The Treatment Sequence in Bladder Exstrophy

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The Treatment Sequence inBladder
Exstrophy
philip ransley
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What do you see ?
What do you think about ?
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The Exstrophy Jigsaw
Penis
Sex
Fertility
Age
Osteotomy
CIC
Voiding
Augment?
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Terminology
Continence Voiding Residual
Dryness Augmentation Cystoplasty
Colocystoplasty
Ileocystoplasty Mitrofanoff
CIC Bladder Bladder Neck Verumontanum
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The Dilemma
The struggle For Voiding continence
The elegant Security of Augmentation Mtroff CIC
risks
risks
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Pleasewould someone show me..
An 18-30 y Male Exstrophy With Umbilical
Reconstruction who Is Dry Day and Night Who
does not wear protection Who does not get up at
night Who can sit through a major epic film Who
empties by proven detrusor contraction And has
good sex
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Contemporary Problems
Diminishing Numbers Increased Expectations Dilut
ed Experience
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Ambition
To provide the best solution for an individual
child as early as possible with the fewest
operations and the least suffering. Recognising
that Unrealistic Ambition creates unnecessary
hardship and disappointment. Not all bladders
are the same just as no two diabetics are alike.
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Reflections
Sorting the jigsaw puzzle Sequence Outcome
Tempered ambition
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Jigsaw Pieces

• Osteotomy

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The Exstrophy Jigsaw

• Osteotomies Anterior Oblique
• Overall Cosmesis
• Penis
• Vaginal Prolapse
• Hips
• Gait
• Age.. Neonatal or later ?

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The Exstrophy Jigsaw
Osteotomies Anterior Oblique Outside the
neonatal period But before weightbearing External
fixator Frog plaster
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Jigsaw Pieces

• Osteotomy
• Outflow Resistance
• Bladder Neck
• Sphincter

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Natural Sphincter

• Mitchell Total Disassembly
• Kelly Procedure

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Bladder Neck Reconstruction

• A Misnomer ?

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The Young-Dees-Leadbetter

• Is Trigonal Tubularisation
• .Not a bladder neck reconstruction

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Disturbs Contractility
Creates Fixed Resistance
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Fixed Outflow Resistance
No Stress Leak
Obstructed Voiding
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Obstructed Voiding

• . Leads to
• Detrusor Failure

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Pleasewould someone show me..
An 18-30 y Male Exstrophy With Umbilical
Reconstruction who Is Dry Day and Night Who
does not wear protection Who does not get up at
night Who can sit through a major epic film Who
empties by proven detrusor contraction And has
good sex
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The Exstrophy Jigsaw
Outflow Resistance Gentle Smooth
Progressive
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The Alternatives
Rely on a developed preserved BN
sphincter..Mitchell / Kelly Loose BN surgery
below the trigone and boost with Injectables (
AUS )
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Mood
Relax Inform Contract Hold on Co-ordinate
Urine
Temp
Skin
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B N R
Capacity Compliance Must develop rapidly
Bladder Neck Reconstruction Has some problems
Must leak at night
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One Additional Factor
The site of the continence mechanism Must be at
or above the veru Or Epididymitis follows .
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Bulking Agents

• Poor Reputation
• Require Critical Patient Selection
• Anatomy is Important

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Jigsaw Pieces

• Osteotomy
• Bladder Outflow Resistance
• Epispadias Repair

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Jigsaw Pieces

• Osteotomy
• Bladder Outflow Resistance
• Epispadias Repair
• Augmentation
• Cup Colocystoplasty
• RIF Mitrofanoff

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Hydrostatic pressure
No Siphon
. . . .
. .
. . .
.
Mucus
Siphon
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Flush Stoma
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VQZ Stoma
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VQZ Stoma
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Our Journey Begins
Together
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Closure lt48h
Bladder only No osteotomies Frog Plaster 1
month
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PGR Treatment Sequence
Neonatal Closure w/o Osteotomies _at_ 3/12
Ultrasound EUA Cystoscopy
/cystogram Testosterone 25 mg X 3 _at_ 6-8/12
Osteotomies Epi Repair Loose
BNR
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PGR Treatment Sequence
Neonatal Closure w/o Osteotomies _at_ 3/12
Ultrasound EUA Cystoscopy
/cystogram Testosterone 25 mg X 3 _at_ 6-8/12
Osteotomies Epi Repair Loose
BNR
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Treatment Sequence 2
_at_ 9/12 Ultrasound and bladder assessment _at_
12/12 Cystoscopy / Cystogram and
bulking agent for progressive
resistance
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If You are Making Progress..
Then the sequence can continue And Anticipate
natural progress at Puberty However.if you are
not.
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Not all patients with bladder exstrophy will
achieve continence with voiding circa
25 Some exstrophy patients are best
served by continent diversion with a Mitrofanoff
channel for intermittent Catheterisation at an
early age .
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Augmentation / Mitrofanoff
_at_ 6 /12 _at_ 12 /12 _at_ 3 5 years _at_ 10
years After 18 years ( success or failure ? )
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Continent Urinary Diversion

• In The Very Young

philip ransley
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Early Continent Diversion
11 cases 6 36 months
median 13 months Exstrophy 7 Cl
Exstrophy 2 Valve/Reimplant 1
Reimplant(s) 1
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Exstrophy Early Augmentation
Initial Failure Capacity / UT diln
Later Failure Continence/Capacity
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Bladder Exstrophy n7
Early Failure 4 Late Failure 3
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Bladder Exstrophy n 7
Ost Mitrf Augmnt Bldr Nk Other
TUU . Epi
C C I/C C C C I/C


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Augmentation in the very young
Adequate Parents Dedicated Anaesthesia ITU
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AUS In Exstrophy/Epispadias
N 20 17 male 3 female 13
exst 7 epi F / U 3 10 y
mean 6.25y
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AUS In Exstrophy/Epispadias

• 4 removed
• 16 4 cic
• 12 void
• 6 augmented void

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Pleasewould someone show me..
An 18-30 y Male Exstrophy With Umbilical
Reconstruction who Is Dry Day and Night Who
does not wear protection Who does not get up at
night Who can sit through a major epic film Who
empties by proven detrusor contraction And has
good sex
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It is crucial that
We develop centres of excellence We promote
centres for adult follow up We take note of what
is found
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Treatment Sequence 2
_at_ 9/12 Ultrasound and bladder assessment _at_
12/12 Cystoscopy and bulking agent Sequential
upper and lower tract assessment
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Bladder Neck Reconstruction

• Any procedure which requires
• Ureteric Reimplantation
• Is not a bladder neck reconstruction !

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PGR Treatment Sequence
Neonatal Closure w/o Osteotomies _at_3/12
Ultrasound EUA Cystoscopy
/cystogram T 25 mg X 3 _at_6-8/12 Osteotomies
Epi Repair BNR
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Early Continent Diversion
11 cases 6 36 months
median 13 months Exstrophy 7 Cl
Exstrophy 2 Valve/Reimplant 1
Reimplant(s) 1
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PGR Treatment Sequence
Neonatal Closure w/o Osteotomies _at_3/12
Ultrasound EUA Cystoscopy
/cystogram T 25 mg X 3 _at_6-8/12 Osteotomies
Epi Repair BNR
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Treatment Sequence 2
_at_ 9/12 Ultrasound and bladder assessment _at_
12/12 Cystoscopy and bulking agent Sequential
upper and lower tract assessment
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Dilemma 2
A classical treatment sequence A newer approach
Mitchell
Kelly