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Infants with In Utero Renal Insufficiency Secondary to Posterior Urethral Valves: A New and Complex Patient Population

Saturday, October 20, 2012: 1:40 PM
Napoleon Ballroom (Hilton Riverside)
Abigail C. Coots, MD1, Shumyle Alam2, Pramod Reddy2, Foong-Yen Lim1, Maria Alonso1, Jens Goebel3, Jaimie Nathan1, Curtis Sheldon, MD2 and Greg Tiao1, (1)Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, (2)Pediatric Urology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, (3)Nephrology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

Purpose: Posterior urethral valves (PUVs) are the most common cause of pediatric obstructive uropathy with almost 20% of patients progressing to end-stage renal disease (ESRD).  PUV causing in utero or perinatal ESRD is less frequently seen as these children most often succumb to respiratory hypoplasia.  With the advent of fetal intervention, advances in ventilatory support, and neonatal critical care, more afflicted newborns are surviving.  These patients have profound urinary tract abnormalities and represent a unique and challenging ESRD population.  The optimal management of these patients has yet to be established.

Methods: An IRB approved retrospective review was conducted. Since the inception of a Fetal Care Center in 2004, 11 patients with prenatally detected PUV induced renal insufficiency were identified.

Results: Ten patients (born at EGA 34.1 ± 0.65 weeks) had prenatal imaging demonstrating oligo- or anhydramnios and renal dysplasia consistent with in utero renal insufficiency.   Seven patients underwent fetal interventions (amnioinfusion (4), fetal vesicostomy (2), and vesicoamniotic shunt (1)) for decreasing amniotic fluid volumes and non-reassuring fetal urine electrolytes. After delivery, ten infants required ventilatory support.  All patients required renal replacement therapy via peritoneal dialysis.  Three patients required hemodialysis secondary to complications of peritoneal dialysis.  Mean age at initiation of dialysis was 49.7 days (range 2-210 days).  Anatomic urinary tract abnormalities were seen in all patients.  Prenatal cross-sectional imaging demonstrated echogenic kidneys (11) and hydroureteronephrosis (7).  Postnatally, all underwent placement of a urinary catheter until valve ablation was possible.  Imaging demonstrated dilating vesicoureteral reflux (VUR) in 6 patients initially that persisted in 5 after valve ablation.   To improve bladder compliance before transplant, clean intermittent catheterization and anticholinergics (7) or vesicostomy (3) was required. Growth and development was a priority before transplant.  To facilitate adequate enteral intake, gastrostomy tubes were placed in 9 infants.  Gastrostomy-tubes were placed in the mid-body of the stomach to preserve greater curvature blood supply to allow potential future gastrocystoplasty.  Five patients had inguinal hernia repair to facilitate peritoneal dialysis. One patient also had a diaphragmatic hernia repair.  All patients have undergone workup for living-related kidney transplantation; three have been transplanted at mean age 20 ± 3.2 months and weight 12 ± 0.423kg.  All three underwent ipsalateral native nephroureterectomy at the time of transplant.

Conclusion: Infants with in utero renal insufficiency secondary to PUVs have improved survival in the neonatal period when appropriate intervention and ventilatory support is utilized.  A multi-disciplinary approach has optimized growth and allowed some to proceed to renal transplantation. The timing of transplantation versus the determination of the optimal management algorithm of the bladder and urinary tract dysfunction requires definition.  The impact of the lower urinary tract on long term graft survival remains to be determined.