3.

5mm
spine

Journal of Indian Association of Pediatric Surgeons • Volume 24 • Issue 1 • January-March 2019 • Pages ***-***
Review Article

Consensus on the Management of Posterior Urethral Valves from

Antenatal Period to Puberty
Shilpa Sharma, Manoj Joshi1, Devendra K. Gupta, Mohan Abraham2, Praveen Mathur3, J. K. Mahajan4, A. N. Gangopadhyay5,
Simmi K. Rattan6, Ravindra Vora7, G. Raghavendra Prasad8, N. C. Bhattacharya9, Ram Samuj4, K. L. N. Rao4, A. K. Basu10

Department of Pediatric The need for successful management of posterior urethral valves always

Abstract
Surgery, All India Institute of
Medical Sciences, New Delhi,
captivates the minds of pediatric surgeons. Its success, however, depends on
India, 1Consultant Pediatric several factors ranging from prenatal preservation of upper tracts to postoperative
Surgeon, Department of pharmacological compliance. Regardless of measures available, some cases
Pediatric Surgery, King Saud do not respond and progress to end stage. The management depends on several
Hospital, Uneyzha City, issues ranging from age and severity at presentation to long‑term follow‑up and
Kingdom of Saudi Arabia prevention of secondary renal damage and managing valve bladder syndrome. This
2
Department of Pediatric
Surgery, Amrita Institute of
article is based on a consensus to the set of questionnaires, prepared by research
Medical Sciences, Kochi, section of Indian Association of Paediatric Surgeons and discussed by experienced
Kerala, 3Department of pediatric surgeons based in different institutions in the country. Standard operating
Pediatric Surgery, SMS procedures for conducting a voiding cystourethrogram and cystoscopy were
Medical College, Jaipur, formulated. Age‑wise contrast dosage was calculated for ready reference. Current
Rajasthan, 4Department of evidence from literature was also reviewed and included to complete the topic.
Pediatric Surgery, Advanced
Pediatric Centre, Postgraduate
Institute of Medical Education
and Research, Chandigarh,
5
Department of Pediatric
Surgery, Institute of Medical
Sciences, Banaras Hindu
University, Varanasi, Uttar
Pradesh, 6Department of
Pediatric Surgery, Maulana
Azad Medical College, Delhi,
7
Department of Paediatric
Surgery, Paediatric Surgery
Centre and PG Institute,
Sangli, Maharashtra,
8
Department of Paediatric
Surgery, Deccan College of
Medical Sciences, Hyderabad,
9
Department of Paediatric
Surgery, Gauhati Medical
College, Guwahati, Assam,
10
Consultant Pediatric Surgeon,
Institute of Child Health,
Kolkata, West Bengal, India
Received: July, 2018.
Keywords: Antenatal obstructive uropathy, chronic kidney disease, posterior
Accepted: September, 2018. urethral valves, renal dysplasia, valve bladder

Address for correspondence: Dr. Shilpa Sharma,

Room No. 4001, Department of Pediatric Surgery,
All India Institute of Medical Sciences, New Delhi, India.
E‑mail: drshilpas@gmail.com
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Quick Response Code: remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is
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How to cite this article: Sharma S, Joshi M, Gupta DK, Abraham M, Mathur P,
DOI: 10.4103/jiaps.JIAPS_148_18 Mahajan JK, et al. Consensus on the management of posterior urethral valves
from antenatal period to puberty. J Indian Assoc Pediatr Surg 2019;24:4-14.

4 © 2018 Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow
 Sharma, et al.: Posterior urethral valve management
Introduction
P osterior urethral valves  (PUV) are a common
anomaly that presents to a pediatric surgeon or
pediatric urologist for management. They are the most
common cause of chronic kidney disease due to urinary
tract obstruction in children. The antenatal cases are
far more than those that actually reach term and are
delivered live. The prognosis can be improved if the
anomaly is tackled soon after diagnosis. Morbidity
in terms of recurrent urinary tract infection  (UTI),
deteriorating renal functions, and growth failure may be
reduced by timely and aggressive interventions, which
involves surgery, pharmacology, and clean intermittent
catheterization  (CIC). The long‑term outcome, however,
remains poor if there is associated renal dysplasia. Due
to inherited spectrum of this disease, the management
and outcome often differ in the hands of different
surgeons and different institutes. This topic and the
surrounding controversies were prepared on the basis
of questionnaires for a panel discussion. The responses
were subsequently compiled and supplemented with
a thorough literature review in an effort to formulate
uniform guidelines and depth in understanding.
Antenatal Diagnosis
The antenatal sonographic findings in PUV include
overdistended bladder (megacystis) and thickened bladder
wall (normally <2 mm, pathologic >3 mm), bilateral
or unilateral hydroureteronephrosis, oligohydramnios,
and poor bladder emptying over a 30 min scan. Other
findings include hyperechoic kidneys  (renal dysplasia,
renal cortical cyst), oligohydramnios, urinoma, or fetal
ascites. The sonographic finding of an enlarged fetal
bladder may simply be a transitory normal variant.
However, it may also be secondary to reflux, obstructive,
neurogenic, or myopathic causes. Repeated sonographic
monitoring for persistence of findings, for changes in
bladder enlargement, and for changes in the volume
of amniotic fluid is required as these signs may be the
indicators of abnormalities of renal function and risk
factors for a poor prognosis.[1]
After exclusion of transient physiological dilatation,
a careful examination of the fetal spine should
be performed. The presence of hydronephrosis in
association with enlarged bladder is indicative of
obstructive uropathy or serious malformation and its
absence is likely indicative of normal variation in
size. The sensitivity and specificity of the antenatal
diagnosis of PUV have been reported as 94% and
43%, respectively.[2] Increased bladder wall thickness
and bladder dilatation were strongly associated with
the diagnosis of PUV  (P < 0.001).[2] On the contrary, a
Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
thick‑walled bladder was observed in 39% and a dilated
bladder in 48% of the infants, with a postnatal diagnosis
other than PUV.[2]
The keyhole appearance due to a distended posterior
urethra is suggestive of obstructive uropathy. The
most common cause is PUV. Other causes include
vesicoureteral reflux, bilateral junction stenosis,
Prune–Belly syndrome, primary megaureter, and ectopic
obstructive ureteral implantation.[2] In females, the
most common cause is urethral atresia. Other causes
of fetal lower urinary tract obstruction include but are
not limited to obstructive ureterocele, urethral stricture
or agenesis, persistent cloaca, and megalourethra.
The ultrasound findings of many of these conditions
are similar, and it is often difficult to differentiate the
cause of the urinary obstruction until after delivery.
The presence of the keyhole sign alone was not found
to be predictive of a diagnosis of PUV  (P = 0.27).[2]
A “keyhole” bladder was more likely to be associated
with urethral atresia  (54.5%) than PUVs  (40.9%).[3] A
neurogenic bladder may also have a keyhole appearance.
The reason for this discrepancy is that the hypotonic
bladder neck may give the sonological picture of a
keyhole.
Poor Prognostic Markers
These include oligohydramnios  (low amniotic fluid
volume defined as the maximum vertical pocket
≤2.0  cm), urinary ascites, perinephric urinoma
(maybe pop off), increased renal echogenicity, and
cortical cysts. Diagnosis of PUV up to the second
trimester is associated with high perinatal mortality and
risk of end‑stage renal disease (ESRD), particularly
if there is severe bilateral hydronephrosis with
oligohydramnios, and findings suggestive of renal
dysplasia.[4‑6] When oligohydramnios is detected in
the second trimester, the mortality may be as high as
90%–95%.[4]
Counseling and Intervention
Even in the era of antenatal diagnosis and early
intervention, the long‑term prognosis of PUV is far from
satisfactory. At follow‑up after 18 years of age, chronic
renal failure was detected in 54%, hypertension in
37.5% with ESRD developing in 21%, and presence of
lower urinary tract symptoms in 29%.[7] The diagnosis is
based on sonological findings which should be repeated
by two different observers, one of which should be a
trained radiologist in Level 2 antenatal sonography. In
India, medicolegal termination in view of bad prognosis
for a fetal condition is permitted as per law. Furthermore,
the sex determination is prohibited and a punishable
5
 Sharma, et al.: Posterior urethral valve management
offense. Accordingly, if an obstructive uropathy is
diagnosed before 20 weeks, the prognosis of the
anomaly is explained to the parents. Most young parents
with an insignificant obstetric history, in consultation
with a gynecologist, after counseling from a pediatric
surgeon and a radiologist opt for medical termination
of pregnancy. Few parents with bad obstetric history,
following in vitro fertilization, late conception, precious
baby, Muslim religion prefer to continue pregnancy with
a guarded prognosis.
In cases diagnosed between 20 and 24 weeks, with
oligohydramnios, echogenic kidneys, bladder urine tap
showing poor prognosis, a guarded medical termination
or watchful attitude for intrauterine demise may be
adopted. The outcome of pregnancy and long‑term
prognosis in cases of PUV diagnosed antenatally
at <24 weeks are extremely poor primarily due to the
prolonged oligohydramnios and the damage to the fetal
kidneys due to the involvement of the upper tracts. In
cases diagnosed between 20 and 24 weeks, without any
poor prognostic factors, the pregnancy may be continued
until term with a repeat ultrasonography  (USG) at
monthly intervals for any signs of poor prognosis.
When the pregnancy is between 24 and 34 weeks, fetal
intervention may be offered if indicated and available.
However, it has been realized that antenatal resection
of valve has high complication and does not improve
prognosis. In patients with severe oligohydramnios,
amnioinfusion may be considered to continue pregnancy
till fetal lung maturity and prevent any mishap.
Beyond 34  weeks, intensive 2  weekly monitoring is
required for amniotic fluid content. If oligohydramnios
or other signs of poor prognosis appear, induced
delivery or cesarean section in case of failure may be
offered based on fetal indication. If the amniotic fluid is
adequate, the pregnancy may be continued till term.
Indications and Role of Fetal
Intervention
1. Diagnosis of obstructive uropathy confirmed on two
separate scans by two experts individually
2. Oligohydramnios
3. Gestation <34 weeks
4. Unilateral or bilateral hydroureteronephrosis without
abnormal karyotyping
5. Detailed USG showing no evidence of renal dysplasia
6. Absence of ruptured membranes, chorioamnionitis,
placental abruption, or active labor
7. Cordocentesis: If the serum beta‑2‑microglobulin
level drawn from the fetal umbilical cord blood is
<5.6 mg/L, then fetal therapy may be offered
6 Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
Table 1: Threshold values of fetal urine parameters that
predict good renal function
Parameter Common units SI Units Other units
Sodium <100 mEq/L <100 mmol/L <230 mg/dl
Chloride <90 mEq/L <90 mmol/L <319.5 mg/dl
Calcium <4 mEq/L <2 mmol/L <8 mg/dl
Osmolarity <210 mOsm/L <210 mOsm/L ‑
Β2‑Microglobulin <10 mg/L <0.01 g/L <1 mg/dl
Total protein <20 mg/dl <0.2 g/L <200 mg/L
8. Fetal vesicocentesis: The fetal bladder is tapped,
and the urine is analyzed for biochemistry and fetal
electrolyte levels. Table 1 depicts the parameters
showing that the kidney is salvageable and fetal
intervention can be planned.
The prenatal USG may not be highly reliable in
differentiating fetuses with obstruction and those without
obstruction.[8] Hence, fetal intervention based on USG
should be done with caution. An initial study on fetal
urine biochemistry had suggested a beta‑2‑microglobulin
>2 mg/L as an indicator of poor renal function.[9] The
threshold was increased to 4 mg/L, and now, it is
10 mg/L. In case some parameters are deranged, a repeat
bladder tap can be performed after an interval of 48 h.
If the fetal urine becomes progressively hypoosmolar
as indicated by better biochemical analysis results,
intervention may be planned.
Besides termination of pregnancy, the options for
intervention include vesicoamniotic shunting and fetal
endoscopic ablation of valves. The first case of prenatal
surgery to relieve obstructive uropathy was performed in
1981.[10] Surgical fetal intervention primarily in the form
of vesicoamniotic shunt placement was based upon the
rationale that restoring amniotic fluid to normal levels by
shunting fetal urine from the obstructed urinary system
to the amniotic space would prevent lung hypoplasia
and thus improve neonatal survival. However, over
the past three decades since the introduction of fetal
intervention for obstructive uropathy, data on outcome
of fetal surgery for PUV suggest that it is associated
with an increased risk of fetal and maternal morbidity
without proven benefit for long‑term renal outcome.
Fetal endoscopic ablation is a relatively newer
interventional modality first reported in 1995 by
Quintero et  al.[11] Most of the available outcome data
relate to vesicoamniotic shunting. It has been seen
that vesicoamniotic shunting in PUV does correct
the existing oligohydramnios, although the perinatal
mortality and long‑term survival after this procedure
remain poor.[12,13] This fact is important as the parents
need to be counseled that the incidence of renal failure
in childhood, even after vesicoamniotic shunting, could
 Sharma, et al.: Posterior urethral valve management
Table 2: Varied nonurological and urological presentations in posterior urethral valves
Age Non urological
Neonates Poor breathing movements, Small chest cavity, respiratory
distress due associated pulmonary hypoplasia, urinary
ascites, Potter’s facies, limb deformities e.g., indentation
of knee and elbow due to compression
Infant Failure to thrive, vomiting, dehydration, hypotonia, fever,
uremia, metabolic acidosis, electrolyte imbalance
Older boys Poor growth, hypertension, lethargy, large abdominal
mass in suprapubic region
Incidental
be as high as 50%.[14] Moreover, the procedure has a
45% complication rate which includes shunt blockage,
preterm delivery, and urinary ascites. Thus, while
shunting improves the pulmonary outcome and not renal
outcome, fetoscopic valve ablation may improve both.
Currently, fetal surgery for PUV should be considered
only for fetuses who have a high risk of in utero
or neonatal death due to midtrimester severe
oligohydramnios and who have both a normal karyotype
and evidence of good renal function based upon fetal
urinary evaluation.
Need for Early Referral
There is a dire need for good collaboration between
the pediatric surgeon with the triad of gynecologist,
radiologist, and perinatologist or pediatrician for
diagnosis and timely referral. There should be joint
meetings, case discussions, participation in postgraduate
teaching, and creation of awareness about the possible
treatment options available from the pediatric surgeon
perspectives. The parents should be informed in detail
about the various situations and the protocols to be
followed. They are the best ones to convey the plans of
the different specialists to each other.
Postnatal Management
If the baby after birth is unstable, in metabolic acidosis
and uremia, urinary catheterization is done immediately.
Once drainage is established, the patient may have
large urinary water and solute losses because of tubular
dysfunction, resulting in an inability to concentrate the
urine or normally absorb solutes (sodium and potassium).
In addition, some patients with PUV due to urinary
obstruction may have type IV renal tubular acidosis.
As a result, careful monitoring of serum electrolyte
including serum bicarbonate and fluid status is required
Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
Urological
Poor Stream
Palpable bladder
Palpable kidneys
Weak stream or dribbling of urine
Urosepsis
Straining or grunting while voiding
Weak or abnormal urinary stream, Urinary tract
infections, Diurnal enuresis (incontinence) in boys older
than 5 years, Secondary diurnal enuresis, voiding pain
or voiding dysfunction, Urgency, frequency, hesitancy.
Incidentally diagnosed hydronephrosis on ultrasound
done for unrelated condition, Urinary tract infections,
Proteinuria, Increased serum creatinine
with timely replacement of fluid and electrolytes as
needed. One may have to put two intravenous lines
for chasing the output and supplementing bicarbonate.
Diversion is to be considered if there is persistent
elevation of serum creatinine. Primary ablation is the
preferred mode of management with urinary catheter
drainage for at least 72 h.
Postnatal Diagnosis
The various common modes of presentation according to
age are given in Table 2.
Investigations
1. USG of the kidneys, ureters, bladder  (KUB), and
posterior urethra
2. Renal function tests and serum electrolytes
3. Blood gas analysis if baby is sick
4. Voiding cystourethrogram (VCUG)
5. Renal dynamic scan to assess renal function after the
baby is 1 month of age. The scan should be done with
a catheter in situ in cases with hydroureteronephrosis
6. Dimercaptosuccinic acid (DMSA) scan to assess
scarring in the presence of reflux
7. Diagnostic cystoscopy may be done in cases who are
already diagnosed during antenatal scans. Nakai et al.
described a top‑down approach where they picked up
some patients with mild concomitant PUV among
patients with primary vesicoureteral reflux (VUR).[15]
Age‑wise Standard Operating
Procedures for Conducting a Proper
Voiding Cystourethrogram
Ideally, a pediatric surgeon and a radiologist should
perform the procedure in conjunction. Once the protocol
of the investigation has been established as a routine
and workforce is trained, the responsibility may be taken
7
 Sharma, et al.: Posterior urethral valve management

Table 3: The age-wise maximum dose of the contrast agent that can be used and the formulae for calculating the
maximum expected bladder capacity
Age Urografin 30% (max dose in ml/kg) Urografin 76% (max dose in ml/kg) Expected maximum Bladder capacity
Newborn to 3 months 6 2.4 7 ml/kg
3-12 months 6 2.4 7 ml/kg
1‑8 years 4 1.6 1 <2 years: 7 ml/kg >2 years: (Age +
2) x 30ml
Over 8 years 3 1.2 (Age + 2) x 30ml

Table 4: The weight-wise maximum dose of the contrast agent that can be used and the suggested dilution to attain a
volume near the maximum expected bladder capacity
Body Maximum Bladder capacity Urografin 30% Urografin 76%
weight in kg in ml (Approx. Age in years) Maximum dose in ml Minimal Dilution Maximum dose in ml Minimal Dilution
3 21 18 1:1 7 1:3
5 35 30 1:1 12 1:3
10 90 (1) 40 1:2 16 1:4
15 150 (3) 60 1:2 24 1:4
20 210 (5) 80 1:2 32 1:4
25 300 (8) 100 1:2 40 1:4
30 360 (10) 90 1:2 36 1:4

over by the radiologist. Following are the guidelines for
conducting the procedure. Preprocedure antibiotics should
be given 1–2 days prior and 2–3 days after the procedure.
Older children are instructed to clear their bowel before
the investigation. If urosepsis is present, it should be
treated with intravenous antibiotics, and the urine should
be sterile before the procedure. All equipment should be
laid out on a sterile trolley by a nurse/technician. The
suite should be warmed for babies and infants.
Important Information of the Contrast
Agent
• Urografin 30% contains 40  mg of sodium diatrizoate
and 260 mg of meglumine diatrizoate per mL[16]
• Urografin 76% contains 100 mg of sodium diatrizoate
and 660 mg of meglumine diatrizoate per mL.[16]
The inactive ingredients include sodium calcium
edentate and water for injection.
The child with renal dysfunction, diabetes, repeated
studies, and hypertension should be properly hydrated
before and after the study. Contrast media which are
warmed to body temperature before administration
are better tolerated and can be injected more easily
because of reduced viscosity. Using an incubator,
only the calculated number of bottles needed for the
same examination day should be warmed up to 37°C.
Pretesting sensitivity testing using a small test dose
of contrast medium is not recommended as it has
no predictive value. Furthermore, sensitivity testing
itself has occasionally led to serious and even fatal
hypersensitivity reactions.
Side effects may present in the form of nausea, vomiting,
sensation of pain, a general feeling of warmth, mild
swelling of the face, lips, tongue, or throat, conjunctivitis,
coughing, itching, running nose, sneezing, hives, headache,
disturbance in breathing, difficulty in breathing, and skin
redness. Allergic reaction may occur rarely in the form
of allergic rash, other kinds of skin rash, cramp, and
whitening eyes. Severe reactions requiring emergency
treatment can occur in the form of a circulatory reaction
accompanied by blood vessel dilation and subsequent low
blood pressure, increase in heart rate, difficulty in breathing,
agitation, confusion, and “turning blue,” possibly leading to
unconsciousness. Allergic reactions occur more frequently
in patients with an allergic disposition. These reactions
can be aggravated in patients taking beta‑‑blockers for
maintenance of blood pressure. Temporary renal failure
may occur in rare cases. Preventive measure against acute
renal failure following contrast medium administration
includes ensuring adequate hydration.
Fluid and electrolyte balance must be corrected before
the examination. Newborns and infants are susceptible to
electrolyte imbalance. Care should be taken regarding the
dose of contrast medium to be given [Tables 3 and 4], the
technical performance of the radiological procedure, and
the patient status. Anxiety and pain may increase the risk of
side effects or intensify contrast medium‑related reactions.
Newborn to 3 Months Age or up to 5 kg
1. Two attendants should hold the newborn in the supine
position. A plain skiagram covering the kidneys,
ureters and bladder region and the lumbosacral spine

8 Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019

 Sharma, et al.: Posterior urethral valve management
is taken in anterior‑posterior and lateral position to
assess the spine and bowel gases
2. The genitalia should be cleaned with betadine scrub
and dried
3. A 1–2 ml syringe should be used to instill 1 ml of
xylocaine jelly in the urethra or layered over an
infant feeding tube no.  5/6 French  (Fr). The feeding
tube should be inserted gradually till it reaches the
bladder, 4 cm proximal to the black mark
4. The position of the baby is made in such a manner
that the pelvis is covered in an oblique manner with
one leg extended and the other abducted and knee
flexed. The direction of the urethra should all clear
of the pubic rami and in line with the bladder
5. The contrast is prepared in a dilution of 1:1 initially.
This is so as in cases of PUV, the bladder is usually
distended and may contain a postvoid residue.
Further, in the presence of reflux, the urine may
dilute the contrast. The dilution may be increased
to up to 1:3 if there is minimal urine in the bladder
and the presence of high‑grade reflux requires more
volume of contrast preparation. Higher concentration
may mask lesions such as diverticula and ureteroceles
6. About 10 ml of diluted contrast is slowly injected to
outline the bladder and assess its size. The contrast
may also be given as a slow infusion. An AP film
may be taken in the filling phase
7. Another 10 ml is then slowly injected/infused till the
bladder is full. Right oblique and left oblique views
are taken
8. Another 5–10 ml is slowly injected/infused to assess
the presence of reflux. In case higher grade reflux is
present, more contrast can be injected till both the
kidneys are visualized
9. The catheter is then slowly removed and a container
is placed below the baby to collect the urine. The
baby is encouraged to pass urine, and the voiding fill
is captured in the right or left oblique manner
10. During the whole procedure, intermittent still shots
are captured as instructed to the radiographer on
the machine. Relevant fluoroscopic videos may also
be obtained taking care not to expose the baby to
unnecessary radiation exposure.
The baby is cleaned with clean water, wiped, and dried
after the procedure. The parents are encouraged to give
excessive fluids.
Infants
The procedure is same as above. The infant feeding
tube may be of size 7/8 Fr. The expected maximum
bladder capacity of 7 mL/kg should be kept in mind
while injecting contrast preparation. The volume may be
higher in the presence of reflux.
Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
Toddlers
These are most difficult to manage and may require
video games of smartphones to keep them occupied
during the procedure. The infant feeding tube may
be of size 9/10 Fr. Koffman formula for the expected
maximum bladder capacity of  (age  +  2) × 30  ml should
be kept in mind while injecting contrast preparation. The
volume may be higher in the presence of reflux.
Boys More than 3 Years
The preputial skin should be clean and retractable before
subjecting them to VCUG. The procedure should be
explained to them. The parents may be asked to bring
an extra pair of clothing of the child. If the child is
uncomfortable to void in a supine or semireclining
position, the machine may be adjusted so that he can
stand and void. The lateral oblique films enable us to
pick up the diverticulae if any. In the presence of higher
grade reflux in older boys, the dilution may be up to
1:4–5. The child is encouraged to drink excessive fluids
and triple void postprocedure.
Standard Operating Procedure
Guidelines for Cystoscopy for
Posterior Urethral Valve
Zero/Six degree 6.5 Fr panendoscope is good for
cystoscopy in neonates <3 kg weight. An 8/9 Fr
miniature pediatric cystourethroscope with a 4/5 Fr
working channel may allow insertion of a Bugbee
electrode to fulgurate the valves under vision. Zero/thirty
degree 8.5/9.5 Fr cystourethroscope/resectoscope may
be used for diagnosis in babies over  3.5  kg. The
8/10/11 Fr resectoscope with 0° telescope is preferred
for visualization and fulguration/incision of the valves.
The sickle‑shaped cold knife is preferred by most
surgeons. Others may use the cutting loop, hook with
or without ball‑tip electrode, sharp‑angled electrode, or
blunt‑angled electrode. For infusion, warm normal saline
may be used. Some prefer 1.5% glycine for fulguration.
The neodymium‑doped yttrium‑aluminum‑garnet laser
may be used for fulgurating the PUV in smaller caliber
urethra as this can be performed with the smaller
available cystoscope that has a side channel, admitting
the laser fiber.[17] The appropriate instruments are
chosen and lined up on the sterile instrument tray in
chronological order. An antibiotic dose of co‑amoxiclav
at 30 mg/kg intravenous is given at the time of induction.
After induction under general anesthesia and caudal
analgesia, the baby is put in lithotomy position with
feet supported in stirrups. The genital area is cleansed
with an antiseptic solution taking care to retract the
prepuce while cleaning and pulling it back and draped.
9
 Sharma, et al.: Posterior urethral valve management
Xylocaine (lidocaine monohydrochloride) jelly 2% is
instilled in the urethra with a 5 ml syringe in the maximum
dose of 4.5 mg/kg/dose or maximum 300 mg/dose. Each
ml of 2% lidocaine contains 20 mg of lidocaine HCl. An
infant may require 3–5 mL (60–100 mg of lidocaine HCl).
The endoscope is well lubricated and inserted into the
urethra to the bladder, and the urine sample is collected
for analysis and culture and sensitivity if required. The
ureteric orifices are seen for size and position. The
bladder wall is inspected for trabeculations, sacculations,
and diverticulae. The scope is gradually withdrawn along
the posterior bladder wall, bladder neck into the posterior
urethra till the verumontanum bulge is appreciated on the
PUV. The configuration of the bladder neck is noted. The
posterior urethra should be carefully inspected, and the
valve configuration is noted as it appears just distal to
the verumontanum. The resectoscope is assembled with
either the cold/sickle blade or Bugbee electrode. The
resectoscope sheath is advanced into the bladder. The
video camera is connected for better visibility of all on
the screen. The mobility and direction of the cold knife/
electrode are examined in the bladder. The electrode/knife
is withdrawn and the resectoscope is brought into the
posterior urethra. The knife/electrode is again advanced
and brought to hook against the valve at 5 o’ clock
position. The procedure is repeated for the other two
positions at 7 and 12 o’clock positions. The resectoscope
is gradually removed from the urethra, and the stream of
urine is visualized by gentle Crede’s maneuver. A check
scopy may be done for assessing the effectiveness of the
procedure. The urinary catheter is left for 72 h or till the
diuresis has subsided whichever is later.
Alternatives to Primary Fulguration
If the smallest size scope is not available for preemie,
one should put in a catheter and drain the bladder. The
parents are counseled. One option is to wait and reassess
after 2–4 weeks, and if still not feasible, a vesicostomy
may be done. The other option is to refer to a center with
availability. Valvotomy with indigenous valvulotomes
(Chooramani or Abraham) is a blind procedure but safe
in selected experienced hands. However, with recent
developments, most centers have appropriate‑sized
instruments. The use of a Fogarty catheter to disrupt
valve in low birth weight babies is not recommended.
Indications for Diversion
If primary ablation cannot be done due to nonavailability
of appropriate instruments or due to technical reasons,
the next preferred procedure is vesicostomy particularly
in very preterm infants in whom visualization or ablation
of the valve is not possible.
10 Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
Higher diversion is not indicated if the renal parameters
have become normal and the upper tracts are not
tortuous as it does not improve outcome as compared
to vesicostomy. It may affect bladder cycling and may
have an impact on bladder function. However, if the
baby is in urosepsis and the upper tracts are dilated
and tortuous with deranged renal parameters despite
1 week of catheterization, higher diversion in the form
of ureterostomy is indicated. It may be done on the one
side to allow the bladder to grow with urine from the
other kidney and also allowing the distal ureterostomy to
act as a pop‑off mechanism for reflux. In severe cases,
bilateral ureterostomy may prove to be life‑saving.
Vesicoureteral Reflux in Posterior
Urethral Valves
VUR is present in about ⅓ to ½ of the patients of PUV.[18]
Half of these will have unilateral reflux and the other half
would have bilateral. VUR will resolve in at least one‑third
of the patients with relief of obstruction. The remaining
two‑thirds would require deflux injection or surgery.
Resolution of reflux has also been seen with addition of
alpha‑blockers. High‑grade reflux with dysplasia leading
to a nonfunctioning kidney may require nephrectomy. It
is unclear whether VUR in PUV independently affects
long‑term functions. The incidence of ESRD before
16 years was highest in a patient with bilateral VUR up
to 25% followed by 7% with unilateral VUR. Serum
creatinine level was persistently elevated in patients with
VUR at diagnosis, after 6 months, and after 12 months.[19]
Bladder Dysfunction in Posterior
Urethral Valves
Approximately one‑third of the patients have persistent
bladder dysfunction after PUV ablation, and these require
pharmacological therapy and CIC. In one case series,
severe bladder dysfunction  requiring CIC was predictive
of ESRD.[18,20] A high incidence of patients up to 50%–
70% have high postvoid residue due to bladder neck
hypertrophy. Radiological indicators of bladder dysfunction
include persistent upper tract dilatation, posterior urethral
dilatation, VUR, trabeculation and diverticula in bladder,
and significant postvoid residual urine.
Renal Dysplasia, Chronic Kidney Disease,
and End‑Stage Renal Disease in Posterior
Urethral Valves
Prenatal renal parenchymal changes consistent with renal
dysplasia are seen in about 60% of infants in prenatally
diagnosed PUV patients.[21] A study on the histology
of kidney specimens from children with PUV who
underwent nephrectomy reported that approximately
 Sharma, et al.: Posterior urethral valve management
80% of the specimens showed primary dysplastic
malformations (mesenchymal or fetal cartilage tissue
or dysplastic glomeruli and tubuli) in the presence of
well‑developed renal parenchyma and all specimens
showed secondary pathologies such as renal‑cortical
atrophy, interstitial fibrosis, and interstitial‑nephritis
atrophy.[22] Organogenesis of the kidney is terminated at
the 12th gestational week and secondary renal damage
is irreversible at the 20th gestational week, but prenatal
urinary diversion of the upper urinary tract is feasible in
the 20th gestational week at the earliest. These facts must
be taken into account when considering intrauterine
urinary diversion. About 15%–20% cases of PUV
progress to ESRD.[18,21] Recurrent infection with scarring
is thought to be a risk factor for ESRD in the long
term. However, in one study of 119 patients, recurrent
infection was present in almost half patients group and
was not associated with increased risk of ESRD.[21]
Follow‑up
The protocols may vary from center to center and
among different surgeons/nephrologists with the main
aims remaining universal.
The principles of follow‑up for PUV are to:
1. Maximize renal function
2. Minimize urinary infections
3. Minimize renal scarring
4. Assess voiding dysfunction
5. Attaining urinary continence
6. Assess bladder growth
7. Assess need for renal replacement therapy and renal
transplant.
• Postfulguration: 3 months, 6 months, 9 months, 1 year,
annually up to 15  years of age: Urea, creatinine,
complete blood count, electrolytes, midstream urine
for analysis, USG with postvoid residue measurement,
examination of urine stream/uroflowmetry were noted.
More than 10% of the prevoid volume is abnormal
and described as significant postvoid residue
• Check cystoscopy/VCUG at 3  months
postfulguration: If symptoms persist/routine
(optional). Morphological normalization of the
posterior urethra after fulguration on VCUG has
been described as a significant factor in prognosis[23]
• Corrected glomerular filtration rate  (GFR)  –  1, 3, 5,
10, 13, 15 years
• Renal scan  (diethylenetriaminepentaacetic acid/)
Technetium-99m ethylene dicysteine (EC)/ 99mTc-
mercaptoacetyltriglycine (MAG3) – 3 months and 1,
3, 5, 8, 11, 13, 15 years
• DMSA – If Hydroureteronephrosis/VUR are present,
then at 3 months and 1, 3, 5, 10, 15 years
• If HDUN/VUR present, then voiding diary for
1 week at 1, 3, 5, 10, and 15 years
Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
• If dribbling/reflux present, urodynamic study at 3, 5,
10, and 15 years.
Indications for Repeat Voiding
Cystourethrogram during Follow‑up
1. At 3  months postfulguration: If symptoms
persist/routine (optional). Check VCUG should
be mandatory before labeling the patient as valve
bladder or any other sequelae[23]
2. If HDUN present postfulguration on USG, repeat
VCUG at 2, 5, and 10 years
3. To assess structural anomalies such as diverticuli,
obstructive lesions – residual valves, and strictures
4. To assess if VUR is present during bladder filling,
voiding, or both
5. If deflux/ureteral reimplantation planned
6. To study the bladder anatomy before bladder
augmentation or renal transplant.
Urodynamic Study
Urodynamic studies are helpful in with radiological signs
of persistent bladder dysfunction, significant postvoid
residue, persistent VUR on anticholinergics and CIC. The
other indications for urodynamic study are as follows:[24]
1. Persistent daytime urinary incontinence beyond the
age of 5 years
2. Deterioration in renal function (rising creatinine or drop
in GFR) with no obvious cause such as growth spurt
3. Increase in upper tract dilatation in the absence of
ongoing outflow obstruction
4. Before renal transplantation to ensure a safe,
compliant low‑pressure urinary tract
5. To assess the efficacy of treatment or intervention
6. As a research tool to study the evolution or natural
history of developing bladder function in boys with
successfully treated PUV
7. Functional problems – detrusor‑sphincter
discoordination or suspected dyssynergia
8. Bladder emptying, true and false residuals.
Drug Treatment
A variety of agents have been used to treat bladder
overactivity.
Anticholinergics
Anticholinergic agents are commonly used to treat
reduced bladder compliance and the overactivity during
bladder filling. The most common side effect of the
anticholinergics is dryness of mouth. During the initial
treatment period, there is more symptomatic improvement
when anticholinergics are combined with bladder training
as against each modality alone. Agents such as imipramine,
oxybutynin chloride, and tolterodine tartrate have been
11
 Sharma, et al.: Posterior urethral valve management
found to be effective for noncompliant or overactive
bladder.[25] The safe dose in children is oxybutynin
0.2 mg/kg/day in 2–4 divided doses and imipramine
0.7–1.2 mg/kg/dose, 2–3 doses per day. Pro‑Banthine may
be given in a dose of 0.5 mg/kg/dose twice a day although
it is not popular among pediatric surgeons.
Oxybutynin inhibits stretch‑induced bladder smooth
muscle cell proliferation in vitro studies.[26] A protective
effect of oxybutynin on bladder function and structureless
collagen infiltration in the detrusor with fewer glycogen
granules on the hypertrophic and ischemic bladder
changes is an argument for an early start of oxybutynin
treatment in children with urethral valves.[26]
Oral tolterodine may be preferred over oxybutynin by some
because of the reduced risk of dry mouth. It has been seen
in adults that extended‑release preparations of oxybutynin
or tolterodine are preferred to immediate‑release
preparations because there is less risk of dry mouth.[27]
The use of two anticholinergic medications simultaneously
(oxybutynin, tolterodine, and/or solifenacin) could
optimize the medical therapy for children in whom
single‑agent anticholinergic therapy has failed.[28]
Inappropriate use of anticholinergic medications
may induce iatrogenic myogenic failure. Therefore,
they should be used only with urodynamic
monitoring.[29] Fortunately, this drug‑induced myogenic
failure is reversible on stopping treatment.[30]
Alpha‑adrenergic blockers
Terazosin in doses ranging from 0.25 to 2 mg has proved
to be safe and results in significant improvement in
bladder emptying in PUV. There was a reduction of 85%
in the pretreatment postvoid residual urine volume.[31]
Hypotension was not a common side effect in children.
Chemoprophylaxis
Night‑time uroprophylaxis is prescribed in cases with
associated VUR. The cyclical chemoprophylaxis of 3 weekly
cephalexin, septra, and amoxicillin is usually prescribed
Role of Nephrologist
The nephrologist should be involved earlier in cases
of deranged renal parameter. They take care of timely
institution of renal replacement therapy including Vitamin
D, soda bicarbonate, calcium, erythropoietin, and manage
renal tubular acidosis. Proteinuria depicts early renal
damage. Measuring plasma renin level and screening for
microalbuminuria are not a routine practice.
Refluxing Loop Ureterostomy
The valve bladder in the initial 1–2 years following valve
ablation has a reduced capacity with detrusor overactivity
12 Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
and high voiding pressures. In the presence of gross reflux
and impaired renal function, a low refluxing ureterostomy,
proposed by Philip Ransley, helps to neutralize the
abnormal bladder dynamics and at the same time maintains
bladder cycling.[24] The ureterostomy can be closed at
a later date; alternatively if associated with a poorly
functioning kidney, it can proceed to a nephrectomy.
Timed Voiding
The patients and the parents should be counseled
about bladder functioning, need for adequate fluid
intake, recognizing the urge sensation, need for regular
bladder emptying, and eradication of urine holding
maneuvers.[25] The children with dilated upper tracts
should be encouraged to do double or triple voiding.
Constipation should be avoided. Adequate bladder
emptying can be achieved by timed voiding both
during the waking time and during the sleeping time in
older children. In cases where the postvoid residue is
significant, CIC should be taught. The frequency of CIC
depends on the severity of bladder dysfunction.
Clean Intermittent Catheterization
The bladder may not be able to empty completely due to
inherent ineffective detrusor contractions or as a side effect
of the anticholinergic medications, necessitating the use of
catheters to empty the bladder. CIC should be initiated
at an early age when indicated. It improves the bladder
instability and also the renal function with an increase in
the median differential GFR.[32] It was interesting to note
that in this study, there was functional renal deterioration in
those patients who stopped CIC. The sensate perineum and
the dilated posterior urethra may make doing CIC difficult
in a child, leading to noncompliance and deterioration of
upper tracts. An abdominal catheterizing channel may be a
viable alternative to keep the bladder empty.[33]
Nocturnal Bladder Management
This involves timed emptying of the bladder or
continuous drainage as an adjunct to the treatment
of bladder dysfunction. The valve bladder syndrome
is associated with a persistent bladder dysfunction
characterized by chronic overdistention of the urinary
bladder, which is exacerbated by associated polyuria.
There is a long period of full bladder in the night during
sleep leading to increased pressures that will impair
the upper tract drainage with further deterioration of
renal function. Nocturnal bladder drainage reduces the
frequency of UTIs, improves upper tract dilatation, and
improves continence. Overnight drainage in conjunction
with daytime CIC can be appropriate management in
children with poorly compliant bladders, especially in
the early stages of renal compromise.[34,35]
 Sharma, et al.: Posterior urethral valve management
Biofeedback Therapy and Pelvic Floor
Exercises
Children with urodynamically proved lower urinary
tract dysfunction after successful valve ablation
could be managed by biofeedback therapy and home
pelvic floor exercises, with an overall consistent good
response in 70%.[36] This could possibly avoid or reduce
anticholinergic drugs and avoid CIC.
Bladder Neck Ablation
Secondary bladder neck obstruction has been over
diagnosed in patients with PUV. Earlier, endoscopic
bladder neck incision or open bladder neck repairs used
to be made with poor clinical outcome. There are few
reports of reduced detrusor overactivity, reduced need
for anticholinergic medications, and better bladder
urodynamics following simultaneous bladder neck
incision and valve ablation.[37] The practice has not been
adopted universally as long‑term results are awaited.
Bladder Augmentation
Augmentation of the bladder may be required in PUV
patients with valve bladder when medical management
fails to prevent the deterioration of renal function or
the bladder is very small with thickened wall and renal
transplant has been planned. The parents should be
aware of the associated metabolic consequences and
long‑term complications such as metabolic acidosis and
malignancy. Enterocystoplasty achieves a better storage
function outcome while ureterocystoplasty has shown
a durable functional and urodynamic improvement in
valve bladder patients.[38]
Renal Transplantation
As up to 50% of PUV patients can end in ESRD, renal
transplantation may be required. Preoperative bladder
management and continued monitoring of bladder
and kidney function postoperatively are of paramount
importance in the preservation of allograft function.
This may require bladder augmentation to increase the
bladder capacity. Reconstruction of the lower urinary
tract followed by renal transplantation is a safe and
efficient approach restoring the lower urinary tract before
immunosuppressive therapy and supplying the best
possible reservoir for a transplanted kidney.[39] However,
renal transplantation can be performed safely without
preemptive bladder augmentation as no difference was
seen in renal outcome and bladder capacity. Thus, the
decision about the need for bladder augmentation can be
made after normal diuresis is restored.[40]
Journal of Indian Association of Pediatric Surgeons / Volume 24 / Issue 1 / January-March 2019
Outcome
Poor prognostic factors include prenatal detection at
<24 weeks gestation, respiratory distress at birth, urinary
sepsis, dyselectrolytemia, nadir serum creatinine >0.8 mg/
dL, bilateral VUR, hyperechoic kidneys, and absence
of pop‑off mechanism. It is speculated but it is unclear
that later presentations have better outcome. A serum
creatinine of 0.3–0.7 mg/dL for children under age 3 and
0.5–1.0 mg/dL for children ages 3–18 years is considered
safe. Persistently elevated serum creatinine after procedure
is a risk factor for ESRD. A serum creatinine of 1 mg/dL
or above at 1 year of is poor prognosis.
Conclusion
The management of PUV is from the antenatal period
to puberty. Early fulguration of the valves after
stabilization is the first step in management. It is a
famous aphorism that the management of PUV actually
begins after valve fulguration. A department dealing with
neonatal urological problems should have good antenatal
support system and endourological instrumentation for
timely intervention. It is vital to identify the bladder
dysfunction and manage it appropriately to prevent
any deleterious effects on the upper tracts. A judicious
mix of timed voiding, anticholinergics, and bladder
drainage is important. Postfulguration, a close watch on
upper urinary tract functions and renal tubular acidosis
by regular serum creatinine and soda bicarbonate
levels along with postvoid residue is imperative. Those
showing evidence of noncompliant bladder in spite
of medical management may become candidate for
augmentation. Deterioration of upper function with
persistent high creatinine suggests ESRD and need for
renal replacement. Long‑term follow‑up till adolescence
is therefore a key in management. Appropriate timely
management improves the long‑term survival.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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