Evaluation, treatment & intervention in the pediatric
neuropathic bladder Paul F. Austin, MD, FAAP Professor of Urologic
Surgery Department of Surgery Division of Urologic Surgery St.
Louis Childrens Hospital Washington University School of
Medicine
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Department of Surgery Division of Urologic Surgery ICCS
Standardisation Documents
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Department of Surgery Division of Urologic Surgery ICCS
Standardisation Documents
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Department of Surgery Division of Urologic Surgery Disclaimers
and limitations Not a systematic literature review There is a
paucity of level I or level II levels of evidence publications
These recommendations are a consensus of a compilation of best
practices Review of the literature Relevant research Expert opinion
Current understanding on the pathophysiology of neuropathic bladder
and bowel Draft review document was open to all the ICCS members
via the ICCS web site Feedback was considered by the core authors
and by agreement, amendments were made as necessary
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Department of Surgery Division of Urologic Surgery Objectives
Neuropathic bladder & bowel documents To create an educational
reference document that will guide healthcare providers in the
evaluation and management of children with neuropathic bladder
& bowel dysfunction To provide a consensus view of the members
of the ICCS in the evaluation and management of children with
neuropathic bladder & bowel dysfunction
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Department of Surgery Division of Urologic Surgery Initial
evaluation Determined by several factors: Timing of presentation or
diagnosis infancy vs. older child Etiology
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Department of Surgery Division of Urologic Surgery Open spinal
cord lesion Initial evaluation Check PVR Ultrasound or catheter
Urodynamics Usually 2 -3 months of age Screening for: High pressure
DO contractions Elevated detrusor filling &/or voiding
pressures
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Department of Surgery Division of Urologic Surgery Open spinal
cord lesion Initial evaluation Renal & bladder U/S Screening
for: Hydronephrosis, Ureteral dilation
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Department of Surgery Division of Urologic Surgery Open spinal
cord lesion Initial evaluation Renal & bladder U/S Screening
for: Discrepancy in renal size or contour RK: 9.2 cmLK: 6.7 cm
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Department of Surgery Division of Urologic Surgery Open spinal
cord lesion Initial evaluation Renal & bladder U/S Screening
for: Bladder wall thickness
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Department of Surgery Division of Urologic Surgery Open spinal
cord lesion Initial evaluation VCUG Not routine Indicated when:
Abnormal U/S imaging of kidneys Bladder urodynamic studies reveal
high risk Detrusor overactivity Poor detrusor compliance Elevated
leak point pressure and DSD
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Department of Surgery Division of Urologic Surgery Neuropathic
bladder Video-urodynamics
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Department of Surgery Division of Urologic Surgery Follow-up of
NBD dysfunction Newborn to toddler Urodynamic studies High risk CIC
+/- anticholinergics Low risk Diaper voiding Repeat UDS (with RBUS)
in 2 3 months after initiating therapeutic interventions RBUS every
6 months for child with DO UDS yearly unless changes seen on RBUS
or with lower extremities Rationale: Elevated risk of developing
tethered cord
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Department of Surgery Division of Urologic Surgery Follow-up of
NBD dysfunction Toddler to adolescent Cord tethering risk lessens
RBUS yearly or every 6 months UDS Changes on RBUS Changes in
ambulation or lower extremity function Changes in continence
Increased UTIs
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Department of Surgery Division of Urologic Surgery Follow-up of
NBD dysfunction Adolescent to adult 2 nd time period of growth
spurt and increased risk of tethering RBUS yearly May consider
every 2 years after growth velocity diminishes UDS Changes on RBUS
Changes in ambulation or lower extremity function Changes in
continence Increased UTIs
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Department of Surgery Division of Urologic Surgery Follow-up of
NBD dysfunction Adulthood RBUS every 3 years UDS Changes on RBUS
Changes in continence Increased UTIs
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Department of Surgery Division of Urologic Surgery Evaluation
of neuropathic bowel dysfunction History Frequency of bowel
movements Consistency of feces: Hard Soft Watery Current use of
laxatives Frequency of fecal incontinence Childs ability: To feel
the urge to defecate To sit on the toilet To cooperate with bowel
regimen or program Determine the childs response to prior
treatments Dietary measures Digital rectal stimulation Enemas
Suppositories
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Department of Surgery Division of Urologic Surgery Evaluation
of neuropathic bowel dysfunction History 2-week bowel diary
Validated assessment of a childs defecation habits Although not
mandatory, it is an excellent supplement to history taking
http://i-c-c-s.org/members/Clinical-Tools.cgi
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Department of Surgery Division of Urologic Surgery Treatment:
Neuropathic bladder & bowel
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Department of Surgery Division of Urologic Surgery
Pharmacotherapy Anticholinergics Mainstay of drug therapy Level I
evidence Target muscarinic receptors M2 & M3 Systemic
implications M1-M5 Improve bladder wall compliance Diminish storage
pressures Convert NGB from high to low risk Abolishes detrusor
overactivity Provides time for CIC Provides urinary continence M3
M2 M1M4M2 ACh Anticholinergics
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Department of Surgery Division of Urologic Surgery
Pre-treatmentPost-treatment Anticholinergic effects Detrusor
overactivity
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Department of Surgery Division of Urologic Surgery
Anticholinergic effects Detrusor compliance
Pre-treatmentPost-treatment
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Department of Surgery Division of Urologic Surgery
Pharmacotherapy Botulinum-A-Toxin Inhibits ACh release at NMJ Botox
may modulate both sensory & motor pathways Small, uncontrolled
studies in children with NGB Improved clinical and urodynamic
parameters: Improved continence Reduced max detrusor pressure
Increased detrusor compliance Not approved by FDA or the EMEA for
the treatment of NBD BTX-A use is off-label requiring informed
consent FDA approval in adults 2011 Treatment of urinary
incontinence due to DO associated with a neurologic condition in
adults who have an inadequate response to or are intolerant of an
anticholinergic medication Spinal cord injury Multiple sclerosis
Adult Max dose = 200 U
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Department of Surgery Division of Urologic Surgery
Pharmacotherapy Antibiotics No level I evidence of medical benefit
to using antibiotic prophylaxis in children with NBD who perform
CIC. No difference in the rate of symptomatic or total UTIs Alters
the normal skin and bladder flora Increased selection of virulent
bacterial isolates Klebsiella and Pseudomonas Antibiotic
prophylaxis selective and individualized Focus on better emptying
with CIC
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Department of Surgery Division of Urologic Surgery
Catheterization Non-latex catheters are employed exclusively
Cochrane Review - incidence of UTI Lack of evidence that one
catheter type, technique, or strategy is better Modification of
catheters and catheter regimens should be made on an individual
basis for children with NBD
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Department of Surgery Division of Urologic Surgery
Neuromodulation therapy Intravesical electrical stimulation Labor
intensive & controversial Only one randomized,
placebo-controlled trial No efficacy demonstrated in children with
NBD
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Department of Surgery Division of Urologic Surgery
Neuromodulation therapy Sacral nerve stimulation Primarily been
reported in the treatment of patients with non-neuropathic bladder
Sacral nerve stimulation is considered investigational at this
time
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Department of Surgery Division of Urologic Surgery
Neuromodulation therapy Biofeedback No significant studies of
biofeedback have been reported in children with NBD
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Department of Surgery Division of Urologic Surgery Surgical
intervention Patients who fail medical management Goals: Attaining
safe bladder storage pressures & capacity Increasing bladder
outlet resistance
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Department of Surgery Division of Urologic Surgery Attaining
safe bladder storage pressures & capacity Urethral dilation
Mixed efficacy Selected patients Technically easiest in females
Vesicostomy Excellent temporizing procedure Ideal in infants and
toddlers
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Department of Surgery Division of Urologic Surgery Bladder
augmentation Achieves complete continence in children with
neuropathic bladder Allows independence & self-esteem Requires
patient commitment & compliance
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Department of Surgery Division of Urologic Surgery Bladder
augmentation Definitive method of creating a safe, low-pressure
storage Small bowel Most commonly employed Large bowel Ureter
Auto-augmentation
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Department of Surgery Division of Urologic Surgery Bladder
augmentation Associated complications Acid-Base imbalances UTIs
Stones Bladder augment perforation Cancer risk
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Department of Surgery Division of Urologic Surgery Increasing
bladder outlet resistance Variety of surgical approaches Fascial
sling Artificial urinary sphincter Bladder neck reconstruction
Bladder neck closure Pump Cuf f Reservoir
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Department of Surgery Division of Urologic Surgery Treatment
Neuropathic bowel High fiber diet Digital stimulation / glycerin
suppositories Laxatives Transanal irrigation e.g. cone enema
Colonic irrigation ACE or MACE Chait tube / Cecostomy tube
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Department of Surgery Division of Urologic Surgery Summary
Neuropathic bladder & bowel documents Provide a guideline for
appropriate evaluation and timely surveillance of the various
neuro-urologic conditions that affect children Underscore the
variability and complexity of patients with NBD & bowel
Non-surgical intervention is promoted before undertaking major
surgery CIC +/- anticholinergics are mainstay interventions Dietary
fiber, laxatives and enemas are common in bowel management Surgical
intervention After failure of medical therapy Requires patient
commitment and compliance
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Department of Surgery Division of Urologic Surgery Surgical
reconstruction Neuropathic bladder & bowel
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Department of Surgery Division of Urologic Surgery Bowel
segments
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Department of Surgery Division of Urologic Surgery Bowel
segments
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Department of Surgery Division of Urologic Surgery Mitrofanoff
principal *
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Department of Surgery Division of Urologic Surgery
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Department of Surgery Division of Urologic Surgery Surgical
reconstruction Neurogenic bladder & bowel
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Department of Surgery Division of Urologic Surgery Bowel
segments Preparation
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Department of Surgery Division of Urologic Surgery Monti
Catheterizable channel
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Department of Surgery Division of Urologic Surgery Bowel
segments
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Department of Surgery Division of Urologic Surgery
Catheterizable channels & augmentation
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Department of Surgery Division of Urologic Surgery Continence
mechanism How does it work?
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Department of Surgery Division of Urologic Surgery MACE Malone
Antegrade Continence Enema
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Department of Surgery Division of Urologic Surgery Refractory
constipation Neuropathic bladder & bowel Myelodysplasia
Anorectal malformations
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Department of Surgery Division of Urologic Surgery Patient
selection Refractory constipation Failed all conservative measures
Underlying pathology Chronic idiopathic constipation = poorly
Neuropathic bowel & anorectal malformations = good Age > 5
yo = good results Compliance & Motivation
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Department of Surgery Division of Urologic Surgery Continence
mechanism MACE
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Department of Surgery Division of Urologic Surgery Appendiceal
mesentery MACE
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Department of Surgery Division of Urologic Surgery Mesenteric
windows Dissection
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Department of Surgery Division of Urologic Surgery Mesenteric
windows MACE
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Department of Surgery Division of Urologic Surgery Pre-cecal
wrap MACE
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Department of Surgery Division of Urologic Surgery Cecal wrap
MACE
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Department of Surgery Division of Urologic Surgery MACE Cecal
wrap
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Department of Surgery Division of Urologic Surgery Mitrofanoff
& MACE (Appendix)
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Department of Surgery Division of Urologic Surgery Mitrofanoff
& MACE (Appendix)
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Department of Surgery Division of Urologic Surgery Spiral Monti
Casale, J Urol, 162:1743, 1999
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Department of Surgery Division of Urologic Surgery Spiral
Monti
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Department of Surgery Division of Urologic Surgery Spiral
Monti
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Department of Surgery Division of Urologic Surgery Spiral
Monti
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Department of Surgery Division of Urologic Surgery MACE
alternatives Appendectomy
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Department of Surgery Division of Urologic Surgery Colon
tube
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Department of Surgery Division of Urologic Surgery
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Department of Surgery Division of Urologic Surgery
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Department of Surgery Division of Urologic Surgery
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Department of Surgery Division of Urologic Surgery
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Department of Surgery Division of Urologic Surgery
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Department of Surgery Division of Urologic Surgery Appendiceal
pedicle Limitations
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Department of Surgery Division of Urologic Surgery Stoma
construction V-flap
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Department of Surgery Division of Urologic Surgery Stomas MACE
& Mitrofanoff
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Department of Surgery Division of Urologic Surgery Thank
you!