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Scandinavian Journal of Surgery 100: 256–263, 2011

Neurogenic bladder in children: basic principles,


new therapeutic trends

J. M. Guys, G. Hery, M. Haddad, C. Borrionne


Pole Medico Chirurgical Pédiatrique, Hôpital d’enfants de la Timone, Marseille, France

ABSTRACT

Diagnosis of neurogenic bladder is straightforward in children with myelomeningocele.


However, recognition is more difficult in patients with occult dysraphism or central ner-
vous system disorders since clinico-anatomical correlations are poor. Careful clinical
examination and urodynamic exploration are mandatory for diagnosis and follow-up.
Even if urinary leak is the first symptom, the main goal of the pediatric surgeon must
be to preserve the upper urinary tract. The ideal protection strategy consists of ensuring
that micturition is voluntary and complete and that the bladder capacity is sufficient with
adequate compliance and sphincter outlet resistances. Balancing these functions requires
a combination of medical and surgical treatment.
A variety of techniques can be used depending on gender and age of the patient and
social environment. In most cases, intermittent bladder catheterization is necessary to
obtain complete evacuation of the bladder. Bladder capacity can be increased by anti-
cholinergic drugs, injection of botulinum toxin into the bladder, and augmentation cys-
toplasty. Augmentation of bladder outlet resistances requires endoscopic injection of
bulking agents, surgical bladder neck reconstruction and urethral lengthening, bladder
neck suspension, and artificial urinary sphincter. In difficult cases, continent cystostomy
with closure of the bladder neck can achieve definitive continence.
At the beginning endoscopic treatment combining anti reflux procedure, injection of
the bladder neck and botulinum toxin can be considered as a “total endoscopic manage-
ment” and should be our first line. Other techniques are under evaluation. Sacral neuro-
modulation has given promising results. Artificial tissue engineering will probably be
used in the next future.
Management of neurogenic bladder is not limited to urological considerations. Ortho-
pedic, digestive, and sexual problems must also be taken into account in order to obtain
an “acceptable quality of life”.
Key words: Urinary incontinence; pediatric neurogenic bladder; spinal dysraphism; bladder sphincter
dyssynergy; total endoscopic management; sacral neuromodulation

Correspondence:
Jean Michel Guys, M.D.
Service de Chirurgie Pédiatrique
Hôpital d’Enfants de la Timone
13 385 Marseille Cedex 5
France
Email: jean-michel.guys@ ap-hm.fr
Neurogenic bladder, new trends 257

INTRODUCTION ing from ectopic ureter to exstrophy as well as after


treatment for Hirschsprung disease, upper anorectal
To ensure adequate treatment of neurogenic bladder malformation, sacral coccygeal teratoma, etc. Tran-
in children, management must be proactive rather sient or permanent neurogenic bladder can also be
than reactive. Decision-making must take into ac- observed in association with myelitis and radicu-
count prognostic factors anticipating the consistently loneuritis. Neonatal cerebral anoxia and central ner-
unfavorable natural course of neurogenic bladder. vous system abnormalities including spastic diplegia
The purpose of this article is to show the importance (cerebral palsy) and learning disabilities, severe infec-
of the circumstances surrounding diagnosis and/or tious syndrome (meningitis) can be observed (7).
paraclinical findings in determining the functional
urologic grade of disease as a basis for selecting the­ Diagnostic features
rapeutic modalities. The last part of this article pres-
ents a quick update of current therapeutic indications Neurogenic bladder can also be diagnosed in cases
that have advanced in recent years. involving a more or less long history of micturitional
disturbances considered as benign and treated medi-
cally and even surgically. In most of the cases, the
possibility of a neurological background is raised
Etiologies and diagnostic features
only after repeated therapeutic failure.
Congenital causes Neurogenic bladder may be suspected during di-
agnostic work-up for other symptoms associated or
Myelomeningocele (spina bifida) not with micturitional disturbances, e.g., severe con-
stipation or psychological disorders with encopresia.
For a long time, myelomeningocele, was the main In this setting, it should be pointed out that Hinman
etiology of neurogenic bladder in children (1,2,3). syndrome involves bladder dysfunction that closely
With the advent of prenatal diagnosis, the frequency mimics organic neurogenic bladder even if no organic
of this cause has dropped since, in many countries defect can be found (8). The psychological context of
including France, this in utero finding often leads to these children is special and abuse can be identified
termination of pregnancy. In a recent randomized as a possible cause in numerous cases. Only after
trial of prenatal versus postnatal repair of myelom- completing an exhaustive work-up, it will be possible
eningocele, Adzick et al reported that prenatal repair to speak of «non-neurogenic elimination disorders »
reduced the need for shunting and improved motor that have been indifferently designated under a num-
outcome at 30 months but was associated with a non ber of names including “neurogenic non-neurogenic
negligible maternal and fetal risks (4). bladder”, “occult neurogenic bladder”, “lazy blad-
This finding prompts the question: do the potential der”. All of these names clearly reflect the paucity of
benefits of prenatal coverage of hindbrain herniation, our current knowledge in this domain.
shunt-dependent hydrocephalus, and leg function
justify the associated risks for mother and child (5)?
Pathophysiology and work up
Occult spinal dysraphism
Natural course
This malformation is currently the most common
cause of neurogenic bladder (1). It can be suspected Regardless of etiology of neurogenic bladder, the
based on the presence of characteristic lumbosacral natural course almost always involves altered storage
cutaneous stigmata and confirmed by spinal ultra- function and vesicosphincteric dyssynergia. Overac-
sound in the first two months of life. In many cases, tivity of the detrusor muscle often due to loss of in-
diagnosis is made later upon presentation of mictu- hibition from the pontine and suprapontine centers
ritional disturbances and motor or sensory deficits leads to high intravesicular pressure. As a result, the
involving the lower extremities and perineal region. striated ureteral sphincter exhibits spasticity (1,6).
These manifestations frequently occur around the These changes explain that neurogenic bladder pre-
time of puberty when growth causes increased trac- senting mainly as urinary incontinence actually cor-
tion on the spinal cord. Dysraphism includes tethered responds to an obstructive disorder of the lower tract
spinal cord associated or not with imperforate anus, due to high-grade vesicosphrincteric dyssynergia.
cloacal malformation, diastematomyelia, and sacral
hypoplasia or agenesis (3). Strategy

Acquired neurogenic bladder In this setting, the use of the conventional neurologi-
cal categories, i.e., central, peripheral or mixed has
In addition to being less common than in adults, spi- little bearing on therapeutic decision-making. Strat-
nal cord injuries in children are intrinsically different egy must simply be aimed at achieving ideal micturi-
owing to a number of factors including mechanism tion characterized by voluntary control, low pressure,
of injury. Tumor-related spinal compression is not and complete voiding. Accordingly, assessment must
uncommon in children. Irreversible changes in blad- focus on the three main bladder functions, i.e., reten-
der enervation can occur as complication after pelvic tion, storage and discharge. Capacity must be suffi-
surgery in patients with urologic malformations rang- cient for low-pressure retention and storage with
So : no sphincterian resistances
S+- : variable sphincter
V- : “big” bladder
258 J. M. Guys, G. Hery, M. Haddad, C. Borrionne

Abbreviations: tients with congenital neurogenic bladder. Functional


R : kidney exploration (uro-MRI and isotopic examination) be-
V : “normal” bladder (capacity) comes mandatory if pyelonephritic renal scaring is
V 0 : “small” bladder suspected.
S 0 : no sphincterian resistances
S+ – : variable sphincter
V– : “big” bladder Bladder exploration
The essential examination for bladder exploration is
cystography coupled with urodynamic investigation.
This combination allows complete morphological,
functional, and dynamic assessment of the lower uri-
nary tract.
Cystography
Cystography is usually performed by retrograde
catherization. Catheter diameter must allow micturi-
tion. Bladder features are key findings for diagnosis
of neurogenic bladder: verticalization of the long axis,
thickening of the wall, diverticulas. Vesico-ureteral
reflux, even if only minor, can be evidence of high
bladder pressure. The bladder neck may open spon-
taneously with opacification of the posterior uretra,
since the external sphincter is often hypertonic (9). In
case of particularly low sphincter resistance, it may
be necessary to perform this examination using an
Fig. 1. Urinary incontinence: one symptom, numerous causes.
occlusive catheter at the level of the bladder neck.
Characteristic urodynamic data for diagnosis neu-
rogenic bladder include maximum detrusor pressure,
reflex volume, maximum cystometric capacity, leak
adequate sphincter resistances and emptying control. point pressure, and detrusor activity. Detrusor over-
In our view, functional urologic assessment (Wein) activity is defined as any short-lived pressure rise
provides the soundest basis for therapeutic planning > 15 cm H2O over baseline before capacity is reached
(1, 4). (10, 11).
Urinary incontinence can be caused by a variety of Dynamic electromyography of the striated ureteral
factors (Fig. 1) including: sphincter can be the only way to demonstrate vesico-
sphincterian dyssynergy. However, like study of
1. true sphincter-related incontinence (a) with a
evoked somesthesic sensory and motor potentials,
“normal” bladder (uncommon).
dynamic electromyography is not performed system-
2. undersize bladder resulting in limited retention
atically in children since these examinations are in-
time and need for frequent voiding that is incom-
teresting only if the neurological background is un-
patible with a normal social life (b).
certain (12).
3. oversize hypotonic bladder that empties by over-
flowing (c).
Risk factors and follow-up
Any of these bladder conditions can occur in combi-
nation with sphincter-related incontinence that can The essential goal of workup is to evaluate risk fac-
range from obstructive hypertonia to a total lack of tors for the upper tract deterioration and to follow-up
resistance. All these disorders lead to the same clini- the treatment. For this reason, leak point pressure is
cal finding, i.e., leakage. For this reason, a careful one of the main urodynamic prognostic factors. The
work-up is necessary to determine the exact underly- estimated critical threshold is at 40 cm H2O. Beyond
ing mechanism. this point, upper tract damage appears in 70% of
cases (11). A similar adverse relationship is observed
Work-up between bladder overactivity and pressure peaks
again reaching 40 cm H2O on a regular basis. While
Radiological and urodynamic examination must pro- there is not always a direct link between high bladder
vide a precise idea of the morphological and func- pressure and appearance of reflux, this finding should
tional condition of the upper tract and of lower tract be considered as a high-risk signal of imminent up-
function with regard to storage, discharge and syn- per tract deterioration (13).
ergy with sphincteric resistance (1). In summary, the most detrimental urodynamic 23 fac-
tor is elevated bladder pressure regardless of whether
Exploration of the upper urinary tract this occurs throughout the micturition cycle (“blad-
der hypertonia”), transiently (“bladder overactivity”),
In the early stages of disease, the upper urinary tract or even only in the pre-micturition phase. Early de-
can be studied by ultrasound since the kidneys are tection and treatment of this condition is mandatory.
generally unaffected. This is particularly true in pa- In association with elevated pressure, inadequate
Neurogenic bladder, new trends 259

emptying whether due to dyssynergy or to sphincter smooth muscle relaxants may be useful in some cases
hypertonicity aggravates the effect of the “high-pres- involving hypertonia but indications are limited due
sure syndrome”. Chronic retention of urine in the to adverse effects (cardiac) and poor clinical efficacy.
bladder is the most important clinical prognostic fac-
tor. Urinary incontinence is of limited prognostic Neurotomy and sphrincterotomy
value since it is just a “symptom” of an unbalanced
relationship between the different bladder func- Neurotomy and sphincterotomy have been used to
tions. permanently suppress peripheral resistance. How-
ever, this definitive solution has been largely aban-
doned due to severe side effects (impotence) and too
Treatment definitive incontinence.

In all cases, treatment of micturition disturbances as- Improving bladder capacity


sociated with neurogenic bladder must be guided by
the notion of protecting the upper urinary tract (1). Pharmacological treatment
In older children, management of incontinence is nec-
essary to ensure a normal social life. Continence de- Anticholinergic agents are most commonly used
pends on urinary storage, complete voiding, and suf- medication to improve bladder capacity. Oxybutinin
ficient sphincter resistances. Ideally the goal should chloride is currently the most active agent. Detrus-
be to achieve voluntary control of the micturition via itol® (tolteridine) seems to be best tolerated. Ceris®
the natural passages. Socially compatible continence (trospium chloride) is used in adult patients and ap-
is generally defined as the ability to hold urine for pears to have fewer side effects but has not been ap-
four hours without leakage. Management of neuro- proved for children. In theory more than in practice,
genic bladder should therefore focus on: diazepam is thought to have a relaxant effect on the
detrusor muscle and tonic effect on the sphincter. Im-
1. ensuring bladder emptying; pramine is hazardous at effective doses. Betamimetics
2. improving bladder capacity; (Isoprenaline) also have a relaxant activity on the de-
3. increasing sphincter resistances. trusor muscle but their use is greatly limited by un-
desirable systemic effects.
Ensuring bladder emptying
Botulinum toxin
Therapeutic modality
Botulinum toxin has been proposed for management
The simplest therapeutic modality use expression of neurological detrusor hyperactivity. The first de-
voiding methods such as the Crede and Valsalva ma- scription of this technique was published in 2000 by
neuvers. However Crede voiding is frequently inad- Schurch et al (16) who administered botulinum toxin
equate in older children because most have intact (Botox®, Allergan®) by endoscopic injection and ob-
motor function above L1 and any augmentation in served a significant increase in maximal bladder ca-
abdominal pressure can lead to a reflexive increase in pacity and a decrease in maximal detrusor pressure
urethral sphincter activity, thus producing an increase and number of incontinence episodes. Since 2002,
in bladder outlet resistance (3). prospective studies have confirmed the efficacy of
botulinum toxin in children with a neurogenic detru-
Intermittent catheterization sor overactivity (17–20) with continence rates ranging
from 60 to 83%.
Intermittent catheterization performed 4 to 5 times a In our center, patients undergoing intermittent
day is the easiest-to-use instrumental bladder man- catheterization for urinary incontinence associated
agement technique (14). Current devices have bene- with poorly compliant and overactive bladder that
fited from numerous technological improvements fails to respond to anticholinergic agents are included
(e.g. self-lubricating catheters and built-in drainage in a ongoing prospective trial after informed consent.
receptacle). By allowing the bladder to be emptied at The treatment protocol calls for injection of 12 U/kg
low pressure and removing residues that can be of Botox (maximum 300U) into the detrusor and 2 to
harmful for the upper tract, intermittent catheteriza- 3 cc of polydimethylsolixane into the bladder neck.
tion is the mainstay of management for neurogenic Response is evaluated on the basis of urinary diary
bladder. Catheterization allows complete voiding data and urodynamic testing at 2 and 6 months after
through the natural passages in a manner that can be the 1st injection and 4 months after subsequent injec-
considered as voluntary. There is no age limit for be- tions. Ten patients (7 boys and 3 girls) with a mean
ginning intermittent catheterization that is proposed age of 9.36 years (range, 5.6–16) have been included.
to young children by many groups (15). All but two presented spina bifida. The number of
injections was 1 in 3 patients, 2 in 5, and 3 in 2. From
Pharmacological treatment a clinical standpoint, 8 patients reported a decrease
in leak episodes and 2 reported no response. No pa-
Pharmacological treatment in order to facilitate blad- tient achieved continence even after 3 injections.
der emptying is not widely used in patients with neu- Clinical effects were observed at a mean interval of
rogenic bladder. Alpha-adrenergic blockers and 3.13 ± 3.98 days after injection and lasted for a mean
260 J. M. Guys, G. Hery, M. Haddad, C. Borrionne

duration of 2.87 ± 1.27 months. Follow-up at 2 and 6 Few series describing percutaneous tibial nerve
months showed increased bladder compliance stimulation in children have been published, but ex-
(p < 0.01). A positive effect (not statistically signifi- isting data suggest that improvement in continence
cant) was also observed on maximal bladder capacity was significantly greater for patients with non-neu-
and maximal detrusor pressure. The same results rogenic than neurogenic disease (30).
were noted after repeated injections.
Parasacral transcutaneous electrical nerve stimulation

Bladder autoaugmentation The effectiveness of parasacral transcutaneous electri-


cal nerve stimulation for treatment of overactive
If performed early, auto-augmentation that consists bladder in children was recently evaluated in a pro-
of anteroposterior section of the detrusor muscle fi- spective trial in which 25 girls and 12 boys were ran-
bers can be sufficient to increase bladder capacity. domized into a test and sham group. Thirteen of the
Some evidence indicates that instrumental bladder 16 patients who underwent parasacral transcutane-
dilation using an intravesicular balloon is necessary ous electrical nerve stimulation were cured. Again
to ensureat least short term success of this procedure none of these patients presented neurogenic bladder
(21). (31).
Direct stimulation of the S3 and S4 nerve roots
Bladder enlargement
Direct stimulation of the S3 and S4 nerve roots i.e.,
Bladder enlargement can be performed using various sacral neuromodulation (SNM: “Interstim therapy”)
materials including segments from the colon, ileum, is performed not only to reinforce the sphincter tone
stomach, or dilated ureter. The ileum is the most com- by stimulating contraction of all the muscles of the
monly used tissue reported in the literature (22). En- perineal floor but also to improve bladder compliance
largement is always achieved using the detubularized by reactivating certain efferent and afferent nerves
patch technique to avoid residual hyperactivity due (32). The efficacy of this treatment in children was
to intrinsic intestinal peristalsis. Results after removal assessed in a multicenter, open label, randomized,
of the digestive mucosa are controversial but some crossover study including children older than 5 years
investigators have reported excellent clinical out- in France. The positive response rate was more than
comes (23). As a prerequisite for bladder enlargement, 75% overall, 81% for urinary incontinence and (78%)
it is mandatory for the patient to be able to perform for bowel incontinence. Crossover analysis indicated
effective emptying of the new bladder (1, 3). In par- that SNM is more effective than conservative treat-
ticular, the patient must accept and understand the ment for both types of incontinence (p = 0.001) (33).
need for intermittent catheterization and his/her abil-
Various radiculotomy procedures
ity to use the device must be tested before undertak-
ing bladder enlargement. Disadvantages include mu- Various selective radiculotomy procedures (S2 or S3)
cus production, recurrent urinary infection, electro- have been used in adults. The goal of these proce-
lyte imbalance, stone formation and the risk for the dures is to suppress detrusor contraction and increase
late occurrence of cancer in the augmented segment bladder capacity. Continuous intraoperative monitor-
(25). In the future, it is likely that tissue culture will ing of the bladder pressure is necessary to ensure
provide a new source of material for bladder enlarge- precise selection of the targeted nerve roots. No in-
ment (26). formation is available about the long-term outcome
of these procedures.
Electrical stimulation
Increasing sphincter resistances
Electrical stimulation has been used in adults for
more than 25 years. Pharmacodynamic treatment
Transuretral electrical bladder stimulation Cholinergic and alpha-stimulating agents exhibit
Kaplan was the main proponent of the technique of weak action and have undesirable side effects.
transurethral electrical bladder stimulation in chil-
dren and reported a series of 372 patients with a mean Surgery of the bladder neck
age of 5.5 years and mean follow-up 6.6 years. In
76.9% of these patients, the increase in bladder capac- Surgery of the bladder neck such as anterior wedge
ity was 20% or greater (27). Despite these results, few reinforcement type Young Dees, bladder neck sus-
groups have adopted this technique. pension (rectus facial sling) (Goebbel-Stœckel) or
simple urethropexy (Marshall-Marchetti) are well
Stimulation of the posterior tibial nerve known and have been used for years (34). In a recent
Some investigators have reported statistically sig- 51-case Spanish study using the rectus fascial sling
nificant symptomatic improvement after percutane- technique, good continence was achieved in 88% of
ous tibial nerve stimulation in adult patients with patients with a mean follow-up of 4.16 years (35).
overactive bladder. Twelve weekly procedures Bladder neck reconstruction using the Kropp and
achieved some short term success results for 12 Pippi Salle techniques appeared to be promising. The
months (28). This technique is considered as safe and short-term success rate was 61% in girls but few long-
effective (29). term data have been reported (36).
Neurogenic bladder, new trends 261

Combined techniques, e.g. fascial wrap and ure- but rapid decompression. The only other indications
thral lengthening, appear to give the best results (37). for external urinary diversion are in extreme situa-
In one 19-case series using this approach, complete tions and usually for social reasons (1, 3).
continence was achieved in 15 patients with a mean
± SD follow-up of 35.5 ± 29.1 months (38). Therapeutic indications

Artificial urinary sphincter A succinct description of therapeutic strategies is dif-


ficult because incontinence can be compounded by a
The only model now used is the totally implantable host of other problems associated with the underly-
Scott device (AS800). In children, the cuff is placed at ing disease: digestive, sexual and orthopedic disor-
the level of the bladder neck. Short- and middle-term ders.
results are often good (80% continence) but technical
problems require a mean number of 3 re-operations Emptying of the bladder and protection of the upper
per patient (39). One review in the literature showed urinary tract
that long-term results of artificial urinary sphincter
placement were better and more reproducible in The first step is to ensure adequate bladder emptying
terms of continence, preservation of voluntary void- and to protect the upper urinary tract. In baby, the
ing including CIC (Clean Intermittent Catheteriza- Crede or Vasalva method or, in case of reflux in small
tion) and avoidance of bladder augmentation (34). children, intermittent catheterization can be pro-
Placement of an artificial sphincter does not rule out posed. In cases strictly limited to vesicosphincteric
intermittent catheterization (39). dyssynergy, biofeedback training can be tried in the
older patient. Surgical treatment of reflux more likely
involves a Cohen crossed trigonal ureteral reimplan-
Injection of bulking agents
tation more than the Leadbetter technique. Bilateral
Injection of bulking agents at the level of the bladder treatment of the reflux is performed in all cases. Sub-
neck increase passive resistance to urinary flow. The ureteral injections (Teflon, collagen, or silicone gel)
current popularity of injection (for the authors) is must be used as an alternative to surgery in young
based on the materials used and the minimal inva- children or as a bridge therapy if a bladder surgery
siveness of the technique that can be carried out en- is planned at a later time.
doscopically. The most common materials now in use
are Macroplastic® and Deflux®. Used alone or in com- Improving bladder capacity
bination with other techniques, these materials have The second phase consists of improving bladder ca-
a 30% success rate (40,41). pacity once complete bladder emptying has been en-
abled. If pharmacological treatment using anticholin-
Urinary diversion and continent cystotomy ergic agents fails, botulinum toxin followed in case of
failure by bladder enlargement is mandatory. In our
Urinary diversion and continent cystotomy is ulti- opinion, the preferred method is sigmoid plasty. We
mate procedure for reinforcement of resistance since only use the stomach in association with the bowel in
the bladder neck is divided. This technique has un- case of low-grade kidney insufficiency to alleviate
dergone many technical improvements according to ionic reabsorption problems.
the Mitrofanoff principle to allow creation of a cath-
eterizable urinary stoma by anti-reflux reimplanta-
tion of the appendix or of a tubularized bowel seg- Necessary sphincter resistance
ment (Monti) or a ureter. This procedure was initially If only a slight increase of the sphincter resistances is
used in patients in whom self-catheterization of the needed, our approach consists of injecting silicone gel
urethra was difficult due to obesity, poor eye–hand in the bladder neck. If reinforcement is required dur-
coordination or caretaker issues surrounding genital ing a bladder procedure (anti-reflux or cystoplasty),
organ privacy. In a recent review of their continent we prefer the Young-Dees or Pippi Salles procedure
stomas, Pippi Salle et al reported surgical revision in in boys in association with suspension of the bladder
39% of patients, including stomal revision in 18%, neck in girls. Once again endoscopic injection can
redo in 8%, bulking agent injection in 8% and pro- enhance results in terms of residual continence. In
lapse correction in 4%. No statistically significant dif- our hands, the artificial sphincter remains an excel-
ferences in complication rates were noted between lent tool especially in boys and more as the first line.
appendix and tubularized bowel or different stoma Continent cystostomy is proposed to patients in
locations (42). whom micturition by natural passages is not easy,
e.g., patients who are in wheelchairs or obese. De-
External urinary diversion finitive closure of the bladder neck is performed only
if bladder catheterization through the Mitrofanoff ca-
External urinary diversion, once considered as an ef- nal is fully functional.
fective method to protect the upper tract, is now used
only in special cases involving infants and young Optimizing the patient´s wellbeing
children that develop high intravesicular pressure
syndrome (> 40 cm H2O during filling phase). The Decision-making at each step of treatment must take
adverse effects of high pressure requires temporary into account the cooperation of the patient, his au-
262 J. M. Guys, G. Hery, M. Haddad, C. Borrionne

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Received: July 30, 2011

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