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BACKGROUND:
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.
METHODS:
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.
RESULTS:
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.
CONCLUSIONS:
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.

Table 1. Demographic and clinical data

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	67 50 70.4 30.9
Median age at PSARP in months % PSARP at our institution % reoperative PSARP % laparotomy at PSARP % laparoscopy at PSARP	7.0 72 48 69 23
% recurrent UTIs % recurrent epididymo-orchitis	48 8
% preoperative hydronephrosis % postoperative hydronephrosis % hydronephrosis at most recent follow-up	55 52 45
% preoperative VUR % postoperative VUR % VUR at most recent follow-up % anti-reflux surgery	57 51 37 27
% solitary kidney % upper-tract anomalies % hypospadias % urethral anomalies % undescended testis Most recent GFR in mL/min/1.73 m2	37 24 34 22 34 110.3
% tethered cord % tethered cord release Mean anteroposterior sacral ratio Mean lateral sacral ratio	39 28 0.55 0.60
% anticholinergic use at most recent follow-up % clean intermittent catheterization at most recent follow-up	56 58
% continent catheterizable channel % bladder neck procedure % augmentation cystoplasty	27 6 10

Table 2. Postoperative urodynamic findings

% 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