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Pediatric-Onset Rehabilitation

Genitourinary Function

The bladder performs two functions: storage and elimination of urine. Performance of those functions depends on anatomic and physiologic integrity and neurologic conditions affect the latter. Congenital neural tube defects, such as myelomeningocele, spinal dysraphism, and tethered cord lesions, are the most common causes of neurogenic bladder in children. However, SCI and diseases (stroke, myelopathies, tumors, etc.) also can cause acquired dysfunction, with significant long-term consequences (Lucas 2019). 

Pediatric SCI-related neurogenic dysfunction of the urinary tract differs from the adult type in the fact that it affects a developing organ. Normal voiding in newborns is largely reflexive with some input from the cerebral cortex. Detrusor and sphincter functions remain uncoordinated through the first several years of life (Yeung et al. 1995). As children grow, the brainstem takes more control of the pontine micturition center (Blaivas 1982). In SCI/D (spinal cord injury/dysfunction) related paralysis, the lower urinary tract dysfunction characteristics depend on the type of neurologic injury, specifically, upper and lower motor neuron type, with a small percentage having a mixed pattern (i.e., conus medularis). The upper motor neuron pattern SCI/D-related urinary tract dysfunction, a consequence of injury to the spinal cord itself, is characterized by over-active detrusor/detrusor hyperreflexia, detrusor-sphincter dyssynergia, decreased bladder compliance, and increased detrusor pressures, leading to the risk of developing vesicoureteral reflux. In this instance, the bladder acts under reflex control; as the bladder fills, the sacral micturition reflex triggers an involuntary contraction leading to bladder evacuation, clinically expressed as incontinence. Because the lesion is below the pontine micturition center (thus eliminating its’ control), there is loss of coordinated detrusor/sphincter relaxation; instead, there are intermittent contractions of the external sphincter during a detrusor contraction which can result in high intra-vesical voiding pressures, ultimately leading to upper urinary tract dysfunction and renal deterioration if not addressed. The lower motor neuron type urinary tract dysfunction, characteristic to injuries to cauda equina/nerve roots leads to a flaccid bladder which prevents emptying; external sphincter may be flaccid and incontinence can occur with any increase in intra-abdominal pressure (such as when coughing or straining). The internal sphincter tone may be intact because the sympathetic innervation originates above the conus medullaris, and this may contribute to incomplete emptying.

Most common complications of lower motor neuron type urinary tract dysfunction are recurrent UTIs related to bladder stasis and injury to the bladder wall because of distension. The small number of mixed injury bladder patterns are type A, where the detrusor is rendered flaccid, there is hypertonic external urinary sphincter and incontinence is uncommon; and type B pattern characterized by spastic bladder due to a disinhibited detrusor nucleus, a flaccid external urinary sphincter with resulting incontinence.  Development of pyelonephritis and nephrolithiasis can occur in both upper motor neuron and lower motor neuron types of urinary tract dysfunction.

Goals of treatment change in the pediatric SCI as they advance through life. While preservation of kidney and bladder function remain the most important goals throughout life, social continence becomes essential as the child grows beyond the potty-training period, followed by achieving independence from caregivers. Achieving low-pressure urinary storage associated with bladder continence and efficient and complete emptying with normal pressures and minimizing the number of infections become mainstay of day-to-day bladder management.

Author, Year

Country

Study Design

Sample Size

Population

Intervention

Outcome Measure

Results

(Patki et al., 2006)

United Kingdom

Observational

N=10

Population: Pediatric-onset SCI: Mean age: 28 (18-37) yr; Age at injury: 13.6 (6-16) yr; Time since injury: 13.1 (6-31) yr; Severity of injury: complete=6, incomplete=4.

Group 1: neurogenic detrusor overactivity (NDO) with or without detrusor sphincter dyssynergia (DSD) (n=6), Group 2: acontractile detrusor with or without low bladder compliance (n=4).

Intervention: None. Review.

Outcome Measures: Urodynamics and ultrasound studies (USS), video-urodynamic (VCMG).

Group 1: NDO

1.         2 patients, each on urge/reflex voiding (T2 complete and T5 incomplete) with sheath drainage reported 2-3 urinary tract infections (symptomatic bacteriuria) per month. The patient with T5 incomplete injury underwent sphincterotomy and memokaths stent insertion. The stent had to be removed due to malposition within a year. Subsequently CSIC and oxybutynin was instituted in both patients leading to control of recurrent infections.

2.        Upper tract changes with bilateral renal scarring and reduced glomerular filtration rate (GFR) were reported in 2 patients. In the patient (C3 complete) managed with supra pubic catheter (SPC) and oxybutynin, the changes were secondary to recurrent bilateral reflux with small, contracted, poorly controlled neuropathic bladder. The upper tract deterioration was stabilised with ileal conduit diversion. In the patient (T5 complete), kidney stone and recurrent pyelonephritis were responsible for upper tract changes. Sacral anterior root stimulator implant (SARSI) with posterior rhizotomy resolved the NDO and emptying, and the kidney stone was treated with lithotripsy with no further complications. Incidental bladder stone was picked up on routine ultrasound in the remaining patient with DSD. The stone was treated with cystolitholapaxy with no change in management.

3.        During the follow-up, complications involving upper and lower urinary tracts were reported in 5/6 patients with NDO all of whom also had DSD. The single patient (C3 incomplete) with NDO without DSD has remained complication free on urge/reflex voiding for 13 yr.

4.        Overall, at the last follow-up, 4 patients were continent with normal renal functions and 2 patients have stable renal function with ileal conduit and SARSI.

 

Group 2: acontractile detrusor

5.        2 patients had low compliance on initial VCMG. A kidney stone was detected incidentally in one of them (T12 incomplete) and was treated with extracorporeal shockwave lithotripsy (ESWL). The other patient (T12 complete) changed management from CSIC to indwelling urethral catheter (IDUC). After 10 months of long-term catheter drainage he developed urethral fistula, which was subsequently excised. He is currently managed on CSIC and oxybutynin without further complications.

6.        A small noncompliant bladder was seen in a patient (T12 complete) who on initial VCMG had no loss of compliance. The loss of compliance seen subsequently was secondary to long-term suprapubic catheterisation (3 yr) and noncompliance with SPC clamping routine. The patient required ileocystoplasty and Mitrofanoff procedure for good capacity and low-pressure storage.

7.        During the follow-up, a total of 16 urological interventions were performed. 12/16 (75%) were carried out in NDO + DSD group. 7/12 interventions (58%) in this group were for change of management. In acontractile detrusor group, 2 interventions changed management and 2 were for treatment of complications. Apart from occasional ‘firing off’, continence and renal function was well maintained in all.

(Tanaka et al., 2006)

USA

Observational

N=22

Population: Pediatric patients with transverse myelitis: Age at injury: 8.8 yr (3 mo-18yr); Gender: males=15, females=7; Time since injury: 7.1 yr (6 mo-22 yr); Level of injury: cervical=7, thoracic=9, lumbar=6; Severity of injury: complete=9, incomplete=13.

Intervention: None. Review.

Outcome Measures: American Spinal Injury Association Impairment Scale (AIS), urinary and bowel continence, urodynamic studies, augmentation cystoplasty, upper tract imaging studies, CIC and anticholinergics.

1.         At last follow-up, 19 patients (86%) had persistent bladder dysfunction. Of these patients, 16 (73%) used CIC and 14 (64%) took anticholinergic medications. 3 patients continued to have urinary incontinence despite CIC and anticholinergics. 1 patient with urinary incontinence refractory to medical therapy was successfully treated with detrusor botulinum toxin injection.

2.        All patients with complete injury remained on CIC. When evaluated continence status, they did not significantly correlate with neurological level of lesion (p=0.58).

3.        17 patients (77%) had persistent bowel dysfunction. Of these patients, 15 (68%) followed a daily bowel program, including stool softeners, digital stimulation, suppositories and/or enemas. Of the 7 patients not on a bowel program, only 5 had complete bowel control. Fecal incontinence refractory to medical management resolved with the Malone antegrade continence enema procedure in 1 case.

4.        Urodynamics were performed in 17 patients. Detrusor overactivity was present in 10 of these patients (59%), DSD in 7 (41%), low compliance in 8 (47%) and detrusor leak point DLPP >40 cm water in 2 (12%). Detrusor overactivity, DSD, low compliance and increased DLPP did not significantly correlate with lower extremity tone (p=0.48, p=0.16, p=0.37 and p=0.40, respectively) or ambulatory status (p=0.58, p=0.58, p=0.60 and p=0.26, respectively).

5.        No patient with complete injury had increased DLPP. When patients with complete injury were evaluated the presence of detrusor overactivity, DSD and low compliance did not significantly correlate with the neurological level of the lesion (p=0.58, p=0.58 and p=0.58, respectively).

6.        Augmentation cystoplasty was performed in 4 patients for worsening compliance, incontinence and upper tract changes. 1 male patient had placement of an artificial urinary sphincter during augmentation for a Valsalva leak point pressure of <40 cm water, 1 patient underwent an antegrade continence enema for stool incontinence refractory to medical therapy, and 1 patient with incontinence and detrusor overactivity refractory to anticholinergics underwent endoscopic detrusor botulinum toxin injection.

7.        Of the 19 patients with upper tract imaging studies, 5 (26%) had upper tract changes, consisting of hydronephrosis in 3, reflux in 1, and hydronephrosis and reflux in 1. Chronic renal insufficiency developed in 1 patient during the study period. The patient presented for rehabilitation 2.7 yr after disease onset with a complete thoracic level lesion and no increased lower extremity tone. Initial imaging studies showed hydronephrosis and vesicoureteral reflux; initial urodynamics revealed low compliance. Although CIC and anticholinergics were started, detrusor compliance continued to decrease. The patient ultimately underwent augmentation cystoplasty and bilateral ureteral reimplantation.

8.        5/6 patients in the early CIC group and all of those in the delayed group underwent urodynamic testing. Low compliance was found in 7 of 10 patients in the delayed CIC group and no patient in the early group. Bladder compliance was significantly worse in the delayed CIC group (p=0.02).

9.        None of the 6 patients in the early CIC group had development of upper tract changes. Conversely, 4/10 patients in the delayed group had development of upper tract changes. Renal deterioration correlates with time elapsed between disease onset and institution of CIC (p=0.1, 90% confidence limit). However, standard statistical significance (p≤0.05, 95% confidence limit) was not reached by Fisher’s exact test.

(Johnston et al., 2005)

USA

Post Test

N=3

Population: Age: 17-21 yr; Gender: males=3; Time since injury: 1.0-1.5 yr; Level and Severity of injury: Motor complete T3-T8.

Intervention: Praxis system consists of a 22-channel implant stimulator, extension leads and epineural electrodes. Leads emanating from the stimulator are configured in three tresses: two tresses of nine leads each

for stimulation of lower extremity muscles and one tress of four leads for stimulation for bladder and bowel function (parameters: 0.2–8 mA amplitude, 25–600 ms pulse

duration, 2–500 Hz pulse frequency per channel). After implantation and immobilization participants completed exercise phase (FES strengthening) followed by lower extremity conditioning, standing and upright mobility training (13 wk).

Outcome Measures: Completion of eight upright mobility activities, scored based on completion time and level of independence: donning, stand and reach, high transfer, bathroom, floor to stand, 6m walk, stair ascent, stair descent.

1.         Just one subject demonstrated positive neuromodulation effects of the bladder; stimulation suppressed reflex bladder contractions acutely thereby reducing vesical pressure.

(Generao et al., 2004)

USA

Observational

N=42

Population: Age at injury: 5.3 yr (1 day-14 yr), Age at follow-up: 5.5 (1-15.5) yr; Gender: males=19, females=23; Level of injury: cervical=10, thoracic=26, lumbar=6.

Intervention: None. Retrospective review.

Outcome Measures: Bladder management, infection history, anticholinergic and antibiotic usage, continence, renal ultrasounds, video-urodynamics.

1.         Bladder management included clean intermittent catheterization (CIC) in 40 of 42 patients and antispasmodics in 37.

2.        No patient had reflux, hydronephrosis or renal scarring.

3.        In patients with cervical injuries, safe bladder capacity was less than the expected capacity in 80% but all patients undergoing multiple urodynamics had increasing capacity with time.

4.        In patients with thoracic injuries, 58% had a safe bladder capacity less than expected and 76% of those undergoing multiple urodynamics had increasing capacity.

5.        In patients with lumbar injuries, 50% had a safe bladder capacity less than expected and 67% of those undergoing multiple urodynamics had increasing capacity.

(Vogel et al., 2002b)

USA

Observational

N=216

Population: Age at interview: 28.6±3.4 yr; Age at injury: 14.1±4.0 yr; Gender: males=150, females=66; Time since injury: 14.2±4.6 yr; Level of injury: tetraplegia=123, paraplegia=93. Severity of injury: C1-4 ABC=41, C5-8 ABC=67, T1-S5 ABC=82, tetra/para D=26.

Intervention: None. Survey.

Outcome Measures: Prevalence of urinary tract infections (UTI), hospitalizations, urinary stones, orchitis or epididymitis, bladder incontinence,

dysreflexia.

1.         Most (160/216) of the study subjects experienced at least 1 UTI in the year before their interview.

2.        Among those who had a UTI, the mean number of infections per year was 3.9, and 41 (26%) of these individuals experienced UTIs that required intravenous antibiotics or hospitalization (severe UTI).

3.        Compared with those who did not have UTI, individuals who experienced UTI had significantly lower ASIA motor scores (p<0.001) and were less likely to have AIS scores of D (p<0.001).

4.        Both the ASIA Motor score (p=0.003), ASIA Impairment Scale scores of D (p=0.009), and both the total (P = .013) and motor (P = .017) FIM scores were significantly lower among individuals who experienced severe UTI.

5.        Urinary stones affected 25% of the subjects.

6.        Compared with those without stones, those with stones had significantly more severe neurologic deficits, with a lower mean ASIA Motor score (p=0.007), and lower total (p=0.001) and motor (p<0.001) FIM scores.

7.        Frequency of urinary incontinence greater than monthly was reported by 25% of the subjects; there were no significant associations between bladder incontinence and the demographic, impairment, or functional limitation variables.

8.        Urinary incontinence was not more common in those who experienced UTI (or severe UTI) compared with those who did not have UTI (or severe UTI).

9.        Of the male subjects, 15 (10%) reported having orchitis or epididymitis; those who had experienced either orchitis or epididymitis were older at follow-up (p=0.018), had been injured longer (p=0.04), and more likely to have had severe UTI (p=0.049).

10.      Compared with those with normal bladder function, UTI (but not severe UTI) was more commonly experienced by subjects who performed intermittent catheterization, had indwelling catheters, or who used external collection devices (p<0.001).

11.       Urinary stones were more common in individuals with indwelling catheters compared with those with normal bladder function, and those who were performing intermittent catheterization or using external collection devices (p=0.004).

12.      Bladder incontinence was statistically associated with bladder management, with incontinence most common in those with external collection devices and least common among those with normal bladder control (p=0.050).

13.      There was no significant association between the type of bladder management program and the development of orchitis or epididymitis.

(Anderson et al., 1997)

USA

Observational

N=37

Population: Females with Pediatric-onset SCI: Injured Before Menarche (n=22): Age at injury: 5.8 (0-13 yr); Age at interview: 16.4 (12-25) yr; Level of injury: tetraplegia=3, paraplegia=10; Injured After Menarche (n=15): Age at injury: 14.6 (12-16) yr; Age at interview: 17.6 (16-22) yr; Level of injury: tetraplegia=9, paraplegia=6.

Intervention: None. Survey.

Outcome Measures: Menstrual onset, regularity, frequency, duration, dysmenorrhea, dysreflexia, menstrual flow management and age of menarche of the patients’ mothers.

Injury Before Menarche Group

1.         Mean age of menarche was 12.3 (10-15) yr which was not significantly different from their mother’s age of menarche or the age of menarche of females in this study who were injured after menarche.

2.        The average age of menarche for those injured prior to menarche is comparable to the standard for North American females (12.5 yr).

3.        There was no significant association between age of menarche and age at injury or level of injury.

4.        Characteristics of menstruation included regular cycles, within a normal range of 20-36 days, for 14 of the 22; remaining eight showed a variety of irregularities in their cycles.

5.        Six subjects occasionally miss a month of their cycle or their periods come one to two weeks early or late; for example, one has a sixty-day cycle and another started menstruating immediately after her injury (age 11 yr) but then stopped until she turned 14 yr.

6.        Seven reported having minor dysmenorrhea.

7.        None experienced dysreflexia during their menstrual cycles.

 

Injury After Menarche

8.        Of the 15 females who were injured after menarche, seven reported no interruption in menses while eight had an interruption ranging from one to seven months.

9.        There was no association between length of time until resumption of menstruation and level of injury.

10.      Comparing the characteristics of menstruation pre- and post-injury, 12 females reported no changes in regularity or flow and three reported minor changes including one who became more regular and two less regular after injury.

11.       Six reported changes in dysmenorrhea, including two who had less discomfort and four who had more discomfort; in all cases, dysmenorrhea was mild and none reported dysreflexia or any other complications.

 

Menstrual Flow Management

12.      Of the 36 with SCI who were menstruating at the time of interview, 16 used pads, 11 used tampons, 7 used both; 2 used diapers for menstrual flow management.

13.      Most women reported no medical problems with either pads or tampons, although one person who had initially used pads switched to tampons because she felt she experienced more urinary tract infections with pads.

14.      Another individual switched from tampons to pads because she felt that reduced urinary tract infections.

15.      Five females who had used tampons prior to injury stopped using them after injury because they found them inconvenient; four of those five were tetraplegic.

16.      Five other females with tetraplegia use tampons by choice.

(Fanciullacci et al., 1988)

Italy

Observational

N=18

Population: Age at injury: 5.7 yr (n=18), Age at follow-up: 7.7 yr (n=14); Gender: males=16, females=2; Level of injury: cervical=2, thoracic=8, lumbar=8; Severity of injury: complete=13, incomplete=5.

Intervention: None. Review.

Outcome Measures: Urodynamic studies (UDS), intermittent catheterisation (IC), upper urinary tract (UUT) condition, urinary tract infection (UTI) rate, detrusor/sphincter balance.

 

1.         Generally, the bladder recovered its emptying quite easily.

2.        Spinal shock was shorter in those with upper motor neuron lesions compared to the 4-6 wk period seen in adults.

3.        Urological data at the initial evaluation following spinal shock (2 mo-1.5 yr after trauma) showed that UUT was normal in 13 patients, slight dilation in 2, vesico-ureteral reflux in 2 (ureteral stone in 1), and renal stone in 1 patient. UTI was present in 15/18 (83%) patients.

4.        UDS showed that 12 had hyperreflexia. Of the 12 patients, 6 had bladder/sphincter dyssynergia. 3 had areflexia with denervation of the perineal floor. 3 were not evaluated.

5.        2 children with stones were operated on. One of the 2 patients with reflux later developed a urethrocele and vesical stones (had an indwelling catheter for 1 year). Incontinence was not diagnosed until the child was 5 yr old. Continence was partial in all cases. Only 2 children were given oxybutynin + IC, in 1 case for initial ureteroidronephrosis and in the other to try to obtain good continence.

6.        Follow-up of the conservative management of the neuropathic bladder was carried out in 14 children. UUT was normal in 8/11 patients. Slight dilation in 1, and vesico-urethral reflux in 2 with normal IVP. UTI was present in 7/14 patients (50%). 12 patients had a residue of <200 0 of the bladder capacity (balanced bladder).

7.        2 children who had reflux, have a UUT in good conditions, as shown by IVP. Of the 3 children where the condition of their UUT was not checked, 2 are free of UTI, 1 has asymptomatic bacteriuria and 2 have no residue.

8.        The UUT of the boy managed by IC + oxybutynin, because of initial ureteroidronephrosis, improved.

9.        For all the children, the control of urinary continence is a problem.

10.      UTI was generally asymptomatic except for 2 children.

(Burke, 1974)

US

Observational

N=29

Population: Injury at birth (N=5): Gender: males=2, females=3; Level of injury: cervical=4, thoracic=1; Severity of injury: complete=3, incomplete=2.

Injury post birth (N=24): Gender: males=11, females=13; Level of injury: cervical=9, thoracic=14, lumbar=1; Severity of injury: complete=22, incomplete=2.

Intervention: None. Chart review.

Outcome Measures: Prevalence of scoliosis, urodynamic management.

1.         No patients have died of renal complications, and none had a raised serum creatinine, but 6 patients had serious urological problems which could reduce their life expectancy.

2.        In the group with a survival period of 0-5 yr (N=14), 1 patient died after a few months, another had bilateral vesico-ureteric reflux, but a normal pyelogram after 4.5 yr; one patient had a Leadbetters operation for left-sided vesico-ureteric reflux.

3.        In the group with a survival period of 5-10 yr (N=6), 1 patient had multiple renal and bladder stones, but a normal pyelogram and normal blood chemistry 9 yr after injury; 1 had an early left nephrectomy for hydro-nephrosis, was drained by a suprapubic catheter for some yr, and had a right nephrostomy 9 yr after injury.

4.        In the group with a survival period of 10-15 yr (N=3), all patients had renal damage, infected urine, 1 patient had a suprapubic catheter and 1 had bilateral cutaneous ureterostomies.

5.        In the group with a survival period of more than 15 yr (N=3), 2 had poor function of one kidney, 1 had a U tube draining both suprapubically and through a peno-scrotal fistula.

6.        None of the 6 longest survivors had any elevation of blood urea nitrogen or serum creatinine.

Author, Year

Country

Study Design

Population

Intervention

Outcome Measure

(Abbo et al., 2013)

France

Case Report

N=2

Population: Case I: 23 mo, male, C7-T1 SCI; Case II: 18 mo, female, T6 SCI.

Intervention: Clean Intermittent Catheterization.

Outcome Measures: Detrusor hyperactivity, detrusor-sphincter dyssynergia, urinary retention.

(Tuite et al., 2013)

USA

Case Report

N=1

Population: 10 yr, male, T10-11 SCI.

Intervention: Xiao procedure.

Outcome Measures: Bladder and bowel function, electrophysiological and histological evaluation.

Discussion

Given the low incidence of pediatric SCI/D (Blaivas 1982), it is quite expected that we only found seven observational studies, one post-test, and two case reports looking at individuals with pediatric-onset SCI. Due to the heterogeneity of the presentation of the findings and the small sample size of most studies identified from the literature, conclusions regarding the prevalence of bladder dysfunctions in children with SCI/D cannot be drawn. In a larger-scale study involving 216 adults with pediatric-onset SCI, the majority (74%) of participants reported experiencing at least one UTI in the past year (Vogel et al. 2002b). Etiology-wise, most study participants suffered motor vehicle accidents, either being in the vehicle during accident or as pedestrians struck by cars; some studies included participants with birth injuries (Burke 1974; Chao & Mayo 1994; Fanciullacci et al. 1988; Generao et al. 2004), domestic accidents (Fanciullacci et al. 1988), gunshot wounds (Chao & Mayo 1994; Generao et al. 2004), iatrogenic causes (Fanciullacci et al. 1988; Generao et al. 2004), transverse myelitis (Chao & Mayo 1994; Tanaka et al. 2006).

Population-wise, the papers looked at individuals who sustained SCI between the ages of 0 and 18 years (Generao et al. 2004). The mean time post-injury was 46 months (Chao & Mayo 1994) to 28 years (Patki et al. 2006). Topics of examination consisted of bladder management method (Burke 1974; Chao & Mayo 1994; Fanciullacci et al. 1988; Generao et al. 2004; Patki et al. 2006; Tanaka et al. 2006), and continence (Fanciullacci et al. 1988; Patki et al. 2006; Tanaka et al. 2006; Vogel et al. 2002b), urodynamic studies (UDS; with or without video and cystography) (Chao & Mayo 1994; Fanciullacci et al. 1988; Generao et al. 2004; Patki et al. 2006; Tanaka et al. 2006), upper tract health, as established by renal ultrasound (Chao & Mayo 1994; Generao et al. 2004; Patki et al. 2006; Tanaka et al. 2006) and/or intravenous pyelogram (Burke 1974; Chao & Mayo 1994; Fanciullacci et al. 1988), medical complications like infections (Burke 1974; Fanciullacci et al. 1988; Patki et al. 2006; Vogel et al. 2002b), renal insufficiency (Burke 1974; Patki et al. 2006; Tanaka et al. 2006), lithiasis (Burke 1974; Vogel et al. 2002b), and need for subsequent surgical interventions, like sphincterotomies/stent placement, urinary diversions and bladder augmentation, nephrectomies, etc.

Surprisingly, the severity and level of neurologic injury were not necessarily predictive of urodynamic findings. Detrusor pressures and activity did explain changes in bladder capacity, compliance, and cystometric appearance at times of follow-up (Patki et al. 2006)

Burke et al. (1974) looked at 29 children who sustained C1 to below T12 SCI under the age of 13 years; 26 of them had urologic follow-up. Among the 20 children who were less than 10 years after injury, 17 had normal upper tracts, 15 were using reflex voiding, 10 were having infected urine, and 1 already had a nephrostomy in the single residual kidney he had. In the 6 individuals that suffered the neurologic injury more than 10 years prior to data collection, only 1 had intravenous pyelogram (which was normal), 1 emptied his bladder by reflexive void, 4 had indwelling catheters (urethral or suprapubic) and 1 had bilateral cutaneous ureterostomy.

Fanciullaci et al. (1988) reported on 18 children with SCI seen 1972-1985 in 1 unit in Italy. Participants were first assessed 2-18 months post-injury, and follow-up assessment was done in 14 children 7.7 years post-injury on average, to evaluate results of the conservative management of the neurogenic bladder. Authors noted that the initial indwelling catheter was removed within 4 months in 14 of the participants. In the initial urologic follow-up, the upper urinary tract was normal in 13 patients; slight dilatation and vesicoureteral reflux was noted in 2 each, and renal stone in 1 patient; UTI was deemed present in 15/18 patients, but 13 of the 15 were noted to be asymptomatic bacteriuria. 15/18 underwent UDS which showed over-active detrusor in 12 children. In the 14 children that were evaluated 2-16 years post-injury, the upper urinary tract was deemed normal in 8/11 patients; 1 child was found to have a slight dilatation of the upper tract; vesicoureteral reflux was found in 2 children (with normal intravenous pyelogram) and UTI was present in 7/14 patients, with 5/7 noted to be asymptomatic bacteriuria.

Chao et al. (1994) looked at 40 children with SCI receiving urological care; Initial bladder management was done by reflex voiding (with acceptable voiding pressures <40 cm H2O) in 11 of the participants; the rest of 29 emptied their bladder by intermittent catheterization, utilizing anticholinergic drugs to address higher than 40 cm H2O voiding pressures, presence of detrusor-sphincter dyssynergia and/or having moderate to severe bladder trabeculations. First UDS showed that all 22 patients with cervical injury had over-active detrusor; 8 were reflexively voiding and 14 emptied their bladder by intermittent catheterization; in the thoracic injured category, 11 patients had over-active detrusor and two were areflexic; among them, 2 were reflexively voiding and 11 were using intermittent catheterization; in the 5 individuals with lumbar spine injury, bladder management was by reflexive void in 2 and intermittent catheterization in 3 and UDS showed that all of them had areflexic bladders. 28/40 participants were followed for more than 1 year undergoing bladder management assessment and video UDS; 11/19 patients with cervical injury, 5/6 with thoracic injury and 1/3 with lumbar injury were emptying the bladder using intermittent catheterization/anticholinergic drugs at this follow up; video fluoroscopy during UDS showed the bladder wall to be smooth in 43%, mildly trabeculated in 50% of participants, and markedly trabeculated in 7%. Five patients had a change from a smooth bladder wall to mild trabeculation (four cervical injuries and one thoracic injury). Four patients had a change from a trabeculated bladder wall to a smooth wall (three cervical injuries and one thoracic injury) at six months follow-up after institution of intracranial pressure and anticholinergic therapy. One patient had a grade 2/5 vesicoureteral reflux, which resolved on intermittent catheterization/anticholinergics. The authors noted that neurogenic management treatment failure was related to non-compliance with catheterization or pharmacologic regimen.

Unlike the studies discussed previously, Vogel and colleagues (2002b) work assessed the consequences of pediatric SCI/D-related neurogenic bladder by interviewing 216 adults with pediatric-onset SCI using a structured questionnaire. Mean number of UTI’s/year was reported to be 3.9 and occurred in 74% of individuals; those experiencing more UTIs had lower International Standards for Neurological Classification of Spinal Cord Injury motor scores and were less likely to have AIS D grade injury. In addition, a history of severe UTIs and urinary stones were associated with lower motor scores, higher injury level, and Functional Independence Measure scores. Those who reported having orchitis and epididymitis tended to be older, injured for a longer period of time, and reported having had severe UTIs. Bladder incontinence was reported by 25% of participants, more common in those using external catheters; UTIs were more common in individuals managing bladder with all types of catheters. Severe UTIs, orchitis, and epididymitis were not associated with any specific type of bladder program, but stones were more common in individuals using indwelling catheters.

Generao et al. (2004) performed a retrospective review of 42 pediatric patients who had been living with SCD-related paralysis for a minimum of 1 year. Assessed outcomes were bladder management, continence, medication, UTI occurrence, upper urinary tract health (US), and video-UDS in the context of safe bladder capacity (defined as pressure-specific volume of 40 cm water or less. 40/42 children were managing the bladder using intermittent catheterization and 37 were taking anticholinergic medications. Upper tract exploration showed no vesicoureteral reflux, hydronephrosis, or renal parenchymal scarring.  Bladder capacity was less than expected in 80% of cervical injuries (but increasing with time); it was less than expected in 58% of thoracic injuries (and only 76% were found to progress as expected with time) and was less than expected in 50% of lumbar injuries. Early bladder management with intermittent catheterization and anticholinergic drug usage was considered to prevent hydronephrosis, scarring, reflux, and bladder trabeculation, improve continence and decrease rates of infection. In addition, thoracic injuries were found to have the most variable bladder pattern.

Patki et al. (2006) conducted a retrospective review of urological follow-up in 10 adults with pediatric-onset SC  The group was divided into Neurogenic Detrusor Overactivity with or without Detrusor Sphincter Dyssynergia and a-contractile detrusor with or without low bladder compliance, based on initial UDS studies. It was found that patients with Neurogenic Detrusor Overactivity and Detrusor Sphincter Dyssynergia were at higher risk of developing complications. In addition, those who opt for reflex voiding and SPC were more likely to experience multiple problems requiring hospitalizations and interventions. The authors also noted that over time, most participants required changes to optimize urological management.

Finally, Tanaka et al. (2006) retrospectively examined the long-term urological outcomes in 22 children affected by transverse myelitis, as well as the correlation between bladder dysfunction and lower limbs spasticity and ambulatory status in addition to neurologic level of injury. Lower limb spasticity was documented in 14/22 (61%) and, while none of the children were ambulatory immediately post-onset of neurologic deficit, 9/22 (41%) became ambulatory during the follow-up period. At follow-up, 86% of children reported persistent bladder dysfunction. Bladder management was done by intermittent catheterization in 73% and 64% was using anticholinergic medications. Three were still incontinent despite adequate bladder management and 1 had the incontinence resolved by undergoing intra-detrusor onabotulinumtoxin type A injections. 6/22 were able to void volitionally, 2 of them having incomplete cervical injuries and maintaining continence (one using anticholinergic medication) and 4 of them having incomplete lumbar injuries, with only 2/4 maintaining continence.  All patients with complete injury remained on intermittent catheterization. UDS were completed in 17 at follow-up, an average of 2.2 years after disease onset; detrusor over-activity was present in 10 patients (59%), Detrusor Sphincter Dyssynergia in 7 (41%), low compliance in 8 (47%) and detrusor leak point pressure greater than 40 cm water in 2 (12%). Upper tract was evaluated in 19/22 individuals using renal ultrasound and cystography for vesicoureteral reflux; 26% had upper tract changes (hydronephrosis in 3, reflux in 1, and hydronephrosis and reflux in 1). Chronic renal insufficiency developed in only 1 individual that did not have adequate urologic care for the initial 2.7 years post-injury, developed hydronephrosis and vesicoureteral reflux despite having a complete thoracic injury with no lower limb spasticity; the bladder was found to have low compliance and, despite treatment with intermittent catheterization and anticholinergics, patient needed to undergo augmentation cystoplasty and bilateral ureteral reimplantation to address the complications. 6 individuals that subsequently underwent upper tract and UDS evaluation started intermittent catheterization in the first 9 months post-injury, and another 10 in the first 2.3 years post-injury and were considered early intermittent catheterization users; none of the early catheterization users developed low bladder compliance of upper pathologic tract changes, as opposed to 7 of 10 patients in the delayed catheterization group exhibiting low bladder compliance and 4 of 10  having upper tract changes at follow up.

Conclusion

Major shortcomings of the literature pertaining to management and consequences of neurogenic upper and lower urinary tract dysfunction in children with SCI/D-related paralysis is the retrospective, observational case studies reporting type. That is because high-quality evidence regarding management and follow-up does not exist in the literature (Averbeck & Madersbacher, 2015). There is no standardization of intervention or even follow-up. The approach is not scientifically rigorous but clinically driven and there are no controls. Sometimes, even the definition of terms is variable (e.g., bladder compliance).

With that said, given the fact that pediatric SCI/D is extremely rare, learning from robust case series is the first step toward building knowledge. The reviewed papers consistently point to the lack of predictive value of neurological injury category on bladder function and management. To that point, it does appear that thoracic injuries are less likely to be predictable when it comes to the type and subsequent management of neurologic bladder dysfunction. The ideal urological treatment needs to be individualized and tailored to suit a specific patient’s medical and social needs and both needs are changing as the child grows. Changes in bladder management required at later stage in injury and variation of renal deterioration with different bladder managements make a strong case for regular and long-term follow-up of the pediatric SCI population. Ongoing long-term comprehensive assessment utilizing imaging (renal ultrasound and scan, computed tomography scan, cystometrogram, cystoscopy) and functional (video-urodynamics, creatinine clearance, glomerular filtration rate, cystatin C, radio-isotope studies etc.) tools to assess both upper and lower tracts is strongly recommended for management guidance in the ever-growing and evolving genitourinary system. Preservation of renal function is the most important objective of any long-term treatment plan in children with SCI/D-related neurogenic bladder dysfunction. Early assessment of the bladder function in this population and ongoing treatment compliance monitoring appears to play a major role in preventing long-term medical complications. Asymptomatic bacteriuria and recurrent infections are frequent, especially in the instrumented bladder and prophylactic antibiotic usage is not well supported. There is a great need for developing prospective, controlled studies looking at effect of specific interventions on renal function preservation and prevention of bladder pathologic changes.

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