Etiology and management of early pregnancy renal anhydramnios: Is there a place for serial amnioinfusions?

Abstract

Early pregnancy renal anhydramios (EPRA) comprises congenital renal disease that results in fetal anhydramnios by 22 weeks of gestation. It occurs in over 1 in 2000 pregnancies and affects 1500 families in the US annually. EPRA was historically considered universally fatal due to associated pulmonary hypoplasia and neonatal respiratory failure. There are several etiologies of fetal renal failure that result in EPRA

Fetal interventions for congenital renal anomalies

“…EPRA encompasses the most severe form of CAKUT t h a t r e s u l t s i n f e t a l a n h y d r a m n i o s b y 2 2 w e e k s gestation (3). EPRA is thought to be universally fatal secondary to pulmonary hypoplasia and affects 1 in 2000 pregnancies.…”

“…The main etiologies are congenital bilateral renal agenesis (CoBRA), cystic kidney disease (CKD), and severe LUTO (4-6). In these patients, it is crucial to evaluate the entire genitourinary (GU) tract, including the urethra, bladder, ureters and kidneys, to identify or rule out a specific anomalies that may have renal, urologic or genetic significance (3).…”

“…However, a subset of (non-EPRA) ARPKD patients can also present with respiratory compromise, due to compressive effects of the large kidney. Patients with classic ARPKD detected on ultrasound are not currently eligible for therapy in the RAFT trial (3,24).…”

“…When amniotic fluid is low or absent, lung fluid escapes out of the airway and pulmonary development decreases or may cease altogether. Pregnancies complicated by anhydramnios secondary to severe CAKUT prior to 22 weeks GA constitute early pregnancy renal anhydramnios (EPRA) (3). The main etiologies of EPRA are congenital bilateral renal agenesis (CoBRA), cystic kidney disease (CKD), and severe lower urinary tract obstruction (LUTO) (4)(5)(6).…”

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

“…The NICHD-funded prospective Renal Anhydramnios Fetal Therapy (RAFT) trial (NCT03101891) [3,4] is the "North American Fetal Therapy Network Collaborative … prospective study enrolling patients whose fetuses DOI: 10.1159/000509749 have BRA" to which we believe the authors refer. This trial is currently enrolling patients and is slated to include 35 fetuses with prenatally diagnosed BRA, with a primary outcome of neonatal respiratory survival to successful dialysis.…”