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

Anhydramnios caused by early anuria is thought to be universally fatal due to pulmonary hypoplasia. Bilateral renal agenesis and early fetal renal failure leading to anhydramnios constitute early pregnancy renal anhydramnios (EPRA). There have been successful reports of amnioinfusions to promote lung growth in the setting of EPRA. Some of these successfully treated EPRA fetuses have survived the neonatal period, undergone successful dialysis, and subsequently received a kidney transplant. Conversely, there are no reports of untreated EPRA survivors. This early success of amnioinfusions to treat EPRA justifies a rigorous prospective trial. The objective of this study is to provide a review of what is known about fetal therapy for EPRA and describe the Renal Anhydramnios Fetal Therapy trial. We review the epidemiology, pathophysiology, and genetics of EPRA. Furthermore, we have performed systematic review of case reports of treated EPRA. We describe the ethical framework, logistical challenges, and rationale for the current single center (NCT03101891) and planned multicenter trial.

Optimal timing for elective resection of asymptomatic congenital pulmonary airway malformations.

PURPOSE: We sought to determine optimal timing for CPAM resection within the first year of life. METHODS: We queried the National Surgical Quality Improvement Program pediatric database from 2012 to 2015 for elective CPAM resections on patients less than 1year of age. Patients were divided by age in months: 1-3 (n=57), 4-6 (n=135), and 6-12 (n=214). Patient operative variables and 30-day postoperative outcomes were compared. RESULTS: A total of 406 patients were included with no differences in demographics or comorbidities. Median operative time increased with each older age category (115min, 152min, 163min, respectively; p<0.01). Thoracoscopic approach was less utilized in 1-3months (40.4%) compared to the older two age categories (65.9% and 69.6%, respectively; p<0.01). There were no differences by age in major complications, conversion to open, or readmissions. On multivariate analysis, ASA class≥3 (p<0.01) and prolonged operative time (p<0.01) were associated with a major complication. Furthermore, operations on patients aged 6-12months were associated with increased operative time (p<0.01) regardless of operative approach. CONCLUSION: Elective CPAM resections are equally safe in patients 1-12months of age. Earlier resection including both open and thoracoscopic resection is associated with decreased operative time. LEVEL OF EVIDENCE: IIc, Outcomes Research.