2. Clinical Presentation
Boys with PUV can present with a variety of symptoms
and at various ages
They range from newborns with life-threatening renal
and pulmonary conditions to older children with minor
voiding dysfunction
In general, the symptoms are age dependent; the more
severely affected boys present earlier in life
Antenatal hydronephrosis diagnosed with prenatal
ultrasonography is the most common presentation
3. Clinical Presentation
Obstruction leads to decreased fetal urine output and
results in oligohydramnios
The observation of marked hydro, a distended bladder,
and a thickened bladder wall in utero strongly supports
the diagnosis of valves
Neonates with sever PUV can present with Pulmonary
Hypoplasia , intrauterine growth retardation, failure to
thrive, lethargy, and poor feeding
Older children can present with urinary tract infection,
voiding dysfunction or renal insufficiency
4. Approach
Hx :Pre and postnatal
Exam: Abdomen,genitalia and back
Blood work: CBC,lytes,Cr,?acid base
Bladder drainage
Abx prophylaxis
US
VCUG
Careful monitoring
9. VCUG
Bladder is thickened and trabeculated, diverticula
may be present, bladder neck is elevated and the
proximal urethra is dilated, and the actual valve
structure is often visible
Vesicoureteral reflux is present in at least 50% of
valve patients at the time of diagnosis
The incidence of reflux has been found to be higher
in neonates than in older children
There is an 80% incidence of reflux on the left side in
patients with unilateral reflux for no apparent reason
10.
11.
12.
13. Investigations
Initial laboratory evaluation of the newborn with valves is
usually misleading because of the effects of maternal renal
function mediated through the placenta
It will take at least 48 hours for the serum levels of creatinine
and blood urea nitrogen to accurately represent the child's
intrinsic renal function
Creatinine, blood urea nitrogen, and electrolyte values should
be determined twice daily for the first few days of life until they
plateau
14. Bladder Drainage
Initial management of all patients with PUV requires immediate
bladder drainage
This should be performed even if the diagnosis has not been
confirmed by VCUG
Neonates can be catheterized with a 3.5 or 5 French pediatric feeding
tube
Foley catheters have been used with success, but there have also been
reports that the balloon causes irritation and resultant bladder spasms
After successful initial bladder drainage and when the patient's
medical condition has stabilized, the next step is to permanently
destroy the valves
15. Valve Ablation
Transurethral valve ablation is the 1st
treatment choice
A Bugbee electrode or a pediatric resectoscope with a hook or cold
knife can be used to incise the valves
A number of authors report use of a cystoscope and laser to disrupt
valves
Some surgeons prefer incision at 12-o'clock position; others prefer
incisions at 4- and 8-o'clock, and others all three
Although most valves are thin and do not bleed at surgery, it is
preferable to leave a catheter in place for 24 hours after incision
The valve remnants resolve after incision, and there is often no
evidence of them on later cystoscopic examination
16.
17. Cutaneous Vesicostomy
If the infant is too small for safe instrumentation for valve
ablation, a cutaneous vesicostomy can be performed as a
temporary measure
The vesicostomy provides adequate drainage of the upper
tracts in more than 90% of cases
There has been concern that vesicostomy would cause
permanent loss of bladder volume, but this has not proved
to be true, and vesicostomy does not significantly affect
bladder capacity
18.
19.
20. Upper Tract Diversion
There is controversy about the superiority of upper tract diversion vs.
vesicostomy regarding long-term results and measured renal function,
bladder function, and somatic growth in each group
the current consensus is that neither initial treatment is superior in
promoting renal function and somatic growth
The current consensus is that both approaches eventually yield similar
results and that infants who undergo initial upper tract diversion are at
the disadvantage of needing more surgical procedures
Today, upper tract diversion is usually limited to those patients who fail
to respond to bladder-level drainage
Upper tract diversion Is considered if bladder-level drainage is
insufficient to prevent infection or to drain the upper tracts adequately
21. Upper Tract Diversion
If the serum creatinine concentration drops below 2.0 mg/dL (150
μmol/L), it is safe to rely on improved bladder drainage for additional
kidney improvement
If the creatinine concentration remains above 2.0 mg/dL (150 μmol/L)
after 10 days of adequate bladder decompression and if
hydronephrosis is unimproved, upper tract diversion may be
considered
The type of diversion remains the surgeon's choice, options are high
loop ureterostomy, ring ureterostomy, pyelostomy, and end
ureterostomy
If upper tract diversion is performed, reconstructive surgery to
internalize the urinary tract should be delayed until the bladder and
upper tracts have improved as much as can be expected
22.
23. Management of VUR
Reflux in PUV is considered secondary to bladder
outlet obstruction
the initial management of reflux is relief of
obstruction
Reflux resolves after valve ablation in between 20%
and 32% of refluxing ureters
Most reflux resolves within several months, but some
can take as long as 3 years
Reflux is more likely to resolve when it is associated
with a better functioning kidney
24. Management of VUR
Children with initial bilateral reflux are more likely to
have reflux resolve than are those with unilateral reflux
As for any child with vesicoureteral reflux, they must be
maintained on prophylactic antibiotics to prevent
infection
If persistent high-grade reflux is a clinical problem
because of urinary tract infections or incontinence,
bladder function and drainage must be reviewed
Inadequate emptying and high storage pressures are the
usual causes of persistent reflux
25. Management of Hydronephrosis
Nonrefluxing hydronephrosis resolves in 49% of patients
and may do so rapidly after valve ablation
This leaves a significant population of valve patients with
persistent hydronephrosis for years despite adequate
bladder emptying
The majority of patients with persistent hydronephrosis
do not have obstruction at either the bladder outlet or the
ureterovesical junction
26. PROGNOSTIC FACTOR
Good Factors
• Nadir creatinine < 0.8 mg/dl
• S. creatinine < 1 mg/dl
• Pop-off mechanism
- VURD
- Ascitis
- Large bladder diverticulum
27.
28.
29. Bad Factors
• Age
• Delayed correction
• GFR < 50 % of normal in infancy
• VUR
- Bil -----> 57 % mortality
- Uni. -----> 17 %
- Non -----> 9 %
• Loss of cortico medullary junction
• delayed incontinence beyond 5 years
30.
31. Summary
PUV is one of the trickiest conditions to treat
It requires a very careful initial and long-term
management
Our goal is to preserve all remnant kidney function by
relieving obstruction and preventing infection
Special attention and long-term bladder management is a
key in treating those patients