End stage renal disease and kidney transplant in patients with anorectal malformation: is there an alternative route?

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Web End = Pediatr Surg Int (2015) 31:725728 DOI 10.1007/s00383-015-3734-8

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Web End = End stage renal disease and kidney transplant in patients with anorectal malformation: is there an alternative route?

Andrea Bischoff1 William DeFoor2 Brian VanderBrink2 Jens Goebel3

Jennifer Hall1 Maria Alonso1 Pramod Reddy2 Alberto Pea1

Accepted: 23 June 2015 / Published online: 5 July 2015 Springer-Verlag Berlin Heidelberg 2015

AbstractBackground/purpose Mortality from end stage renal disease (ESRD) in patients with anorectal malformation (ARM) is reported to be between 2.5 and 6 %. The risk differs depending on the type of ARM (6.4 % high vs.1.1 % low). The purpose of this study was to review the characteristics of the ARM patients who received a kidney transplant (KT) to potentially identify if any modiable factors existed that may have prevented ESRD.

Methods The Colorectal Center and the kidney transplant databases at Cincinnati Childrens Hospital were queried to identify patients with ARM and a KT. Data obtained included: gender, type of ARM, associated characteristics, urological status at birth, surgical and medical management, age at KT, and possible interventions that could have prevented or delayed the KT.

Results 20 patients with ARM who underwent KT were identied. 16 were females, thirteen of whom had a cloaca; the average common channel length in these patients was 5 cm, ranging from 2 to 8 cm. Nine cloaca patients had hydrocolpos and 5 of them were not drained at birth. Eleven patients presented with renal failure at birth. Five patients

with cloaca had common channel atresia/stenosis, 2 male patients had severe urethral atresia, 2 patients had absent bladders, and 1 patient had bilateral blind ureters. At birth, 6 patients had single functional kidneys, 4 had bilateral hydronephrosis with megaureters, 3 patients had bilateral dysplastic kidneys, 1 patient had a single kidney with hydronephrosis, and 1 patient had a single hypoplastic kidney. The average age at KT was 10.9 years (range 221 years.); in 3 patients, the KT was performed before the repair of the ARM. One patient died after the KT and one...