Impact of spinal cord malformation on bladder function in children with anorectal malformations

doi:10.1016/j.jpedsurg.2009.03.001

Journal of Pediatric Surgery

Volume 44, Issue 9, September 2009, Pages 1778-1785

https://doi.org/10.1016/j.jpedsurg.2009.03.001 Get rights and content

Abstract

Purpose

Risk factors for the presence of neurogenic bladder dysfunction (NBD) in children born with high anorectal malformations (ARMs), were investigated, to identify the need for urodynamics in these patients.

Material and Methods

The study included 37 patients with high ARMs (21 boys and 16 girls). Bladder function was evaluated with urodynamics both before and after anorectoplasty (posterior sagittal anorectoplasty [PSARP]). All patients were investigated with spinal radiograph. Spinal ultrasound was performed in the neonatal period, and magnetic resonance imaging was added in case of abnormal ultrasound or urodynamics and in case of cloacal malformation.

Results

In ARM patients with rectourethral and vestibular fistulas and cloacas, NBD was identified in 9 children (25%). The bladder dysfunction was innate in all cases except in one girl with cloaca, indicating that the risk of iatrogenic denervation seems minimal using the PSARP technique. All children with innate NBD had a spinal cord malformation either as spinal cord regression or tethering with or without a lipoma. Concerning vertebral status, almost all children with NBD had partial sacral agenesis. Abnormal perineal appearance was highly correlated to NBD in boys, especially in those with a spinal cord regression malformation. Innate NBD was not found in any child with normal spinal cord.

Conclusion

From these results, we suggest that spinal ultrasound and perineal inspection are used as screening procedures for NBD in children with ARM. Urodynamic investigation is recommended only when spinal cord anomalies or other signs indicative of NBD are present. In case of spinal cord malformation, repeated urodynamics during follow-up is mandatory because of the risk for developing tethered cord syndrome.

Section snippets

Patients

This material was based on a prospective study of patients with ARM referred to the Department of Pediatric Surgery, the Queen Silvia Children's Hospital Gothenburg, Sweden, from 1995 to 2005. The department is a regional referral center in the southwestern part of Sweden, serving a population of approximately 2.5 million.

During this period, 105 patients with ARM, representing virtually all patients with anal anomalies in the region, were treated. Forty-five cases were categorized as high

Methods

Investigations in the neonatal period included plain abdominal film, colostogram via a transperineal punction through the fistula or the distal colostomy, spinal and sacral radiograph, ultrasound of urinary tract, and voiding cystourethrography. Spinal ultrasound was routinely performed in the neonatal period in 24 patients. The limited number of patients investigated is because the method was only available from 1999. Magnetic resonance imaging (MRI) of the spine was added in 14 patients owing

Statistical methods

For comparison between groups, Mann-Whitney U test was used for continuous variables and Fisher's Exact test for dichotomous variables. For analyses of difference of before and after, Wilcoxon's signed rank test was used. All tests were 2-tailed and conducted at 5% significance level.

Anorectal malformation in boys (n = 21)

Sixteen (8 prostatic, 5 bulbar, and 3 no fistula) of 21 boys with ARM were considered to have normal urodynamics both before and after surgical reconstruction. Seven of these boys were found to have mild detrusor overactivity (4 before and 6 after surgery), which was considered to be of nonneurogenic origin.

Five boys with fistulas (1 bladder neck, 4 prostatic) were considered to have NBD both before and after reconstructive surgery. The urodynamic pattern was characterized by neurogenic

Discussion

In this prospective study of children with ARM, 25% of the children were found to have NBD. There is wide variation reported in the prevalence of neurogenic bladder dysfunction in children with ARM, from 17% to 50% [2], [3], [4], [5]. The variation seems to depend on factors such as small material, selection of patients, retrospective studies, differences in investigation techniques, and inconsistency in classification and follow-up program.

Regarding NBD in the present study, the problem was

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Cited by (33)

Reoperative Anorectal Procedures in Patients with Anorectal Malformations – Is Bladder Function Affected?
2023, Journal of Pediatric Surgery
Anorectal malformations (ARM) are associated with neurogenic bladder. The traditional surgical ARM repair is a posterior sagittal anorectoplasty (PSARP), which is believed to have a minimal effect on bladder dynamics. However, little is known about the effects of reoperative PSARP (rPSARP) on bladder function. We hypothesized that a high rate of bladder dysfunction existed in this cohort.
We performed a retrospective review of ARM patients undergoing rPSARP at a single institution from 2008 to 2015. Only patients with Urology follow-up were included in our analysis. Data was collected regarding original level of ARM, coexisting spinal anomalies and indications for reoperation. We assessed urodynamic variables and bladder management (voiding, CIC or diverted) before and after rPSARP.
A total of 172 patients were identified, of which 85 met inclusion criteria with a median follow-up of 23.9 months (IQR, 5.9–43.8 months). Thirty-six patients had spinal cord anomalies. Indications for rPSARP included mislocation (n = 42), posterior urethral diverticulum (PUD; n = 16), stricture (n = 19) and rectal prolapse (n = 8). Within 1 year following rPSARP, 11 patients (12.9%) had a negative change in bladder management, defined as need for beginning intermittent catheterization or undergoing urinary diversion, which increased to 16 patients (18.8%) at last follow-up. Postoperative bladder management changed in rPSARP patients with mislocation (p < 0.0001) and stricture (p 0.005) but not for rectal prolapse (p 0.143).
Patients who undergo rPSARP warrant especially close attention for bladder dysfunction as we observed a negative postoperative change in bladder management in 18.8% of our series.
Level IV.
The Importance of Screening for Additional Anomalies in Patients with Anorectal Malformations: A Retrospective Cohort Study
2023, Journal of Pediatric Surgery
In children with anorectal malformations (ARM), additional anomalies can occur within the VACTERL-association. Routine screening is of great importance for early identification and potential treatment. However, uniformity in screening protocols is lacking and only small cohorts have been described in literature. The aim of this study was to assess and describe a unique large cohort of ARM patients who underwent VACTERL screening in the neonatal period.
A retrospective mono-center cohort study was performed. Included were all neonates born between January 2000 and December 2020 who were diagnosed with ARM and screened for additional anomalies. Full screening consisted of x-ray and ultrasound of the spine, cardiac and renal ultrasound, and physical examination for limb deformities, esophageal atresia, and ARM. Criteria for VACTERL-classification were predefined according to the EUROCAT-definitions.
In total, 216 patients were included, of whom 167 (77.3%) underwent full VACTERL-screening (66% in 2000–2006 vs. 82% in 2007–2013 vs. 86% in 2014–2020). Median age at follow-up was 7.0 years (IQR 3.0–12.8). In 103/167 patients (61.7%), additional anomalies were identified. Some 35/216 patients (16.2%) fulfilled the criteria of a form of VACTERL-association. In 37/216 patients (17.1%), a genetic cause or syndrome was found.
The majority of ARM patients underwent full screening to detect additional anomalies (77%), which improved over time to 86%. Yet, approximately a quarter of patients was not screened, with the potential of missing important additional anomalies that might have severe consequences in the future. Forms of VACTERL-association or genetic causes were found in 16% and 17% respectively. This study emphasizes the importance of routine screening.
III.
A prospective cohort study of assisted bladder emptying following primary cloacal repair: The Children's National experience
2023, Journal of Pediatric Urology
Although the combination of bladder dysfunction and upper tract anomalies puts patient with cloaca at risk for renal disease, the rarity of this condition makes it difficult to study empirically. As a high-volume center, we uniquely capture bladder function outcomes following our growing number of cloacal repairs.
1) Describe the rates of incomplete bladder emptying following primary cloacal repair (at 2–3 months after repair and last follow up), and 2) identify clinical factors associated with assisted bladder emptying.
We performed a prospective cohort study of patients undergoing primary cloaca repair by our Children's National Colorectal Center team between 2020 and 2021. The primary outcome was assisted bladder emptying at 2–3 months postoperatively and last visit. Covariables included preoperative characteristics (cloacagram measurements), ARM complexity (moderate = common channel [CC] <3-cm, severe = CC ≥ 3-cm), vesicoureteral reflux (VUR) status, sacral ratio (good ≥0.7, intermediate 0.7–0.4, poor ≤0.4), spinal cord status, means of preoperative bladder emptying, and operative details (age at repair, repair type, & concomitant laparotomy).
Eighteen participants were eligible. A majority had moderate cloaca (78%), VUR (67%), spinal cord abnormalities (89%), and good sacral ratios (56%). Preoperatively, 10 patients were diapered for urine and 8 had assisted bladder emptying. Surgical repairs were performed at a median age of 8 months (range 4–46). Nine (50%) patients underwent urogenital separation (UGS), eight (44%) total urogenital mobilization, and 1 (6%) perineal sparing posterior sagittal anorectoplasty with introitoplasty. Exploratory laparotomy was performed in 7 (39%) patients. At 2–3 months, 7 patients were voiding and 11 required assisted bladder emptying. Median length of long-term follow up was 12 months (range 5–25), and 8 patients were voiding and 10 required assisted bladder emptying. Postoperative need for assisted bladder emptying was significantly associated with assisted bladder emptying preoperatively, a shorter urethra and increasing common channel length, UGS and exploratory laparotomy. Spinal cord imaging findings were not associated.
Bladder emptying following cloaca repair is likely a result of congenital function and surgical effects. Indeed, increasingly cloaca complexity requiring UGS and laparotomy was associated with both pre- and post-operative assisted bladder emptying. The lack of association with spinal cord imaging may reflect a divergence between anatomy and function.
Approximately half of patients required assisted bladder emptying in this study. Associated factors included urethral and common channel length, the need for assisted bladder emptying preoperatively, the type of surgical approach and additional laparotomy. Being diapered with seemingly normal voiding prior to surgery did not guarantee normal bladder function postoperatively.
Four-hour voiding observations detect neurogenic lower urinary tract dysfunction in neonates with anorectal malformation
2021, Journal of Pediatric Urology
Citation Excerpt :
Since bladder capacity is age dependent, we also gave bladder capacity as a percentage of the capacity expected for age, using the formula [30 + 2.5 × age in months] recommended by the ICCS standardisation document [11]. Pressure measurement (MMS, UDS 0075-Solar) was performed according to a routine method described previously [3]. To calculate reference values for normal/abnormal urodynamic findings, results from earlier studies in infants of comparable age were used [11,14].
Neurogenic lower urinary tract dysfunction (LUTD) has been reported in 20–50% of children with anorectal malformations (ARM). As neurogenic LUTD represents an inherent risk of renal deterioration and urinary tract infections, an early diagnosis is important. The gold standard for evaluating neurogenic LUTD involves invasive urodynamic testing but a useful addition should be an easy-to-perform, non-invasive method of screening.
In this study, we evaluate non-invasive 4 h voiding observations as a screening method for neurogenic LUTD in ARM children.
Thirty-four patients with ARM, excluding those with perineal fistulas, were evaluated using both 4 h voiding observation and urodynamic testing before and after posterior sagittal anorectoplasty (PSARP) at median ages of 0.3 and 1.1 years. In the urodynamic assessment, the gold standard for neurogenic LUTD, nine children received the diagnosis, eight innate and one post-surgery.
Five boys with a high urethral fistula and anomalies of the spinal cord had urodynamically diagnosed neurogenic LUTD, a dysfunction also identified in the 4 h voiding observations. The pattern was characterised both by an increase in the number of voiding and the number of interrupted voiding, urinary leakage and elevated residual urine (Figure). In three girls with a vestibular fistula and tethered cord, an urodynamic investigation identified suspected mild neurogenic LUTD. In the voiding observations, an abnormal voiding pattern was not as obvious in the girls as in the five males. One girl with cloaca showed signs of postsurgical denervation damage, which was easily identified in the 4 h voiding observations (high capacity and elevated residual urine).
In the present study, gender differences in the severity of dysfunction reflected in the free voiding pattern in infants with ARM and neurogenic LUTD is probably the result of the different underlying causes of neurogenic LUTD in boys and girls. Boys with the condition have a congenital malformation of the caudal part of the spinal cord and girls a tethering of the cord. The most obvious limitation of the study was the low number of patients. Despite this, we consider the results worth reporting, since we found that results in the free voiding observations effectively confirmed what was established in the urodynamic investigations.
In pre-PSARP patients, 4 h voiding observations can be used to screen for severe neurogenic LUTD requiring attention and treatment. When post-surgical denervation is suspected, the voiding observation is also a good method for indicating the diagnosis.
Predictors of upper tract damage in pediatric neurogenic bladder
2017, Journal of Pediatric Urology
Citation Excerpt :
The reported incidence of NVD in ARM varies from 17% to 50% and occurs more commonly with supralevator anomalies as was also noticed in this study [3,12]. Underlying intraspinal abnormality (18–50%), sacral agenesis, and damage to the afferent pelvic nerve during reconstructive procedures contribute to NVD in ARM [12]. The peculiar sex predilection of detrusor overactivity with failure of storage in boys and incomplete evacuation in girls was not seen in this study [12].
Upper tract damage (UTD) is a life-threatening complication of neurogenic bladder (NB). Early identification of risk factors for UTD and institution of remedial measures may probably prevent UTD. The aim was to study the predictors of UTD in children 2 years or older with NB.
This cross-sectional, observational study over 2 years included 30 children. UTD was defined as serum creatinine of >1 mg/dL or society of fetal urology grade III–IV hydronephrosis or hydroureteronephrosis on ultrasonography or renal scars on ⁹⁹^mtechnetium dimercaptosuccinic scan or subnormal glomerular filtration rate (GFR) for age. The evaluated clinical variables were age at presentation, gender, palpable bladder lump, and recurrent urinary tract infection (UTI). Bladder wall thickness (BWT), grade and laterality of vesicoureteric reflux (VUR), status of the bladder neck, post-void residue (PVR), and level and type of intraspinal lesions were also noted. Urodynamic studies were performed for functional bladder assessment. A p-value <0.05 identified the risk factors.
UTD was detected in 15 (50%) with serum creatinine >1 mg% (2, 6%), SFU III–IV (11, 36%), renal scars (12, 40%), and subnormal GFR in (2, 6%) patients. Clinical risk factors for UTD were delayed presentation (p = 0.034), palpable bladder lump (p ≤ 0.001; OR 38.5; CI 5.6–262.5), and recurrent UTI (p = 0.033, OR 4.125, CI 0.913–18.630). The presence of significant PVR, trabeculated bladder, spin-top urethra, and bilateral VUR were identified as radiological risk factors for UTD. Mean BWT in patients with and without UTD was 4.69 ± 1.78 mm and 2.91 ± 1.08 mm respectively. BWT predictive of UTD was 3.05 mm (Figure). The mean detrusor leak point pressure (DLPP) did not vary significantly in those with and without UTD (36.82 ± 14.74 and 29.09 ± 10.44 cmH₂O, respectively), yet 75% patients with DLPP > 40 cmH₂O had UTD (p = 0.038, OR 5.4, CI 0.84–34.84). DLPP <40 cmH₂O was associated with UTD in 35% patients.
The incidence of UTD in this series is in accordance with that reported with expectant management (40%) and is much higher than the 17% stated with proactive management. A limitation of this study is the small number of patients and heterogeneous clinical diagnosis.
Delayed presentation with palpable bladder lump, recurrent UTI, increased BWT, bilateral VUR, increased PVR, and DLPP > 40 cm H₂O were identified as potential risk factors for UTD. This study highlights the significance of BWT as a predictor of UTD in NB.
Manometric findings in relation to functional outcomes in different types of anorectal malformations
2017, Journal of Pediatric Surgery
To compare anorectal manometry (AM) in patients with different types of anorectal malformations (ARMs) in relation to functional outcomes.
A single-institution, cross-sectional study. After ethical approval, all patients ≥ 7 years old treated for anterior anus (AA), perineal fistula (PF), vestibular fistula (VF), or rectourethral fistula (RUF) from 1983 onwards were invited to answer the Rintala bowel function score (BFS) questionnaire and to attend anorectal manometry (AM). Patients with mild ARMs (AA females and PF males) had been treated with minimally invasive perineal procedures. Females with VF/PF and males with RUF had undergone internal-sphincter saving sagittal repairs.
55 of 132 respondents (42%; median age 12 (7–29) years; 42% male) underwent AM. Patients with mild ARMs displayed good anorectal function after minimally invasive treatments. The median anal resting and squeeze pressures among patients with mild ARMs (60 cm H2O and 116 cm H2O respectively) were significantly higher than among patients with more severe ARMs (50 cm H2O, and 80 cm H2O respectively; p ≤ 0.002). The rectoanal inhibitory reflex was preserved in 100% of mild ARMs and 83% of patients with more severe malformations after IAS-saving sagittal repair. The functional outcome was poor in 4/5 patients with an absent RAIR (BFS ≤ 11 or antegrade continence enema-dependence). Rectal sensation correlated significantly with the BFS.
Our findings support the appropriateness of our minimally invasive approaches to the management of mild ARMs, and IAS-saving anatomical repairs for patients with more severe malformations. Level of evidence: III.

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Original articleImpact of spinal cord malformation on bladder function in children with anorectal malformations

Abstract

Purpose

Material and Methods

Results

Conclusion

Section snippets

Patients

Methods

Statistical methods

Anorectal malformation in boys (n = 21)

Discussion

J Pediatr Surg

J Urol

J Urol

J Urol

J Urol,

J Urol

J Urol

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Original article
Impact of spinal cord malformation on bladder function in children with anorectal malformations