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.

1. EVALUATIONAND MANAGEMENT
OFPOSTERIORURETHRALVALVE.
PRESENTED BY : DEVASHISH CHOUBEY

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

5. Young’s classification

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.

7. EMBRYOLOGY
TYPE-3 :(5%)
Incomplete dissolution of the urogenital membrane

8. Pathophysiology
Primitive tissues mature in an abnormal environment of high
intraluminal pressures and organ distention.

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.

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

13. EFFECT CONT….
DETRUSOR DECOMPENSATES FURTHER….
INEFFICIENT BLADDER PRESSURES
INCREASED PVR
TYPEIII INFILTRATES DETRUSOR MUSCLE
TYPEIII
TYPEI
ECM

14. EFFECTON RENAL/BLADDERFUNCTION
• 0-1 YEAR
HYPERTONIC
SMALL CAPACITY
DETRUSOR OVERACTIVITY
• 1-3 YEARS
HYPERREFLEXIC BLADDER
UNINHIBITED
CONTRACTIONS
• 4-12 YEARS
• INCREASED RESIDUAL URINE
MYOGENIC FAILURE
OVERFLOW

15. ClinicalPresentation
In utero
Older
Child
Neonatal

16. ClinicalPresentation
Variable
Age dependent
= Prenatally : 70%
of PUV by
ultrasound
= Newborn: -
Abdominal mass-
ascites-respiratory
distress-Urosepsis

17. ClinicalPresentation
= Infant
Urinary
dribbling
• Enuresis
= Failure to
thrive/renal
failure
• Urosepsis
= Toddler
UTI voiding
dysfunction
= School-age
boy
Urinary
incontinence

18. MANAGEMENT
•PRENATAL
@
•POSTNATAL
@

19. INUTERO(PRENATAL)
BY ANTENATAL U.S.G.
Oligohydramnios
Marked hydroureteronephrosis
Distended bladder
Thickened bladder wall

20. INUTERO

21. FETAL MRI
DILATED URETHRA
DISTENDED BLADDER
REDUCED AMNIOTIC FLUID LEVELS
LUNG HYPOPLASIA
CYSTIC CHANGES IN RENAL PARENCHYMA

22. VoidingCystourethrography
•Gold standard for diagnosing PUV
• Typically shows :

23. VCUGFINDINGS
Thickened and trabeculated bladder
High grade vur-50% patients
Grossly dilated posterior urethra

25. Functional Assessment
Diuretic Radioisotope Scan
 - DTPA OR MAG-3
 - with urethral catheter in place
 -Assess split renal function
 INDICATIONS:
 Usg s/o renal dysplasia
 usg s/o loss of CMD
 USG S/O persistent hydronephrosis

26. Urine Biochemistry
Good Prognosis Bad Prognosis
Sodium < 100 mEq/L > 100 mEq/L
Chlorine < 90 mEq/L > 90 mEq/L
Osmolality < 210 mOsm/L > 210 mOsm/L
Calcium < 2 mmol/L > 2 mmol/L
Phosphate < 2 mmol/L > 2 mmol/L
To. protein < 20 mg/dl >20 mg/dl

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).

29. Needle Aspiration of Bladder

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

33. SONOGRAPHIC FACTORS
Good prog factors
◦ Normal fluid
◦ Diagnosis after 24 wk
◦ Asymmetric hydronephrosis
◦ Urinary ascites
◦ Isolated
Poor prog factors
◦ Oligohydramnios
◦ Diagnosis before 24 wks
◦ Echogenic kidneys
◦ Perinephric urinoma
◦ Associated abnormalities

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

36. Vesicoamniotic Shunt

37. COMPLICATIONS
Premature labor
Fetal bowel injury
Fetal loss
Infection

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.

40. InitialDiagnosticEvaluation
(POSTNATAL)
Ultrasound
Voiding Cystourethrography
Radionuclide Renal Scan(functional assesment)

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.

42. Postnatal Ultrasound

44. PROGNOSTIC FACTOR
GOOD FACTOR
Nadir creatinine < 0.8 mg/dl
S. creatinine < 1 mg/dl
Pop-off mechanism
 -VURD
 -Ascitis
 -Large bladder
 diverticulum.
BAD FACTOR
Young age
 Delayed correction
 GFR < 50 % of normal in
infancy
VUR – mortality (b/l - 57% u/l -
17%, non - 9%)
Loss of cortico medullary junction

45. Postnatal management
Initial management
- bladder drainage
- Tt life threatening condition by:-
*maximal ventilatory support
*extracorporeal membrane oxy-
genation.
*dialysis & parenteral nutrition
*control of hypertension
- Valve Ablation

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.

48. BEFORE ABLATION AFTER ABLATION

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

50. OPTION FOR URINARY DIVERSION

51. Vesicostomy
Distal ureterostomy
Proximal loop ureterostomy
Cutaneous pyeloplasty
Ring ureterostomy
Sober y ureterostomy

52. BLOCKSOMCUTANEOUS VESICOSTOMY

54. INDICATIONS
VERY LOW BIRTH WEIGHT INFANTS
CONTINUED IMPAIRED RENAL FUNCTION
HIGH BLADDER VOLUMES
UPPER TRACT DETERIORATION AFTER VALVE ABLATION/URETHRAL
CATHETERIZATION

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.

63. Vesicoureteral Reflux
50% VUR at time of diagnosis
Primary or Secondary

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.

66. VALVEBLADDER
CLINICAL FEATURES
Typically comfortable with large bladder volumes at high
pressure
Overflow incontinence
Incomplete void
Increased frequency
Nocturia

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

68. TO SUMMARISE…..
POLYURIA BLADDERINSENSITIVITY
RESIDUAL URINE
VOLUME

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

71. @
• anticholinergics
• α-blockers
@
• timed voiding
• double voiding
@
• intermittent catheterization
• Cont. nocturnal blad. drainage
@
• Augmentation bladder
• Appendicovesicostomy

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

75. END STAGE BLADDER:
VESICOSTOMY
UPPER TRACT
DIVERSION
ILEAL
CONDUIT

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