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VALVE BLADDERVALVE BLADDER
Dr. Patrick Dr. Patrick MahMah
MBBS (Melbourne)MBBS (Melbourne)
FRCS (FRCS (EdinEdin). AM ). AM
(Mal)(Mal)
POST VALVE ABLATIONPOST VALVE ABLATION
No improvement:No improvement:
–– Pre renal causePre renal cause-- post obstructive post obstructive diuresisdiuresis
–– Renal Renal dysplasiadysplasia
–– Sepsis and infectionSepsis and infection
–– Post renal cause:Post renal cause:
HydronephrosisHydronephrosis--vesicovesico uretericureteric obstruction obstruction (anatomic or functional)(anatomic or functional)
Severe refluxSevere reflux
Valve bladderValve bladder
Residual posterior urethral valveResidual posterior urethral valve
VALVE BLADDERVALVE BLADDER
Posterior urethral valve affect no organ Posterior urethral valve affect no organ
more consistently and more profoundly more consistently and more profoundly
than the bladder.than the bladder.
Degree of damage varies considerablyDegree of damage varies considerably
Most patients suffer significant life long Most patients suffer significant life long
bladder dysfunction.bladder dysfunction.
VALVE BLADDERVALVE BLADDER
Two long term problemsTwo long term problems
Upper tract drainage affectedUpper tract drainage affected
IncontinenceIncontinence
50 % will reach ESRF in their lifetime50 % will reach ESRF in their lifetime
–– Need dialysis or transplantation during first 2 Need dialysis or transplantation during first 2 decades of life.decades of life.
Abnormal bladder will be a threat to the Abnormal bladder will be a threat to the success of transplantationsuccess of transplantation
Upper Tract DamageUpper Tract Damage
Can occur at Can occur at
–– Newborn stage Newborn stage
–– Later childhoodLater childhood
Newborn: Newborn: HypercontractileHypercontractile bladderbladder
–– Bladder thickened and Bladder thickened and trabeculatedtrabeculated
–– Contracts with great forceContracts with great force
–– Compliance lowCompliance low
–– Situation may persist for months even after successful Situation may persist for months even after successful
valve ablation.valve ablation.
Upper Tract DamageUpper Tract Damage
Later childhoodLater childhood-- Rigid BladderRigid Bladder
–– Contraction is lessContraction is less
–– Poor compliancePoor compliance
–– Poor sensationPoor sensation
DEFINITIONDEFINITIONMitchell 1982Mitchell 1982
Chronic conditionChronic condition
Successful valve ablationSuccessful valve ablation
Bladder dysfunctionBladder dysfunction
Deterioration of upper urinary Deterioration of upper urinary
tractstracts
Urinary incontinenceUrinary incontinence
PATHOPHYSIOLOGYPATHOPHYSIOLOGYMitchell 1982Mitchell 1982
Poor bladder sensationPoor bladder sensation
High bladder volumesHigh bladder volumes
Poor compliancePoor compliance
High storage pressuresHigh storage pressures
Poor drainage of upper tractsPoor drainage of upper tracts
PATHOPHYSIOLOGYPATHOPHYSIOLOGYMitchell 1982Mitchell 1982
Comfortable with large bladder Comfortable with large bladder
volumes at high pressurevolumes at high pressure
Overflow incontinenceOverflow incontinence
Void infrequently and incompletelyVoid infrequently and incompletely
Full Valve Bladder SyndromeFull Valve Bladder SyndromeDuckettDuckett & Snow 1986& Snow 1986
Upper tract dilatation progresses despite Upper tract dilatation progresses despite adequate valve ablationadequate valve ablation
Sensation of bladder decreaseSensation of bladder decrease
Increase intra Increase intra vesicalvesical pressure occur at low pressure occur at low filling volumefilling volume
VUJ obstructs with bladder fillingVUJ obstructs with bladder filling-- no more urine no more urine enters bladderenters bladder
Upper tract fills with inherent dilatationUpper tract fills with inherent dilatation
After voiding, bladder fills up rapidly with After voiding, bladder fills up rapidly with drainage form upper tractsdrainage form upper tracts
Worsened by Worsened by polyuriapolyuria
Full Valve Bladder SyndromeFull Valve Bladder SyndromeDuckettDuckett & Snow 1986& Snow 1986
UrodynamicsUrodynamics::
–– Poor compliancePoor compliance
–– Delayed sensation of fullnessDelayed sensation of fullness
Treatment:Treatment:
–– Double/ triple voidingDouble/ triple voiding
–– CICCIC
–– Anti Anti cholinergicscholinergics
–– Bladder augmentationBladder augmentation
PATHOPHYSIOLOGYPATHOPHYSIOLOGYKoffKoff et al 2002et al 2002
Induced condition. Combination of:Induced condition. Combination of:
–– PolyuriaPolyuria
–– Impaired bladder sensationImpaired bladder sensation
–– Residual urineResidual urine
Bladder Bladder decompensationdecompensation
–– Increased residual urineIncreased residual urine
–– Upper tract dilationUpper tract dilation
–– Renal injuryRenal injury
PATHOPHYSIOLOGYPATHOPHYSIOLOGYKoffKoff et al 2002et al 2002
HydronephrosisHydronephrosis-- persistent or persistent or
progressiveprogressive
Bladder Bladder overdistentionoverdistention::
–– PolyuriaPolyuria (24 urine volume > 2 liter (24 urine volume > 2 liter
(10/18)(10/18)
–– Impaired bladder sensation (18/18)Impaired bladder sensation (18/18)
–– Residual urine (14/18)Residual urine (14/18)
DetrusorDetrusor DysfunctionDysfunction
Smooth muscle infiltrated with increased Smooth muscle infiltrated with increased
amount of Type III collagen and amount of Type III collagen and elastinelastin
–– Cause loss of complianceCause loss of compliance
–– Small capacity bladder Small capacity bladder
–– High pressure bladderHigh pressure bladder
Changes in neurotransmitter Changes in neurotransmitter receptroreceptro
concentration in obstructed bladderconcentration in obstructed bladder
–– HyperreflexiaHyperreflexia ((ElbadawiElbadawi 1989)1989)
Valve BladderValve Bladder
Is it a permanent disability?Is it a permanent disability?
Can the valve bladder improve?Can the valve bladder improve?
Can renal deterioration be prevented?Can renal deterioration be prevented?
Can bladder dysfunction normalize?Can bladder dysfunction normalize?
EVALUATIONEVALUATION
Voided volume chartVoided volume chart
UltrasoundUltrasound-- assess degree of assess degree of hydronephrosishydronephrosis
VideourodynamicssVideourodynamicss::
–– Ability of bladder to hold and empty adequate Ability of bladder to hold and empty adequate
volume at acceptable pressuresvolume at acceptable pressures
LasixLasix renographyrenography: relative renal function and : relative renal function and
drainagedrainage
Whitaker perfusion test Whitaker perfusion test ––measure measure intrarenalintrarenal
pressures at a given drainage ratepressures at a given drainage rate
Diuretic Diuretic RenogramRenogram
DTPA / MAG3DTPA / MAG3
URODYNAMICSURODYNAMICS
WHITAKER TESTWHITAKER TEST
DIAGNOSISDIAGNOSISGhanemGhanem et al 2004et al 2004
Video Video urodynamicsurodynamics
Correlation between abnormal Correlation between abnormal
urodynamicsurodynamics (poor compliance and (poor compliance and
detrusordetrusor overactivityoveractivity) with poor renal ) with poor renal
function.function.
UrodynamicsUrodynamics in Childrenin Children
TechniqueTechnique
–– Supra pubicSupra pubic
–– Double lumenDouble lumen-- one for infusion and one for one for infusion and one for bladder pressure monitoringbladder pressure monitoring
–– Rectal lineRectal line
Indication:Indication:
–– Deterioration of renal function in spite of Deterioration of renal function in spite of adequate valve ablationadequate valve ablation
–– In children > 6 years and still incontinentIn children > 6 years and still incontinent
URODYNAMICS URODYNAMICS –– 3 PATTERNS3 PATTERNS(Peters 1990)(Peters 1990)
MyogenicMyogenic failurefailure
DetrusorDetrusor hyperreflexiahyperreflexia
Decreased compliance/ small capacityDecreased compliance/ small capacity
URODYNAMICSURODYNAMICS((HolmdahlHolmdahl 1996)1996)
Dominant pattern changed with ageDominant pattern changed with age
Stages of developmentStages of development
InfantsInfants-- poor compliancepoor compliance
Older childrenOlder children--instability from instability from
hypercontractilityhypercontractility
Post pubertal boysPost pubertal boys-- myogenicmyogenic failurefailure
URODYNAMICSURODYNAMICS((HolmdahlHolmdahl 1996)1996)
First 3 years of life:First 3 years of life:
–– Vanishing Vanishing hypercontractilityhypercontractility and increasing and increasing
bladder capacity, even though instability bladder capacity, even though instability
remain unchangedremain unchanged
4 4 –– 12 years12 years
–– Decreasing instability and Decreasing instability and hypercontractilityhypercontractility
–– Major problemMajor problem-- emptying difficultiesemptying difficulties
URODYNAMICSURODYNAMICS((HolmdahlHolmdahl 1996)1996)
Voiding patterns during day and nightVoiding patterns during day and night
–– 10 incontinent and 6 continent boys with PUV10 incontinent and 6 continent boys with PUV
–– Small frequent voiding during the day with Small frequent voiding during the day with fewer or no fewer or no voidingsvoidings during night. High during night. High bladder volumes in morning bladder volumes in morning
–– Bladder unstable during day and stable at Bladder unstable during day and stable at night night
–– Voiding Voiding detrusordetrusor pressure was higher and pressure was higher and functional bladder capacity lower during the functional bladder capacity lower during the day than at nightday than at night
URODYNAMICSURODYNAMICS
Need regular Need regular urodynamicsurodynamics
Track changes in patternTrack changes in pattern
Alter managementAlter management
First 2 decades of lifeFirst 2 decades of life
AIMS OF TREATMENTAIMS OF TREATMENT
Preserve renal functionPreserve renal function
Achieve a Functional bladderAchieve a Functional bladder
–– StorageStorage
–– Continence Continence
RENAL INJURYRENAL INJURY
GlomerularGlomerular insults insults
–– Renal Renal dysplasiadysplasia-- irreversibleirreversible
Affect renal growth and developmentAffect renal growth and development
–– Obstructive Obstructive uropathyuropathy
Produce ongoing injuryProduce ongoing injury
ReversibleReversible
–– Infections:Infections:
RefluxReflux
Incomplete bladder emptyingIncomplete bladder emptying
MANAGEMENTMANAGEMENTAustin et al 1999Austin et al 1999
Aim to lower bladder pressure and Aim to lower bladder pressure and
promote bladder emptying:promote bladder emptying:
–– Timed voidingTimed voiding
–– αα blockersblockers
–– Clean intermittent Clean intermittent catheterisationcatheterisation
MYOGENIC FAILUREMYOGENIC FAILURE
Older childrenOlder children
Overflow incontinenceOverflow incontinence
Incomplete bladder emptyingIncomplete bladder emptying
–– Ensure that there is no residual obstructionEnsure that there is no residual obstruction
–– Not due to bladder neck hypertrophyNot due to bladder neck hypertrophy
Treatment:Treatment:
–– Timed voidingTimed voiding
–– Double voiding/ Triple voidingDouble voiding/ Triple voiding
–– αα blockersblockers
–– CICCIC
DetrusorDetrusor HyperreflexiaHyperreflexia
Adequate emptyingAdequate emptying
Urine frequencyUrine frequency
Urine incontinenceUrine incontinence
Treatment:Treatment:
–– Anti Anti cholinergicscholinergics
POORLY COMPLIANT BLADDERPOORLY COMPLIANT BLADDER
Treatment:Treatment:
–– AntiAnti--cholinergicscholinergics
–– Augmentation Augmentation cystoplastycystoplasty
Clean Intermittent Clean Intermittent CatheterisationCatheterisation
Is it effective?Is it effective?
What time of regimen?What time of regimen?
–– Day time onlyDay time only
–– Day time and night timeDay time and night time
MANAGEMENTMANAGEMENT
KoffKoff et al 2002et al 2002
–– Not a permanent prenatal alteration in Not a permanent prenatal alteration in
bladder anatomy and functionbladder anatomy and function
–– Sustained postnatal bladder over Sustained postnatal bladder over
distentiondistention-- polyuriapolyuria, impaired bladder , impaired bladder
sensation and residual urine volumesensation and residual urine volume
–– Prevent bladder normalization after valve Prevent bladder normalization after valve
ablationablation
–– Bladder Bladder decompensationdecompensation-- upper tract upper tract
dilatation and renal injurydilatation and renal injury
MANAGEMENTMANAGEMENT
KoffKoff et al 2002et al 2002-- 18 boys for 11 yr18 boys for 11 yr
Treat Treat overdistensionoverdistension
–– Daytime alone unsuccessful. Leaves the bladder Daytime alone unsuccessful. Leaves the bladder
full throughout the nightfull throughout the night
–– Nocturnal bladder emptying Nocturnal bladder emptying –– indwelling night time indwelling night time
catheter, intermittent nocturnal catheter, intermittent nocturnal catheterizatoncatheterizaton +/+/--
frequent nocturnal double voidingfrequent nocturnal double voiding
Once nocturnal bladder emptying was started , Once nocturnal bladder emptying was started ,
hydronephrosishydronephrosis markedly improved ( comparable to markedly improved ( comparable to
urinary diversion)urinary diversion)
MANAGEMENTMANAGEMENT
HolmdahlHolmdahl et al 2003:et al 2003:
–– 35 boys with valve35 boys with valve
–– Intermittent CatheterizationIntermittent Catheterization
Improve GFRImprove GFR
Improve bladder compliance and capacityImprove bladder compliance and capacity
INCONTINENCEINCONTINENCE
81 % delayed day and night continence at 81 % delayed day and night continence at
5 years (Smith et al 1996)5 years (Smith et al 1996)
Only 53 % dry at 12 years (Churchill et al Only 53 % dry at 12 years (Churchill et al
1990)1990)
Improves by 20 yearsImproves by 20 years
Psychological impact during adolescence Psychological impact during adolescence
((ParkhouseParkhouse 1988)1988)
INCONTINENCEINCONTINENCE-- CAUSECAUSE
? Sphincter injuries? Sphincter injuries
? Bladder neck dysfunction? Bladder neck dysfunction
MultifactorialMultifactorial –– functional ability exceededfunctional ability exceeded
–– Poor bladder sensationPoor bladder sensation
–– Poor bladder compliancePoor bladder compliance
–– DetrusorDetrusor instabilityinstability
–– PolyuriaPolyuria ––renal tubular damage (several renal tubular damage (several litreslitres/day)/day)
HYDRONEPHROSISHYDRONEPHROSIS
Almost all have severe Almost all have severe hydronephrosishydronephrosis at at diagnosis (96.5%diagnosis (96.5%-- Scott 1985)Scott 1985)
Resolve after relieve of bladder outlet Resolve after relieve of bladder outlet obstructionobstruction
May or may not be associated with VURMay or may not be associated with VUR
Non refluxing Non refluxing hydronephrosishydronephrosis resolves in 49 % resolves in 49 % after Valve ablationafter Valve ablation
50 % have persistent 50 % have persistent hydronephrosishydronephrosis for yearsfor years
55--15 years later 25 % still have 15 years later 25 % still have hydronephrosishydronephrosis(Hulbert & (Hulbert & DucketDucket 1988)1988)
Only 8 % have obstruction at Whitaker testOnly 8 % have obstruction at Whitaker test
HYDRONEPHROTIC UPPER TRACTS HYDRONEPHROTIC UPPER TRACTS
–– 3 TYPES (3 TYPES (GlassbergGlassberg 1982)1982)
Drains independent of bladder volumeDrains independent of bladder volume
Drains only when bladder emptyDrains only when bladder empty
Obstructed independent of bladder volumeObstructed independent of bladder volume
High DiversionHigh Diversion
Failure to improve after valve ablationFailure to improve after valve ablation
No improvement in 2 to 3 weeksNo improvement in 2 to 3 weeks
–– VesicostomyVesicostomy
No improvement after 4 to 6 No improvement after 4 to 6 weeeksweeeks
–– Higher diversionHigher diversion
VESICOSTOMYVESICOSTOMY
Small, sick infantsSmall, sick infants
To small for safe instrumentationTo small for safe instrumentation
Preserve renal functionPreserve renal function
Somatic growthSomatic growth
90 % adequate drainage90 % adequate drainage
10 % inadequate drainage10 % inadequate drainage
? Loss of bladder volume/ capacity? Loss of bladder volume/ capacity
? Decrease bladder compliance? Decrease bladder compliance
UPPER TRACT DIVERSIONUPPER TRACT DIVERSION
Decompress upper tractsDecompress upper tracts
Control infectionControl infection
Long term results Long term results vsvs valve ablationvalve ablation
–– Krueger et al 1980 Toronto experienceKrueger et al 1980 Toronto experience
Better eventual renal function and somatic growthBetter eventual renal function and somatic growth
–– ReinbergReinberg, , DuckettDuckett & & HendrenHendren
No significant differenceNo significant difference
UPPER TRACT DIVERSIONUPPER TRACT DIVERSION
INDICATIONSINDICATIONS
Fail to respond to bladder level drainageFail to respond to bladder level drainage
Renal function deteriorateRenal function deteriorate
Recurrent urinary tract infectionRecurrent urinary tract infection
Increasing Increasing hydronephrosishydronephrosis
UPPER TRACT DIVERSIONUPPER TRACT DIVERSION
INDICATIONSINDICATIONS
Bladder drainageBladder drainage
–– Fall in Fall in creatininecreatinine by 10 % a day to a nadir of < 0.8 by 10 % a day to a nadir of < 0.8 mg/dl (70 mg/dl (70 umol/lumol/l))
–– Assess Assess creatininecreatinine at Day 10at Day 10< 20 mg/dl (176 < 20 mg/dl (176 umol/lumol/l) ) –– continue bladder drainagecontinue bladder drainage
> 20 mg/dl, and > 20 mg/dl, and hydronephrosishydronephrosis not improved, consider not improved, consider upper tract diversionupper tract diversion
Options of Upper tract diversionOptions of Upper tract diversion
–– Loop Loop ureterostomyureterostomy
–– PyelostomyPyelostomy
–– Sober Roux en YSober Roux en Y
IleocystoplastyIleocystoplasty
UreterocystoplastyUreterocystoplasty
Auto AugmentationAuto Augmentation
SeromuscularSeromuscular EnterocystoplastyEnterocystoplasty ––
Sigmoid colonSigmoid colon
THANK YOUTHANK YOU