Neonatal Renal Failure in the Setting of Anorectal Malformation: A Case Report and Literature Review

Anorectal malformations (ARMs) can occur in isolation or in association with other anomalies, most commonly those of the genitourinary systems. Morbidity and mortality are highest among patients who develop end-stage renal disease (ESRD) either from severe congenital anomalies (dysplastic kidneys) or from repeated infections in those who have vesicoureteral reflux or persistent recto-urinary fistulas. We describe our management strategy for a patient born with an ARM and bilateral dysplastic kidneys to highlight the nuances and complex decision-making considerations required in taking care of this complex patient population. Our patient is a male twin born at 32 weeks' gestational age who was found to have bilateral dysplastic kidneys on prenatal ultrasound. On initial examination, an imperforate anus was identified along with a severe urethral stricture. Full workup also revealed sacral dysgenesis and confirmation of the dysplastic kidneys. On day of life 3, a laparoscopic diverting sigrnoid colostomy was performed; urologic evaluation confirmed the severe urethral stricture, which required dilation to place an SF council Lip catheter. Due to his small size, peritoneal dialysis could not be initiated until five weeks of age. As full volumes could not be reached with peritoneal dialysis, he was soon transitioned to continuous renal replacement therapy. At five months of age, a laparoscopic-assisted posterior sagittal anorectoplasty (PSARP) was performed. As his urethral stricture had worsened, a suprapubic catheter had been placed for bladder decompression. Reversal of his colostomy was performed 15 days after PSARP. Unfortunately, the patient required three further surgical interventions due to abdominal wall and inguinal hernias contributing to filling and emptying dysfunction when utilizing peritoneal dialysis. He is currently 16 months of age and remains inpatient due to intermittent hemodialysis requirements along with autocycling of his peritoneal dialysis. He is working on developmental milestones, can pull to a stand, and is currently being evaluated for kidney transplantation. The development of ESRD in a neonate or infant with an ARM is rare and can be due to congenital dysplasia or agenesis of bilateral kidneys. While peritoneal dialysis is the preferred approach, catheter dysfunction can result from intra-abdominal adhesions or inadequate fluid removal from inguinal or abdominal wall hernias that form in the setting of increased infra-abdominal pressure required for peritoneal dialysis. Close collaboration is required between pediatric surgeons, nephrologists, and urologists to facilitate colonic and urologic reconstruction and manage catheter-related complications.