Survival in a functionally anephric neonate with autosomal recessive polycystic kidney disorder (ARPKD)
Sarah L. Hecht MD*, Vijaya M. Vemulakonda, MD
Division of Urology, Children's Hospital of Colorado
INTRODUCTION:
The functionally anephric fetus traditionally has been considered nonviable. Some centers have begun offering aggressive perinatal care for patients with bilateral renal agenesis with rare case reports of survival past the neonatal period(1,2). We present a patient with prenatally diagnosed ARPKD who successfully underwent postnatal nephrectomy and neonatal dialysis as a bridge to kidney transplant.
CASE:
A 27-year-old G2P1 mother presented to our fetal care center after 26-week prenatal ultrasound revealed enlarged echogenic kidneys and new anhydramnios, consistent with ARPKD. Fetal MRI confirmed enlarged, hyperintense kidneys (Figure 1). Neonatal respiratory survival was deemed likely based on fetal lung volumes. Parents elected to pursue any available medical interventions. The child was born at 36 weeks. She was anuric as expected. She underwent left nephrectomy on day of life 2 to create space for peritoneal dialysis. A peritoneal dialysis catheter and temporary hemodialysis line were placed at that time. Her course was complicated by bacteremia, line thrombosis, bacterial peritonitis, feeding intolerance, and difficulty achieving therapeutic peritoneal dialysis volumes requiring intermittent hemodialysis. At 10 months of age, she is tolerating goal enteric feeds and is on a stable home peritoneal dialysis regimen.
CONCLUSION:
We report survival in a functionally anephric neonate with ARPKD managed with unilateral nephrectomy and peritoneal dialysis. Pulmonary survivors eligible for future kidney transplant may be candidates for aggressive perinatal management in a condition previously considered universally fatal. Efforts are underway to further study who is a candidate for such intervention, and whether offering such care is ethical(3,4).
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