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

Abstract

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/cmH2O, interquartile range [IQR] 4–13.6 ml/cmH2O), reduced age-adjusted bladder capacity (59%, IQR 22–85%), elevated end-fill pressure (22 cmH2O, IQR 11–28 cmH2O), 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.

Contractile and Structural Properties of Detrusor from Children with Neurogenic Lower Urinary Tract Dysfunction

“…Connective tissue contains collagen fibrils, embedded in a ground substance, that have a greater intrinsic elastic modulus than muscle tissue [ 23 , 24 ]. This is consistent with poor compliance and upper tract damage in a large proportion of patients with myelomeningocele or sacral agenesis [ 25 , 26 ], as well as increased connective tissue deposition in animal models and humans with spinal cord injury, in particular deposition of the subtype of collagen (type-I) with greater intrinsic stiffness [ 27 , 28 ]. Medical management of neurogenic bladders with antimuscarinic agents or botulinum toxin can improve continence and renal outcomes by reducing detrusor overactivity and improving compliance.…”

What's inside

“…Sinha et al . [ 1 ] in their study reported that children with sacral agenesis may also have neurogenic bladders that could lead to renal damage, similar to that seen in children with anorectal malformations.…”

Caudal Regression Syndrome—A Narrative Review: An Orthopedic Point of View

“…An unfavorable factor is vesicoureteral reflux, which, together with frequent urinary tract infections, may affect kidney function in the future. Sinha, in his interesting research, observed a long-standing lower urinary symptoms in all patients with sacral agenesis, as well as high prevalence of upper tract changes [ 50 ]. Isolated sacral agenesis may be a cause of neurogenic bladder that often presents itself late and may result in renal damage [ 50 ].…”

“…Sinha, in his interesting research, observed a long-standing lower urinary symptoms in all patients with sacral agenesis, as well as high prevalence of upper tract changes [ 50 ]. Isolated sacral agenesis may be a cause of neurogenic bladder that often presents itself late and may result in renal damage [ 50 ]. Esposito recommended a special diagnostic protocol for all newborns at increased risk of urologic anomalies.…”