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POSTERIOR
URETHRAL
VALVES
DR.LEELA KRISHNA
 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.
INTRODUCTION
 Seen in 1.6 – 2.1 % in 10,000 births .
 Most common pathology in LUTO in congenital
anomalies of the urinary tract.
TYPES OF PUV
PATHOLOGY
DAMAGE CAUSED BU PUV
LOWER URINARY TRACT
 Bladder dysfunction – urinary reflux,renal dysfunction
,worsening hydronephrosis.
 Valve bladder syndrome – voiding dysfunction ,urinary
reflux, renal dysplasia , obstructive uropathy.
UPPER URINARY TRACT
 Increased pressure over prolonged intervals transmits
to ureter,renal pelvis and glomerular units -
architectural and functional changes of ascending
structure.
URETER
 ureteral wall thickening
 Loss of peristalsis
 Loss of mucosal coapatation
 Leading to urinary stasis, infection, increased pressures
in renal system .
RENAL DYSFUNCTION
 Renal dysplasia
 Obstructive uropathy
 Angiotensin II – renal damage – hemodynamic
changes in glomerular flow - induction of
transforming growth factor beta and tumour necrosis
factor alpha.
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
CYSTOSCOPIC FINDINGS
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 .
ULTRASOUND
CONTD…
KEYHOLE SIGN –
ANTENATAL SCAN
FETAL MRI
VOIDING
CYSTOURETHROGRAM
VURD IN VCUG
VCUG
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 .
RADIONUCLIDE RENAL
SCAN
 Quantification of differential renal function and cortical
defects implying renal dysplasia in neonatal period .
 Mercaptoacetyltriglycerine
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
PULMONARY HYPOPLASIA
 Perinatal mortality
 Ventilator support,delays attention away from PUV
 Etiology – unclear, multifactorial.
 Reduced expansion of alveoli
 Renal growth factor .
URINOMAS
 Seen in 3% to 10 %
 Forniceal rupture , distorted renal parenchyma,
contained with in renal capsule.
 Transperitoneal transudation of fluid or bladder
rupture – neonatal ascitis .
 Percutaneous drainage /tapping of ascitis – respiratory
distress .
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.
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.
CYSTOSCOPY – INCISION OF
PUV
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
.
 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.
CYSTOSCOPY
 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.
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 .
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.
UPPER TRACT DIVERSIONS
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 .
NEPHROURETECTOMY
 Non functioning kidney with dilated urinary reflux
leading to infections and sepsis .
 PUV + VURD .
 Prevention – circumcision and proper bladder
emptying .
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.
BLADDER DYSFUNCTION
 Voiding dysfunction
 urinary reflux
 worsening of renal dysplasia
 obstructive uropathy .
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.
FOLLOW UP
 Renal ultrasonography
 Uroflow, PVR .
 Toilet training ,adequate fluid intake ,practice double
voiding , pelvic floor muscle excercises,biofeed back
therapy.
 Alphablockers /anticholinergics
CONTD….
 Routine – height ,weight ,blood pressure, serum
creatinine and elecrolytes .
 Indicated – isotope renography ( MAGE 3 or DMSA ),
 Formal estimate of GFR .
VALVE BLADDER
SYNDROME
 Polyuria
 Poor bladder compliance with high pressure voiding
and elevated wall tension bladder .
 Residual urine volume
VICIOUS CYCLE
TREATMENT
 CIC
 Overnight bladder drainage .
 Appendicovesicostomy
 Augmentation cystoplastly
VESICOAMNIOTIC
SHUNTING
ANTENATAL MANAGEMENT
 Antenatal scan – oligohydraminos ,dilated
bladder,severe HUN with out renal cortical cystic
lesions in a fetus with a normal karyotype.
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
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 .
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.
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
 CAUSE – thickened bladder wall may increase
incidence of ureteral obstruction .
 Video urodynamics
 Overnight bladder drainage / CIC .
 Pretransplant augmentation – rare, done in
immunocomprimised child.
LONG TERM RESULTS
 LUTS
 Bladder dysfunction
 UTI
 Renal dysfunction
 Erectile dysfunction
 Infertility .
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
 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 .
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
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

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POSTERIOR URETERAL VALVES (PUV )

  • 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.
  • 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
  • 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 .
  • 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 .
  • 26.
  • 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.
  • 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.
  • 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 .
  • 37.
  • 38.
  • 39.
  • 40.
  • 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.
  • 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
  • 52. TREATMENT  CIC  Overnight bladder drainage .  Appendicovesicostomy  Augmentation cystoplastly
  • 54. ANTENATAL MANAGEMENT  Antenatal scan – oligohydraminos ,dilated bladder,severe HUN with out renal cortical cystic lesions in a fetus with a normal karyotype.
  • 55.
  • 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