Summary
Background
End stage renal disease (ESRD) requiring kidney transplant (KT) remains an important
cause of morbidity in anorectal malformations (ARM) patients. Current literature is
scarce on defining the risk factors for ESRD and the outcomes of KT in ARM patients.
Objective
This study aimed to identify predisposing factors to ESRD in ARM patients and verify
if the long term outcome of KT in these patients differs from pure urological anomalies
(UA).
Study design
Databases of ARM and KT patients treated at our center between 2000 and 2016 were
used for comparing characteristics of ARM cases which developed ESRD and those who
did not, and the outcome features of MAR-KT vs UA-KT.
Results
Out of 117 ARM patients, 9 developed ESRD. All of them had a complex ARM. Association
with UA were significantly higher in ARM-KT compared to other ARM patients (100% vs.
52%, p = 0.001). The most common UA associated to KT in ARM patients was renal dysplasia.
During the same period 23 patients underwent KT as a sequelae of pure urological anomalies
(UA-KT group). The most represented UA were primary vesico-ureteral reflux (65.2%)
and posterior urethral valves (14%) in UA-KT (table 2). ARM-KT patients required more
often hemodialysis before KT (50% vs. 8.7%, p = 0.05) and an aorto-caval anastomosis
at the transplant (75% vs. 30%, p = 0.04) compared to UA-KT. Moreover ARM-KT patients
experienced more often graft failure and and the need for a second KT (50% vs. 8.6%,
p = 0.02).
Discussion
To our knowledge, this is the first study describing the differences in terms of risk
factors and outcomes of KT in ARM patients. We observed a need for KT in 7% of ARM,
which is at the higher end of the range reported in the literature. Bilateral dysplasia
and cloaca malformation seem the leading cause to ESRD for ARM patients compared to
vesico-ureteral reflux and posterior urethral valve in UA-KT. Our experience showed
that KT has worst outcome in ARM vs pure UA patients.
Conclusion
Patients with complex ARM are more frequently associated to renal dysplasia which
lead to KT. Graft in ARM patients seems to be the more vulnerable and prone to failure.
Keywords
Abbreviations:
ESRD (End-stage renal disease), ARM (anorectal malformations), KT (kidney transplant), UA (urological anomalies), ARM-KT (patients with anorectal malformation who underwent kidney transplant), UA-KT (patients with urological anomalies but no ARM who underwent kidney transplant), eGFR (estimated glomerular filtration rate), VUR (vesico-ureteral reflux), PUV (posterior urethral valve), UPJO (ureteropelvic junction obstruction), MRI (magnetic resonance imaging)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: June 23, 2022
Accepted:
June 20,
2022
Received in revised form:
June 17,
2022
Received:
February 1,
2022
Footnotes
☆Previous communication: The paper has been accepted as oral communication at the 5th Joint Meeting of ESPU-SPU September 23–25th,,2021-Virtual.
Identification
Copyright
© 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.