Pediatric urology :Posterior Urethral Valve (PUV)- diagnosis & management

DIAGNOSIS AND MANAGEMENT OF
POSTERIOR URETHRALVALVE
Dept of Urology
Govt Royapettah Hospital and Kilpauk Medical College
Chennai
1

Moderators:
Professors:
• Prof. Dr. G. Sivasankar, M.S., M.Ch.,
• Prof. Dr. A. Senthilvel, M.S., M.Ch.,
Asst Professors:
• Dr. J. Sivabalan, M.S., M.Ch.,
• Dr. R. Bhargavi, M.S., M.Ch.,
• Dr. S. Raju, M.S., M.Ch.,
• Dr. K. Muthurathinam, M.S., M.Ch.,
• Dr. D. Tamilselvan, M.S., M.Ch.,
• Dr. K. Senthilkumar, M.S., M.Ch.
Dept of Urology, GRH and KMC, Chennai. 2

POSTERIOR URETHRAL VALVES
• Congenital obstructing membranous folds within the
lumen of posterior urethra causing LUTO starts in foetus
in utero.
• Primary tissues mature in an abnormal environment of
high intraluminal pressures and organ distension
Universal injury in the urinary tract
3
Dept of Urology, GRH and KMC, Chennai.

POSTERIOR URETHRAL VALVES
• MC structural cause of urinary outflow obstruction in paediatric
practice
• MC type of obstructive uropathy leading to childhood renal
failure.
• Incidence - 1.4-2.1 per 10,000 live births
• 10 % of prenatally diagnosed hydronephrosis.
• Higher incidence in Blacks
4

HISTORY
• First recognized by Morgagni in 1769.
• Confirmed by Langenbeck in 1802
• First endoscopic description and the term posterior urethral
valve coined by Hugh Hampton Young in 1919
• Young classified valves based on endoscopic appearance into 3
types
• First endoscopic valve ablation by Randall in 1920
5

PUV – CLASSIFICATION
Young classified valves based on endoscopic appearance into 3 types:
Type – 1
• 95% cases
• Valves arise from veru and insert in midline proximal to EUS
• Distal end of WD into UGS
Type – 2
• Valve extends from veru proximally, posterosuperiorly to BN
Type – 3
• Annular ring
• Persistence of urogenital membrane after uro-rectal septum divides
cloacal membrane
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EMBRYOLOGY
Theories
• Hypertrophy of urethral mucosal folds
• Cloacal remnants after division of urogenital membrane
• Abnormal development of WD/MD
• Persistent oblique urogenital membrane
13

PATHOPHYSIOLOGY OF PUV
PUV cause BOO in early life
Bladder hypertrophy
High voiding pressures to maintain complete voiding of
bladder ( compensated phase)
Gradual remodelling of bladder wall ( deposition of ECM )
Further increase in voiding pressures
14

Incomplete emptying of bladder as bladder begins to fail
High PVR
Overflow incontinence
Urine reflux and upper tract dilatation
Further renal damage
Inc urine output
( normal growth
and development)
Polyuria
1. Existing dysplasia
2. Inc glomerular
pressure with
tubular injury
15

Bladder in a state of partial or complete stretch
dilated bladder with poor contractility
( decompensated phase)
16

Lower Urinary Tract
Root cause Dysfunctional bladder
BOO Bladder overdistention with bladder wall remodelling
UUT dilation Renal dysplasia
Features of BOO on cystoscopy
•Posterior urethral dilation
•BN Hypertrophy
•Flattening of veru
•Dilated ejaculatory ducts
17

Upper urinary tract
Ureteral dillation
•Direct transmission of pressure
•VUR  70%
Chronic ureteral dilation
•Wall thickening
•Loss of co-aptation, peristalsis
• Urine stasis/infection
• Intra-renal pressure  Renal dysfucntion
18

Renal dysfunction in PUV
Obstructive uropathy
•Damage to luminal cells in renal tubule
•Concentration defects
•Loss of medullary gradient
•Polyuria
Renal Dysplasia
•In-utero obstruction during renal development
•Irreversible
19

20

VURD Syndrome
Vesicoureteral Reflux and
Dysplasia
13% patients with PUV
Theory
•Dysplastic kidney with VUR
protects C/L kidney
•Pop-off mechanism
Disproved now  C/L
kidney also at risk of renal
dysfunction and ESRD
21

POP-OFF MECHANISMS
• Reflux – VURD syndrome-high bladder pressures on the
refluxing kidney, sparing and protecting the nonrefluxing
kidney.
• Bladder diverticula
• Urinary ascites
• Patent urachus -RARE
22

POP- OFF MECHANISMS
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PRE-NATAL DIAGNOSIS
• 1 in 1250 on USG
• 10% of all antenatal GU
anomalies
• 1/3rd of B/L renal disease
• Pathognomonic Findings
B/L HUN
Oligohydramnios
Dilated posterior urethra
(KEY-HOLE SIGN)
renal echogenecity
26

OLIGOHYDRAMINOS-DEFINATION
• Amniotic fluid volume < 500ml at 32-36wks gestation
• AFV < 5th percentile for gestational age
• AFI < 5 ( normal= 7-25 )
• Single vertical pocket < 2cms
27

AMNIOTIC FLUID INDEX
• Four quadrant technique
• AFI is measured by
dividing the uterus into four
quadrants
• Linea nigra- divides into
right and left halves
• Umblicus serves as the
dividing point for the upper
and lower halves
• AFI= A+B+C+D Each individual pocket of fluid
should be= 2-8cm
28

FETAL MRI
– To distinguish the degree of
obstruction based on urethral
dilation
– Distended bladder size with
thickening
– Reduced amniotic fluid levels.
• Lung hypoplasia and cystic
changes in renal parenchyma
are also apparent on MRI
• Do not provide added
analysis of causes of
obstruction, in diagnosing the
actual cause of LUTO
29

MANAGEMENT- APPROACH AT BIRTH
• Management starts immediately after birth
• Establish urinary drainage( 5-6fr feeding tube/coude tip )
• Sample for urine routine and urine c/s collected
• IV line secured and antibiotics started/ IV fluids
• Blood sample withdrawn after 48-72hrs for CBC, S.
creatinine, and electrolytes
• Look for dehydration and acidosis with other electrolyte
imbalance
30

MANAGEMENT-APPROACHAT BIRTH
Sonography KUB:
• Kidneys for cortical echogenicity, CMD, degree of HN,
• Dilatation of ureters
• Bladder for distension, thickness, presence of diverticula,
high bladder neck with hyperplasia
• Posterior uretheral dilatation,
• Urinoma, urinary ascities and other anomalies
31

Urinoma  3-10%
•Forniceal rupture
•Trans-peritoneal transudation
•Bladder rupture
Neonatal ascites
32

MANAGEMENT-APPROACHAT BIRTH
• VCUG- is a definitiveinvestigation
• Timing: hemodynamicallystable
• Antibiotic prophylaxis: not needed if urine c/s report is sterile and within
72hrs of this sterile report
• How to make baby to urinate: suprapubic massage, pinching glans,
sprinkling cold water on glans
o Condition of bladder, diverticula
o Reflux
o Bladder neck hypertrophy
o Dilatation and length of posterior urethra
o Abrupt funneling and configurationof obstruction and its level
33

MANAGEMENT- APPROACH AT BIRTH
• Definitive study  VCUG
• Findings on VCUG
Thickened trabeculated
bladder with multiple diverticulae
High grade VUR – 50%
Hypertrophied elevated bladder
neck
Grossly dilated posterior urethra
Abrupt funneling of urethra at
level of valve
34

MCU COMPLICATION
• Rupture of bladder due to over
distension
EBC-
 NEONATES(< 1 mth);
infants(1mth-1yr)= 7x wt in kgs
 Rest capacity= (age+2)x30,
capacity in mls
5-6fr catheter, passed aseptically.
Water soluble contrast 20%(w/v),
slow filling with gravity under
fluoroscopic control
• Other complications: dysuria,
infection, hematuria, retention of
urine
35

RADIONUCLIDE RENAL SCAN
• The radionuclide renal scan offers
• quantification of differential renal function
• cortical deficits seen on the study may imply renal dysplasia.
• MAG3 or DMSA
• Placement of a urinary catheter is essential in a patient
with VUR to minimize error in the calculation of renal
function.
36

• Definitive management
Cystoscopy + Endoscopic valve ablation  First line option
To be done in hemodynamically stable pts
37

ADVANTAGESOF EARLY VALVEABLATION
• Bladder cycling, obstruction free drainage
• Thus improves
- Complaince
- Bladder stability in infancy with a possibility of less
bladder dysfunction in older children
38

PUVFULGRATION-PRECAUTIONS
• Use appropriate size equipment
• Pure current
• Engage leaflets adequately for optimum cut
• Never an over doing i.e avoid aggressive resection
39

PUVFULGRATION-INSTRUMENTATION
• Urethrocystoscopes- mostly 7-7.5fr used
• Resectoscopes- 9.5fr
40

BUGBEE- ELECTRODE
41

PUV FULGRATION- WHERE TO ABLATE
• 5 and 7o’clock- most time
• 5,7 and 12o’clock- if
promonent 12o’clock
leaflet
42

Fogarty or Foley's balloon catheter
• A size 4 Fr Fogarty balloon catheter or an appropriate size
Foley's catheter is placed into the bladder
• balloon inflated with 0.75 ml of saline.
• With gentle withdrawal, the operator places the balloon at
the level of the valves.
• Sharp withdrawal of the catheter ruptures the membrane
without injury to the urethra.
• This procedure should be preferably done under
fluoroscopic or ultrasonographic guide to ensure that the
balloon is only inflated proximal to the valve to avoid
urethral injury.
43

44

PUV FULGRATION- FOLLOW UP
• Post fulgration= in 2-3days, usg/s.creatinine/s.electrolytes
• Monthly urine analysis, urine c/s, ht and wt
• 3 monthly USG KUB to look for
- dilatation of PCS and ureters
- residual urine within the bladder
• Repeat MCUG at 3 months for any residual valve
• Cystoscopy only if MCUG shows persistent significant
obstructive changes
• DMSA at 3 months for cortical functiopn and quantification
of scars
45

• Vesicostomy
•VLBW infant not accomodating endoscope
•Severe urosepsis
•Failed ablation
Continued impaired renal function
High bladder urine volumes
Upper tract deterioration
•BLOCKSOM TECHNIQUE
Dome of bladder fixed to fascia
Most important Ensure taut posterior wall
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• Supravesical Diversion
•Direct low pressure renal drainage to optimise renal function
•Indication Complete decompression of the lower
urinary tract with
Worsening renal function
Increasing upper tract dilation
Clinical picture of sepsis
•Possible cause  Compression of intramural ureter by
thick walled bladder
•Disadvantages
Complicatedundiversion procedure needed
Defunctionalised bladder Loss of contractilityand compliance
51

vesicostomy End stomal
ureterostomy
loop ureterostomy
52

Sober Y
ureterostomy
Ring ureterostomy
Cutaneous
pyelostomy
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• Nephroureterectomy
•Historically for VURD
•Nowadays only in intractable UTI
•Preserve ureter for later use in augmentation
56

Management of VUR
•Seen in 50-80% cases
•Resolves in 25-40% post valve ablation
•Focus Maintaining low storage pressures
•High complication rates of VUR surgery
Persistence of VUR:
• Residual valve ( 15-33%)
• Altered bladder dynamics mainly low complaint bladder
• Paraureteric diverticulum
57

Bladder neck incision
•No evidence of benefit
•Not recommended routinely
Circumcision  Recommended
-Risk of UTI  50-60%
-Incidence reduced by 90% with circumcision
-should certainly be completed before giving any consideration to a
ureteral reimplant in a scenario of frequent febrile urinary tract
infections despite conservative measures.
58

BLADDER DYSFUNCTION IN PUV
• Cascading pathological changes
•3 distinct evolutionary patterns
Infancy and Early Childhood
•Detrusor hyperreflexia
Late Childhood
•Decreasing intravesical pressures and improving
compliance
Adolescence
•Increased capacity bladder with hypocontractility and
atony
59

• Long term follow-up mandatory
Clinical examination
Periodic assessment of voiding  Observation, UFR, PVR,UDS
Upper tract assessment  USG
•Bladder Management
Voiding training
•Timed voiding and Double voiding
•CIC if necessary
Anti-cholinergics
•Oxybutynin 0.1mg/kg preferred
•Stop if no effect or high PVR
α – blockade
•To relax bladder neck
•Tamsulosin 0.2mg
60

• Valve Bladder Syndrome
Coined by Mitchell in 1982
Worsening renal function and  HUN
No evidence of BOO
Three important determinants
•Polyuria
•Poor bladder compliance with high-pressure voiding and elevated wall
tension
•Residual urine volume
61

62

VALVE BLADDER- DEFINITION
• Low capacity
• High pressure
• Low complaince bladder with
• Upper tract deterioration
• In the absence of outlet obstruction
63

• Management
Routine bladder management
CIC mandatory
Nocturnal bladder drainage
•Breaks the cycle
•Extender period of bladder decompression
Inability to catheterise  Appendicovesicostomy
(Mitrofanoff Procedure)
Refractory to therapy with small contracted bladder
•Augmentation cystoplasty
•Ureteral augmentation preferred if possible
64

INDICATORS OF RENAL FUNCTION
•Incidence of ESRD  20-50%
•Nadir creatinine at 1 year of age
< 0.8  Minimal risk
> 1.2  High risk
•Other factors
Age at diagnosis
Renal dysplasia
Recc UTI
Bladder dysfunction
65

FAVOURABLE PROGNOSTIC FACTORS
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TRANSPLANTATION IN PUV PATIENTS
• The 2006 annual report of the North American Pediatric
Renal Trials and Collaborative Studies listed obstructive
uropathy as the second most common cause for
transplantation, accounting for 1424 of 8990
transplantation cases (15.8%) since 1987 (Smith et al,
2007).
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Several comorbidities:
• High-grade vesicoureteral reflux into native,
nonfunctioning kidneys and valve bladder syndrome with
a thick-walled, poorly contractile or hypercontractile
bladder.
• The thickened bladder wall of PUV patients may
contribute to the significantly increased incidence of
ureteral obstruction
71

RENAL Tx IN PATIENT TREATEDFOR PUV
72

• Video-urodynamics should be obtained for transplant
candidates to determine
– the safe storage pressures
– contractile function of the future reservoir.
• Overnight bladder drainage or CIC - prior to
transplantation to optimize the reservoir and establish
proper bladder management.
• Pretransplantation nephrectomy- rarely required and only
considered in cases in which proteinuria or severe
polyuria is creating hemodynamic challenges.
73

• Augmentation is necessary if unsafe storage pressures in
the bladder.
• Pretransplantation augmentation is preferable
– to prevent the undertaking in a immunocompromised child
– one too young to take responsibility for catheterization and pouch
management,
• Recent experience argues that transplantation into even a
vesicostomy is a safe alternative until the child grows to
an appropriate age for cystoplasty and the attendant
reliance on CIC.
74

AUGMENTATION OF BLADDER
PRE TRANSPLANT
• Augment before
immunosupression
• To young to take
responsibility of CIC or
manage stoma
• Increase risk of UTI
POST TRANSPLANT
• Risking graft loss by doing
Tx in unsafe bladder
• Tx in vesicostomy is safe
• Later cystoplasty and CIC
can be practiced
effectively and safely as
child is more prepared
and complaint
• Infection rates are less or
same
75

ANTENATAL INTERVENTION
CONTROVERSIAL
• Accurate diagnosis with current technology is difficult
• Natural history of each disease process causing AH is
variable and has not been fully elucidated
• Lack of data regarding the success and complications of
intervention in cases of PUV
76

WHY??
• An infant affected by
posterior urethral valves
may be affected by serious
comorbidities
– pulmonary hypoplasia
– physical stigmata of
oligohydramnios, including
• Potter facies
• clubfeet and deformed
hands
• poor abdominal muscle tone
• require intensive initial
management.
77

ANTENATAL INTERVENTION
•Indications
Oligohydramnios
Dilated bladder with severe HUN
No renal cortical cystic lesions
Normal Karyotype
Fetal urine analysis after 20 weeks ( 2-3 samples at the
interval of 24-48hrs)
•Na < 100meq/L
•Cl < 90 meq/L
•Osmolarity < 200meq/L
•β2 microglobulin < 6mg/L
•43% fetal mortality rate
78

VESICOAMNIOTIC SHUNTING
• Improves survival in worst cases by improving pulmonary function
and renal function
• But high complication rate:
- 40% migration, obstruction, and displacement
79

FETAL CYSTOSCOPY
• 8.8% fistula formation rates
• 5.9% recurrence of LUTO
• Preterm delivery and death after birth
80

SFU- STAGES OF FETAL LUTO
81

PLUTO TRIAL
• Percutaneous vesicoamniotic shunting vs conservative
management for LUTO
• It was RCT
• Paucity of data: poor recruitment and pregnancy
termination
• Only 12 live births in each group
• Improved survival in shunted group for at 28 days, but
overall survival was same
• High mortality bec of pulmonary hypoplasia
• Greater risk of pregnancy loss and maternal
complications
82

PLUTO TRIAL
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FINAL TAKE
• Primary valve ablation is the preferred T/t
• Those unstable bladder- bladder level diversion
• Supervesical div.- pts who fail to respond to bladder level
diversion
• Diversion yields similar results- disadv of needing more
surgical procedure
• At puberty
- 2/3rd progress to CKD
- 1/3RD progress to ESRD
• PUV- is it never ending story??
• Life long follow up
“Primary lesion is simple to treat but total care of boy with PU
valve is complicated undertaking”- SIR DAVID INNES WILLIAM
86

THANK U
87

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