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Review
. 2020 Apr;40(5):528-537.
doi: 10.1002/pd.5658. Epub 2020 Feb 19.

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

Angie C Jelin  1   2 , Katelynn G Sagaser  1 , Katherine R Forster  3 , Tochi Ibekwe  1 , Mary E Norton  4 , Eric B Jelin  1   5
Affiliations
Review

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

Angie C Jelin et al. Prenat Diagn. 2020 Apr.

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 including bilateral renal agenesis, cystic kidney disease, and lower urinary tract obstruction. Appropriate sonographic evaluation is required to arrive at the appropriate urogenital diagnosis and to identify additional anomalies that allude to a specific genetic diagnosis. Genetic evaluation variably includes karyotype, microarray, targeted gene testing, panels, or whole exome sequencing depending on presentation. Patients receiving a fetal diagnosis of EPRA should be offered management options of pregnancy termination or perinatal palliative care, with the option of serial amnioinfusion therapy offered on a research basis. Preliminary data from case reports demonstrate an association between serial amnioinfusion therapy and short-term postnatal survival of EPRA, with excellent respiratory function in the neonatal period. A multicenter trial, the renal anhydramnios fetal therapy (RAFT) trial, is underway. We sought to review the initial diagnosis ultrasound findings, genetic etiologies, and current management options for EPRA.

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Conflict of interest statement

CONFLICT OF INTEREST

The authors declare no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Etiologies of early pregnancy renal anhydramnios (EPRA) 1a. Bilateral renal agenesis: absence of kidneys with “lying down adrenals” 1b. Polycystic kidney disease: large hyperechoic kidneys 1c. Multicystic kidney disease: kidneys with multiple cysts of variable size 1d. Lower urinary tract obstruction (LUTO): large bladder with “keyhole” appearance
FIGURE 2
FIGURE 2
Flow sheet for evaluation, diagnosis of early pregnancy renal anhydramnios (EPRA), and candidacy for renal anhydramnios fetal therapy (RAFT)
See this image and copyright information in PMC

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MeSH terms

Supplementary concepts