Pediatric urology : PUV- overview

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

• This obstructive membrane has no active
function,simply a passive barrier to urine
flow
• DR.HAMPTON YOUNG -described and
classified in 1919 .
• Incidence -1 in 25,000 live births
• 10% of urinary obstruction in utero
3
Dept of Urology, GRH and KMC, Chennai.

Posterior urethral valves
Young’s classification system
Type I valves:
Most common type -95%
Ridge lying in floor of urethra continuous with veru .
Takes anterior course and divides into two fork like processes
In the bulbomembranous jn and is directed upwards and
forward attached to the urethra throughout its entire
circumference.
Two leaflets  Type Ia or as a unicuspid leaflet Type Ib
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5

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 Abnormal insertion of the mesonephric
duct into the uro genital sinus
7

Arises from veru and extending along
posterior urethral wall towards bladder
neck
-Non obstructing
-Due to hypertrophy of muscles of
superficial trigone and prostatic urethra
due to distal obstruction.
8

 Can be seen in many obstructive
conditions such as urethral strictures
,posterior urethral valves ,anterior
urethral valves,detrusor-sphincter
dyssynergia.
 Type 2 folds are no longer referred to as
valves.
9

10

-5%
-Membrane lying transversely across
urethra with a small perforation near its
center
-Distal or proximal to veru.
- Distal  Type IIIa or Proximal Type IIIb
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Embryology
-Persistence of the urogenital membrane
-Type 3 valves present in the same manner
and are managed in the same way as
type 1.
Type 3 valves have a worse prognosis.
14

 PUV damage the entire urinary tract
above the valves.
 Reduced Glomerular function
 Abnormal renal tubular function
 Hydronephrosis
 Vesico ureteral reflux
 Vesical dysfunction
15

Bladder
-hypertrophy and hyperplasia of the detrusor
muscle with increased connective tissue.
- The bladder neck is rigid and hypertrophied .
Bladder neck appearance and function improve
after ablating valves
Prostatic urethra
-High voiding pressures distend and thins.
The storage capacity sometimes exceeds that of
the bladder
Verumontanum -distorted
Ejaculatory ducts - dilated due to refluxing urine.
16

Upper Urinary Tract
 Ureter –severe damage.
- direct transmission of pressure from the dysfunctional
bladder
- VUR seen in 70%
Chronicity of ureteral dilatation leads to
 Ureteral wall thickening
 Loss of peristalsis
 Loss of mucosal coaptation
 Increasing the risk of urine stasis
17

Two distinct components.
1) Obstructive uropathy - by persistent high
pressure.
reversible with relief obstruction.
2) Renal dysplasia-
-Increased pressure during the
development of the kidney
- Abnormal embryologic development.
- Not reversible.
18

 Hoover and duckett hypothesized that
the reflux served a pop-off mechanism in
which the dysplastic kidney with reflux
served as a pressure reservoir mitigating
damage to the contralateral kidney
 VURD found in 13 % of valve patients .
 Refluxing kidney is on the left side in 92 %.
 No protective effect on long term renal
prognosis
19

 Prenatal usg
oligohydramnios , Hydroureteronephrosis,
distended bladder, thickened bladder wall .
 Neonates - Pulmonary Hypoplasia
most common cause of mortality in valve patients.
etiology – distended bladder & gross HUN along
with increased uterine pressure reduces
diaphragmatic expansion and affects lung volume
& growth
20

 signs of severe systemic illness
 Intrauterine growth retardation, failure
to thrive, lethargy, and poor feeding
 Classic signs of oligohydramnios: Potter
facies, bowed and deformed limbs, with
pressure dimples over knees and elbows
from intrauterine pressure
21

 40% of neonatal ascites- PUV.
 High intraluminal pressure forces urine to
extravasate usually across a renal fornix, enters the
retroperitoneum and travels across the peritoneum
as a transudate
 The urine within the peritoneum is subject to the
large absorptive mesothelial surface that quickly
normalizes the creatinine and electrolyte values,
masking the identity of ascitic fluid as urine.
 Lowers urinary pressure and offer protection to the
developing kidneys but urinary ascites alone has a
poorer prognosis.
22

 Urinary tract infection
 Voiding dysfunction
 35 % of Patient presenting at school
age -renal insufficiency.
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24

 10% of all fetal uropathy
 Highly sensitive –fetal Hydronephrosis but
specific diagnosis of PUV is more difficult
 Timing of screening for PUV is after 24
weeks
 Renal echogenicity important
parameter
- increased echogenicity
25

 B/L hydroureteronephrosis
 Dilated /Thick walled bladder
 Dilated posterior urethra
 Hypertrophic bladder neck
 Keyhole sign: Dilated bladder above
dilated prostatic urethra.
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27

 Defines the anatomy and gross function of the
bladder, bladder neck, and urethra.
 On Imaging the bladder and upper tracts of
children with neuropathic bladder, urethral
stricture, anterior urethral obstruction, and posterior
urethral valves are identical
 Images during voiding are necessary to make
correct diagnosis
 Bladder is thickened , trabeculated, bladder
diverticula and severe vesicoureteral reflux.
28

• Lateral projection- bladder neck is elevated
, proximal urethra is dilated, and actual
valve structure is often visible
• Crescentic radiolucent defect bulges out
into the contrast column below the veru the
SPINNAKER SAIL appearance-type 1 valve
• Type III valves when they prolapse into the
bulbous portion of urethra gives wind sock
appearance
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30

 MAG-3 (mercaptoacetyltriglycine)-most
useful agent
 functional data ,adequate imaging
of both parenchyma and collecting
system
 Provides differential renal function
Photopenic area -scarring
-Dysplasia
Bladder must be emptied during the entire
study with a catheter- AVOID reflux.
31

 Bladder storage and capacity
 HIGH FILLING /VOIDING PRESSURE
3 Types of Detrusor dysfunction
 MYOGENIC FAILURE :Weak bladder
contraction
PVR :Large
 HYPERREFLEXIA : Unstable bladder
contraction
 POOR COMPLIANT : Reduced capacity
32

 Valve visualisation
 Dilated / Elongated Posterior urethra
 Hypertrophied bladder neck
 Trabeculated bladder
 Diverticulae
 VUR
33

 Initial creatinine and blood urea niteogen
are low due to effects of maternal renal
function mediated through the placenta.
- Test urea and creatinine after 48 hrs -
represent the child's intrinsic renal function.
-Creatinine, blood urea nitrogen, and
electrolyte values- Twice daily for the first
few days of life until they plateau.
- Serum bicarbonate, sodium, and
potassium concentrations are critical
factors
34

• Initial management
- Catheterisation with 3.5fr to 5fr infant feeding
tube/foleys catheter-difficult due to bladder
neck elevation
To avoid placing catheter in the dilated
prostatic urethra- Confirm position with bladder
imaging and one shot cystogram is often
advisable.
-Pulmonary hypoplasia and renal insufficiency:
ventilatory Support,extracorporeal membrane
oxygenation,dialysis,parenteral nutrition,control
of hypertension
35

Child stabilised
PRIMARY ABLATION
OR
DIVERSION
36

 Ind - sr creat < 1.5 mgs
No urosepsis
Urethra admits scope
No other co morbid factors
37

• permanently destroy the valves
• To destroy PUV
-Hooks, balloon catheter and valvulotomes
• Bugbee electrode or pediatric resectoscope with a hook or cold
knife used to incise the valves
• Retrograde –viewed through urethra
• antegrade approach-through vesicostomy or supra pubic puncture
of the bladder.
38

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 Complete resection of valves –stricture
urethra due to electrosurgical and
instrument damage .
 The goal is not to remove the valves but to
incise them so that they are not suspended
across the urethra obstructing urine flow.
 Point of incision-12 O’ clock or some prefer
4 and 8
 Valves are thin ,do not bleed,preferable to
keep the catheter in place for 24 hrs
41

Post PUV ablation
• signs of successful relief of obstruction:
Decreased trabeculation, resolution of
reflux, uniform urethral diameter
• Ratio of diameter of the posterior urethra to
anterior urethra as an indicator of obstruction.
post ablation urethral ratio of 2.5 to 3.0 at 12
weeks is acceptable
• transurethral incision of bladder neck at the
time of valve ablation improve the bladder
performance
42

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INDICATIONS
- Sr creatinine > 1.8 mgs
-Urosepsis
- pt is not responding to catheter
drainage
- infant too small for safe instrumentation
- infants too ill for valve ablation
-Better decompression of kidneys
45

Cutaneous VESICOSTOMY –
Safe and efficient for preserving renal
function
Adequate upper tract drainage in 90%.
No permanent loss of bladder volume.
Technique –BLOCKSOM method.
46

vesicostomy 47

48

Adv -
• Quick & prompt renal decompression
Improve renal function , Reduce infection
Disadvantage-
• Difficult reconstruction later in life as an
additional procedure.
49

 INDICATIONS -Persistant upper tract dilation
after vesicostomy
Scr->2 mg% after 10 days of bladder
decompression.
OPTIONS : Pyelostomy
Pyelo ureterostomy
Ureterostomy- Distal, Proximal
loop, Ring, Sober y ureterostomy
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assess renal function-aspirate fetal
bladder urine-sodium-high-poor function.
-vescicoamniotic shunt
-restoration of amniotic fluid-bet 20-32
weeks
-oligohydramnios earlier than 20 weeks –
incompatible with life
-latter than 32 weeks early delivery is
preferable for fetal intervention
52

50-70% of PUV have reflux
32% is bilateral
Secondary to bladder outlet obstruction
Most reflux resolve within several months,
some can take as long as 3 years.
B/L reflux are more likely to resolve than
unilateral reflux
High grade reflux have renal dysplasia
53

INVESTIGATIONS :
VCUG-residual valves and grade of reflux
UDE- evaluate bladder function
Management
Low grade reflux- Antibiotics
High grade-Surgery – Reimplantation
Higher failure and complication rate.
54

Valve bladder syndrome - coined by Mitchell
Three process contributes to the devolution of
bladder into a valve bladder
1. Polyuria
2. Poor bladder compliance with high pressure
voiding & elevated wall tension
3. Residual urine volume
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 Management-
 Timed voiding
 Alpha blockers- reduces post void
residual urine and improving the
urinary stream .
 Clean intermittent catheterisation
 The mean reduction of residual volume
was 85%
57

Bladder dysfunction-
Peters –three urodynamic patterns
1.Myogenic failure-older children –leads to
over flow incontinence and incomplete
emptying
Management; timed voiding,double
voiding,alpha blockers,and intermittent
catheterisation
58

2.Detrusor hyperrefexia-urinary
frequency,urge incontinence managed
with anticholinergics
3.Decreased compliance/small capacity
Boys with puv if bladder function is not
adequate to protect the upper tracts
CIC,anticholinergics
augmentation cystoplasty
59

 Ileal augmentation-disadvantage
mucous production,risk of rupture,stone
,bladder cancer
 Ureteral augmentation-advantage
Leaving the bladder lined only with the
native urothelium and avoiding
mucous,stone and cancer
60

1) Age at diagnosis- poor <1 yr
2) Presence of reflux- poor
3) USG-amount of Dysplasia
4) Serum bio chemistry-Scr-0.8 mg% at 1st yr
-good
61

THANK YOU
62

Pediatric urology : PUV- overview

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