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Correspondence to: Konrad M. Szymanski, Division of Pediatric Urology, Riley Hospital for Children at IU Health, 705 Riley Hospital Dr., Suite 4230, Indianapolis, IN 46202, USA, Tel.: +1 317 944 7446; fax: +1 317 944 7481
We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals
with myelomeningocele (MMC) followed by urology in the modern medical era and to assess
if ESKD risk was higher after surgery related to a hostile bladder.
We retrospectively reviewed patients with MMC followed by urology at our institution
born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of
age (when mortality is highest, sometimes before establishing urology care). ESKD
was defined as requiring permanent peritoneal/hemodialysis or renal transplantation.
Early surgery related to hostile bladder included incontinent vesicostomy, bladder
augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy
for recurrent urinary tract infections. Survival analysis and proportional hazards
regression were used. Sensitivity analyses included: risk factor analysis with only
vesicostomy, timing of surgery, including the entire population without minimal follow-up
(n = 1054) and only patients with ≥ 5 years of follow-up (n = 925).
Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female,
76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis,
1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was
0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old,
p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD
risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender,
birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16).
Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without
one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had
a higher risk of ESKD. No other statistically/clinically significant differences were
Patients with MMC remain at risk of progressive renal damage throughout life. We relied
on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular
filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent
documentation of early urodynamic findings and indications for bladder-related surgery.