Outcomes of kidney transplants in pediatric patients with the vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities association

Abstract

In this single‐center retrospective study, we analyzed kidney transplant outcomes in nine pediatric patients with VACTERL [vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities] association—making this the largest study of its kind. Of 743 pediatric kidney transplant recipients at our center (1980‐2017), nine had documented diagnoses of VACTERL association. All nine had congenital anorectal malformations and renal anomalies, five had vertebral defects, and one had a bifid thumb and tracheoesophageal fistula. Renal anomalies included dysplasia (n = 6), aplasia (n = 3), and horseshoe kidney (n = 2). Congenital lower urinary tract anomalies included neurogenic bladder (n = 6), obstructive uropathy (n = 4), anovesicular fistula (n = 1), rectourethral fistula (n = 1), and posterior urethral valves (n = 1). Age at transplant ranged from 1.2 to 15 years (mean, 7.3; standard deviation [SD], 5.5); 6 (67%) were male, and 3 (33%) were female; 6 (67%) had a living related donor, and 3 (33%) had a deceased donor. The overall graft survival rate was 78% (range, 1.5 to 25.2 years; mean, 10.5; SD, 8.9). One month post‐transplant, one recipient died with a functioning graft. At 3.7 years post‐transplant, one graft failed because of recurrent pyelonephritis. Post‐transplant urologic complications included pyelonephritis (n = 6), vesicoureteral reflux (n = 5), and graft hydronephrosis (n = 4). We conclude that pediatric patients with VACTERL association can be safely transplanted—careful patient selection with vigilance and intervention for pre‐ and post‐transplant urologic complications is essential.

Substantial incidence of bladder dysfunction in patients with VACTERL association: Implications for surveillance

“…Diagnosing occult renal anomalies is equally important, as these malformations may increase the risk of future morbidity such as chronic renal insufficiency, even in the absence of NB. Moreover, some studies have raised the hypothesis that patients with the VACTERL association progress to endstage renal failure at an increased rate and present a higher incidence of complications compared with non-VACTERL patients with kidney disease and have an increased incidence of post-transplant urologic complications (Ahn et al, 2009;Diaz et al, 2019). Akl et al (2015) showed that NB was the cause of 25% of children with end-stage renal failure.…”

“…Akl et al (2015) showed that NB was the cause of 25% of children with end-stage renal failure. In the study conducted by Diaz et al (2019), 67% of a cohort of VACTERL patients that underwent renal transplants had NB. Ahn et al (2009), in a study of children with VACTERL association and chronic kidney disease, 5 of 12 (41.6%)…”