Posterior urethral valves & VUR

Presenter : Dr.Dhabessa M.(Resident)

Moderator : Dr.Asheber B.(Surgeon)

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 • Outline
• Introduction
• Embryology and anatomy
• Diagnosis
• Management
• Complications
• outcomes

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 Posterior urethral valves
• Introduction
– Most common cause of bladder outlet obstruction in boys
– Incidence of 1 in 5,000 to 8,000 male births.
– Majority of boys with PUV are diagnosed antenatally.
– The most common obstructive cause of ESRD in children
– 35% of children who require renal transplantation.

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• EMBRYOLOGY AND ANATOMY
– Several disruptions of the male
embryologic urethral development
• proposed as the mechanism
– Persistence of the urogenital memb. with
abnormal canalization of the urethra.
– Overgrowth of urethrovaginal folds
– Abnormal integration of the Wolffian
duct into the posterior urethra
• At 5 to 6 weeks, mesonephric duct (Wolffian
ducts) migrates
– To the posterior wall of the urogenital sinus.
– Remnants as plicae colliculi.
• Too anterior insertion
– ducts fuse anteriorly, resulting in PUV

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• Classification
• 1919, by Young (traditional).
– Type I is an obstructing membrane (95% )
– Type III .
• congenital obstructing posterior urethral
membrane.
– Type II valves
• prominent longitudinal folds of hypertrophied
smooth muscle
• Dewan & Doh
– Single pathologic condition of an oblique
membrane (COPUM)

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• DIAGNOSIS
• Prenatal
– Typical findings on ultrasound (US)
• Bilateral hydroureteronephrosis,
• Distended bladder, and a dilated
prostatic urethra,
– ‘keyhole’ sign.
• Discrete focal cysts in the renal
parenchyma
– Diagnostic of renal dysplasia.
• Amniotic fluid volume is variable.
– Oligohydramnios.

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• Fetal renal function assesseent
• Normally, fetal urine is hypotonic (favorable prognosis)
– sodium less than 100 mEq/L,
– chloride less than 90 mEq/L,
– osmolality less than 210 mEq/L, and
– β2-microglobulin levels less than 6 mg/L.5

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• Neonates
– Symptoms of delayed voiding
– reduced urinary stream.
– Respiratory distress.
– Abdominal mass
• palpable walnut-sized bladder
– secondary to the hypertrophic detrusor muscle.
– failure to thrive
– lethargy, poor feeding,
– urinary tract infection (UTI)
– urinary ascites
• Older boys
– persistent diurnal incontinence
– abdominal distention

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• RADIOGRAPHIC EVALUATION
– Ultrasound
– Voiding cystourethrogram (VCUG)
• Definitively establishes the diagnosis of
PUV.
• Unilateral VUR is present in 25% and
• bilateral VUR in 25% of infants with
PUV.
• 50% NO reflux

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• Renal nuclear scintigraphy.
– Should be delayed until 6 to 8 weeks
– Effective in establishing baseline differential renal function.
– technetium-99m– labeled dimercaptosuccinic acid (99mTc-DMSA)

• Dynamic contrast-enhanced magnetic resonance urography (MRU).

– High resolution renal images
– Assessment of differential renal function, but requires an anesthetic.

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 MANAGEMENT
• Prenatal Intervention • Early delivery
– Controversial • vesicoamniotic shunting
– Prenatal detection of PUV and
• Amnioinfusion
technological advances in surgical
equipment. • percutaneous fetal cystoscopy
– Nonobstructive disorders prunebelly • open fetal surgery
syndrome and high-grade VUR
• May be difficult to differentiate from
PUVs
– Screen for associated anomalies

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• POSTNATAL MANAGEMENT
• Initial Management
– Decompress the bladder
• 5 French or 8 French feeding tube.
– Antibiotic prophylaxis.
– Electrolytes, BUN (blood urea nitrogen), creatinine, and fluid status.
– Metabolic acidosis and hyperkalemia
– Immediate pulmonary resuscitation
– Urinary ascites
• paracentesis

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• Primary Endoscopic Valve Ablation
– after the neonate is stabilized.
– Dilatation the meatus and glandular urethra.
– Preferred initial surgical option in any neonate.
– The treatment goal
• restore flow of urine through the urethra and
• enable normal cyclic filling and emptying of the bladder
– which is superior to urinary diversion and passive urine drainage
– Availability of a 7.5- or 9-Fr infant cystoscope

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• Endoscopic Valve ablation
– 5 o’clock and 7 o’clock positions
• with or without an incision at the 12 o’clock
position.
– 12 o’clock position alone
– successful in more than 90% of patients.
– Incomplete valve ablation.
– Urethral stricture.

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• Temporary Urinary Diversion
– Cutaneous vesicostomy
• Small or premature neonate
– When cystoscope is too large for the urethra
• severe hydroureteronephrosis,
• urinary ascites,
• high-grade VUR
• Poor renal function are present.
• Blocksom technique is favored
– Bringing the bladder dome to the skin
– decrease the chance of bladder prolapse
• Stomal stenosis, and prolapse
• Antibiotic prophylaxis

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• Proximal diversion
– cutaneous pyelostomy
– cutaneous ureterostomy
– Theoretically provides better renal drainage
– optimizes the potential for renal function and somatic growth.
– Does not prevent ESRD
• Because at least 85% have renal dysplasia.
– reserved only when valve ablation or vesicostomy fails to improve upper tract
drainage.
– Regular cyclical bladder filling and contraction does not occur
• results in a smaller, less compliant bladder.

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• Sober-en-T ureterostomy
– preferred method of proximal
diversion
– proximal ureter is divided and
exteriorized on the abdominal wall.
– The proximal end of the distal
ureteral segment is then
anastomosed to the renal pelvis.
– allows urine to drain into the bladder
• Maintain bladder cycling
• Provide good upper tract drainage

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• Urinary extravasation and
transudation
– 5% to 15% of neonates with PUV.
– perirenal urinoma or urinary ascites.
– In urinary ascites
• significant electrolyte abnormalities
– from urinary reabsorption,
• respiratory compromise
– from the abdominal distention.
– Ultrasound, VCUG, and renal
scintigraphy.
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• Decompress the bladder and upper
urinary tract suffciently
– forniceal extravasation stops
• Percutaneous drainage
– serum creatinine worsening
– respiratory compromise
– infection, hypertension
– significant parenchymal compression
• Percutaneous nephrostomy
– If extravasation persists
• Drain can be inserted into the urinoma
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• Prognosis After Initial Therapy
• The prognosis for satisfactory renal function
– A serum creatinine concentration less than 0.8 mg/dL one month after initial
treatment, or at age 1
– Visualization of the corticomedullary junction differentiation on renal ultrasound
– Achieving diurnal continence by the age of 5 years indicates
– Presence of a pressure pop-off mechanism
• Massive VUR into a nonfunctioning kidney (termed the VURD syndrome: valves, unilateral reflux,
dysplasia),
• urinary ascites, or
• a large bladder diverticulum

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• Adverse prognostic factors
– bilateral VUR,
– persistence of the serum creatinine higher than 1.0 mg/dL after initial
therapy,
– Identification of small subcapsular renal cysts (indicative of renal
dysplasia),
– Increased renal echogenicity
– loss of corticomedully differentiation.

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• VESICOSTOMY CLOSURE
– Decision to close the vesicostomy should be made carefully.
• If febrile UTIs are noted with the vesicostomy.
• As a prerequisite to renal transplantation.
• Upper urinary tracts have stabilized and the child is large enough to undergo simultaneous valve ablation
– generally between ages 1 and 3 years.
– Preoperatively, a cystogram using a Foley catheter , introduced via the vesicostomy
– with the balloon inflated to avoid leakage of contrast
– assess whether signifcant VUR is present and to evaluate the appearance of the bladder.
– Monitor for
• worsening hydronephrosis,
• Incomplete bladder emptying, and
• significant change in serum creatinine.

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• Associated Vesicoureteral Reflux
– in approximately 50% of boys.
– After valve ablation, nearly all patients will show improvement in reflux grade at 1
year.
– Antibiotic prophylaxis is continued, and periodic upper tract imaging and
cystography should be performed.
– Renal deterioration without infection may be a sign of bladder dysfunction.
• Lower tract evaluation with videourodynamics is important.
– VUR should be corrected if breakthrough infections occur or if it remains high
grade.

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• Reimplanting thick, dilated ureters into the abnormal bladder
– challenging.
– A 15–30% complication rate
• most often persistent reflux or ureteral obstruction.
• Bilateral high-grade VUR is found
– Transureteroureterostomy can be performed
• With a single, long, tapered reimplant and a psoas hitch.
• Unilateral high-grade reflux into a kidney with reasonable function
– transureteroureterostomy into the nonrefluxing ureter is an option.
• Boys with unilateral VUR into a dysplastic kidney
– Nephrectomy

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• Bladder Dysfunction After Initial Therapy
– The prognosis for boys with PUV depends on the status of the kidneys
and the bladder at the time of diagnosis.
– In 40% with PUV, ESRD or chronic renal insuffciency develops
• Majority of these boys have voiding dysfunction.
– The bladder abnormalities
• Manifested as incontinence and/or persistent hydronephrosis.
• Can be life long problem

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• Long-term Risk Of End-stage Renal Disease
– Study Finland reported outcomes in 193/200 males with PUV
• With a median age of 31 years (range 6–69 years)
• The lifetime risk of ESRD in this cohort was 28.5%.
• The time to progression to ESRD correlated with the lowest serum creatinine value during the
first postoperative year.
• An increased risk of ESRD was associated with
– early presentation,
– pneumothorax,
– bilateral VUR, and
– recurrent UTI following valve ablation.
– No patient progressed to ESRD after their mid 30s.

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• RENAL TRANSPLANTATION
– In many cases, impaired renal function can be stabilized during
childhood.
– During adolescence, there may be insufficient renal reserve
• Dialysis or renal transplantation becomes necessary.
– Valve bladder may have a detrimental effect on graft survival.

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• Adult Sexual Function And Fertility
– Theoretically, prostate function is affected.
• Elevated urethral pressure during embryonic development and ongoing
voiding dysfunction.
• reflux into the seminal vesicles and ejaculatory ducts
– In a recent report
• no increase in ejaculatory problems
• Paternity rates were similar to the general Finnish population
• The prevalence of lower urinary tract symptoms (LUTS) was increased about
twofold in PUV men

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• VESICOURETERAL REFLUX
– Retrograde passage of urine from the
bladder into the ureter.
• Pathophysiology
– The normal UVJ is characterized by
• an oblique entry of the ureter into the
bladder and
• high ratio of tunnel length to ureteral
diameter.
• Passive valve mechanism.
– Active effects of ureterotrigonal muscle
contraction and ureteral peristalsis.

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• CAUSES •
– Attenuation of the trigone and its contiguous intravesical
ureteral musculature.
• The major causes of VUR
– Any condition that shortens the intravesical ureter
• Less common causes

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• Congenital Causes
–Trigonal Weakness (Primary Reflux)
• Most common
–Familial Reflux
–Ureteral Abnormalities
• Complete ureteral duplication
• Ectopic ureteral orifice
• Ureterocele
• Voiding Dysfunction
• Vesical Trabeculation
–spastic neurogenic bladder and severe distal
obstruction
• Edema of the Vesical Wall Secondary
to Cystitis
• Iatrogenic Causes

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• Classification
– Many attempts at classification
• Low pressure (occurring during the fling phase of the VCUG) vs high pressure
(occurring only during voiding).
• Primary reflux vs secondary reflux.
• Simple reflux vs complex reflux.

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• Degree of reflux - graded from I to V
– Grade I - visualization of a nondilated ureter
only
– Grade II - visualization of a nondilated renal
pelvis, calyceal system & ureter.
– Grade III- mild to moderate dilatation or
ureteral tortuosity with mild to moderate
dilatation of the renal pelvis and calyces.
– Grade IV-forniceal angle is completely
blunted.
– Grade V-Loss of the papillary impressions
along with increased dilatation and tortuosity.

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• Epidemiology
– Incidence quite low in normal
children.
– Higher incidence of VUR (30–40%), in
patients undergoing evaluation for UTI
– Incidence rises as age decreases.
• infant most vulnerable.
– Females account for the majority of
reflux patients.
– Significant risk of VUR in family
members of patients with reflux

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 Diagnostic Evaluation
• X-Ray Findings
• The plain film
–evidence of spina bifida, meningomyelocele, or the absence of the sacrum
• pointing to a neurologic deficit.
• Excretory urograms
–may be normal, clues to the presence of reflux
•Persistently dilated lower ureter
•Areas of dilatation in the ureter
•Ureter visualized throughout its entire length
•Changes of healed pyelonephritis (caliceal clubbing with narrowed infundibula or cortical
thinning).
–A normal intravenous urogram does not rule out reflux.

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• Reflux demonstration
– Simple or delayed cystography
– Voiding cystourethrography
– Radionuclide scanning
– Indigo carmine

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• Instrumental Examination
– Cystoscopy
• Chronic cystitis
• Ureteral duplication,
• Ureterocele
• Ectopic orifice.
• Morphologic characteristics of the ureteral
orifice
• Should not be performed as a part of the
workup for reflux.
– prior to surgical correction
» To define the anatomy
» To rule out other bladder and ureteral anomalies.

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• Natural History
– Extremely variable
• Spontaneous resolution without nephropathy
• clinically silent scar formation
• recurrent pyelonephritis with hypertension and end-stage renal disease.
– Have better prognoses for resolution
• lower reflux grades
• Younger children(infant males)

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• Treatment
– Nonoperative management
• Successful in most patients.
• Four stages:
– diagnostic evaluation
– avoidance of infection
» low-dose continuous
suppressive antibiotics
– voiding dysfunction treatment
– active surveillance
» long-term, strict
surveillance

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• Once a nonoperative regimen is selected, the patient
is committed to long-term, strict surveillance.
– Renal imaging every six to 12 months
• renal growth
• detection of focal scarring.
– Voiding cystourethrography once a year.
– The child’s growth, renal function, and
– blood pressure are monitored.

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• Antireflux surgery
– Absolute indications for surgical correction of VUR
• Progressive renal injury
• Documented failure of renal growth
• Breakthrough pyelonephritis
• intolerance or noncompliance with antibiotic suppression.
– Relative indications
• high grade (IV–V) reflux in young children after a year of conservative follow-up,
• pubertal age with nephropathy at diagnosis,
• parental preference, and
• failure to spontaneously resolve with watchful waiting.

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• The principles of surgical correction of reflux include
– Exclusion of causes of secondary VUR
– Adequate mobilization of the distal ureter without tension or damage to its delicate blood supply
– Creation of a submucosal tunnel that is generous in caliber and satisfes the 5:1 ratio of length to
width
– Attention to
• the entry point of the ureter into the bladder (hiatus),
• the direction of the submucosal tunnel, and
• the ureteromucosal anastomosis to prevent stenosis, angulation, or twisting of the ureter
– Attention to
• the muscular backing of the ureter to achieve an effective antireflux mechanism
– Gentle handling of the bladder to reduce postoperative hematuria and bladder spasms

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• The surgical procedures can be
classified on the basis of the
approach to the ureter
• intravesical, extravesical, or
combined.
– On the basis of the position of
the submucosal tunnel in
relation to the original hiatus
• suprahiatal or infrahiatal.
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• Intravesical ,suprahiatal tunnels
– Politano-Leadbetter Technique
• Intravesical,Infrahiatal tunnels
– Glenn-Anderson technique
– Cohen Cross-Trigonal Technique
• Extravesical Procedures
– Modifed Lich-Gregoir/detrusorrhaphy technique
• Intra-extravesical techniques
– Paquin Technique
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• Operative procedures differs
– Presence or absence of a ureteral anastomosis
– Need for detrusor closure
– Whether the neohiatus is fashioned by an appropriately sized detrusor incision or by the closure
of the detrusor around the ureter.
• Excellent results are nearly uniformly attainable
– Overall success for an open ureteral reimplantation to be 96%.
• 99% with grade I,
• 99.1% with grade II,
• 98.3% with grade III,
• 98.5% with grade IV and
• 80.7% in grade V.

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• Extravesical detrusorrhaphy approach
– Lumen of the bladder is not entered
• No postoperative hematuria With minimal bladder spasms
– The absence of a uretero-vesical anastomosis
• Decreases the risk of postoperative obstruction.
– No ureteral stents, suprapubic catheters, or perivesical drains are
needed.

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• Politano-Leadbetter
technique

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• Glenn-Anderson
technique

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• Cohen cross-trigonal
technique

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• Modifed Lich-Gregoir
/detrusorrhaphy technique

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 Complications Of Ureteral Reimplantation
• Early Complications • Long-term Complications
– Obstruction
– Persistent Reflux • Suprahiatal
• Hiatus
– Contralateral Reflux • Tunnel
– Obstruction • Orifce
– Recurrent or Persistent Reflux

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• Persistent reflux
– Insufficient tunnel length to ureteral diameter ratio.
– High-pressure voiding dynamics
• uninhibited bladder contraction,
• detrusor sphincter dyssnergia, and/ or
• urinary retention.
• Ureteral obstruction
– Ureteral kinking (at the neohiatus or obliterated umbilical artery),
– An excessively high placed neohiatus,
– Construction of a tight neohiatus,
– Anastomotic stricture,
– Devascularization,
– Tight tunnel.

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• REDO REIMPLANTATION
– Technically more challenging
– Requires careful attention to detail
and meticulous surgical technique
• The psoas hitch
• Boari flap
• transureteroureterostomy
• reconfgured segment of bowel

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• Minimally invasive endoscopic
procedures
– Subureteral transurethral injection
(STING)
• Polytetrafluoroethylene (Teflon).
• Dextranomer/hyaluronic acid
copolymer (Deflux)
– Success rates of around 70–80%
– Larger injected volumes and multiple
injection sites
– Long-term reflux resolution rates -
stable, but recurrence

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• Prognosis
– Resolution rates
– Complication(renal damage)
• Pyelonephritis
• Hydroureteronephrosis

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References

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