention” by Riddle and colleagues [1]. In this single-center experience of fetuses diagnosed with bilateral renal agenesis (BRA) over a 13-year period, the investigators document many granular details about maternal and fetal demographic characteristics and the prenatal, perinatal, and neonatal clinical course, which greatly enriches the current fledgling literature.…”