Pulmonary hypoplasia correlates with the length of anhydramnios in patients with early pregnancy renal anhydramnios (EPRA)

Eric B. Jelin, Jody E. Hooper, Eleonora Duregon, Alex K. Williamson, Sarah Olson, Kristin Voegtline, Angie C. Jelin

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Early pregnancy renal anhydramanios (EPRA) occurs when the fetus is anuric before 22 weeks gestational age (GA) and is considered universally lethal. Serial amnioinfusions have successfully ameliorated the lethal pulmonary hypoplasia associated with EPRA and have resulted in cases of neonatal survival, peritoneal dialysis, and renal transplant. Objective: We sought to evaluate the lung pathology of untreated fetuses and neonates that had EPRA. Study design: This is a retrospective case series of all fetuses and neonates diagnosed with isolated EPRA that underwent autopsy at a single tertiary care center between 1987 and 2018. Autopsy data were correlated with ultrasound findings and GA at delivery. Fetal weights, lung weights, and lung developmental stage were recorded. Results: Nineteen cases met criteria for analysis and ranged from 16 to 38 weeks GA at termination or birth. The observed-to-expected (O/E) lung-to-body-weight ratio was significantly associated with GA (r = −0.51, p = 0.03), such that as GA increased the O/E ratio decreased. When limited to patients >22 weeks, this relationship strengthened (r = −0.75, p = 0.01). Importantly, overall O/E body weight had no relationship with GA. Conclusion: This study shows that the degree of pulmonary hypoplasia in EPRA increases with the length of anhydramnios. This suggests that amnioinfusions are likely to be of most benefit the soonest they can feasibly be initiated.

Original languageEnglish (US)
Pages (from-to)1924-1929
Number of pages6
JournalJournal of Perinatology
Volume41
Issue number8
DOIs
StatePublished - Aug 2021

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Obstetrics and Gynecology

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