Neurogenic Bladder

Case Presentation

Tommy Alexander
1606970285

Supervisor:
dr. Endang Lestari Andayani, Sp.A (K)
Dr. Ina Zarlina, Sp.A (K)

DIVISI NEFROLOGI
DEPARTEMEN ILMU KESEHATAN ANAK
FACULTY OF MEDICINE UNIVERSITY INDONESIA
2018
ABSTRACT

Background: Neurogenic bladder sphincter dysfunction (NBSD) can cause severe and
irreversible renal damage and bladder-wall destruction years before incontinence becomes an
issue. Therefore, the first step in adequate management is to recognize early the bladder at risk for
upper- and lower-tract deterioration and to start adequate medical treatment proactively. Clean
intermittent catheterization combined with anticholinergics (oral or intravesical) is the standard
therapy for NBSD. Early institution of such treatment can prevent both renal damage and
secondary bladder-wall changes, thereby potentially improving long-term outcomes.

Case Illustration: a six years old female controls to policlinic of nephrology in april 19th 2018
with a medical history of neurogenic bladder, spinal bifida (agenesis coccygeus S2-S4), CKD G4,
and Hypertension. This medical condition is begin when patients at three years old, preceded by
complaints about lower abdominal pain, cloudy urine, and excrement like goat feses. With a
history of body weight did not increased and getting weaker. Patient was brought to emergency
department of mother and child of Harapan Kita hospital. After 3 days observation and treatment,
patient was discharged with several planning therapy.

Conclusion: A cases were reported with a diagnosis of neurogenic bladder, with spinal bifida,
CKD and hypertension. Diagnosis is made from clinical approach, laboratorium biomarker, and
radiography. The management provided is supportive based on the underlying disease, to relieved
obstruction in the urinary flow and to prevent worsening renal function

Keywords: Neurogenic bladder, Urinary continence, Anticholinergics, Intermittent

catheterization, Urodynamics

2
Introduction

Neurogenic bladder sphincter dysfunction (NBSD) can develop as a result of a lesion at any level
in the nervous system, including the cerebral cortex, spinal cord, or peripheral nervous system.
Neurologic conditions in children leading to neurogenic bladder dysfunction are predominantly
congenital neural tube defects (including myelomeningocele, lipomeningocele, sacral agenesis,
and occult lesions causing tethered cord). Acquired causes such as spinal cord tumors or trauma
or sequelae of transverse myelitis are less frequent. Whereas from an etiologic standpoint
neurogenic bladder dysfunction is a heterogeneous group, medical management will be similar
irrespective of the underlying cause. The vast majority of knowledge about NBSD management
comes from longterm experience with myelomeningocele (MMC), the most common neural tube
defect.

Following the institution of a general treatment policy with advances in neurosurgical and
orthopedic treatments in previous decades, governing the associated NBSD has become crucial for
improving quality of life and life expectancy in children with neural tube defects. In MMC patients,
disordered innervation of the detrusor musculature and external sphincter adversely affects bladder
function, which if untreated not only leads toincontinence but also will cause secondary damage
and dysfunction of both the upper and lower urinary tracts. Key elements in optimal NBSD
management are early diagnosis (including NBSD typology) and early (presymptomatic)
institution of adequate medical treatment. There is indeed growing evidence that management
decisions made during infancy, which prevent both renal damage and secondary bladder-wall
changes, potentially impact long-term outcomes for renal function and safe urinary continence.

Epidemiology
NGB dysfunction may arise as a result of several neurologic conditions. NGB has been found
in 40% to 90% of patients in the United States with multiple sclerosis (MS), 37% to 72% of
patients with parkinsonism, and 15% of patients with stroke.1,2 It is estimated that 70% to 84%
of patients with spinal cord injuries have at least some degree of bladder
dysfunction.1,3 Bladder dysfunction is also frequently seen in patients with spina bifida, with
vesicoureteral reflux present in up to 40% of children affected by 5 years of age and with up
to 60.9% of young adults with spina bifida experiencing urinary incontinence.1,4 Less common

3
scenarios for NGB may include diabetes mellitus with autonomic neuropathy, unintended
sequelae following pelvic surgery, and cauda equina syndrome resulting from lumbar spine
pathology.1 Many patients with NGB, especially those with multiple sclerosis, cerebrovascular
accidents, and spinal cord injury, experience uninhibited bladder contractions.1,5 Bothersome
urinary symptoms associated with NGB include urinary incontinence (UI), frequency, and
urgency.5 Patients also may have increased risk and incidence of urinary tract infections
(UTIs) and bladder outlet obstruction. If not treated optimally, patients with NGB may also be
at risk for sepsis and renal failure, and these patients have higher numbers of clinical office
and ED visits annually, with up to one-third of these visits leading to a need for
hospitalization.1,3 In addition to the physical and clinical burden associated with NGB, the
associated urinary incontinence can negatively impact a patient’s quality of life, causing
embarrassment, depression, and social isolation.1,6 As diagnostic and treatment options
continue to advance for patients with NGB, it is important to reassess its epidemiology and
physiology, diagnosis, assessment, and classification, and the impact of the symptoms and
complications associated with NGB on the patients affected by this disorder.

Neurologic Disorders and Neurogenic Bladder

Multiple Sclerosis

Normal urinary tract function is dependent on neural integration between the central and
peripheral nervous systems.7 MS, the most common neuroinflammatory disorder of the CNS,
may cause lower urinary tract dysfunction and NGB as a result of a disruption of this
integration. Urinary symptoms in MS are likely caused by neural demyelization and axonal
degradation, and patients with MS lesions in specific CNS regions (encephalic, spinal
suprasacral regions) may be more likely to experience major urinary symptoms.7,8 It has also
been hypothesized that CNS lesions from MS may exert a local effect on bladder function.8 A
variety of patterns may be seen, with detrusor overactivity of the bladder noted in 50% to 90%
of patients with MS and detrusor areflexia in 20% to 30% of patients with MS. 1,3 Neurogenic
lower urinary tract dysfunction is often noted during the first 10 years following MS diagnosis
and tends to increase as the patient’s level of disability worsens. 7,9 This urinary tract
dysfunction can lead to substantial limitations in daily activity for patients with MS.7 More than

4
80% of patients with MS report genitourinary symptoms, with voiding dysfunction impacting
the vast majority of these patients.9,10 In addition, bladder symptoms are frequently
mismanaged in patients with MS, often leading to urinary retention and/or subsequent
UTI.11 Early and accurate assessment of potential lower urinary tract dysfunction is essential
to protect the upper urinary tract, optimize management, and improve quality of life for these
patients.7

Idiopathic Parkinson’s Disease

Idiopathic Parkinson’s disease (IPD) presents as an extrapyramidal neurologic syndrome,

most commonly associated with prominent motor symptoms. However, non-motor symptoms
have been recognized in parkinsonism, and urinary symptoms are frequently present in these
patients.12 Urinary dysfunction as a manifestation of autonomic failure is common in patients
with IPD.13 Studies have shown that urinary storage symptoms (frequency, urgency, urge
urinary incontinence) are present in 57% to 83% of patients with IPD, and voiding symptoms
(poor force of stream, hesitancy, incomplete emptying) are seen in 17% to 27% of this patient
population.12 For most patients, the onset of bladder dysfunction occurs after motor
symptoms are evident, and voiding dysfunction tends to increase with neurologic impairment
as opposed to disease duration.14 As with NGB associated with other disorders, a patient’s
renal function and long-term health may be compromised if urinary dysfunction in IPD is not
recognized and addressed promptly. One confounding factor impacting correct diagnosis and
management is the potential for clinicians to confuse patients with pure IPD with those who
have multiple symptom atrophy, a disorder that manifests with parkinsonian-like motor
symptoms but with substantial differences in both neurologic progression and urinary
disturbances.12,14 Bladder symptoms may also be the result of coexistant disease processes
such as UTI, diabetes, or in men, benign prostatic hypertrophy, which can complicate
accurate diagnosis. Appropriate diagnosis is key to management of urinary dysfunction in
IPD, and a multidisciplinary approach may be needed for symptom management and optimal
patient quality of life.14

The primary urinary complaints in patients with urinary dysfunction due to IPD include
urgency, frequency, UI, and nocturia. This is likely due to the urodynamic finding of detrusor

5
overactivity, which may be seen in 45% to 93% of patients with IPD.12 UI in patients with IPD
may be of multifactorial origin; not only do the patients have significant bladder dysfunction,
but there also are functional problems such as impaired mobility and poor manual dexterity,
which can impact the patient’s ability to perform appropriate toileting. Some patients with IPD
also experience sleep disturbances and nocturnal polyuria. Management of these patients
must take into consideration how IPD influences the lower urinary tract as well the possible
pharmacologic effects of anti-parkinsonian agents the patient may be taking.14

Spinal Cord Injury

It is estimated that there are more than 200,000 patients with traumatic spinal cord injury
(SCI) in the United States, with an incidence of approximately 12,000 new cases estimated
annually.15 Urinary dysfunction is very common in these individuals; approximately 81% of
patients with SCI report at least some degree of impaired bladder function within 1 year after
injury.16,17 The expected level of bladder dysfunction may potentially be determined by the
spinal level at which the SCI occurred. Injury proximal to the sacral spinal cord should lead to
an upper motor neuron lesion and detrusor overactivity. Injuries that involve the sacral spinal
cord or cauda equina should result in a lower motor neuron lesion and detrusor areflexia.
Both of these classifications assume the presence of a complete neurologic lesion; however,
the presence of a complete lesion may be variable. In addition, patients with a suprasacral
SCI are at risk for detrusor-external sphincter dyssynergia, which can place the patient at risk
for incomplete bladder emptying and elevated bladder pressures.1,18 Many patients with SCI
do not have well-defined complete lesions, and although the majority of patients demonstrate
fairly consistent bladder and sphincter behavior based on neurologic deficit, this is not
definitive for all patients with SCI. Thorough urodynamic evaluation to evaluate bladder and
sphincter behavior needs to be performed to better assess urinary dysfunction prior to
initiation of therapy.18 Management of NGB dysfunction is a critical component of a
rehabilitation program for a patient with SCI, as NGB contributes significantly to the overall
morbidity of these patients. Losing normal bladder function is disabling and may lead to
urinary tract deterioration, urinary incontinence, and reduced quality of life. Principal goals for
management are preservation of renal function, improved continence, and reduction of
urinary complications such as kidney stones and UTI. Bladder management focuses on

6
therapy to facilitate bladder filling and storage of urine and treatment to facilitate bladder
emptying that assists in preserving both renal function and social functioning to allow patients
to enjoy a healthier life.16

Spina Bifida

Spina bifida is a common neurologic abnormality, with worldwide incidence estimated at 0.3
to 4.5 per 1000 births.19 Whereas barely 10% of these patients survived infancy prior to 1960,
most patients today have a normal expected lifespan. This extended life expectancy also now
means that many patients with this disease and complex disabilities survive well into
adulthood with increasing expectations of life.4 The disorder is associated with prenatal folate
deficiency, and with government-mandated folate supplementation in foods in the United
States, the incidence of spina bifida has been decreasing.20 UI is a common symptom related
to NGB in patients with spina bifida, and possible urodynamic findings include detrusor
overactivity, poor bladder compliance, and a fixed, obstructing outlet that may be incompetent
as well. NGB secondary to spina bifida may result in a high-pressure bladder, which places
the patient at risk of upper urinary tract damage.19,20 The primary goal for therapy is to convert
the bladder into a low-pressure reservoir and protect the upper urinary tract. Preservation of
renal function is key in the management of urinary dysfunction related to spina
bifida.19 Adjustments in patient management must be made as the patient grows and
progresses into adolescence and adulthood to allow the patient independence with respect
to bladder management.19Successful treatment of UI related to spina bifida can be complex
and will continue into adulthood.20 In 1 study, UI was found in nearly 61% of young adults
with the disorder. Bladder management techniques must be individualized in patients with
spina bifida to preserve both renal function and quality of life.4,20

1. Dorsher PT, McIntosh PM. Neurogenic bladder [published online February 8,

2012]. Adv Urol. 2012;2012:816274. doi:10.1155/2012/816274.
2. Lansang RS, Krouskop AC. Bladder management. In: Massagli TL et al,
eds. eMedicine. 2004.
3. 3. Manack A, Mostko SP, Haag-Molkenteller, et al. Epidemiology and healthcare
utilization of neurogenic bladder patients in a US claims database. Neurourol
Urodyn. 2011;30:395-401.

7
 4. Verhoef M, Lurvink M, Barf HA, et al. High prevalence of incontinence among young
adults with spina bifida: description, prediction and problem perception. Spinal Cord.
2005;43:331-340.
5. Linsenmeyer TA, Culkin D. APS recommendations for the urological evaluation of
patients with spinal cord injury. J Spinal Cord Med. 1999;22:139-142.
6. O’Leary M, Dierich M. Botulinum toxin type A for the treatment of urinary tract
dysfunction in neurological disorders. Urologic Nursing. 2010;30:228-234.
7. Del Popolo G, Panariello G, Del Croso F, et al. Diagnosis and therapy for neurogenic
bladder dysfunctions in multiple sclerosis patients. Neurol Sci. 2008;29(suppl
4):S352-S355. doi:10.1007/s10072-008-1042-y.
8. Stoffel JT. Contemporary management of the neurogenic bladder for multiple
sclerosis patients. Urol Clin North Am. 2010;37:547-557.
9. Pentyala S, Jalali S, Park J, et al. Urologic problems in multiple sclerosis. Open
Androl J. 2010;2:37-41.
10. Litwiller SE, Frohman ER, Zimmern PE. Multiple sclerosis and the urologist. J Urol.
1999;161:743-757.
11. Holland NJ, Reitman NC; for the National Multiple Sclerosis Society. Bladder
dysfunction in multiple sclerosis. http://www.nationalmssociety.org/search-
results/index.aspx?q=bladder+dysfunction&x=-980&y=-19&start=0&num=20.
Accessed April 8, 2013.
12. Yeo L, Singh R, Gundeti M, et al. Urinary tract dysfunction in Parkinson’s disease: a
review. Int Urol Nephrol. 2012;44:415-424.
13. Blackett H, Walker R, Wood B. Urinary dysfunction in Parkinson’s disease: a
review. Parkinsonism Relat Disord. 2009;15:81-87.
14. Kapoor S, Bourdoumis A, Mambu L, Barua J. Effective management of lower urinary
tract dysfunction in idiopathic Parkinson’s disease. Int J Urol. 2013;20:79-84.
15. Jeong SJ, Cho SY, Oh SJ. Spinal cord/brain injury and the neurogenic bladder. Urol
Clin North Am. 2010;37:537-546.
16. Ku JH. The management of neurogenic bladder and quality of life in spinal cord
injury. BJU Int. 2006;98:739-745.
17. Stover SL, DeLisa JA, Whiteneck GG. Spinal Cord Injury: Clinical Outcomes From
the Model Systems. Gaithersburg, MD: Aspen Publishers; 1995.
18. Kaplan SA, Chancellor MB, Blaivas JG. Bladder and sphincter behavior in patients
with spinal cord lesions. J Urol. 1991;146:113-117.
19. de Jong TPVM, Chrzan R, Klijn AJ, Dik P. Treatment of the neurogenic bladder in
spina bifida. Pediatr Nephrol. 2008;23:889-896.
20. Mourtzinos A, Stoffel JT. Management goals for the spina bifida neurogenic bladder:
a review from infancy to adulthood. Urol Clin North Am. 2010;37:527-535.

CASE ILLUSTRATION

A six years old female controls to policlinic of nephrology in april 19th 2018 with a medical
history of neurogenic bladder, spinal bifida (agenesis coccygeus S2-S4), CKD G4, and

8
Hypertension. This medical condition is begin when patients at three years old, preceded by
complaints about lower abdominal pain, cloudy urine, and excrement like goat feses. With a
history of body weight did not increased and getting weaker. Patient was brought to emergency
department of mother and child of Harapan Kita hospital.
His physical examination showed a fully alert patient with blood pressure of 130/100 mmHg,
heart rate of 117 beat per minutes, respiration rate of 24 time per minutes, and 100% peripheral
oxygen saturation in room air environment. Other physical examination did not show a remarkable
findings except tenderness in lower abdominal

Figure 1. Chest x-ray

Chest X-ray examination displayed chest thoracic ratio of 60%, normal aortic and normal
pulmonary segment, with no sign of congestion nor infiltrate.

9
 Figure 2. USG Tractus Urinarius

Figure 3. USG Traktus Urinarius

USG Traktus Urinarius

10
 • Buli
• Dinding menebal tebal +/- 0,4cm
• Tampak debris yang berkurang dibandingkan pemeriksaan sebelumnya
• Volume prevoiding 76,03 cm
• Ureter Kanan –kiri
• Proximal tampak dilatasi
• Distal tampak dilatasi
• Ginjal Kanan- Kiri
• Ukuran ginjal kanan 4,85 x 2,28 cm, ukuran ginjal kiri 6,11x3,37 cm
• Echostruktur parenkim tak tampak lesi patologis
• Sistem pelviokalises tampak dilatasi
• Diameter AP pyelum kanan +/- 2,14cm
• Diameter AP pyelum kiri +/- 3,15cm
Kesan : Hydronefrosis & hydroureter bilateral
Cystitis
Working diagnosis in this patient was anterior neurogenic bladder, spinal bifida (agenesis
coccygeus S2-S4), CKD G4, and Hypertension. After 3 days observation and treatment, patient
was discharged with several planning therapy.

Literature Review

Historical Evolution

The management of NBSD in children has undergone major changes over the years. A first
milestone was the introduction of clean intermittent catheterization (CIC) in 1972. CIC (combined
with anticholinergics if required) has made “conservative” (medical) management a successful
treatment option, with a good outcome for quality of life and kidney protection. Further important
breakthroughs were the wider application of urodynamic testing in the evaluation of infants and
young children with suspected NBSD and the better pathophysiological understanding of the
natural history of NBSD in patients with spina bifida. In spina bifida, the natural history of the
urinary tract in untreated NBSD is one of progressive deterioration by the age of 3 years in up to
58% of patients.

11
Several reports have shown this deterioration to be directly related to increased intravesical
pressure. In 1981, the bladder pressure at which urethral leakage occurred was found to be a useful
predictor of unsafe bladder function. The leak-point pressure, as it is now commonly referred to,
has become accepted as one of the urodynamic parameters that allows clinicians to differentiate
patients with relatively low or high risk for subsequent upper urinary tract deterioration. In 1984,
detrusor external sphincter dyssynergia (DSD) was identified as an important factor leading to
functional obstruction, and intravesical pressure was recognized as the pathophysiological
mechanism of subsequent upper urinary tract deterioration. Shortly thereafter, urodynamics in
infants and children was shown to allow a functional classification of NBSD that correlated with
clinical entities of incontinence and obstruction, an approach that has allowed the concept of
individualized and presymptomatic therapy in high-risk patients. 1

Pathophysiology of the neurogenic bladder

Under normal conditions, the detrusor muscle, bladder neck, and striated external sphincter
function as a synergistic unit for adequate storage and complete evacuation of urine. In healthy
bladders, the change in bladder-filling pressure between empty and full is normally less than 10–
15 cm H2O. Normal voiding pressures for males and females are from 50 to 80 cm H2O and from
40 to 65 cm H2O, respectively. In patients with NBSD, disordered innervation of the detrusor
musculature and external sphincter adversely affects bladder function. In recent years, it has
become clear that children with this condition can be categorized into high- and low-risk groups
for secondary damage from a neurogenic bladder based on intravesical pressure. When the detrusor
(filling) pressure exceeds 40 cm H2O, glomerular filtration rate decreases and pyelocaliceal and
ureteral drainage deteriorates, leading to obstructive hydronephrosis and/or vesicoureteral reflux.
Even in the absence of reflux or upper urinary tract dilatation, high intravesical pressure can impair
drainage of urine into the bladder.

Any pathophysiologic process that causes either intermittent or continuous elevation of bladder
pressure above 40 cm H2O places the child at risk for upper urinary tract dysfunction, urinary tract
infections, and ultimately renal failure. Intermittent elevation of bladder pressure may occur from
detrusor hypertonia, hyperreflexia, or both. Hyperreflexia may cause intermittent elevation of
bladder pressure, especially if the external sphincter acts reflexively and tightens rather than

12
relaxes in an attempt to prevent micturition [detrusor sphincter dyssynergia (DSD)]. Over a long
period of time, hyperreflexia with pressures greater than 40 cm H2O may result in detrusor
decompensation (areflexia from myogenic failure) or in detrusor hypertrophy with associated
sacculations and subsequent diverticula formation. These pathophysiologic changes affect the
elastic and vesicoelastic properties of the bladder and also result in mechanical ureterovesical
junction obstruction. Continuous elevation of bladder pressure above 40 cm H2O may occur from
a hypertonic detrusor or a hypertrophic small-capacity bladder secondary to outflow obstruction.
Bladder outlet obstruction is caused by DSD, or by fibrosis of the external urethral sphincter
secondary to partial or complete denervation. 2

Bladder outlet obstruction will lead to elevated (pathologic) voiding pressures, which will
contribute to either detrusor decompensation or hypertrophy. Finally, recurrent urinary tract
infections due to bladder residue may aggravate damage to the neurogenic bladder through
processes of transmural inflammation and fibrosis. Together with high intravesical pressures
and/or vesicoureteral reflux, these lower urinary tract infections will lead to episodes of acute
pyelonephritis and irreversible renal damage.

Figure 4. Madersbacher classification system with typical neurogenic lesions

13
Heavy lines symbolize overactivity, thin lines underactive or acontractile and green lines normal
function of the relevant structure

Management of the neurogenic bladder

General principles and treatment goals
The cornerstone of optimal NBSD management is early identification and characterization
(typology) and the institution of proactive therapy. Crucial for long-term prognosis of patients with
NBSD is the fact that the management must start before consequences of bladder dysfunction
become apparent. From the outset, the goals of management are to prevent or minimize secondary
damage to the upper urinary tracts and bladder from the primary neurogenic bladder dysfunction
and to achieve safe social continence. Thus, long before continence becomes an issue, starting
from the first year of life, management is directed at creating a low-pressure reservoir and ensuring
complete and safe bladder emptying.
Clean intermittent catheterization (CIC) or selfcatheterization (CISC) in combination with
anticholinergics (oxybutynin) is the standard therapy for children with neurogenic bladder
dysfunction with detrusor hyperactivity and/or DSD. This treatment is also feasible and effective
in developing countries, where untreated neuropathic bladder is an important cause of preventable
chronic renal failure.3 CIC enables complete bladder emptying and thus avoids bladder residues
and consequent risks for infections. In the highrisk bladder with DSD, CIC also allows bladder
emptying before the occurrence of otherwise “spontaneous” highpressure voiding, which is known
to be detrimental for kidney function and drainage. Oxybutynin, a bladder smooth-muscle relaxant,
is used to improve bladder dynamics through suppression of detrusor hypertonicity and
hyperreflexia. By doing so, oxybutynin eliminates (high-pressure) uninhibited detrusor
contractions (and thus urinary leakage) and prevents high-pressure bladder storage (due to detrusor
hypertonicity or low bladder compliance) and high-pressure emptying (in case of DSD).4

Early management, including diagnosis and identification of the high-risk bladder

At birth, the majority of patients with neurogenic bladder has normal upper urinary tracts. Without
proper management, urinary tract infections and elevated bladder pressures with secondary
bladder-wall changes may cause upper urinary tract deterioration within 3 years in up to 58%. One
third of children who develop impaired kidney drainage do so within the first year of life. The
specific abnormalities vary considerably and are not predicted by the level of the spinal cord defect.

14
Furthermore, the dysfunctional pattern may be dynamic, influenced by spinal cord surgery,
tethering, and denervation. In the initial baseline evaluation, clinical observations must be
completed with urinalysis (microscopy and culture), renal/ bladder ultrasound, and
cystourethrogram. These allow the experienced clinician to suspect the type of NBSD and to
identify the high-risk subgroup. 5

Clean intermittent catheterisation

In children with neurogenic bladder, CIC is the first-choice treatment to empty the bladder
adequately (no residue, no infection) and safely (prior to high-pressure voiding), and it is a valuable
tool for achieving continence. The wide variety of used materials and techniques for CIC does not
seem to affect efficacy and safety as long as some basic principles are applied: proper education
and training, clean and atraumatic application, and achievement of good patient compliance on a
long-term basis. For education, training, and further guidance during follow-up, a dedicated
continence nurse is invaluable. Patients and caregivers must understand what is wrong with the
bladder/sphincter and why CIC is proposed for treatment, and they have to learn how to catheterize
properly. CIC has been successfully used by parents even in newborns and infants, becoming a
part of their everyday routine. Some authors prefer early institution of CIC in all infants with
NBSD, given the fact that by the age of 3 years, CIC will be required in all for achieving
continence, and given the difficulties of starting CIC at toddler age. Such early institution of CIC
seems to improve family compliance and their ability to assist the child in coping with their disease
and with CIC.6

Pharmacologic treatment: anticholinergics

Of the anticholinergic agents available, oxybutynin hydrochloride is most commonly used, and
long-term experience supports its safety also in newborns and infants. Oxybutynin is a tertiary
amine with a well-documented therapeutic effect on detrusor hyperactivity, and its effectiveness
is attributed to a combination of anticholinergic (M3-selective receptor subtype antagonism),
antispasmodic, local anesthetic and calcium-channel-blocking activity. Several studies have
shown its efficacy for decreasing the filling pressure, increasing the capacity of the neurogenic
bladder, and preserving renal function.7 The usual dose regimen of oral oxybutynin is 0.3–0.6
mg/kg per day in three doses. In children with insufficient response or significant systemic side

15
effects to oral oxybutynin, intravesical instillation of oxybutynin has been shown to be a highly
efficacious, reliable, and well-tolerated therapy for children who would otherwise require surgical
therapy. Because a solution suitable for intravesical instillation was not available, crushed
oxybutynin tablets were used in the earlier trials, with consequent problems of inconvenience and
impracticability, and it was the belief of several authors that poor patient compliance could be
resolved by an optimized drug preparation. To date, the vast majority (∼ 90%) of patients can be
treated successfully with the gold standard treatment of oxybutynin (oral or intravesical) and CIC.
Other bladderrelaxant drugs include propiverine (10–15 mg b.i.d. or t.i.d., adult dose), trospium
(20 mg b.i.d., adult dose), extended release oxybutynin, and tolterodine (children 0.25–1 mg b.i.d.,
adults 1–2 mg b.i.d.). The current experience with compounds other than oxybutynin is still limited
in children with neurogenic bladder. 8

Medical management of NBSD in clinical practice

Optimal management involves first, early diagnosis, including recognition of high-risk subtypes,
and second, proactive institution of adequate treatment. Early proactive treatment of high-pressure
dyssynergic lower urinary tracts is important in the long term, not only to preserve renal function
but also to prevent poor bladder compliance and the subsequent need for bladder augmentation.9
Urodynamic assessment is used in newborns and infants to come to a functional classification of
NBSD, allowing presymptomatic interventions in the high-risk groups and individualized
treatment planning according to the type of dysfunction. In clinical practice, four major subtypes
can be used to describe NBSD (Fig. 1): sphincter overactivity combined with detrusor
underactivity (type A) or overactivity (type B), and sphincter underactivity combined with detrusor
underactivity (type C) or with detrusor overactivity (type D).

The easiest type to treat is type A. This bladder type requires early treatment because of urine
retention with high filling pressure and continuous leaking. Here, CIC alone is effective and
sufficient and will make the bladder safe and infection free, and the patient will be dry in between
(social continence). Good care to empty the bladder totally is most important to avoid bladder
infections caused by residual urine. Dysfunctional type B will have high filling and high voiding
pressures, being very unsafe from birth onward due to DSD. Here, the act of voiding has to be
prevented. With oxybutynin, the overactive detrusor can be “pharmacologically converted” to an

16
inactive reservoir (situation similar to type A), which has to be emptied with CIC. In type C, CIC
reduces the degree of incontinence and offers much better control over urinary tract infections. To
achieve continence, this type will at a later age need surgical intervention on the sphincter (e.g.
sling operation). An important caveat here is that detrusor instability may emerge only after
surgical improvement of outlet resistance. If this detrusor instability would remain unrecognized
and untreated (with oxybutynin), bladderoutlet surgery would have converted a “wet but safe” into
a “dry but unsafe” bladder. In the last dysfunctional subtype (type D), the bladder leaks due to
detrusor instability and gradually becomes unsafe due to secondary bladder-wall changes with
detrusor hypertrophy and loss of bladder compliance. Therefore, treatment consists of CIC
combined with oxybutynin and, at a later age, bladder-outlet surgery. 10

Figure 5. Classification of neurogenic bladder, with four subtypes (a-d) according to

dysfunctional activities of sphincter and detrusor. For each subtype, clinical implications if
untreated and principles of management are summarized

17
 References

1. van Gool JD (1986) Spina bifida and neurogenic bladder dysfunction—a urodynamic
study. Utrecht, Impress
2. Bauer SB, Joseph DB (1990) Management of the obstructed urinary tract associated with
neurogenic bladder dysfunction. Urol Clin North Am 17:395–406
3. Jeruto A, Poenaru D, Bransford R (2004) Clean intermittent catheterisation: overview of
results in 194 patients with spina bifida. Afr J Pediatr Surg 1:20–3
4. Bauer SB, Joseph DB (1990) Management of the obstructed urinary tract associated with
neurogenic bladder dysfunction. Urol Clin North Am 17:395–406
5. Baskin LS, Kogan BA, Benard F (1990) Treatment of infants with neurogenic bladder
dysfunction using anticholinergic drugs and intermittent catheterisation. Br J Urol 66:532–
4
6. Kessler TM, Lackner J, Kiss G, Rehder P, Madersbacher H (2006) Early proactive
management improves upper urinary tract function and reduces the need for surgery in
patients with myelomeningocele. Neurourol Urodyn 25:758–62
7. Dik P, Klijn AJ, van Gool JD, de Jong-de Vos van Steenwijk CC, de Jong TP (2006) Early
start to therapy preserves kidney function in spina bifida patients. Eur Urol 49:908-13.
8. Haferkamp A, Staehler G, Gerner HJ, Dörsam J (2000) Dosage escalation of intravesical
oxybutynin in the treatment of neurogenic bladder patients. Spinal Cord 38:250–4
9. Youdim K, Kogan BA (2002) Preliminary study of the safety and efficacy of extended-
release oxybutynin in children. Urology 59:428–32
10. Buyse G, Verpoorten C, Vereecken R, Casaer P (1995) Treatment of neurogenic bladder
dysfunction in infants and children with neurospinal dysraphism with clean intermittent
(self) catheterisation and optimized intravesical oxybutynin hydrochloride therapy. Eur J
Pediatr Surg 5:31–4

18