This document provides an overview of the evaluation and management of posterior urethral valves. It begins with an introduction discussing the history and embryology of PUV. It then covers the clinical presentation, diagnostic evaluation, management including in utero and postnatal approaches, complications, and long-term outcomes. Key points include that PUV causes obstructive changes that damage the urinary tract, early diagnosis and relief of obstruction is important to preserve renal function, and bladder dysfunction often persists long-term requiring lifelong management.
2. INTRODUCTION
Langenbeck : first reported congenital obstruction of the prostatic
urethra in 1802.
Hugh Hampton Young: define and name the condition as posterior
urethral valves(1919).
Incidence is 1.6 to 2.1 per 10000 births.
PUV M/C cause for LUTO followed by urethral atresia and prune belly
syndrome.
3. INTRODUCTION
1 in 1250 fetal ultrasound screenings.
Inheritance is poorly understood.
Affect siblings, twins, and successive generations.
They become obstructive during or after the eighth week of life.
When PU has developed
4. GENETIC BASIS
Short arm of chromosome 11
Inheritance may be autosomal recessive,x linked recessive
6. EMBRYOLOGY
TYPE-1:(95%)abnormal insertion of the mesonephric
ducts into the fetal cloaca.
TYPE-2: hypertrophy of muscles of the superficial
trigone and prostatic urethra in response to high
voiding pressure from distal obstruction.
9. Pathophysiology
Bladder dysfunction is usually a lifelong problem resulting in
incontinence and poor emptying.
Persistent hydronephrosis is common and may be due to either ureteral
or bladder dysfunction.
Reflux usually improves and often resolves after valve ablation.
10. Pathophysiology
Posterior urethral valves damage the entire urinary tract proximal to the
valve.
Pulmonary hypoplasia is the most common cause of mortality in valve
patients.
Most renal damage occurs early in fetal life.
11.
12. EFFECTONBLADDERDEVELOPMENT
With puv normal bladder
recycling fails and
function is altered
Initial period:near
complete bladder
emptying with high
voiding pressures
Compensated
phase:impairement in
bladder capacity and
compliance
Decompensated phase:
bladder capacity
compliance,contractility
27. INDICATIONS OF UDS
Persistence of day time incontinence beyond 5 years of age
Increasing upper tract dilatation
Deterioration of renal function
To asses the efficacy of medical treatment
Prior to renal transplant to optimize the result
28. IN UTERO
1ST STEP-( U.S.G.)
Evaluated bladder before and after fine needle vesicocentesis.
Rule out other anamalies like NTD,CVS.
Kidney condition(large hyperechogenic or small hyperechogenic).
30. IN UTERO
2ND STEP:-
Prenatal evaluation for fetal karyotype.
Amniocentesis if fluid available.
FINAL STEP:-
Evaluation of fetal kidney function with sequential vesicocenteses
31. IN UTERO
Completely drain fetal bladder at 48-72 hr intervals at a minimum of
three occasions.
progressive hypotonicity and values that fall below threshold benefit
from in utero intervention i.e. shunt placement.
32. Prognosis
Outcome depends upon severity
Classified as good or poor
Poor prognostic factors :
- Diagnosis before 24 wks
- Oligohydramnios
- Renal dysplasia
- Marked hydronephrosis
34. PLAN
Poor prognosis group – may offer termination or conservative
management.
Fetuses with normal fluid and stable hydronephrosis – serial u/s until
delivery.
Fetuses with good prognosis – placement of VAS with Rodeck catheter
(double pigtail) or fetoscopic/endoscopic valve ablation
35. Vesicoamniotic Shunt
Non cystic kidney
Bilateral hydronephrosis
Single pregnancy
Decreased amniotic fluid volume
Absent other anomalies
46 XY karyotype
Na<100 Ch<90 Osmolarity<210mosm/dl
IDEAL TIME FOR INTERVENTION BETWEEN 20-32 WEEKS
38. PLUTO TRIAL
“PLUTO TRIAL comprehends the fact that
Vesicoamniotic shunting may have a survival
benefit for the infant in select cases,but there is no
clear benefit in the risk for renal failure.”
LIMITATIONS:
Poor enrolment
Pre mature termination of trial
39. PLAN
Consultation with pediatric urologist
Route of delivery – routine obstetric indications
Average age of delivery due to spontaneous rupture of membranes =
33-35 wks
Following delivery – sterile ostomy bag to abdomen until renal
function and anatomical evaluation by pediatric urologist.
41. Postnatal Ultrasound
To evaluate the effect of PUV on the urinary tract rather than to
diagnose PUV.
Typical finding: wide prostatic urethra,thick-walled bladder and upper
tract dilatation.
Assessment of renal parenchyma.
46. Bladder Drainage
By infant feeding tube or catheter.
Monitor Creatinine, BUN, and electrolytes should be followed twice
daily for the first few days of life until they plateau.
Serum bicarbonate, sodium, and potassium are all critical factors to
monitor.
47. Valve Ablation
By hooks,balloon catheters, and valvulotomes.
A bugbee electrode or a pediatric resectoscope with a hook or cold
knife.
Incision prefer 12 or 4 & 8 o” clock position.
leave a catheter in place for 24 hrs.
49. Re-evaluation
By cystoscope and VCUG after 12wks.
VCUG debatable but use to see urethra, bladder capacity & reflux.
signs of successful relief of obstruction
- Decreased trabeculation
- Resolution of reflux
- Uniform urethral diameter
- PUD/AUD ratio 2.5 to 3
55. COMPLICATION AND ITS
MANAGEMENT
I. RENAL DYSPLASIA
II. RENAL FUNCTION
III. RENAL TUBULAR FUNCTION
IV. HYDRONEPHROSIS
V. VUR
VI. VESICAL DYSFUNCTION
VII. VALVE BLADDER
56. Renal Dysplasia
Histological diagnosis
Cause 1- high pelvic pressure during nephrogenesis
2-primary embryologic abnormality from abnormal position of
uteteric bud .
Severity will determine ultimate renal function
57. Renal Function
Children with PUV may demonstrate gradual loss of renal function over time
Cause:
1- Renal parenchymal dysplasia
2- Incomplete relief of obstruction
3-parenchymal injury from :
* UTI
*HTN
*Progressive glomerulosclerosis from hyperfiltration
* Obstruction
58. Renal Tubular Function
50% of patients with PUV have impairment concentration ability
Persistently high urinary flow rate regardless of fluid intake or state
of hydration
severe dehydration and electrolyte imbalance
ureteral dilatation and high resting vesical pressure
59. ESRD
Occurs in 25% - 40%
1/3 soon after birth
2/3 during late teenager
Predictor : -Nadir serum creatinine level
Severe bladder dysfunction
Bilateral reflux
60. primary goal of management is preserve renal function and to
maximize renal growth and development.
provide low-pressure storage and drainage of urine to prevent
further renal damage.
Monitor bladder function, control infection & HTN.
61. Hydronephrosis
Significant urethral obstruction variable degree of ureteral dilatation.
After relief of obstruction : gradual but substantial reduction of
hydronephrosis .
If not reduced we have to rule out:
1-High intravesical pressure
2- ureteral muscle weakness
3- UVJ obstruction
62. Vesicoureteral Reflux
Secondary to B.O.O.
resolves after valve ablation in between 20% and 32%
If persistent high-grade reflux bladder function and drainage must be
reviewed.
64. Vesical Dysfunction
Usually secondary to irreversible change in organization and function of
the smooth muscle from outlet obstruction
Present as as urinary incontinence (20%)
Bladder dysfunction persist in 75 % of cases after
valve ablation
65. VALVEBLADDER
Definition:
A CHRONIC CONDITION IN VALVE PATIENTS WHERE DESPITE
SUCCESFUL VALVE ABLATION,INTRINSIC BLADDER
DYSFUNCTION LEADS TO DETERIORATION OF THE UPPER
URINARY TRACTS AND INCONTINENCE.
67. VALVE BLADDER
term coined by Mitchell.
HIGH
VOIDING
PRESSURES
INCREASED
URINE
PRODUCTI
ON
NEPHROGENIC
DIABETES
INSIPIDUS
NO PERIODS
OF
RELAXATION
DETRUSOR
FIBRES
INJURY
IMPARED
CONTRACTILIT
Y
69. VALVE BLADDER
Even after relief of obstruction a significant number of patient will
continue to have hypertonia and detrusor hyperreflexia and low
compliance
Physiological obstruction of the ureter associated with bladder filling
persistent hydronephrosis and/or urinary incontinence
70. MANAGEMENT OF BLADDER DYSFUNCTION.
Intensively individualized
Close monitoring by
-Urodynamics
- Urine volumes
- Renal function
-Infections
-Hydronephrosis
72. INDICATIONS OF BLADDER
AUGMENTATION
Poor bladder compliance
Failed medical management/anticholinergics
Repeated UTI
Persisting VUR threatening kidney function( GFR)
73. BIOFEEDBACK AND PELVICFLOOR
EXERCISE
Provide significant and durable relief for persistent lower urinary tract
dysfunction
Consistent good response seen in 70% of patients
Alpha blockers/terazosin(0.25-2mg) can be added for bladder
empyting.
INDIAN JOURNAL OF UROLOGY OCT-DEC 2010 VOL 26 ISSUE 4
74. ROLE OF CIC
Increases GFR
Decreases UTI
Decrease upper tract deterioration
Increase continence
Increase compliance
76. TRANSPLANTATION IN
VALVE PATIENTS
50% of puv patients will have CRF/ESRD
Mean age of ESRD is 15-20 years
Most of these will need dialysis or transplantation, first two decades of life.
better quality of life and optimal somatic growth.
technical challenge for renal transplantation.
principal cause of graft failure was chronic rejection
77. Bottom Line Clinical Outcome
MONITOR BLADDER & RENAL FUCTION.
TREAT ACCORDINGLY FOR PRESERVATION OF UPPER URINARY TRACT
AND ITS FUCTION.
TREAT COMPLICATION.
MAINTAIN BETTER QUALITY OF LIFE