posterior urethral valves stephen confer, md ben o. donovan, md brad kropp, md dominic frimberger,...
TRANSCRIPT
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Posterior Urethral Valves
Stephen Confer, MDBen O. Donovan, MD
Brad Kropp, MDDominic Frimberger, MDUniversity of Oklahoma Department of Urology
Section of Pediatric Urology
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Case Report
• 29 day old infant presents with fever of unknown origin X 3 days.
• Admitted by pediatrics team for sepsis workup.
• Urine culture positive.
• Urology consulted.
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Case Report
• PMH: Normal prenatal U/S per report.
• FMH: non contributory
• ROS: + fevers, poor feeding, +lethargy
• PE: Normal except palpable bladder.
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Case Report
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Case Report
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Posterior Urethral Valves (PUV)
• Congenital Proximal Urethral Obstruction
• Abnormal congenital mucosal folds in the prostatic urethra that look like a thin membrane that impairs bladder drainage
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PUV Defined• Type I
– Obstructing membrane that extends distally from each side of the verumontanum towards the membranous urethra where they fuse anteriorly
• Type II– Described as folds extending cephalad from the
verumontanum to the bladder neck
• Type III– Represent a diaphragm or ring-like membrane with a
central aperture just distal to the verumontanum– Thought to represent incompelte dissolution of the
urogenital membrane
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Type I PUV
Obstructing membrane radiating distally from the posterior edge of the verumontanum to the membranous urethra
During voiding, the fused anterior portion bulges into the urethra with a narrow posterior opening
Possibly due to anomalous insertion of the mesonephric ducts into the primitive fetal cloaca
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Type I PUV
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Type III PUV
Represent incomplete dissolution of the UG membrane
Distal to the verumontanum at the membranous urethra
Ring-like with a central opening, “wind sock valve”
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Incidence• Males only
• 1:5000 – 8000 male births
• Type I > 95%
• Type III - 5%
• Children with Type III PUVs have a worse prognosis as a group
• 50% of patients with PUV will have vesicoureteral reflux– 50% unilateral, 50% bilateral
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Clinical Presentation
• Varies by degree of obstruction– Symptoms vary by age of presentation
• Antenatal– Bilateral hydronephrosis– Distended and thickened bladder– Dilated prostatic urethra– Oligohydramnios - accounts for co-presentation
of pulmonary hypoplasia.
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Clinical Presentation• Newborn
– Palpable abdominal mass• Distended bladder, hydronephrotic kidney• Bladder may feel like a small walnut in the suprapubic area
– Ascites • 40% of time due to obstructive uropathy
– History of Oligohydramnios– Respiratory distress from pulmonary hypoplasia
• Severity often does not correlate with degree obstruction• Primary cause of death in newborns
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Clinical Presentation• Early Infancy
– Dribbling / poor urinary stream
– Urosepsis
– Dehydration
– Electrolyte abnormalities
– Uremia
– Failure to thrive; due to renal insufficiency
• Toddlers
– Better renal function (less obstruction)
– Febrile UTI
– Voiding dysfunction – incontinence
– Daytime incontinence may be the only symptom in boys with less severe obstruction
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Initial Management
• Bladder Drainage– A 5 or 8 Fr pediatric feeding tube is ideal– A Foley catheter should not be used, due to the
tendency of the balloon to occlude the ureteral orifice and cause a bladder spasm.
• Secondary obstruction
– Broad spectrum antibiotic coverage– Metabolic panel
• Assess renal function and metabolic abnormalities• Acidosis, hyperkalemia common problems
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Radiologic Evaluation of the Lower Tract
• VCUG– Mandatory for all PUV evaluations
– Showing a dilated prostatic urethra, valve leaflets, detrusor hypertrophy, bladder diverticula, bladder neck hypertrophy, and narrow penile urethra stream, as well as possible incomplete emptying
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Radiologic Evaluation of the Lower Tract
• U/S– Examining the prostatic urethra for
characteristic dilation and thickening of the bladder wall
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VCUG
dilated prostatic urethra valve leaflets detrusor hypertrophy cellules or bladder
diverticula bladder neck hypertrophy narrow penile urethra
stream possible incomplete
emptying
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Radiologic Studies- Upper Tract
• Renal Ultrasound– Examination for bilateral hydronephrosis and
signs of lower tract obstructive process
• Renal Scan– Assesses the function of the kidneys
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Management• Transurethral Valve Ablation
– Incise at 4, 8 & 12 o’clock positions via Pediatric resectoscope
• Avoid urethral sphincter
• Catheter drainage for 1-2 days
• VCUG at 2 months to ensure destruction of valves
• Regular U/S to evaluate resolution of hydronephrosis
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Management
• Transurethral Valve Ablation– Alternatively, 8F cystoscope with a Bugbee
electrode adjacent– Insulated crochet hook (“Whitaker hook”)
• When urethra too small to accommodate cystoscope/Bugbee
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Case study
• Our patient had a very narrow urethra and therefore the approach was with the 8F cystoscope with a Bugbee electrode attached.
QuickTime™ and aTIFF (Uncompressed) decompressor
are needed to see this p icture.QuickTime™ and aTIFF (Uncompressed) decompressor
are needed to see this picture.
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Case study
• Operative images captured by cystoscope camera.
QuickTime™ and aTIFF (LZW) decompressor
are needed to see this picture.
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Case Report
QuickTime™ and aTIFF (LZW) decompressor
are needed to see this picture.
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Vesicoureteral Reflux• Present in 33 - 50%
• Usually Secondary– High intravesical pressures
• 33% resolve spontaneously when obstruction treated
• 33% do well on prophylactic antibiotics
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Vesical Dysfunction• 50% have abnormal bladder function
• Presents as incontinence– Not due to sphincter dysfunction or damage
• Primary myogenic failure
• Uninhibited contractions
• May lead to progressive renal deterioration
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Adverse Prognostic Factors
• Presentation after the age of 1 year
• Failure of Cr to fall below 1.0 1 month following initiation of therapy/drainage
• Bilateral vesicoureteral reflux
• Diurnal incontinence beyond 5 years of age
• Prenatal diagnosis in the second trimester
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Favorable 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
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“Pop-off” Valves
• A mechanism by which high intravesical or intrapelvic pressure is dissipated
• Allows for normal development of one or both kidneys by one of three mechanisms– (1) Urinary ascites
• Urine leaks from the fornices of the kidneys or from a bladder rupture
– (2) “VURD” syndrome• Massive unilateral reflux into a non-functioning kidney
– (3) Large bladder diverticulum• Causing aberrant micturition into the diverticulum, thereby
taking pressure off the developing renal units
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Conclusions:Posterior Urethral Valves
• Two PUV types, Type I the most common, Type III with a worse prognosis
• Prognosis improved with improved symptoms within 1 month of therapy or the presence of a “Pop-off” valve
• Drainage, antibiotics and correction of metabolic disturbances key to initial care
• VCUG, U/S and renal nuclear scan to evaluate• Majority managed by valve ablation• Long-term sequelae significant, primarily renal
disease
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Questions???
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Questions???