Abstract: FR-PO0926
Complexities of an Infant's Care After Serial Fetal Amnioinfusion for Bilateral Renal Agenesis
Session Information
- Pediatric Nephrology: Genetic Diseases, Development, Neonatal Nephrology, Glomerular Diseases, and More
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1800 Pediatric Nephrology
Authors
- Mowery, Ashley, University of Utah Health, Salt Lake City, Utah, United States
- Lomanta, Francis Vincent, University of Utah Health, Salt Lake City, Utah, United States
- Uber, Amanda M., University of Utah Health, Salt Lake City, Utah, United States
Introduction
Serial fetal amnioinfusion therapy (SAT) for bilateral renal agenesis (BRA) has led to successful delivery of viable neonates via mitigation of fatal pulmonary hypoplasia. Unfortunately, these neonates experience significant morbidity and mortality; often requiring prolonged hospitalization for comorbidity management and renal replacement therapy. Current literature has a paucity of long-term survival outcomes to kidney transplantation and beyond.
Case Description
A female fetus was diagnosed with BRA at 20 weeks. Parents were extensively counseled by a multidisciplinary team on management options. Family chose serial amnioinfusions at an outside hospital beginning at 25 weeks, and delivery at our hospital which had never cared for an infant with BRA immediately after birth. Mom underwent 10 weekly amnioinfusions until the infant was born at 35 weeks. Postnatally, she was found to have multiple congenital anomalies consistent with VACTERL. On day of life (DOL) 2 she started Aquadex. She has refractory hypotension requiring support with angiotensin II infusion, norepinephrine, vasopressin, hydrocortisone, fludrocortisone, salt loading, midodrine and epinephrine intermittently. On DOL 168 she started peritoneal dialysis (PD) concurrently with Aquadex. Given her need for continued pressor support, achieving full enteral nutrition has been challenging. Reliance on high volume parenteral nutrition had made full transition to PD impossible despite daily ultrafiltration of >500mL. She remains admitted to the NICU with goals of care aimed toward discharge and possible kidney transplantation. Her prognosis is guarded as she needs stable blood pressures without pressors, toleration of full enteral feeds, and resumption of PD.
Discussion
Overall, SAT promotes adequate lung development to sustain life after birth. However, patients with BRA have nuanced complexities including blood pressure regulation given the absence of a functioning RAAS system. These complexities greatly affect renal replacement therapy options. This case also highlights ethical concerns given SAT is not standard of care, especially for infants with BRA due to poor outcomes in the RAFT trial. Additionally, data regarding long term survival and quality of life is limited. Management of patients with BRA continues to require further investigation and discussion to ensure appropriate and sustainable care for this unique population.