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Posterior Urethral Valve
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
- A (PUV) is an abnormal congenital obstructing
membrane that is located within the posterior male
urethra; this valve is the most common cause of
bladder outlet obstruction in male children. [1, 2, 3]
- The valve is believed to result from abnormal
embryologic development of the fetal posterior
urethra.
- These valves essentially obstruct normal bladder
emptying. This mechanical obstruction increases
voiding pressures and may alter normal
development of the fetal bladder and kidneys.
- Children with higher degrees of obstruction
present earlier with the most severe
symptoms.
- A spectrum of signs and symptoms, ranging
from mild obstructive symptoms of voiding
dysfunction to severe obstruction with resultant
renal failure and pulmonary hypoplasia , may be
noted. (3)
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
Incidence :
- 1 per 5000 to 8000
- More than 50 % are diagnosed in the 1st year
with more severe obstruction .
- Renal insufficiency is caused by PUVs in
approximately 10-15 % of children
undergoing renal transplant .
- approximately 1/3 of patients born with
PUVs progress to end stage renal disease
(ESRD)
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
- The classic categorization
of posterior urethral valves
into types I, II, and III was
developed by H. H. Young in
1919[4] and has undergone
modification over time based
on clinical observation and a
better understanding of the
embryologic events that lead
to normal urethral
development.
Type I PUV
- Type I valve accounts for 95 % of all valves
- Secondary to abnormal insertion and absorption of most distal
aspects of the Wolffian ducts during bladder development .
- Bicuspid valve that radiates distally from the
posterior edge of the verumontanum to the anterior
proximal membranous urethra
Type II PUV
- Very infrequent
- Non obstructive urethral
folds
- It's thought to be a sequela of
voiding dysfunction ,
- therefore these valves can
be differentiated from Type I
& Type III valves by their
location proximal to the
verumontanum (extends
proximaly from the
verumontanum to the
Type III PUV
- Membrane in the posterior
urethra believed to originate
from incomplete canalization
between the anterior &
posterior urethra.
- This valve is a
circumferential membrane or
diaphragm that is located at
the membranous urethra .
- Type III valves account for
almost 5 % of all valves
- Worst Prognosis (1)
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
Voiding cystourethrogram
(VCUG)
is the best imaging technique for the
diagnosis of PUV and can show :
- visualizations of the valve
leaflets
- Thickened & trabeculated
bladder
- Dilated or elongated posterior
urethra
- Hypertrophied bladder neck
- Diverticula
- Vesicoureteral reflux and reflux into the ejaculatory ducts
secondary to elevated bladder and urethral pressure
Associated findings
- VUR 40 %
- Renal dysplasia
- hydroureteronephrosis
- ARF
Antenatal & Postnatal Ultrasound
- Marked distention and hypertrophy of the bladder
- Hydronephrosis and hydroureter may or may not be present
- In severe cases oligohydramnios and renal dysplasia (2)
- Keyhole sign may be seen on ultrasound due to the distention of
both the bladder and the urethra immediately proximal to the valve(5) .
** Unfortunately, such findings are generally not seen before 26 weeks
of gestation. (5)
Delayed presentation
- UTI
- Diurnal enuresis in boys older than 5 years
- Secondary diurnal enuresis
- Voiding pain or dysfunction
- Decreased force of stream may indicate the
presence of PUVs
- Discovered during evaluations of abdominal mass
or renal failure.
Differential Diagnosis
- In this age group and with clear dilatation of the
posterior urethra there is usually little differential other
than urethral atresia, which is far less common (2).
- When only the bladder is clearly abnormal - thick walled
and trabeculated, other conditions to be considered
include (5):
* Neurogenic bladder
* Prune-belly syndrome
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
Primary Valve ablation
Vesicostomy
Cutaneous uretrostomies
Antireflux Surgery
Surgical Treatment
Medical Treatment :
- Antibiotics
- Correction of Metabolic disturbances
Primary valve ablation
Vesicostomy
Cutaneous Uretrostomies
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
- PUV has 3 types I , II &III
- Type I is the most common , Type III has the worst
prognosis.
- A spectrum of signs and symptoms, ranging from mild
obstructive symptoms of voiding dysfunction to severe
obstruction with resultant renal failure and pulmonary
hypoplasia.
- Long term sequelae are significant especially renal
disease.
- VCUG is the best modality of diagnosis.
- Majority are managed by valve ablation
Overview
Epidemiology
Classification
Diagnosis
Treatment
Conclusion
References
Contents
1. Berrocal T, López-pereira P, Arjonilla A et-al. Anomalies of the distal ureter,
bladder, and urethra in children: embryologic, radiologic, and pathologic features.
2. Radiographics. 22 (5): 1139-64. Radiographics (full text) –
3. Chudleigh P, Thilaganathan B, Chudleigh T. Obstetric ultrasound, how, why and
when. Churchill Livingstone. (2004) ISBN:0443054711.
4. Bruyn RD. Pediatric ultrasound, how, why and when. Churchill Livingstone. (2005)
ISBN:0443072752.
5. Haller JO, Slovis TL, Joshi A. Pediatric radiology. Springer Verlag. (2005)
ISBN:3540213546.
6. Blews DE. Sonography of the neonatal genitourinary tract. Radiol. Clin. North Am.
1999;37 (6): 1199-208, vii. –
.
PUV presentation.pptx

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PUV presentation.pptx

  • 4. - A (PUV) is an abnormal congenital obstructing membrane that is located within the posterior male urethra; this valve is the most common cause of bladder outlet obstruction in male children. [1, 2, 3]
  • 5. - The valve is believed to result from abnormal embryologic development of the fetal posterior urethra. - These valves essentially obstruct normal bladder emptying. This mechanical obstruction increases voiding pressures and may alter normal development of the fetal bladder and kidneys.
  • 6.
  • 7. - Children with higher degrees of obstruction present earlier with the most severe symptoms. - A spectrum of signs and symptoms, ranging from mild obstructive symptoms of voiding dysfunction to severe obstruction with resultant renal failure and pulmonary hypoplasia , may be noted. (3)
  • 9. Incidence : - 1 per 5000 to 8000 - More than 50 % are diagnosed in the 1st year with more severe obstruction .
  • 10. - Renal insufficiency is caused by PUVs in approximately 10-15 % of children undergoing renal transplant . - approximately 1/3 of patients born with PUVs progress to end stage renal disease (ESRD)
  • 11.
  • 13. - The classic categorization of posterior urethral valves into types I, II, and III was developed by H. H. Young in 1919[4] and has undergone modification over time based on clinical observation and a better understanding of the embryologic events that lead to normal urethral development.
  • 14. Type I PUV - Type I valve accounts for 95 % of all valves - Secondary to abnormal insertion and absorption of most distal aspects of the Wolffian ducts during bladder development .
  • 15. - Bicuspid valve that radiates distally from the posterior edge of the verumontanum to the anterior proximal membranous urethra
  • 16. Type II PUV - Very infrequent - Non obstructive urethral folds - It's thought to be a sequela of voiding dysfunction , - therefore these valves can be differentiated from Type I & Type III valves by their location proximal to the verumontanum (extends proximaly from the verumontanum to the
  • 17. Type III PUV - Membrane in the posterior urethra believed to originate from incomplete canalization between the anterior & posterior urethra. - This valve is a circumferential membrane or diaphragm that is located at the membranous urethra . - Type III valves account for almost 5 % of all valves - Worst Prognosis (1)
  • 18.
  • 20. Voiding cystourethrogram (VCUG) is the best imaging technique for the diagnosis of PUV and can show : - visualizations of the valve leaflets - Thickened & trabeculated bladder - Dilated or elongated posterior urethra - Hypertrophied bladder neck - Diverticula - Vesicoureteral reflux and reflux into the ejaculatory ducts secondary to elevated bladder and urethral pressure
  • 21.
  • 22.
  • 23. Associated findings - VUR 40 % - Renal dysplasia - hydroureteronephrosis - ARF
  • 24. Antenatal & Postnatal Ultrasound - Marked distention and hypertrophy of the bladder - Hydronephrosis and hydroureter may or may not be present - In severe cases oligohydramnios and renal dysplasia (2) - Keyhole sign may be seen on ultrasound due to the distention of both the bladder and the urethra immediately proximal to the valve(5) . ** Unfortunately, such findings are generally not seen before 26 weeks of gestation. (5)
  • 25.
  • 26.
  • 27. Delayed presentation - UTI - Diurnal enuresis in boys older than 5 years - Secondary diurnal enuresis - Voiding pain or dysfunction - Decreased force of stream may indicate the presence of PUVs - Discovered during evaluations of abdominal mass or renal failure.
  • 28. Differential Diagnosis - In this age group and with clear dilatation of the posterior urethra there is usually little differential other than urethral atresia, which is far less common (2). - When only the bladder is clearly abnormal - thick walled and trabeculated, other conditions to be considered include (5): * Neurogenic bladder * Prune-belly syndrome
  • 30. Primary Valve ablation Vesicostomy Cutaneous uretrostomies Antireflux Surgery Surgical Treatment
  • 31. Medical Treatment : - Antibiotics - Correction of Metabolic disturbances
  • 36. - PUV has 3 types I , II &III - Type I is the most common , Type III has the worst prognosis. - A spectrum of signs and symptoms, ranging from mild obstructive symptoms of voiding dysfunction to severe obstruction with resultant renal failure and pulmonary hypoplasia. - Long term sequelae are significant especially renal disease. - VCUG is the best modality of diagnosis. - Majority are managed by valve ablation
  • 38. 1. Berrocal T, López-pereira P, Arjonilla A et-al. Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. 2. Radiographics. 22 (5): 1139-64. Radiographics (full text) – 3. Chudleigh P, Thilaganathan B, Chudleigh T. Obstetric ultrasound, how, why and when. Churchill Livingstone. (2004) ISBN:0443054711. 4. Bruyn RD. Pediatric ultrasound, how, why and when. Churchill Livingstone. (2005) ISBN:0443072752. 5. Haller JO, Slovis TL, Joshi A. Pediatric radiology. Springer Verlag. (2005) ISBN:3540213546. 6. Blews DE. Sonography of the neonatal genitourinary tract. Radiol. Clin. North Am. 1999;37 (6): 1199-208, vii. – .