Unilateral Nephrectomy: An Effective Temporizing Measure for Refractory Hypertension in ARPKD

Introduction: Neonatal autosomal recessive polycystic kidney disease (ARPKD) can be complicated by respiratory failure, refractory hypertension, and feeding intolerance due to renal failure and mass effect from the kidneys. Current recommendations lack consensus regarding the benefit of unilateral versus bilateral nephrectomy.


Description: We describe our experience with a neonate with prenatally diagnosed ARPKD and associated pulmonary hypoplasia. The patient initially required intubation at birth but tolerated weaning, extubation, and a 9 week period of minimal non-invasive respiratory support. During this period, she developed severe hypertension refractory to high doses of anti-hypertensives, including amlodipine, diuretics, labetalol, minoxidil, clonidine, and parenteral drips. She also had rapid cystic kidney growth and developed respiratory failure secondary to mechanical thoracoabdominal competition requiring re-intubation and a positive-end expiratory pressure of at least 12 cm H2O to maintain lung expansion. Due to respiratory failure and inadequate blood pressure control, the decision was made to undergo nephrectomy via a staged approach. Prior to surgery, renal scintigraphy showed differential renal function of 30% on the left and 70% on the right. She had an uncomplicated total left nephrectomy on DOL 96. Euvolemia was maintained post-operatively through a combination of peritoneal dialysis ultra-filtration and robust urine output from the remaining kidney. Post-nephrectomy, her hypertension was adequately treated with enteral medications and a single anti-hypertensive infusion. Tolerance of enteral feeds also improved. She continued to require mechanical ventilation but was successfully supported on a conventional ventilator.


Discussion: Palliative unilateral nephrectomy is an effective tool for blood pressure control and temporizing of thoracoabdominal competition, allowing time for growth until renal transplant is feasible. Delaying eventual bilateral nephrectomy is beneficial given concerns surrounding effective dialysis in an anuric neonate.