Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger,...

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Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section of Pediatric Urology

Transcript of Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger,...

Page 1: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Posterior Urethral Valves

Stephen Confer, MDBen O. Donovan, MD

Brad Kropp, MDDominic Frimberger, MDUniversity of Oklahoma Department of Urology

Section of Pediatric Urology

Page 2: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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.

Page 3: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Case Report

• PMH: Normal prenatal U/S per report.

• FMH: non contributory

• ROS: + fevers, poor feeding, +lethargy

• PE: Normal except palpable bladder.

Page 4: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Case Report

Page 5: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Case Report

Page 6: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 7: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 8: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 9: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Type I PUV

Page 10: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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”

Page 11: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 12: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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.

Page 13: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 14: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 15: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 16: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 17: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Radiologic Evaluation of the Lower Tract

• U/S– Examining the prostatic urethra for

characteristic dilation and thickening of the bladder wall

Page 18: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

VCUG

dilated prostatic urethra valve leaflets detrusor hypertrophy cellules or bladder

diverticula bladder neck hypertrophy narrow penile urethra

stream possible incomplete

emptying

Page 19: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 20: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 21: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 22: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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.

Page 23: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Case study

• Operative images captured by cystoscope camera.

QuickTime™ and aTIFF (LZW) decompressor

are needed to see this picture.

Page 24: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Case Report

QuickTime™ and aTIFF (LZW) decompressor

are needed to see this picture.

Page 25: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Vesicoureteral Reflux• Present in 33 - 50%

• Usually Secondary– High intravesical pressures

• 33% resolve spontaneously when obstruction treated

• 33% do well on prophylactic antibiotics

Page 26: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 27: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 28: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 29: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

“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

Page 30: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

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

Page 31: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Questions???

Page 32: Posterior Urethral Valves Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section.

Questions???


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