Successful In Utero Intervention for Bilateral Renal Agenesis

Abstract

Serial amnioinfusion appears to have mitigated the severe pulmonary compromise that has, in the past, led to the death of newborns with bilateral renal agenesis.

Amnioinfusions to Treat Early Onset Anhydramnios Caused by Renal Anomalies: Background and Rationale for the Renal Anhydramnios Fetal Therapy Trial

“…This case justified a later intervention by Bienstock et al [14] to promote lung growth in CoBRA with amnioinfusions. This and 8 other case reports have documented adequate neonatal lung function when amnioinfusions were given prenatally in the setting of EPRA [12, 14-16]. These outcomes raise the possibility of pulmonary survival for infants with a disease previously thought to be fatal.…”

“…In 1994, a neonate with CoBRA survived early neonatal life after amnioinfusions were administered from 17 to 33 weeks GA, but ultimately succumbed to dialysis complications after 23 days of life [15]. In 2014, Bienstock et al [14] reported the case of CoBRA in a 34-year-old woman treated with weekly serial amnioinfusions beginning at 24 weeks GA. The infant was born at 29 weeks GA with age-appropriate lung function and received a kidney transplant from her father after 3 years on chronic peritoneal dialysis [12].…”

“…Amnioinfusions are routinely used in ultrasound assessments and diagnoses for fetuses with low fluid volumes [12], but the risk of preterm premature rupture of membranes (PPROM) is still at least 1/300 per infusion. However, the safety of repeated amnioinfusions as a preventative method has been illustrated in multiple studies [2, 4, 8, 14], including a retrospective study of 61 patients presenting with severe oligo-anhydramnios from PPROM who received amnioinfusions with no fatalities recorded as a direct result of treatment. Mothers receiving amnioinfusions for pregnancies complicated by oligohydramnios resulting from early PPROM (16–26 weeks GA) had similar fetal survival as pregnancies complicated by PPROM without oligohydramnios [44].…”

Severe antenatally diagnosed renal disorders: background, prognosis and practical approach

“…Experimental evidence is in favour of its use, which also seems to be supported by recent studies, at least in some cases after the premature rupture of membranes [37][38][39]. Systematic studies on fetuses with ROH have not been performed, yet a recent case report indicates a potential benefit even in a fetus with bilateral renal agenesis [40]. However, amnioinfusion is not without risk and may lead to abortion or rupture of membranes [41].…”

Types and Outcome of Fetal Urinary Anomalies in Low Resource Setting Countries: A Retrospective Study

“…Spaggiari et al [11] reported that 30 of 34 cases (88.2 %) with hypoplastic kidneys complicated by oligohydramnios or anhydramnios were associated with poor outcomes. Bienstock et al [12] reported a case of bilateral renal agenesis treated with serial amnioinfusion in which the newborn survived the newborn period and was able to undergo peritoneal dialysis followed by a planned renal transplantation.…”