Symptomatic lower urinary tract dysfunction in sacral agenesis: Potentially high risk?

INTRODUCTION: Sacral agenesis (SA) is a caudal regression anomaly that can cause neurogenic bladder but is not generally recognized as high risk. We studied the clinical presentation, upper urinary tract, bone and spine abnormalities, and urodynamic findings in patients with SA and compared them with related high-risk conditions, anorectal malformation (ARM), and cloacal malformation. MATERIALS AND METHODS: Patient records between May 2011 and December 2015 were identified and grouped into isolated SA without an overt anomaly (Group I), SA with overt caudal regression anomalies (Group II), and ARM or cloacal malformation without the SA (Group III). Distribution of clinical and urodynamic findings and factors associated with reduced eGFR were tested with rank sum test, t-test, and unadjusted odds (P < 0.05 significant) using R statistical program (version 3.1.3). RESULTS: Of 605 neurogenic bladder patients treated in the study period, 39 fulfilled the inclusion criteria. 12 were Group I, 5 Group II, and 22 Group III. Long-standing lower urinary symptoms were noted in all SA patients. Group I patients were older (14.5 years vs. 6 years and 5 years for II and III). Patients with SA (Group I and II) had poor compliance (6.7 ml/cmH(2)O, interquartile range [IQR] 4–13.6 ml/cmH(2)O), reduced age-adjusted bladder capacity (59%, IQR 22–85%), elevated end-fill pressure (22 cmH(2)O, IQR 11–28 cmH(2)O), hydronephrosis (88%), and reduction in eGFR (29%), all comparable to Group III. Most had Renshaw type II SA and tethered spinal cord rather than wedge-shaped termination. Limitations include small numbers and significant selection bias. CONCLUSIONS: Symptomatic neurogenic bladder due to SA may cause renal damage similar to ARM but often eludes diagnosis.