most commonly encountered congenital condition in children

1. POSTERIOR
URETHRAL
VALVES
DR.LEELA KRISHNA

2.  First recognised in 1769, by morgagani,confirmed by
langenbeck in 1802.
 First endosopic diagnosis of puv – hugh hampton
young.
 First endoscopic resection of valves in 1920 – randall.

3. INTRODUCTION
 Seen in 1.6 – 2.1 % in 10,000 births .
 Most common pathology in LUTO in congenital
anomalies of the urinary tract.

4. TYPES OF PUV

5. PATHOLOGY

6. DAMAGE CAUSED BU PUV

7. LOWER URINARY TRACT
 Bladder dysfunction – urinary reflux,renal dysfunction
,worsening hydronephrosis.
 Valve bladder syndrome – voiding dysfunction ,urinary
reflux, renal dysplasia , obstructive uropathy.

8. UPPER URINARY TRACT
 Increased pressure over prolonged intervals transmits
to ureter,renal pelvis and glomerular units -
architectural and functional changes of ascending
structure.

9. URETER
 ureteral wall thickening
 Loss of peristalsis
 Loss of mucosal coapatation
 Leading to urinary stasis, infection, increased pressures
in renal system .

10. RENAL DYSFUNCTION
 Renal dysplasia
 Obstructive uropathy

11.  Angiotensin II – renal damage – hemodynamic
changes in glomerular flow - induction of
transforming growth factor beta and tumour necrosis
factor alpha.

12. VURD
 Hoover and duckett hypothesized that reflux served as
a pop off mechanism in which the dysplastic kidney
with reflux served as a pressure reservoir migitating
damage to the contralateral kidney.
 Does not improve renal prognosis.
 Contralateral ,nonrefluxing kidney – high risk of
congenital renal cortical damage

13. CYSTOSCOPIC FINDINGS

14. ULTRASONOGRAPHY
 PUV – detected in 1 in 2,500.
 Accounts for 10 % of screened antenatally
genitourinary disease, 1/3 rd of bilateral renal disease .
 Fetal MRI – degree of obstruction based on urethral
dilatation ,distended bladder and reduced amniotic
fluids .

15. ULTRASOUND

16. CONTD…

17. KEYHOLE SIGN –
ANTENATAL SCAN

18. FETAL MRI

19. VOIDING
CYSTOURETHROGRAM

20. VURD IN VCUG

21. VCUG

22. LABARATORY EVALUATION
 After 48 hrs maternal blood mediated through
placenta should clear, base line labaratory values are
monitored.
 Nadir Creatinine value – at 1 year of age is important
diagnostic tool .

23. RADIONUCLIDE RENAL
SCAN
 Quantification of differential renal function and cortical
defects implying renal dysplasia in neonatal period .
 Mercaptoacetyltriglycerine

24. CLINICAL PRESENTATION
 Associated with pulmonary hypoplasia , physical
appaerence due to oligohydraminos such as potter
facies -clubfeet,deformed hands ,poor abdominal
muscle tone
 Difficulty with voiding
 Weak urinary stream
 5 or 7 F feeding tube / coude tipped catheter, stylet to
curl tip of feeding tube

25. PULMONARY HYPOPLASIA
 Perinatal mortality
 Ventilator support,delays attention away from PUV
 Etiology – unclear, multifactorial.
 Reduced expansion of alveoli
 Renal growth factor .

27. URINOMAS
 Seen in 3% to 10 %
 Forniceal rupture , distorted renal parenchyma,
contained with in renal capsule.

28.  Transperitoneal transudation of fluid or bladder
rupture – neonatal ascitis .
 Percutaneous drainage /tapping of ascitis – respiratory
distress .

29. DELAYED PRESENTATION
 Postnatal period
 UTI,ARF,voiding complaints .
 HIGH DEGREE SUSPICION – presenting with lower
urinary tract symptoms especially with recurrent UTI,
gross hematuria,overflow incontinence,renal
dysfunction.

30. SURGICAL TREATMENT
 VALVE ABLATION : cystoscopy and valve ablation
 GOAL – to restore normal flow of urine through
urethra.
 Crocket hook
 7.5 F/ 9 F cystoscope with an offset lens, for passage of
ablating devices including bugbee electrodes.

31. CYSTOSCOPY – INCISION OF
PUV

32. MOHAN VALVOTOME
 Hollow tube with ends shaped like hooks designed to
catch only a floating valve.
 Does not use diathermy .
 Set of two one 3 mm and 2mm diameter in size.
 Handle is turned laterally,downward
 Suprapubic pressure is applied while pulling valvotome
.

33.  Lasers – ND : YAG , Holmium : YAG laser.
 Whitaker and sherwood – modified the hook insulating
the wire except for the very distal portion of the hook
of 6/7 F, applying diathermy when ablating the valves.

34. CYSTOSCOPY

35.  Thin ,associated with minimal vascularity and
aggressive resection should be avoided .
 Cold knife and cutting resectoscope loop .
 Hot loop resectoscope – urethral stricture
 Urethral catheter – 24 hrs
 VCUG – repat after 1 month.

36. VESICOSTOMY
 INDICATIONS : LBW infant whose urethra cannot
accomadate an endocope
 Impaired renal function
 High bladder volumes
 Upper tract detoriation after valve ablation or urethral
catherization .

41. UPPER TRACT DIVERSIONS -
INDICATIONS
 Direct compression of the kidney will produce low
pressure urinary drainage ,allowing optimization of
renal function.
 Complete decompression of the lower urinary tract
 Sepsis
 Increasing upper tract dilatation
 Worsening renal function.

42. UPPER TRACT DIVERSIONS

43. CIRCUMCISION
 Prophylactic measure for any boy + puv .
 Reduces UTI by 83% to 92 %.
 Overall risk of UTI – 50 to 60 %.
 Assoc with upper tract dilatation , VUR ,incomplete
bladder emptying.
 Progress to pyelonephritis,sepsis .

44. NEPHROURETECTOMY
 Non functioning kidney with dilated urinary reflux
leading to infections and sepsis .
 PUV + VURD .
 Prevention – circumcision and proper bladder
emptying .

45. VUR
 recurrent UTI + reflux - elevated bladder pressures
 Conservatively – anticholinergic agents .
 Treating underlying bladder dysfunction.
 Requires Ureteric reimplantation.
 Complications – stricture, persistent reflux.
 Endoscopic > open – low risk.

46. BLADDER DYSFUNCTION
 Voiding dysfunction
 urinary reflux
 worsening of renal dysplasia
 obstructive uropathy .

47. PATHOLOGY OF BLADDER
DYSFUNCTION
 Detrusor hyperreflexia in infancy and early childhood
 Decreased intravesical pressures and improved
compliance
 Increased bladder capacity with hypocontractility
,atony in adolescence.

48. FOLLOW UP
 Renal ultrasonography
 Uroflow, PVR .
 Toilet training ,adequate fluid intake ,practice double
voiding , pelvic floor muscle excercises,biofeed back
therapy.
 Alphablockers /anticholinergics

49. CONTD….
 Routine – height ,weight ,blood pressure, serum
creatinine and elecrolytes .
 Indicated – isotope renography ( MAGE 3 or DMSA ),
 Formal estimate of GFR .

50. VALVE BLADDER
SYNDROME
 Polyuria
 Poor bladder compliance with high pressure voiding
and elevated wall tension bladder .
 Residual urine volume

51. VICIOUS CYCLE

52. TREATMENT
 CIC
 Overnight bladder drainage .
 Appendicovesicostomy
 Augmentation cystoplastly

53. VESICOAMNIOTIC
SHUNTING

54. ANTENATAL MANAGEMENT
 Antenatal scan – oligohydraminos ,dilated
bladder,severe HUN with out renal cortical cystic
lesions in a fetus with a normal karyotype.

56. FETAL URINE SAMPLING –
FAVOURABLE PROGNOSIS
 Fetal urine sample – 20 weeks of gestational age
 Urinary sodium less than 100 meq/L.
 Chloride less than 90 meq/L
 Osmolarity less than 200 meq/L
 Beta microglobulin less than 6 mg/L

57. PROGNOSTIC INDICATORS
FOR RENAL FUNCTION
 ESRD + PUV – 20 to 50 %.
 Serum creatinine at one year of age – 0.8 mg/dl –
minimal risk
 >1.2 mg/dl – high risk .

58. PREDICTORS OF POOR
PROGNOSIS FOR RENAL
FUNCTION
 PRENATAL – history of maternal
oligohydraminos,regardless of gestational age at
onset,early detection of prenatal u/s and other
prognostic features of fetal urinary tract.
 POST NATAL – clinical presentation in the first 6
months of life,proteinuria,bilateral VUR,impaired
continence at 5 yrs of age.

59. TRANSPLANTATION IN PUV
 Prevalence of ESRD + PUV – 50 % .
 Second most CC – obstructive uropathy .
 PUV – VUR + NFK + Bladder valve syndrome ( thick
walled, poorly contractile or hypercontractile bladder )
 Pretransplant evaluation – throughly

60.  CAUSE – thickened bladder wall may increase
incidence of ureteral obstruction .
 Video urodynamics
 Overnight bladder drainage / CIC .
 Pretransplant augmentation – rare, done in
immunocomprimised child.

61. LONG TERM RESULTS
 LUTS
 Bladder dysfunction
 UTI
 Renal dysfunction
 Erectile dysfunction
 Infertility .

62. POP OFF VALVES
 A mechanism by which high intravesical or intrapelvic
presure is dissipated.
 Allows for normal development of one or both kidneys
by one of three mechanisms
 1) urinary ascitis – urine leaks from the fornices of the
kidney or from a bladder rupture

63.  2) VURD syndrome – massive unilateral reflux into a
non functioning kidney
 3) large bladder diverticulum – causing aberrant
micturation into diverticulum there by taking pressure
off the developing renal units .

64. FAVOURABLE PROGNOSTIC
FACTORS
 Creatinine falling below 1.0 one month after treatment
initiated
 Absence of VUR
 Preservation of the corticomedullary junction of the
kidneys by renal U/S .
 Radiologic evidence of a pop off valve

65. ADVERSE PROGNOSTIC
FACTORS
 Prsentation after the age of 1 year
 Failure of cr to fall below 1.0 1 month following
initiation of therapy/drainage
 Bilateral VUR
 Diurnal incontinence beyond 5 yrs of age
 Prenatal diagnosis in the second trimester