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## Complete Summary

#### GUIDELINE TITLE

Posterior urethral values. In: Guidelines on paediatric urology.

#### BIBLIOGRAPHIC SOURCE(S)

Posterior urethral valves. In: Tekgul S, Riedmiller H, Gerharz E,
Hoebeke P, Kocvara R, Nijman R, Radmayr C, Stein R. Guidelines on
paediatric urology. Arnhem, The Netherlands: European Association of
Urology, European Society for Paediatric Urology; 2008 Mar. p. 72-7.
\[30 references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Posterior urethral valves

#### GUIDELINE CATEGORY

Diagnosis\
Evaluation\
Management\
Screening\
Treatment

#### CLINICAL SPECIALTY

Obstetrics and Gynecology\
Pediatrics\
Surgery\
Urology

#### INTENDED USERS

Physicians

#### GUIDELINE OBJECTIVE(S)

-   To outline a practical and preliminary approach to paediatric
    urological problems

-   To increase the quality of care for children with urological
    problems

#### TARGET POPULATION

-   Infants born with posterior urethral valves

-   Fetuses identified antenatally with posterior urethral valves

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Diagnosis/Evaluation**

1.  Antenatal and postnatal ultrasonography

2.  Voiding cystourethrography

3.  Nuclear renography with split renal function

4.  Blood tests: creatinine, blood urea nitrogen, electrolytes

**Treatment/Management**

1.  Antenatal treatment

    -   Placement of a vesicoamniotic shunt

2.  Postnatal treatment

    -   Bladder drainage

    -   Valve ablation

    -   Vesicostomy

    -   High diversion

    -   Life-long monitoring for bladder dysfunction, urinary infection,
        renal function

    -   Treatment of bladder dysfunction: clean intermittent
        catheterization, overnight drainage, alpha-blockers, or
        anticholinergics for overactive bladder

#### MAJOR OUTCOMES CONSIDERED

-   Renal function

-   Bladder function

-   Morbidity and mortality of treatment

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

The guidelines were based on current literature following a systematic
review using MEDLINE.

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

**Levels of Evidence**

**1a** Evidence obtained from meta-analysis of randomized trials

**1b** Evidence obtained from at least one randomized trial

**2a** Evidence obtained from at least one well-designed controlled
study without randomization

**2b** Evidence obtained from at least one other type of well-designed
quasi-experimental study

**3** Evidence obtained from well-designed non-experimental studies,
such as comparative studies, correlation studies and case reports

**4** Evidence obtained from expert committee reports or opinions or
clinical experience of respected authorities

#### METHODS USED TO ANALYZE THE EVIDENCE

Systematic Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Application of a structured analysis of the literature was not possible
due to a lack of well-designed studies. Whenever possible, statements
have been classified in terms of level of evidence and grade of
recommendation. Due to the limited availability of large randomized
controlled trials -- influenced also by the fact that a considerable
number of treatment options relate to surgical interventions on a large
spectrum of different congenital problems -- this document is therefore
largely a consensus document.

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

-   The first step in the European Association of Urology (EAU)
    guidelines procedure is to define the main topic.

-   The second step is to establish a working group. The working groups
    comprise about 4-8 members, from several countries. Most of the
    working group members are academic urologists with a special
    interest in the topic. In general, general practitioners or patient
    representatives are not part of the working groups. A chairman leads
    each group. A collaborative working group consisting of members
    representing the European Society for Paediatric Urology (ESPU) and
    the EAU has gathered in an effort to produce the current update of
    the paediatric urology guidelines.

-   The third step is to collect and evaluate the underlying evidence
    from the published literature.

-   The fourth step is to structure and present the information. The
    strength of the recommendation is clearly marked in three grades
    (A-C), depending on the evidence source upon which the
    recommendation is based. Every possible effort is made to make the
    linkage between the level of evidence and grade of recommendation as
    transparent as possible.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

**Grades of Recommendation**

A.  Based on clinical studies of good quality and consistency addressing
    the specific recommendations and including at least one randomized
    trial

B.  Based on well-conducted clinical studies, but without randomized
    clinical studies

C.  Made despite the absence of directly applicable clinical studies of
    good quality

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

There is no formal external review prior to publication.

The Appraisal of Guidelines for Research and Evaluation (AGREE)
instrument was used to analyse and assess a range of specific attributes
contributing to the validity of a specific clinical guideline.

The AGREE instrument, to be used by two to four appraisers, was
developed by the AGREE collaboration
([www.agreecollaboration.org](http://www.agreecollaboration.org/)) using
referenced sources for the evaluation of specific guidelines. (See the
\"Availability of Companion Documents\" field for further methodology
information).

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

**Diagnosis**

An obstruction above the level of the urethra affects the whole urinary
tract in varying degrees.

-   The prostatic urethra is distended and the ejaculatory ducts may be
    dilated due to urinary reflux. The bladder neck is hypertrophied and
    rigid.

-   The hypertrophied bladder occasionally has multiple diverticula.

-   Nearly all valve patients have dilatation of both upper urinary
    tracts. This may be due to the valve itself and the high pressure in
    the bladder, or due to obstruction of the ureterovesical junction by
    the hypertrophied bladder.

-   If there is secondary reflux, the affected kidney functions poorly
    in most cases.

During prenatal ultrasonography screening, bilateral
hydroureteronephrosis and a distended bladder are suspicious signs of a
urethral valve. If a dilated posterior urethra and a thick-walled
bladder (\'keyhole\' sign) are seen, a posterior urethral valve (PUV) is
likely. In the presence of increased echogenity of the kidney,
dilatation of the urinary tract and oligohydramnion, the diagnosis of a
PUV should strongly be considered.

A voiding cystourethrography (VCUG) confirms a PUV diagnosis. This study
is essential whenever there is a question of an infravesical
obstruction, as the urethral anatomy is well outlined during voiding. A
secondary reflux is observed in at least 50% of patients with PUV.
Reflux is consistently associated with renal dysplasia in patients with
PUV. It is generally accepted that reflux in the renal units acts as a
\'pressure pop-off valve\', which would protect the other kidney,
leading to a better prognosis. Other types of pop-off mechanism include
bladder diverticula and urinary extravasation, with or without urinary
ascites. However, in the long-term, a supposed protective effect did not
show a significant difference compared to other patients with PUV.

Nuclear renography with split renal function is important to assess
kidney function. Creatinine, blood urea nitrogen and electrolytes should
be monitored closely during the first few days. A nadir creatinine of 80
micromol/L is correlated with a better prognosis.

**Treatment**

**Antenatal Treatment**

About 40-60% of PUV are discovered before birth. The intrauterine
obstruction leads to a decreased urine output, which could result in an
oligohydramnios. Amnion fluid is necessary for normal development of the
lung and its absence may lead to pulmonary hypoplasia, causing a
life-threatening problem. Intrauterine attempts have been made to treat
a fetus with PUV.

As renal dysplasia is not reversible, it is important to identify those
fetuses with good renal function. A sodium level below 100 mmol/L, a
chloride value of \<90 mmol/L and an osmolarity below 200 mOsm/L found
in three fetal urine samples gained on three different days are
associated with a better prognosis.

The placing of a vesicoamniotic shunt has a complication rate of 21-59%,
dislocation of the shunt occurs in up to 44%, mortality lies between 33%
and 43%, and renal insufficiency is above 50%. Although shunting is
effective in reversing oligohydramnios, it makes no difference to the
outcome and long-term results of patients with PUV.

**Postnatal Treatment**

*Bladder drainage*. If a boy is born with suspected PUV, drainage of the
bladder and, if possible, an immediate VCUG is necessary. A neonate can
be catheterized with a 3.5-5 F catheter. A VCUG is performed to see if
the diagnosis is correct and whether the catheter is within the bladder
and not in the posterior urethra. An alternative option is to place a
suprapubic catheter, perform a VCUG and leave the tube until the neonate
is stable enough to perform an endoscopic incision or resection of the
valve.

*Valve ablation*. When the medical situation of the neonate has
stabilized and the creatinine level decreased, the next step is to
remove the intravesical obstruction. Small paediatric cystoscopes and
resectoscopes are now available either to incise or to resect the valve
at the 4-5, 7-8 or 12 o\'clock position, or at all three positions,
depending on the surgeon\'s preference. It is important to avoid
extensive electrocoagulation as the most common complication of this
procedure is stricture formation.

*Vesicostomy*. If the child is too small and/or too ill to undergo
endoscopic surgery, a vesicostomy is used to drain the bladder
temporarily. If initially a suprapubic tube has been inserted, this can
be left in place for 6-12 weeks. Otherwise, a cutaneous vesicostomy
provides an improvement or stabilization of upper urinary tracts in over
90% of cases. Although there has been concern that a vesicostomy could
decrease bladder compliance or capacity, so far there are no valid data
to support these expectations.

*High diversion*. If bladder drainage is insufficient to drain the upper
urinary tract, high urinary diversion should be considered. Diversion
may be suitable if there are recurrent infections of the upper tract, no
improvement in renal function and/or an increase in upper tract
dilatation, despite adequate bladder drainage. The choice of urinary
diversion depends on the surgeon\'s preference for high loop
ureterostomy, ring ureterostomy, end ureterostomy or pyelostomy, with
each technique having advantages and disadvantages. Reconstructive
surgery should be delayed until the upper urinary tract has improved as
much as can be expected.

Reflux is very common in PUV patients (up to 72%) and it is described
bilaterally in up to 32%. High-grade reflux is mostly associated with a
poor functioning kidney. However, early removal of the renal unit seems
to be unnecessary, as long as it causes no problems. It may be necessary
to augment the bladder and in this case the ureter may be used.

Life-long monitoring of these patients is mandatory, as bladder
dysfunction is not uncommon and the delay in day- and night-time
continence is a major problem. Poor bladder sensation and compliance,
detrusor instability and polyuria (especially at night) and their
combination are responsible for bladder dysfunction. Between 10% and 47%
of patients may develop end-stage renal failure. Renal transplantation
in these patients can be performed safely and effectively. Deterioration
of the graft function is mainly related to lower urinary tract
dysfunction.

#### CLINICAL ALGORITHM(S)

The original guideline document contains an algorithm providing
information on assessment, treatment and follow up of newborns with
possible posterior urethral valves.

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of supporting evidence is not specifically stated for each
recommendation.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

-   Appropriate and timely diagnosis, treatment, and management of
    posterior urethral valve in neonates and children

-   Improvement in urinary tract and renal function

#### POTENTIAL HARMS

-   The placing of a vesicoamniotic shunt has a complication rate of 21%
    to 59%, dislocation of the shunt occurs in up to 44%, mortality lies
    between 33% and 43%, and renal insufficiency is above 50%.

-   Stricture formation is the most common complication of valve
    ablation procedures in which electrocoagulation is used.

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

The purpose of these texts is not to be proscriptive in the way a
clinician should treat a patient but rather to provide access to the
best contemporaneous consensus view on the most appropriate management
currently available. European Association of Urology (EAU) guidelines
are not meant to be legal documents but are produced with the ultimate
aim to help urologists with their day-to-day practice.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

The European Association of Urology (EAU) Guidelines long version
(containing all 19 guidelines) is reprinted annually in one book. Each
text is dated. This means that if the latest edition of the book is
read, one will know that this is the most updated version available. The
same text is also made available on a CD (with hyperlinks to PubMed for
most references) and posted on the EAU websites Uroweb and Urosource
([www.uroweb.org/professional-resources/guidelines/](http://www.uroweb.org/professional-resources/guidelines/)
& <http://www.urosource.com/diseases/>).

Condensed pocket versions, containing mainly flow-charts and summaries,
are also printed annually. All these publications are distributed free
of charge to all (more than 10,000) members of the Association. Abridged
versions of the guidelines are published in European Urology as original
papers. Furthermore, many important websites list links to the relevant
EAU guidelines sections on the association websites and all, or
individual, guidelines have been translated to some 15 languages.

#### IMPLEMENTATION TOOLS

Clinical Algorithm

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness\
Patient-centeredness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Posterior urethral valves. In: Tekgul S, Riedmiller H, Gerharz E,
Hoebeke P, Kocvara R, Nijman R, Radmayr C, Stein R. Guidelines on
paediatric urology. Arnhem, The Netherlands: European Association of
Urology, European Society for Paediatric Urology; 2008 Mar. p. 72-7.
\[30 references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

2008 Mar

#### GUIDELINE DEVELOPER(S)

European Association of Urology - Medical Specialty Society\
European Society for Paediatric Urology - Medical Specialty Society

#### SOURCE(S) OF FUNDING

European Association of Urology

#### GUIDELINE COMMITTEE

Not stated

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Primary Authors*: S. Tekgül; H. Riedmiller; E. Gerharz; P. Hoebeke; R.
Kocvara; R. Nijman; Chr. Radmayr; R. Stein

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

All members of the working group submit a conflict of interest form. The
information is kept on file in the European Association of Urology (EAU)
Central Office database. This guidelines document was developed with the
financial support of the EAU. No external sources of funding and support
have been involved. The EAU is a non-profit organisation and funding is
limited to administrative assistance, travel, and meeting expenses. No
honoraria or other reimbursements have been provided.

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: Available in Portable Document Format (PDF) from the
[European Association of Urology Web
site](http://www.uroweb.org/fileadmin/tx_eauguidelines/19 Paediatric Urology.pdf).

Print copies: Available from the European Association of Urology, PO Box
30016, NL-6803, AA ARNHEM, The Netherlands.

#### AVAILABILITY OF COMPANION DOCUMENTS

The following are available:

-   EAU guidelines office template. Arnhem, The Netherlands: European
    Association of Urology (EAU); 2007. 4 p.

-   The European Association of Urology (EAU) guidelines methodology: a
    critical evaluation. Arnhem, The Netherlands: European Association
    of Urology (EAU); 18 p.

Print copies: Available from the European Association of Urology, PO Box
30016, NL-6803, AA ARNHEM, The Netherlands.

#### PATIENT RESOURCES

None available

#### NGC STATUS

This NGC summary was completed by ECRI Institute on November 18, 2008.
The information was verified by the guideline developer on December 19,
2008.

#### COPYRIGHT STATEMENT

This summary is based on the original guideline, which is subject to the
guideline developer\'s copyright restrictions.

Downloads are restricted to one download and print per user, no
commercial usage or dissemination by third parties is allowed.

### DISCLAIMER

#### NGC DISCLAIMER

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Date Modified: 1/19/2009