Clinical and urodynamic outcomes in children with anorectal malformation subtype of recto-bladder neck fistula
Andrew C. Strine, MD, Zaheer Alam, MD, Brian A. VanderBrink, MD, Marion Schulte, MHSA, RN, Paul H. Noh, MD, William R. DeFoor, Jr., MD, MPH, Eugene Minevich, MD, Curtis A. Sheldon, MD, Jason S. Frischer, MD, Pramod P. Reddy, MD.
Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
The incidence of urologic anomalies in patients with anorectal malformations (ARMs) is variable and likely dependent on several factors. Recto-bladder neck fistulas are the most severe ARMs in male patients and known to have an increased risk of urologic anomalies. However, their long-term urologic outcomes are poorly described. We sought to evaluate the clinical and urodynamic outcomes in this cohort of patients with an ARM subtype of recto-bladder neck fistula.
We performed a retrospective review of patients with an ARM subtype of recto-bladder neck fistula who were treated at our institution since 2007. Demographic and clinical data were assessed. Our primary outcomes were the ability to achieve continence after 4 years of age and development of chronic kidney disease (CKD) stage 2 or worse (<89 mL/min/1.73 m2). Continence was defined as the ability to store urine for 3-4 hours during the day and 8 hours overnight without leakage.
A total of 67 patients were identified for inclusion. Demographic and clinical data are provided in Tables 1 and 2. Hydronephrosis and vesicoureteral reflux (VUR) worsened in 14 (28%) and 8 (20%) patients with available imaging before and after posterior sagittal anorectoplasty (PSARP), respectively. CKD stage 2 or worse developed in 13 (24%) patients. In 35 patients who could be assessed for continence, it was achieved after 4 years of age in only 5 (14%) patients voiding spontaneously and 15 (43%) patients performing clean intermittent catheterization. Recurrent urinary tract infections (UTIs) (OR 0.71, p=0.005) and PSARP at our institution (OR 0.73, p=0.02) were negative associated with an ability to achieve continence; while urethral anomalies (1.40, p=0.03) were associated with the development of stage 2 CKD or worse on multiple logistic regression analysis. There was no association between continence and the sacral ratio or presence of tethered spinal cord.
Patients with an ARM subtype of recto-bladder neck fistula had a high rate of urologic anomalies. Our cohort of patients was rarely able to achieve continence with spontaneous voiding alone and was at risk of developing CKD, both of which were likely multifocal in origin. These patients require close urologic follow-up.
|No. total patients|
No. patients with urologic follow-up at our institution
Median follow-up from PSARP in months
Median urologic follow-up at our institution in months
|Median age at PSARP in months|
% PSARP at our institution
% reoperative PSARP
% laparotomy at PSARP
% laparoscopy at PSARP
|% recurrent UTIs|
% recurrent epididymo-orchitis
|% preoperative hydronephrosis|
% postoperative hydronephrosis
% hydronephrosis at most recent follow-up
|% preoperative VUR|
% postoperative VUR
% VUR at most recent follow-up
% anti-reflux surgery
|% solitary kidney|
% upper-tract anomalies
% urethral anomalies
% undescended testis
Most recent GFR in mL/min/1.73 m2
|% tethered cord|
% tethered cord release
Mean anteroposterior sacral ratio
Mean lateral sacral ratio
|% anticholinergic use at most recent follow-up|
% clean intermittent catheterization at most recent follow-up
|% continent catheterizable channel|
% bladder neck procedure
% augmentation cystoplasty
|% detrusor overactivity||75|
|Mean leak point pressure in cm H2O||63|
|Mean functional capacity at 40 cm H2O as % of age-expected capacity||147|
|Mean maximum capacity as % of age-expected capacity||167|
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