In this single-center retrospective review, we studied transplant outcomes in nine pediatric patients with VACTERL association, making this the largest study of its kind. Of 864 pediatric kidney transplants occurring between 1980 and 2017, nine met criteria: pediatric kidney transplant, diagnosis of VACTERL/VATER, at least two qualifying anomalies, and absence of confounding diagnoses. A descriptive analysis was performed via chart review. All nine patients had congenital anorectal malformations and renal anomalies; five patients had vertebral anomalies, one of whom also had a bifid thumb and tracheo-esophageal fistula. The renal anomalies included dysplasia (n=6), aplasia (n=3), and horseshoe kidney (n=2) one with and one without concomitant dysplasia. Prior to transplant, many patients also had congenital lower urinary tract anomalies including neurogenic bladder (n=6), obstructive uropathy (n=4), enterovesical fistula (n=1), rectourethral fistula (n=1) and posterior urethral valves (n=1). Age at transplant was 1.2-15 years (mean 7.3 +/-1.8 years); 6 were boys; 6 and 3 received living related donor (LRD) and deceased donor (DD) kidneys respectively. One recipient died with a functioning graft one month post-transplant; there was only one graft failure at 3.73 years post-transplant (due to recurrent pyelonephritis). All the remaining surviving recipients (n=8) still have functioning grafts ranging from 1.53 to 25.22 years (mean 11.46 years); and all eight suffered recurrent urologic complications: recurrent urinary tract infection (n=6), vesicoureteral reflux (VUR) (n=5), recurrent pyelonephritis (n=5), and hydronephrosis of the graft (n=3). Seven patients required surgical optimization of the lower urinary tract prior to transplant, four of whom required surgical revisions post-transplant, and one patient required surgical optimization post-transplant. Medical and surgical interventions included antibiotic prophylaxis and gentamicin bladder irrigation (n=4), bladder augmentation with Monti (n=1), revision mitrofanoff (n=3), fistula repair (n=2), urethral surgery (n=2), nephrostomy (n=1), and ureteral dilation (n=1). Pediatric patients with VACTERL can be safely transplanted – vigilance and intervention for pre-and post-transplant urologic complications is essential.

CITATION INFORMATION: Diaz J., Chavers B., Chinnakotla S., Verghese P. Outcomes of Renal Transplantation in Pediatric Patients with VACTERL Association Am J Transplant. 2017;17 (suppl 3).

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