Sie sind auf Seite 1von 15

output with normal growth and development combined with the latent polyuria of existing renal

dysplasia and increased glomerular pressure caused by poor bladder emptying conspire to increase
urine volumes stored in the upper urinary tract. As this bolus of urine fills the bladder even as the
bladder empties partially, a lack of extended periods of an empty, relaxed bladder pushes the
compensated bladder toward decompensation. This, of course, leads to even greater postvoid
residuals, overflow incontinence, and further renal damage ( Close et al, 1997 ). The storage and
voiding of urine at high pressures has been simulated in fetal sheep and rabbit models in vitro to
simulate and then investigate the cascade of events effectuating bladder smooth muscle alterations.
Increased bladder dilation is seen following partial bladder outlet obstruction ( Kirsch et al, 2003 ),
and the dilated, poorly contracting bladders (simulating the decompen- sated bladder) lead to more
upper tract dilation. Thus increased bladder dilation places the upper urinary tract at risk. This
model and the work of other researchers confirmed that extracellular matrix elements line the
detrusor smooth muscle cells of the bladder after obstructionas noted in valve bladders
( Workman and Kogan, 1990 ). Importantly, the pathologically signifi cant elevated storage
pressures must be distinguished from the neonatal and infantile elevated voiding pressures that are a
normal feature of bladder development ( Silln et al, 1992 ). There remains some controversy as to
whether the changes in bladder morphology in the valve-affected bladder are reversible, as opposed
to the phenotypic changes in neurogenic bladders that are more permanent ( Keating, 1994 ;
Hutcheson et al, 2004 ). The deposition in the extracellular matrix causes altered contrac- tion and
passive relaxation of the bladder, which may cause multiple intracellular changes ranging from a
reduction in detrusor blood flow causing ischemia to free radical toxicity. These changes alter the
phenotype of detrusor muscle myosin bundles and actin-associated filaments ( Ghafar et al, 2002 ;
Shukla et al, 2004 ; Levin et al, 2005 ). The valvular obstruction will also lead to marked dilation of
the posterior urethra, hypertrophy of the bladder neck, and a flattening of the verumontanum with
dilation of the ejaculatory ductsthe pathognomonic fi ndings of this diagnosis on voiding
cystourethrogram ( Fig. 141-2 and Fig. 141-3 ). These changes seem to return to a more normal
appearance following valve ablation as the offending distal obstruction is removed. Upper Urinary
Tract A bladder exposed to posterior urethral valves undergoes a cascade of changes that alter the
organ s normal function in storing and emptying urine. This bladder dysfunction manifests in
varied ways during infancy and later, and is discussed in a later section. What is apparent is that a
sustained increase in intravesical storage pressures over prolonged time intervals transmits that
pressure to the ureter, the renal pelvis, and ultimately the glomerular unitscausing architectural
and functional changes at each ascending structure ( Koff et al, 2002 ). These architectural changes
can be inferred clinically by the severe hydroureteronephrosis that is often seen in the setting of
posterior urethral valves. Increased echogenicity, parenchymal thinning with cortical cysts, and lack
of corticomedullary differentiation similarly imply significant renal dysplasia ( Fig. 141-4A ).
Ureteral dilation occurs as a direct transmission of pressure from the dysfunctional bladder, as well
as the vesicoureteral reflux that is seen in up to 70% of patients with posterior urethral valves ( Puri
and Kumar, 1996 ; Sarhan et al, 2011 ). Polyuria caused by progressive renal damage and
congenital renal dysplasia compound ureteral dilation as well ( Smyth et al, 1991 ) The chronicity
of ureteral dilation is classically believed to cause ureteral wall thickening, loss of peristalsis, and
loss of mucosal coaptation, increasing the risk of urine stasis, infection, and increased pressures in
the renal units ( Fig. 141-4B ) ( Parkhouse et al, 1988 ; Glassberg, 2001 ). The elevated renal pelvic
pressures secondary to the bladder and ureteral pressure increases lead to significant alterations in
renal morphology and function. The renal dysfunction seen in posterior urethral valves has two
specifi c etiologies: (1) obstructive uropathy and (2) renal dysplasia. Obstructive uropathy causing
renal damage is a well-known phenomenon in various models. Fetal sheep ureteral obstruction
models clearly demonstrate that, although hydronephrosis occurs rapidly after obstruction,
irreversible dysplastic changes in the renal architecture are seen by term, and these changes were
confirmed after outlet obstruction in the same model ( Peters et al, 1992 ; Chevalier, 2004 ).
Apoptosis and increased oxidative stress in mouse kidneys with ureteral obstruction are also seen in
the face of bladder outlet obstruction ( Kawada et al, 1999 ; Chevalier, 2004 ). The increased

pressure from obstruction damages luminal cells in the renal tubules and will also lead to poorly
concentrated urine production ( Li et al, 2004 ; Nguyen et al, 2005a ). Obstruction may also affect
urinary concentration by reducing blood fl ow to the medulla, causing a loss in the medullary
concentration gradient that results in signifi cant polyuria and even in the postobstructive diuresis
seen after catheter placement in an infant with posterior urethral valves ( Dinneen et al, 1995 ).
Although some debate whether the entirety of renal damage seen in posterior urethral valves is
actually a secondary phenomenon resulting from obstruction, the observation that even early
prenatal intervention does not necessarily forestall long-term renal disease posits that renal
dysplasia may be concurrent withrather than a consequence ofposterior urethral valves
( Haecker et al, 2002 ). Others have demonstrated known primary dysplastic malformations of fetal
cartilage tissue or dysplastic glomeruli and tubules in posterior urethral valves that affected renal
tissue at the time of nephrectomy ( Haecker et al, 2002 ). A correlation between severe renal
hypodysplasia and decreased activity of the renin-angiotensin system that modulates renal
development has been noted in posterior urethral valves, as well as a decrease in the angiotensin
receptor type 1 genetic polymorphisms ( Peruzzi et al, 2005 ). Bajpai and associates (2005) found
that plasma renin activity increases precede common clinical findings of renal damage such as
rising serum creatinine, renal scars, and lowering glomerular filtration rate. Although angiotensin II
contributes to renal damage by hemodynamic alterations in glomerular flow, it also induces
profibrogenic actions and inflammation through the induction of transforming growth factor- 1
and tumor necrosis factor- ( Kagami et al, 1994 ; Furness et al, 1999 ; MacRae Dell et al, 2000 ).
These cytokines are considered potential biomarkers that seem to decrease with improvement in
renal function after valve ablation, whereas elevations imply worsening outcomes. Institution of an
angiotensin-converting enzyme inhibitor (ACEI) showed therapeutic potential in experimental
studies by reducing fi brosis ( Yu et al, 2004 ; Gagliardini and Benigni, 2006 ), and seemed to
reduce expression of biomarkers such as transforming growth factor- 1 and tumor necrosis factor in another clinical study, suggesting a potential role in selected children with posterior urethral
valves ( Mandelia et al, 2013 ). Further corroborating the hypothesis that renal damage in valves is
due, in part, to dysplasia and not only obstruction is the finding that the ACE1 gene is expressed
significantly more, and the angiotensin receptor gene ( ATR ) less, in patients with posterior urethral
valves compared to those with other dysplasias and controls ( Peruzzi et al, 2005 ; Laksmi et al,
2010 ). Vesicoureteral Reflux and Dysplasia Hoover and Duckett (1982) observed that the highgrade vesicoureteral reflux seen in children with posterior urethral valves was usually into an
ipsilateral poorly functioning renal unit, whereas the contralateral renal unit appeared to have
preserved renal function ( Fig. 141-5 ). They hypothesized that the reflux served as a pop-off
mechanism in which the dysplastic kidney with reflux served as a pressure reservoir mitigating
damage to the contralateral kidney, and coined the term vesicoureteral reflux and dysplasia
(VURD). This relationship, in that original series, was found in 13% of patients with posterior
urethral valves, and the theory that these children would have better long-term renal function as a
result of the pop-off phenomenon was widely accepted. Longer-term studies have confirmed,
however, that the VURD syndrome does not improve renal prognosis. Fifteen years after the VURD
syndrome was first described, Cuckow and colleagues (1997) found that, whereas 67% of patients
affected by VURD during year 2 of life had a normal serum creatinine, only 30% of these children
had normal values between ages 8 and 10 years. The glomerular filtration rate, importantly, was
already abnormal in 75% of these same patients even in the first 2 years of life. Another observation
that even the contralateral, nonrefluxing renal unit in VURD patients carries a high risk of
congenital renal cortical damageimplying poorer long-term prognosismeans that VURD should
never be allowed to create a false sense of security, and close follow-up as with any other cohort
with posterior urethral valves is mandatory ( Narasimhan et al, 2005 ). DIAGNOSIS
Ultrasonography With widespread access to antenatal sonography, posterior urethral valves and
other LUTOs such as urethral atresia or stenosis are increasingly detected during the fetal period.
Posterior urethral valves are detected in approximately 1 in 1250 ultrasound screenings, accounting
for 10% of significant antenatally detected genitourinary disease and afflicting one third of

surviving infants with bilateral renal disease ( Thomas and Gordon, 1989 ; Gunn et al, 1995 ). The
pathognomonic ultrasound fi ndings of a thickened, dilated bladder along with bilateral hydroureter
and pelvocaliectasis do carry a high sensitivity (95%) and specifi city (80%), and oligohydramnios
and the dilated posterior urethra displaying the keyhole sign further corroborate the presence of
LUTO ( Figs. 141-6 and 141-7 ) ( Peters, 1998 ; Robyr et al, 2005 ). Renal echogenicity will be
increased in posterior urethral valves, and is a reliable indicator to infer renal damage as well.
However, although LUTO may be diagnosed prenatally, differentiating valves from urethral atresia
or prune-belly syndrome, high-grade vesicoureteral reflux, or bilateral primary obstructing
megaureters is much more problematic, reducing the accuracy of diagnosing posterior urethral
valves with prenatal sonography alone to as low as 50% ( Abbott et al, 1998 ). Still, the antenatal fi
nding of a thickened, enlarged bladder and bilateral ureterectasis with pelvocaliectasis with or
without oligohydramnios in a male infant requires an early postnatal sonogram and voiding
cystourethrogram prior to the infant s discharge from the postpartum unit ( Lee et al, 2006 ;
Herndon, 2012 ; St. Aubin et al, 2013 ). Fetal magnetic resonance imaging (MRI) is an adjunct in
prenatal diagnosis and increasingly available at major centers. As with ultrasonography, fetal MRI
is used to distinguish the degree of obstruction based on urethral dilation, distended bladder size
with thickening, and reduced amniotic fluid levels ( Fig. 141-8 ). Lung hypoplasia and cystic
changes in renal parenchyma are also appar- RT TRV bladder Figure 141-7. Sonogram of fetal
bladder shows a thickened bladder with dilated posterior urethra below, suggesting the keyhole
sign. LT, left kidney; RT, right kidney; TRV, transverse. (Courtesy Dr. Mark P. Johnson, Children
s Hospital of Philadelphia.) ent on MRI, though the rate of renal dysplasia does not necessarily
correlate with micro- or macrocystic changes ( Chauvin et al, 2012 ). One study of fetal MRI in
early gestations showed that the modality altered the initial sonographic diagnosis in 30% of the
cases ( Poutamo et al, 2000 ). However, although MRI does provide added analysis of causes of
obstruction in select cases, the utility of MRI is limited, as with sonography, in diagnosing the
actual cause of LUTO ( Miller et al, 2002 ). Voiding Cystourethrography Voiding
cystourethrography (VCUG) remains the defi nitive radiologic study in confi rming the diagnosis of
posterior urethral valves. This study should be completed in the early postnatal period after renal
and bladder sonography, and as soon as an infant with suspected prenatal fi ndings of valves is
hemodynamically stabilized and able to undergo the contrast study. The bladder often appears
thickened and trabeculated with multiple diverticuli, mimicking the appearance of a neuropathic
bladder. High-grade vesicoureteral reflux may be seen in approximately 50% of patients with valves
at the time of diagnosis ( Hassan et al, 2003 ). Images obtained during the voiding phase will show
contrast traveling across a hypertrophied, elevated bladder neck and grossly dilated posterior
urethra ( Fig. 141-9 ). The urethra funnels abruptly at a transverse membrane, or cusp, representing
the obstructing valve leaflets seen at cystoscopy. These are the pathognomonic signs for posterior
urethral valves. The study commences with the insertion of a 6- or 8-Fr feeding tube into the
urethra. This tube may curl within the capacious posterior urethra or hypertrophied bladder neck,
requiring the use of a coud catheter to advance into the bladder. Often a catheter may already be in
place at the time of the study, and it is important that the catheter be withdrawn gradually distal to
the posterior urethra during the voiding phase of the study to offer unobstructed views of that
segment. Radionuclide Renal Scan The radionuclide renal scan offers quantifi cation of differential
renal function, and cortical defi cits seen on the study may imply renal dysplasia when completed in
the neonatal period. Mercaptoacetyltriglycine is a useful agent to evaluate renal functional
contribution, though delayed emptying of nuclear tracer from the often dilated collecting systems
should not be necessarily interpreted as ureterovesical junction obstruction requiring intervention.
Placement of a urinary catheter is essential in a patient with vesicoureteral reflux to minimize error
in the calculation of renal function. Laboratory Evaluation Laboratory evaluation of a newborn with
a diagnosis of posterior urethral valves will, as with any newborn, reflect maternal values and must
be interpreted with caution. After 48 hours, the maternal blood mediated through the placenta
should clear, and the infant's baseline laboratory values may be monitored. The nadir creatinine
value at 1 year of age is considered an important diagnostic tool and this value may be plotted to

evaluate immediate response to treatment in the neonatal period. However, the serum creatinine
plateau even in unaffected children may not be seen until days 65 to 220 of life ( DeFoor et al, 2008
, Boer et al, 2010 ). CLINICAL PRESENTATION AND INITIAL MANAGEMENT An infant
affected by posterior urethral valves may be affected by serious comorbidities such as pulmonary
hypoplasia and physical stigmata of oligohydramnios, including Potter facies, clubfeet and
deformed hands, and poor abdominal muscle tone, and require intensive initial management. The
infant may be noted to have difficulty with voiding, and the urinary stream may be weak or
intermittent. A 5- or 7-Fr feeding tube, or similar caliber urinary catheter, should be inserted per
urethra in an infant presenting to the neonatal intensive care unit with a presumed diagnosis of
LUTO. Catheter placement into the bladder may be impeded by the hypertrophied and often
elevated bladder neck and curling of the catheter within the dilated posterior urethra. In such cases,
a coud-tipped catheter or, alternatively, using a stylet to curl the tip of the feeding tube dorsally
will facilitate bladder drainage. Minimizing any type of catheter balloon fi lling is important to
prevent inducing bladder spasms in the small, hypertrophic bladder and potentially occluding the
ureteric orifi ces ( Jordan and Hoover, 1985 ). Placement of the catheter within the bladder may be
confirmed by bladder sonography or, in some instances, a one-shot cystogram. Pulmonary
Hypoplasia Whereas the focus in posterior urethral valves is too often on the lower urinary tract and
kidneys, the most profound complication and cause of perinatal mortality in infants affected by
severe LUTO remains pulmonary hypoplasia. The frequently cyanotic baby requires complex
ventilatory support, and this is often the factor delaying defi nitive surgical intervention to address
the valves. Pulmonary hypoplasia seen in the infant with a history of antenatally detected
oligohydramnios may be the contributing factor to perinatal mortality in these children, requiring
intensive and rapid supportive treatment ( Pinar, 2004 ). The association between posterior urethral
valves, oligohydramnios, and pulmonary hypoplasia is recognized ( Fig. 141-10 ), but the etiology
of hypoplasia is unclear and likely multifactorial. It is recognized that the reduced expansion of
alveoli because of hypoplasia adversely affects the development of the fetal pulmonary tree, which
requires intraluminal pressure, volume, and flow while providing cellular signaling to the
developing alveoli ( Husain and Hessel, 1993 ; Laudy et al, 2002 ). Although the concept that
grossly hydronephrotic kidneys and a dilated bladder, combined with increased uterine pressure on
the developing fetus resulting from oligohydramnios, reduce diaphragmatic expansion and affect
lung volume and growth seems logical, it is an insufficient explanation for the clear correlation
between pulmonary hypoplasia and oligohydramnios. Rather, the finding that abnormal pulmonary
development commences in early embryogenesis indicates that pulmonary hypoplasia may actually
precede uropathy ( Smith et al, 2006 ). Peters and associates (1991) proposed a two-stage
relationship with early pulmonary development being controlled by a renal growth factor,
whereas lung growth and maturation in the later fetal period was more susceptible to the variations
in amniotic fluid volume. Urinomas A urinoma is associated with posterior urethral valves in 3% to
nearly 10% of cases ( Fig. 141-11 ) ( Greenfi eld et al, 1982 ; Patil et al, 2003 ). The introduction of
renal ultrasonography in 1979 increased the detection of urinomas to 15% in one study ( Heikkil et
al, 2011 ). Forniceal rupture will appear on renal ultrasonography as distorted renal parenchyma
resulting from fluid trapped within the renal capsule, whereas transperitoneal transudation of fluid
or bladder rupture will present as neonatal ascites ( Greenfield et al, 1982 ). Although VCUG or
radionuclide cystography may delineate the site of leakage for ascites, the cause is often difficult to
determine ( Patil et al, 2003 ). Urinomas are usually addressed by restoring urinary flow and
treating the proximate cause of LUTO, allowing the fluid to be reabsorbed. It is only in cases in
which the ascites is causing respiratory distress, severe abdominal distention, or other clinical
symptoms that percutaneous drainage or tapping of ascites becomes necessary; these interventions
are uncommon. There is some debate as to whether a urinoma heralds better or worse renal function
for the affected side. Numerous studies have postulated that the urinoma serves as a pop-off
mechanism, thereby reducing renal dysplasia on a given side, and some studies demonstrate
globally preserved renal function, including an index of long-term renal severity ( Rittenberg et al,
1988 ; Wells et al, 2010 ). Other studies hold that the urinoma, especially one retained within the

renal capsule and compressing the kidney, impairs ipsilateral renal function and is a harbinger for
worsened renal prognosis or has no bearing at all on long-term renal function ( Patil et al, 2003 ;
Kleppe et al, 2006 ; Heikkil et al, 2011 ). Delayed Presentation In the age of extensive antenatal
ultrasonography, delayed postinfancy presentation of posterior urethral valves is assumed to be less
common. Still, Engel and colleagues (2011) reported that 141 of 228 children (62%) undergoing
valve ablation presented with posterior urethral valves with a clinical presentation other than
prenatal hydronephrosis or oligohydramnios. Up to 64% of these children had a normal prenatal
ultrasonography and most presented with urinary tract infections, voiding complaints, and acute
renal failure in 10% ( Engel et al, 2011 ). Another recent study at the Childrens Hospital of
Philadelphia followed a cohort of 138 patients presenting with posterior urethral valves between
1988 and 2011, of whom 60 (43%) presented after 6 months of life ( Pulido et al, 2013 ). A high
degree of suspicion for posterior urethral valves is therefore still warranted in boys presenting with
lower urinary tract symptoms, especially recurrent urinary tract infections but also overflow
incontinence, gross hematuria, renal dysfunction, and less commonly ejaculatory dysfunction
( Bomalaski et al, 1999 ; Schober et al, 2004 ). A renal sonogram in these patients often detects the
telltale bladder wall thickening and distal ureteral dilation that requires a voiding cystourethrogram
for confirmation. SURGICAL INTERVENTION Valve Ablation Today, cystoscopy with ablation of
the valves is considered the preferred initial surgical option in any neonate diagnosed with posterior
urethral valves. The treatment goal is to 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 ( Smith et al, 1996 ; Close et al, 1997 ). Experimental models corroborate clinical evidence
of the importance of bladder cycling, and one model of urinary diversion and undiversion
demonstrated the changes that occur in a diverted bladder prevented from cycling ( Chun et al, 1989
). A fetal sheep model developed an increase in expression of extracellular matrix elements and
apoptosis following a high diversion ( Chun et al, 1989 ). There are several approaches to valve
ablation, which has historically been successfully completed even with a crochet hook passed
retrograde into the urethra and feeling the hook catch the obstructing tissue. Innes Williams first
described the engagement of valves with a hook, and Whitaker and Sherwood (1986) modified the
hook by insulating the wire except for the very distal portion of the hook, which measures 6 to 7 Fr
and could be passed at the bedside without general anesthesia while applying a small amount of
diathermy when ablating the valves. With the miniaturization of endoscopes in the age of fiberoptic
and now digital technology, cystoscopy can be accomplished in even the smallest neonate and
endoscopic valve ablation is the preferred approach at most centers today. Availability of a 7.5- or
9-Fr infant cystoscope with an offset lens facilitates passage of a variety of ablating devices,
including a Bugbee electrode that can be used to incise the valves at the ventral 5 o clock and 7 o
clock positions with or without an incision at the dorsal 12 o clock position. Alternatively, the
valves may be incised at the 12 o clock position alone. A wire bent at the tip and passed through a
3-Fr ureteral catheter is another option, as is the visually guided Fogarty embolectomy catheter
( Soliman, 2009 ). In an infant with a normal-caliber urethra, the 9.5-Fr resectoscope may be used
with a Collins knife working element ( Fig. 141-12 ). Posterior urethral valves are thin and
associated with minimal vascularity, and aggressive resection should be avoided. Use of a hot loop
resectoscope for valve ablation, primarily in older children in whom a resectoscope can be readily
inserted, seems to be associated with a higher risk of urethral stricture and caution should be
exercised with its use ( Sarhan et al, 2010 ). A urethral catheter is typically placed for at least 24
hours after the procedure. It is not necessary, though it is not uncommon, for the catheter to be left
in for a longer period while the infant continues to be monitored for improvements in renal
parameters or respiratory issues, often in an intensive care environment. A VCUG must be repeated
after valve ablation within 1 month to ensure that the valves are no longer visible. It is not
uncommon to see immediate signs of diminished bladder pressures, including some improvement in
renal dilation and in the volume of vesicoureteral reflux. Bladder neck hypertrophy and the
subsequent elevation of the bladder neck dorsal to the posterior urethra, along with the incomplete
emptying that seems to persist on imaging in some boys after valve ablation, prompted an interest in

transurethral incision of the bladder neck during or after primary valve ablation ( Androulakakis et
al, 2005 ; Kajbafzadeh et al, 2007 ). Although some studies confirm that the bladder neck incision
benefits emptying in children with neurogenic bladder, concerns of retrograde ejaculation and the
lack of improvement compared to controls in even short-term pilot studies have limited the adoption
of this technique until longer-term data are available ( Christensen et al, 1985 ; Sarin and Sinha,
2013 ). Vesicostomy SURGICAL INTERVENTION Valve Ablation Today, cystoscopy with
ablation of the valves is considered the preferred initial surgical option in any neonate diagnosed
with posterior urethral valves. The treatment goal is to 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 ( Smith et al, 1996 ; Close et al, 1997 ). Experimental models corroborate
clinical evidence of the importance of bladder cycling, and one model of urinary diversion and
undiversion demonstrated the changes that occur in a diverted bladder prevented from cycling
( Chun et al, 1989 ). A fetal sheep model developed an increase in expression of extracellular matrix
elements and apoptosis following a high diversion ( Chun et al, 1989 ). There are several
approaches to valve ablation, which has historically been successfully completed even with a
crochet hook passed retrograde into the urethra and feeling the hook catch the obstructing tissue.
Innes Williams first described the engagement of valves with a hook, and Whitaker and Sherwood
(1986) modified the hook by insulating the wire except for the very distal portion of the hook,
which measures 6 to 7 Fr and could be passed at the bedside without general anesthesia while
applying a small amount of diathermy when ablating the valves. With the miniaturization of
endoscopes in the age of fiberoptic and now digital technology, cystoscopy can be accomplished in
even the smallest neonate and endoscopic valve ablation is the preferred approach at most centers
today. Availability of a 7.5- or 9-Fr infant cystoscope with an offset lens facilitates passage of a
variety of ablating devices, including a Bugbee electrode that can be used to incise the valves at the
ventral 5 o clock and 7 o clock positions with or without an incision at the dorsal 12 o clock
position. Alternatively, the valves may be incised at the 12 o clock position alone. A wire bent at
the tip and passed through a 3-Fr ureteral catheter is another option, as is the visually guided
Fogarty embolectomy catheter ( Soliman, 2009 ). In an infant with a normal-caliber urethra, the 9.5Fr resectoscope may be used with a Collins knife working element ( Fig. 141-12 ). Posterior
urethral valves are thin and associated with minimal vascularity, and aggressive resection should be
avoided. Use of a hot loop resectoscope for valve ablation, primarily in older children With
miniaturization of endoscopic technology, vesicostomy is reserved primarily for the very low-birthweight infant whose urethra cannot accommodate an endoscope, as well as a child with continued
impaired renal function, high bladder urine volumes, and upper tract deterioration after valve
ablation or urethral catheterization. The vesicostomy does reduce bladder storage pressures and may
optimize glomerular filtration rate in some cases ( Kim et al, 1997 ). The argument that the
vesicostomy defunctionalizes the bladder and leads to decreased compliance in the long term has
been refuted, since a properly created vesicostomy allows bladder filling and preserves contractile
function because urine must be expulsed through the stoma, albeit at a reduced leak point pressure
( Hutcheson et al, 2001 ). The vesicostomy is best seen as a temporary diversion in children with
posterior urethral valves because it does not alter clinical outcomes as compared to primary
ablation, nor does it prevent a bladder from acting as an adequate reservoir for a renal transplant
( Fine et al, 2011 ). The vesicostomy is classically created with a 2-cm midline transverse incision
made midway between the pubic symphysis and the umbilicus ( Fig. 141-13 ). The rectus muscles
are separated, the bladder is exposed with traction sutures, and the peritoneum is mobilized
cephalad and away from the posterior wall and dome of the bladder. The bladder
dome is identified by isolating the urachus, which is ligated so that the dome can be exposed
through the fascial incision. The urachus and a small portion of the bladder dome are excised and
the detrusor is then sutured to the fascia 1 cm below the edge of the cystostomy. The key operative
step in creation of the vesicostomy is to ensure that the posterior wall of the bladder is taut
accomplished by bringing the dome of the bladder to the skinto prevent prolapse of the back wall
of the bladder through the incision ( Hutcheson et al, 2001 ). Upper Tract Diversion Proponents of

supravesical urinary diversion hold that direct decompression of the kidney by a cutaneous
ureterostomy or pyelostomy will produce direct, low-pressure urinary drainage, allowing
optimization of renal function ( Fig. 141-14 ). High diversion, when renal dilation and biochemical
markers of renal function fail to improve despite maximal bladder drainage, historically was
believed to protect the upper urinary tract from ureterovesical junction obstruction caused by a
tortuous intramural ureter passing through a thickened valveaffected bladder. In those cases, a loop
diversion of the ureter or renal pelvis, for example, also allows bladder filling and bladder cycling,
mitigating the concern of defunctionalizing the bladder ( Pinto et al, 1978 ; Churchill et al, 1990 ;
Kim et al., 1997 ). Contemporary studies and long-term follow-up of these patients, however, failed
to detect a long-term renal protective benefit of high diversion, which necessarily requires an often
complicated undiversion procedure as the child matures ( Smith et al, 1996 ). Moreover, the finding
that renal biopsies taken at the time of high urinary diversion uniformly show renal dysplasia, while
the diversion does not seem to protect from renal failure, suggest that renal dysplasia occurring in
early fetal development is the primary mediator of renal outcome, rather than any type of complex
surgical undertaking in the neonate ( Tietjen et al, 1997 ). Upper urinary tract diversion may be
considered in an infant with complete decompression of the lower urinary tract but worsening renal
function, increasing upper tract dilation, and possibly a clinical picture of sepsis. Whether upper
urinary tract diversion or vesicostomy preserves renal function better than valve ablation alone
cannot be definitively concluded because of the lack of controlled comparative studies or other
available studies. Still, upper urinary tract diversion will require secondary surgery, and the bladder
may be exposed to a potentially extended period of defunctionalization with attendant risks of
impairment in compliance and contractility ( Close et al, 1997 ). The preferred initial surgical
intervention for infants with posterior urethral valves is endoscopic valve ablation. The clinician
should be prepared to create a properly constructed vesicostomy or upper urinary tract diversion
when primary valve ablation is impossible, or bladder decompression is not achieved by urethral
catheterization or valve ablation alone. Circumcision A urinary tract infection can quickly progress
to pyelonephritis and sepsis in an infant with posterior urethral valves because of the associated
morbidities of vesicoureteral reflux, incomplete bladder emptying, and severe upper urinary tract
dilation. The overall risk of urinary tract infection in children with posterior urethral valves is 50%
to 60%several magnitudes greater than the 1% risk for unaffected boys ( Mukherjee et al, 2009 ;
Bader and McCarthy, 2013 ). Circumcision reduces that risk of urinary tract infection by 83% to
92%, a reduction to a level of risk similar to that for unaffected boys ( Wiswell et al, 1988 ;
Mukherjee et al, 2009 ). It is recommended that a circumcision be strongly considered as a
prophylactic measure for any boy diagnosed with posterior urethral valves, and it should certainly
be completed before giving any consideration to a ureteral reimplant in a scenario of frequent
febrile urinary tract infections despite conservative measures. Nephroureterectomy
Nephroureterectomy was historically considered an appropriate intervention for posterior urethral
valve patients manifesting elements of the VURD syndrome discussed previously. The
nonfunctioning renal unit in association with dilating urinary reflux was long considered a potential
source for infections and sepsis, and prophylactic excision was considered appropriate. In
contemporary practice, a focus on proper bladder emptying and circumcision has decreased the
incidence of urinary tract infections sufficiently that nephroureterectomy is rarely considered.
Indeed, renal preservation is appropriate for even poorly functioning renal units contributing
moderate polyuria, which is easier to manage than anuria. If frequent urinary tract infections
localizing to the nonfunctioning renal unit necessitate a nephrectomy, preserving the ureter for
potential subsequent reconstruction, such as a ureteral augmentation, is recommended ( Husmann et
al, 2004 ). MANAGEMENT OF VESICOURETERAL REFLUX A voiding cystourethrogram will
reveal vesicoureteral reflux (see Fig. 141-9 ) in 50% to 80% of infants undergoing a workup for
posterior urethral valves ( Puri and Kumar, 1996 ; Tourchi et al, 2014 ). Infants with valves are also
at an increased risk for urinary tract infections as discussed in the previous section, and the
coexistence of reflux and valves presents a clinical scenario that may suggest a role for ureteral
reimplantation. However, understanding that reflux in these infants is a consequence of obstruction

and the secondarily elevated bladder pressures is critical to management, and should render the
ureteral reimplantation an option in atypical cases in which urinary tract infections continue despite
maximal bladder therapy. Indeed, ablation of the valves or vesicostomy alone will resolve ureteral
reflux in 25% to 40% of patients with urinary reflux prior to ablation ( Hassan et al, 2003 ; Tourchi
et al, 2014 ). Hassan and colleagues (2003) found that the presence of urinary reflux did not
correlate with renal outcomes, underscoring that the presence of reflux alone should not be seen as
an indication for intervention. All efforts in a symptomatic posterior urethral valve patient with
vesicoureteral reflux must focus on addressing any persistent bladder outlet obstruction, reducing
intravesical pressures by considering anticholinergic treatment, and treating underlying bladder
dysfunction that is common in bladders exposed to fetal bladder outlet obstruction. Urodynamic
evaluation may help guide management in this regard ( Kim et al, 1997 ). In the rare event that a
clinical scenario of recurrent urinary tract infections mandates intervention in a child with posterior
urethral valves, bladder management in advance of intervention is a critical component in ensuring
favorable surgical outcomes ( Hunziker et al, 2012 ). Consideration of surgical correction for reflux
must also recognize the higher complication rate of stricture and persistent reflux associated with
reimplanting dilated ureters into thick-walled bladders that are not properly rehabilitated prior to
surgery ( Coleman and McGovern, 1978 ; Atwell, 1983 ). Indeed, any treatment of urinary reflux in
a child with profound bowel dysfunctionas commonly seen in boys with posterior urethral valves
potentially puts an already compromised renal unit at risk for further deterioration ( Silln et al,
2010 ; Tekgl et al, 2012 ). Endoscopic correction of reflux seems to carry less of the risks of
myogenic disruption and renal deterioration seen with ureteroneocystotomy in children with valves,
but the overall surgical success rate is lower than that for children without valves ( Puri and Kumar,
1996 ; Tourchi et al, 2014 ). BLADDER DYSFUNCTION AND VALVE BLADDER SYNDROME
Consequent to exposure to obstruction from its earliest development, the bladder is necessarily the
focus of management and rehabilitation throughout the life of a boy diagnosed with posterior
urethral valves ( Parkhouse et al, 1988 ). The extent of remodeling and subsequent functional
compromise may vary, but the bladder and its dysfunction begin a cascade of pathophysiologic
changes, including voiding dysfunction, urinary reflux, and worsening of renal dysplasia and
obstructive uropathy. The ultimate manifestation of this dysfunction is the valve bladder syndrome.
Urodynamics plays an important role in monitoring an affected child s progression through various
well-described changes in bladder function throughout childhood. The bladder evolves through
three distinct contractility patterns through childhood: (1) detrusor hyperreflexia in infancy and
early childhood; (2) decreasing intravesical pressures and improved compliance bladder in
childhood; and (3) increased capacity bladder with hypocontractility and atony in adolescence
( Peters et al, 1990 ; De Gennaro et al, 2000 ). Holmdahl and coworkers (1995) stressed that the
patterns outlined here overlap in most children, emphasizing that the patterns are not arbitrary but
rather are useful guideposts in monitoring and managing children over the long term. Mitchell
(1982) conceptualized a vicious circle in voiding dysfunction wherein the outlet obstruction begins
a cascade of events leading to the end-stage bladder, or valve bladder. Bladder outlet obstruction
leads to detrusor hypertrophy, which increases the voiding pressures initially as the bladder strives
to complete emptying. As further remodeling of the bladder occurs, however, the postvoid residual
begins to increase as the urine output increases. Ultimately, the bladder cannot meet the demand of
emptying and the detrusor decompensates. Bladder dysfunction, even when not detected on clinical
history alone, must always be suspected in children with a history of valve ablation.
One systematic review of 34 studies describing renal function, vesicoureteral reflux, and
urodynamic findings after endoscopic valve ablation in 1474 patients found that, whereas the selfreported history of incontinence ranged widely between 0% and 70%, the mean incidence was 19%.
However, when urodynamic outcomes were examined, the incidence of bladder dysfunction rose to
a mean of 55% ( Hennus et al, 2012 ). Reliance on clinical examination or patient questionnaires
alone may grossly underestimate bladder dysfunction, and obtaining a uroflow and checking
postvoid residuals should be a routine part of follow-up in toilet-trained children with a history of
posterior urethral valves. Upper tract assessment with renal ultrasonography can also be a useful,

simple tool to detect dangerous bladder dysfunction and monitor response to therapy ( Lopez
Pereira et al, 2013 ). Bladder Management The typical follow-up for children with posterior urethral
valves after ablation for bladder dysfunction has focused on observation, clinical history, and
urodynamics. Education of parents and growing children is a critical component of bladder
management and the success of any prescribed behavior modifications. Families are counseled to
not aggressively push an affected child toward toilet training and to expect a lag compared to the
normal population. Daytime incontinence is not uncommon, ranging from 7% to 35%, and
nocturnal enuresis is expected in 1 of 4 children with a history of valve ablation ( Hennus et al,
2012 ). Once toilet training is achieved, children and caregivers are educated to ensure adequate
fluid intake, to void on a timed regimen, and to practice double voiding. Biofeedback therapy and
home pelvic floor exercises have also been shown to be useful ( Ansari et al, 2008 ). The role of
adjunctive medications is unclear. The preferred intervention is predicated also on varying proposed
etiologies of voiding dysfunction after valve ablation: (1) functional obstruction at the bladder neck
as a result of hypertrophy and external sphincter hyperreflexia, or (2) bladder wall thickening
caused by detrusor wall thickening from increased collagen deposition. One study suggests the use
of -adrenergic blockade to relieve sphincteric hypertonicity and relax the bladder neck in children
with high postvoid residuals, finding a significant reduction in residual volumes ( Abraham et al,
2009 ). In contrast, Casey and coworkers (2012) administered oxybutynin at 0.1 mg/kg twice daily
in 18 consecutive infants undergo- ing urodynamic assessments at 3 months after valve ablation and
showing high voiding pressures or small bladder capacity. Although the study found that both of
these parameters improved significantly with oxybutynin, the lack of a control group in both aforementioned studies and the reality that detrusor hypercontractility and elevated voiding pressures are
normal findings in neonates necessitate more rigorous prospective studies ( Silln et al, 1992 ;
Casey et al, 2012 ). If oxybutynin is chosen, its use must be closely monitored for effect, and if a
growing child begins to demonstrate higher bladder residual volumes and capacity, oxybutynin
should be stopped. Therapy was stopped in 4 of 18 patients in the Casey and coworkers (2012)
study, and another study found that myogenic failure required intermittent catheterization ( Kim et
al, 1997 ). It is unclear whether the myogenic failure was a consequence of evolving bladder
dysfunction inherent to posterior urethral valves or secondary to oxybutynin, but caution
nevertheless should be exercised during the treatment period. Valve Bladder Syndrome The term
valve bladder syndrome was coined by Mitchell in 1982 after reviewing his experience with 11
patients in whom hydroureteronephrosis and renal function continued to worsen despite no clinical
evidence of residual bladder outlet obstruction ( Lloyd et al, 2013 ). Mitchell s concept, which is
illustrated in Figure 141-15 and was described earlier, holds that although the bladder initially
compensates for outlet obstruction by generating high voiding pressures, it begins to experience
higher volumes of urine as a result of increasing urine production as the child grows. The polyuria
caused by nephrogenic diabetes insipidus secondary to evolving renal impairment augments the
urine volumes entering a bladder that is increasingly unable to empty completely. As the postvoid
residuals increase, the bladder no longer enjoys periods of complete relaxation, and the detrusor
fibers are continuously in a state of partial or complete stretch, beginning a cascade of gene
expression and phenotypic changes that further impair contractility of the bladder ( Kirsch et al,
2003 ; Hutcheson et al, 2004 ; Shukla et al, 2004 ). When the bladder does empty partially, the urine
already stored in the hydronephrotic kidneys quickly empties into the bladder once again, denying
the detrusor muscle periods of relaxation. The impaired contractility and increasing postvoid
residuals then transmit the increasing bladder pressures to the kidneys, potentially worsening the
already impaired renal function. In sum, then, three processes contribute toward the devolution of a
bladder into a valve bladder in a cohort of patients with posterior urethral valves: (1) polyuria. (2)
poor bladder compliance with high-pressure voiding and elevated wall tension bladder, and (3)
residual urine volume. These three factors conspire to sustain the bladder overdistention that is the
original insult leading to the valve bladder ( Koff et al, 2002 ). Although the goal of intensive
management of bladder dysfunction is to arrest the progression toward full expression of late-term
manifestations of valve bladder syndrome, valve bladder syndrome is actually a broad term

describing a continuum of symptoms of bladder dysfunction. As described in the previous section,


monitoring urine postvoid residuals, flow rates, and voiding pressures, along with timed voiding,
double voiding, and anticholinergics or -blockers, comprises the mainstay of treatment. Clean
intermittent catheterization (CIC) becomes necessary if myogenic failure progresses. Overnight
bladder drainage is an important adjunct in therapy, and increasingly adopted as a standard
intervention in children with classic evidence of developing a valve bladder syndrome ( Koff et al,
2002 ; Nguyen et al, 2005b ). Nocturnal bladder drainage is instituted if ureteral dilation and
hydronephrosis do not respond to behavior modification, or an affected child develops worsening
renal function or recurrent urinary tract infections. The continuous bladder drainage achieved by
leaving a urinary catheter in the bladder over a 7- to 10-hour period allows an extended period of
bladder decompression even as the kidneys empty urine without encountering the increased voiding
or resting bladder pressures they face during the day. This simple step interrupts the vicious cycle
of bladder remodeling and consequent renal effects resulting from chronic bladder distention. Koff
and colleagues (2002) and Nguyen and associates (2005a) both noted significant improvements in
hydronephrosis, continence, and urinary tract infections after instituting overnight bladder drainage.
When CIC or overnight bladder drainage is difficult because of an elevated bladder neck or sensate
urethra, an appendicovesicostomy utilizing the Mitrofanoff principle ( Mitrofanoff, 1980 ) presents
a useful option. Minimally invasive techniques to create this catheterizable channel, utilizing both
laparoscopic and robotic-assisted approaches, are increasingly being adopted at many centers
( Mitrofanoff, 1980 ; Hsu and Shortliffe, 2004 ; Nguyen et al, 2009 ; Famakinwa and Gundeti, 2013
; Famakinwa et al, 2013 ). The robotic-assisted approach potentially limits the field of dissection
and could, in older children, make it difficult to mobilize the appendix and perform bladder
mobilization with anastomosis of the appendix through a single robot docking. In such situations, a
pure laparoscopic approach could be used to mobilize the appendix, followed by docking of the
robot with standard triangulation of port sites focused on the pelvic midline for anastomosis of the
appendix. Augmentation cystoplasty is rarely utilized for a valve bladder in the contemporary era,
perhaps because of improved understanding of bladder dysfunction, behavior modification, and
timely institution of overnight bladder drainage. However, when faced with a small-capacity, highpressure, thick-walled valve bladder with worsening upper tract anatomy refractory to conservative
measures, augmentation may be considered. Ureteral augmentation is preferred in children with
posterior urethral valves because it reduces the risks of mucus production, acidosis, and stones that
are common to ileal augmentation. Moreover, severe ureteral dilation or unilateral VURD seen in
boys with posterior urethral valves offers an ideal clinical scenario wherein the ureter can be
detubularized and patched on a bisected bladder without bowel manipulation. Johal and coworkers
(2008) reported lasting benefits of increased capacity and decreased filling pressures at a mean
follow- up of 4.5 years after ureteral augmentation. ANTENATAL MANAGEMENT Antenatal
intervention in cases of suspected LUTO was popularized in the mid 1990s as advances in
fiberoptics and endoscopic miniaturization enabled even complex fetal procedures. Intervention is
considered in some centers when antenatal sonography detects evidence of oligohydramnios, a
dilated bladder, and severe hydroureteronephrosis without renal cortical cystic lesions in a
fetus with a normal karyotype ( Ruano, 2011 ). A fetal urine sample can also be obtained after 20
weeks gestational age, and favorable prognosis is suggested by a urinary sodium less than 100
mEq/L, chloride
less than 90 mEq/L, osmolarity less than 200 mEq/L, and 2 -microglobulin less than 6 mg/L
( Nicolini and Spelzini, 2001 ). Vesicoamniotic shunting to treat oligohydramnios offers potential
ameliorative effects on pulmonary function and represents the first stage in fetal intervention, with
several hundred shunt procedures reported in the literature ( Ruano, 2011 ). This approach is
corroborated by fetal sheep models demonstrating that restoration of amniotic fluid volume prevents
lung hypoplasia, though the lack of controlled studies in the literature constrains conclusions as to
its effectiveness ( Kitagawa et al, 2006 ). Also, although a systematic review published a survival
advantage in infants having undergone vesicoamniotic shunting, randomized trials were lacking
( Clark et al, 2003 ). The Percutaneous vesicoamniotic shunting versus conservative management

for Lower Urinary Tract Obstruction (PLUTO) trial attempted to fill this void but was limited by
poor recruitment and pregnancy terminations, with only 12 live births in each group studied. The
results showed a trend toward improved survival at 28 days in the shunted group but overall
survival was very poor in both groups, with only 2 infants surviving to 2 years of age with normal
renal function. There was a high mortality owing to pulmonary hypoplasia. There was also a greater
risk of pregnancy loss in the shunt group because of procedure-related complications and early
rupture of membranes ( Morris et al, 2013 ). Biard and colleagues (2005) reported a mean 5.83
years of follow-up on 20 pregnancies with a singleton male fetus that underwent vesicoamniotic
shunting for clear evidence of isolated LUTO and good or borderline urinary sampling parameters.
This study found an overall 1-year survival of 91%, and health-related quality-of-life parameters
were similar to those in the unaffected, healthy child population. Vesicoamniotic shunting, in
contemporary practice, is utilized as a potential intervention in the rare case of LUTO with
oligohydramnios but should be limited to experienced centers with multidisciplinary capabilities.
No studies similar to the PLUTO trial have been attempted for even more complex fetal
interventions such as fetal cystoscopy with valve ablation, or even fetal surgery. Fetal cystoscopy,
performed using a 1.0-mm fetoscope, is carried out in an antegrade fashion by a transuterine
percutaneous incision into the bladder. When the posterior urethra is entered through the bladder
neck, a Nd : YAG laser or cauterizing wire is used to perforate the obstructing membrane (Quintero
et al, 1995, 2000 ; Ruano, 2011 ). Holmes and coworkers (2001) reported a series of 14 fetal
surgical procedures for posterior urethral valves, including antenatal valve ablation, vesicoamniotic
shunting, cutaneous ureterostomy, and vesicostomy. Six infants died as a result of premature
delivery and respiratory failure and 5 of 8 surviving children at a mean 11.6 years of follow-up were
in renal failure. The 43% fetal mortality rate for fetal surgery must be an essential part of any
prenatal counseling prior to considering fetal intervention that offers potential, though yet unproven,
benefits for a very select group of pregnancies. PROGNOSTIC INDICATORS FOR RENAL
FUNCTION Despite numerous advances in antenatal diagnosis and interven- tion, and rapid
postnatal evaluation and treatment, the lifetime prevalence of end-stage renal disease in boys with
posterior urethral valves is between 20% and 50% ( Parkhouse et al, 1988 ; Smith et al, 1996 ;
Sarhan et al, 2011 ). Risk factors known to affect the prognosis of an infant diagnosed with
posterior urethral valves include age at diagnosis, renal dysplasia with or without vesicoureteral
reflux, nadir creatinine during 1 year of life, recurrent urinary tract infections, and bladder
dysfunction. Nadir creatinine has long been considered a relatively easy method of predicting longterm renal outcome in affected children. The nadir creatinine value measured at 1 year of life
appears to be more accurate as a predictive tool than the value obtained at 1 month of age ( Drozdz
et al, 1998 ; Lal et al, 1999 ; Heikkil et al, 2011 ). A serum creatinine of less than 0.8 mg/dL
appears to indicate a minimal risk, whereas a value greater than 1.2 mg/dL at 1 year of age predicts
a higher risk of developing end-stage renal failure ( Drozdz et al, 1998 ; DeFoor et al, 2008 ). In
those studies suggesting that a 1-month post-treatment serum creatinine is a more accurate predictor
of renal function, again the value of less than 0.8 mg/dL at 1 month after treatment seems to
indicate better long-term outcomes ( Rittenberg et al, 1988 ). Age at diagnosis remains an unclear
predictor of future renal outcomes. The assumption that antenatal diagnosis would lead to more
rapid diagnosis of posterior urethral valves and therefore forestall renal injury has not been
sustained. Indeed, Heikkil and associates (2011) found that patients diagnosed in the
presonography era (before 1982) had a risk of end-stage renal disease of 16.8%, as compared to
36.6% developing renal failure if diagnosed during the post-sonography era. Another review found
a similar variation with 41% of those presenting before 1 year of age having poor long-term renal
outcome compared to 15% of those presenting after 1 year of age ( Parkhouse et al, 1988 ). This
difference in outcomes may be explained by the assumption that the critically ill infants in the presonography era likely died before diagnosis was complete, and early interventions, including fetal
measures, in the current era increased survival dramatically. These infants born after 1982 may
previously have died and tended to have more severe manifestations of valve disease and related
comorbidities contributing to worse renal outcomes. Another common assumption, not

corroborated, was that the later-presenting children likely had a milder variant of valve disease,
allowing them to go undetected for some years before presenting with more vague symptoms of
voiding dysfunction. However, some reports found much worse outcomes in children presenting
outside of the neonatal period with posterior urethral valves, with delayed presentation associated
with a significantly higher risk of azotemia, higher serum creatinine, and worse long-term renal
outcomes ( El-Sherbiny et al, 2002 ; Ziylan et al, 2006 ; Sarhan et al, 2011 ). Quantifying renal
dysplasia without a renal biopsy requires reliance on available imaging technology, including renal
sonography and nuclear scintigraphy. Hyperechogenic kidneys, cystic changes in the cortex, and
loss of corticomedullary differentiation are considered to portend a poor prognosis ( Robyr et al,
2005 ). Pulido and colleagues (2013) examined the association of the renal parenchymal area
defined as the area of the kidney minus the area of the pelvicaliceal system on the first postnatal
sonogram with end-stage renal disease. Reviewing the first postnatal ultrasound images of 60
patients followed for 393 person-years, the authors found that, for infants with a serum creatinine
between 0.8 and 1.1 mg/ dL at 1 month of life, each 1-cm 2 increase in renal parenchymal area was
associated with a lower risk of end-stage renal disease ( Pulido et al, 2013 ). The study highlights
that there is a need for novel ways of predicting renal outcomes in children with posterior urethral
valves, and certainly more definitive and powerful predictive tools may lie in the elucidation of
genetic and biochemical markers ( Farrugia et al, 2006 ). TRANSPLANTATION IN POSTERIOR
URETHRAL VALVE PATIENTS The prevalence of end-stage renal disease in boys with a history
of posterior urethral valves is up to 50%, and the 2006 annual report of the North American
Pediatric Renal Trials and Collaborative Studies listed obstructive uropathy as the second most
common cause for transplantation, accounting for 1424 of 8990 transplantation cases (15.8%) since
1987 ( Smith et al, 2007 ). Patients with posterior urethral valves comprise an especially difficult
cohort for receiving a renal transplant. These boys are likely to have several comorbidities,
including high-grade vesicoureteral reflux into native nonfunctioning kidneys and valve bladder
syndrome with a thick-walled, poorly contractile or hypercontractile bladder. A pediatric urologist
must be a critical component of the transplantation team and should carefully examine the
prospective recipient as part of the pretransplantation evaluation. Transplant recipients in whom
bladder dysfunction is incompletely managed or the bladder reservoir is not optimized have
significantly higher complication rates and graft loss rates ( Sheldon et al, 1994 ; Mendizabal et al,
2005 ). Outcomes after renal transplantation in children with posterior urethral valves have been
mixed. The thickened bladder wall of posterior urethral valve patients may contribute to the
significantly increased incidence of ureteral obstruction on univariate and multivariate analysis
compared to a nonposterior urethral valve transplantation cohort, but recent studies saw no risk of
increased graft loss or patient death despite ureteral obstruction, stenting, or dilation ( Indudhara et
al, 1998 ; DeFoor et al, 2003 ; Smith et al, 2010 ; Fine et al, 2011 ). Fine and colleagues (2011)
reported on 59 valve patients who underwent renal transplantation with 8-year follow-up and found
that outcomes were similar whether a boy underwent an initial valve ablation, vesicostomy, or
supravesical diversion; also, though bladder dysfunction increased the risk of graft failure, the effect
did not reach significance. Video-urodynamics should be obtained for transplant candidates to
determine the safe storage pressures and contractile function of the future reservoir. Overnight
bladder drainage or CIC may be initiated prior to transplantation
to optimize the reservoir and establish proper bladder management skills that will be essential for
graft success after transplantation. Pretransplantation nephrectomy is rarely required and only
considered in cases in which proteinuria or severe polyuria is creating hemodynamic challenges. If
augmentation is believed necessary based on unsafe storage pressures in the bladder, this
reconstruction can be considered before or after transplantation. Whereas previous dogma suggested
that a pretransplantation augmentation is preferable to prevent the undertaking in an
immunocompromised child or one too young to take responsibility for catheterization and pouch
management, recent experience argues that transplantation into even a vesicostomy is a safe
alternative until the child grows to an appropriate age for cystoplasty and the attendant reliance on
CIC ( Rigamonti et al, 2005 ; Christman et al, 2013 ). QUALITY OF LIFE WITH POSTERIOR

URETHRAL VALVES It must be emphasized that posterior urethral valves have lifetime
repercussions. Understanding these long-term risk factors and their impact on quality of life is
necessary for counseling, preparing, and treating valve patients as they reach adulthood. The
evolution of the valve bladder and the associated comorbidities of renal transplantation that many
valve patients face are associated with well-known risks, including erectile dysfunction and
infertility. The lower urinary tract symptoms that affect children with valves also affect them as
adults two to three times more often than those symptoms affect the general population ( Tikkinen
et al, 2011 ). A survey of 67 adult patients with posterior urethral valves found that the overall rate
of erectile dysfunction or infertility was not different than that in the general population ( Taskinen
et al, 2012 ). However, a subgroup analysis of these same patients with urinary incontinence or
renal insufficiency showed that valve patients had the greatest evidence of impaired quality of life,
underscoring the necessity of long-term follow-up and active treatment well into adulthood
( Jalkanen et al, 2013 ). Indeed, the chronicity of bladder dysfunction, risk of urinary tract
infections, and sequelae of renal dysfunction require communication between pediatric and adult
urologists, even as the adult urologist should be knowledgeable of the pathophysiology of posterior
urethral valves and well prepared to provide care to these patients after their transitions into
adulthood. OTHER URETHRAL ANOMALIES Anterior Urethral Valves Anterior urethral valves
are the most common congenital obstructive lesion of the anterior urethra, but are 25 to 30 times
less common than posterior urethral valves ( Confer et al, 2010 ). Since the condition is often found
in association with a large anterior urethral diverticulum, the valve itself is varyingly described as
an obstruction resulting from a wall of the diverticulum obstructing flow or a semilunar fold
draping down from the wall of the anterior urethra and interrupting urinary flow ( Tank, 1987 ;
Paulhac et al, 2003 ). The embryology of anterior urethral valves is not clear, but abortive corpus
spongiosum over the affected portion of the anterior urethra indicates a place on the hypospadias
spectrum or a faulty union between urethral mucosa and the epithelium of the fossa navicularis. A
rupture of dilated bulbourethral glands has also been suggested as an etiology ( McLellan et al,
2004 ). The valves may be located at the bulbar urethra, the penoscrotal junction, or the penile
urethra ( Firlit et al, 1978 ). Patients present with anterior urethral valves at different ages based on
the severity of the obstructive process. Symptoms may consist of postvoid dribbling and mild
incontinence, significant bulging of the distal penis, palpable bladder with obstruction or even renal
insufficiency, and urinary tract infections ( Cruz-Diaz et al, 2013 ). Diagnosis requires a careful
examination of the external genitalia, and compression of the distal shaft may result in expressing of
urine as seen in a diverticulum. A voiding cystourethrogram is required to confirm the diagnosis and
may demonstrate a dilated anterior urethra with proximal signs of chronic obstruction, including
bladder diverticula and massive vesicoureteral reflux. The treatment approach to anterior urethral
valves varies according to age of presentation, extent of upper tract damage, and extent of anterior
urethral deformity. In a premature or small infant, a vesicostomy may be required to facilitate relief
of obstruction until the infant can accommodate a cystoscope or undergo further reconstruction. In
the majority of cases, cystoscopy with valve ablation using a Bugbee electrode or laser is possible
as an initial treatment, and when successful, no further surgery of the urethra is required ( CruzDiaz et al, 2013 ). In more severe cases in which a gross urethral diverticulum is seen, surgical
reconstruction over a urethral catheter may be required. Up to 80% of children with anterior urethral
valves will develop bladder dysfunction, and bladder instability, hyperreflexia, and diminished
compliance and capacity will be seen on urodynamics ( Kajiwara et al, 2007 ). As with posterior
urethral valves, long-term renal function is contingent upon preoperative creatinine and glomerular
filtration rate. Routh and colleagues (2010) performed a multivariate analysis of data available from
97 studies including 229 patients with anterior urethral valves and found that pretreatment azotemia,
vesicoureteral reflux, and urinary tract infection together increased the risk of poor renal outcome
25-fold. Still, because of the milder and more subtle presentation, the overall incidence of preserved
renal function in anterior urethral valves is better than that in posterior urethral valves, with 78% of
patients having normal renal function after treatment ( Routh et al, 2010 ). Urethral Atresia Urethral
atresia or congenital urethral stricture is a rarely described entity, likely because of its high

associated mortality. When an infant survivesbecause the obstruction is incomplete or because


there was decompression owing to an antenatal shunt placement or a patent urachusthe outcome
can be similar to that of a child with obstructing posterior urethral valves ( Gonzlez et al, 2001 ).
An obstructing membrane is typically seen at the distal end of the prostatic urethra and the urethra
distal to that point may be hypoplastic. As a part of the LUTO spectrum seen in antenatally detected
anomalies of the lower urinary tract, the obstruction is confirmed after birth with cystoscopy ( Fig.
141-16 ) and a vesicostomy is usually required ( Freedman et al, 1999 ). Whether urethral atresia is
a precipitating factor for development of prune-belly syndrome in certain cases remains
controversial, but infants with urethral atresia or congenital urethral stricture often will present with
oligohydramnios, bilateral hydroureteronephrosis, and weak abdominal wall musculature. The
progressive augmentation by dilating the urethra anterior (PADUA) procedure is considered a safe
alternative for restoring urethral continuity in selected cases without complex reconstructive surgery
( Passerini-Glazel et al, 1988 ; Stalberg and Gonzlez, 2012 ). Urethral Duplication Urethral
duplication is another rare anomaly of the urethra with several known anatomic variants. The
duplication may begin at the bladder neck or within the more distal urethra. Whereas one urethra
usually terminates on the glans near its orthotopic position, another urethra may end in a meatus
placed on the glans or more ventrally along the shaft of the penis. In the most severe cases, the
duplicated urethra may even be as proximal as the anal sphincter. The duplication occurs in a
sagittal plane, with the ventral urethra usually the functional meatus containing the sphincteric
complex and verumontanum. Effmann and associates (1976) are credited with the most widely used
classification system for urethral duplications. Broadly, the type I abnormality includes a blind
incomplete urethral duplication or accessory urethra. Type II is a complete patent urethral
duplication with four subtypes and type III refers to urethral duplication as a component of partial
or complete caudal duplication ( Fig. 141-17 ). The Y -type duplication refers to a type IIA2 in
which the duplicated urethra arises from the first urethra but diverges away to open into a second
meatus that opens as ventral as the rectum. Diagnosis is readily made in some cases when two
distinct meatal openings are seen on the glans, but in other cases requires a high index of suspicion
when examining what appears to be an atypical case of proximal hypospadias with a patentappearing opening on the glans. A voiding cystourethrogram will confirm the diagnosis during the
voiding phase in many cases, though a retrograde injection of the distinct urethra can also be
accomplished ( Fig. 141-18 ) ( Hoekstra and Jones, 1985 ; Podesta et al, 1998 ). Surgical
management is complex and may require a variety of single or multiple-stage repairs. Whereas the
small, blind-ending accessory urethra may be treated expeditiously with simple coagulation of the
mucosal tract with a Bugbee electrode, a patent duplicated urethra connected to the bladder with a
distinct bladder neck will require a planned reconstruction. When two urethral openings are found
on the glans, the more dorsal urethra may mimic the appearance of a distal epispadias. Alanee and
colleagues (2012) recently described a technique by which the septum between the two urethral
channels is incised and the dorsal defect is repaired with reverse glans wings to cover a dorsal
urethroplasty. In cases in which the urethral openings are separated by the Y type arrangement, a
staged reconstruction using preputial or buccal
mucosa flaps is often required to bring the more ventral, but functional, meatus to the glans penis
where it can be insinuated into the dorsal, usually atretic, urethra. The anterior sagittal trans-anorectal approach (ASTRA) is also recommended as a means to mobilize the meatus placed in the
anal sphincter ( Macedo et al, 2012 ). Urethrorrhagia Urethrorrhagia, usually referred to as
idiopathic urethrorrhagia, describes a spotting of blood on the underwear after urination, or voiding
of clear urine followed by a few drops of blood. The visible blood tends to raise alarm within
families, but the condition is typically considered benign and self-limited. Urethrorrhagia is most
commonly seen in boys. The etiology of urethrorrhagia is unclear, though various hypotheses have
been offered. Meatal stenosis and dysfunctional elimination syndrome have been suggested as
inciting factors ( Herz et al, 2005 ). Proponents of voiding dysfunction as the etiology of
urethrorrhagia hold that increased voiding pressures caused by incomplete relaxation of the external
urethral sphincter lead to turbulent flow that creates a negative intraluminal urethral pressure. That

negative pressure causes an engorgement of the sinuses of the urethral mucosa and a small
extravasation of blood ( Docimo et al, 1998 ; Herz et al, 2005 ). Meatal stenosis is similarly
proposed as a cause of increased voiding pressures. Adult urologists consider cystoscopy and upper
urinary tract evaluation a necessary step for the evaluation of hematuria, but the high rate of
spontaneous resolution of urethrorrhagia in symptomatic adolescent malesup to 92%means that
routine cystoscopy is not necessary. Rather, evaluation should focus on a detailed history of bowel
and bladder function, renal and bladder ultrasonography, and an office evaluation of urinary flow
rate and postvoid residual. If urethrorrhagia becomes atypicalaccompanied by symptoms of
urethral stricture or increased urethral bleeding - then cystoscopy should be performed. Although
the majority of cases of urethrorrhagia will have an idiopathic origin, urethral strictures are
diagnosed during evaluation in 14% to 60% of patients ( Dewan and Wilson, 1996 ; Poch et al, 2007
). This strong association has led to some debate as to whether cystoscopy itself, in traversing
abnormal inflamed epithelium, initiates stricture formation. Poch and associates (2007) , in a review
of 66 boys with urethrorrhagia, found that cystoscopy in atypical cases of urethrorrhagia identified
varying levels of bulbar urethral inflammation, with 24% of patients having a white membranous
exudate noted at cystoscopy subsequently being diagnosed with a urethral stricture over a mean of 5
years. In the same cohort, 12% had a stricture found on cystoscopy without any prior history of
instrumentation. These findings seem to support a perspective that, although cystoscopy is benign in
most cases, the procedure may exacerbate the inflammation of an already inflamed urethra in a
small population that cannot readily be distinguished by any radiologic modality. A careful analysis
of voiding habits and baseline uroflow is therefore imperative before cystoscopy is considered
( Poch et al, 2007 ). Urinary Fistula in Boys with Anorectal Malformation The vast majority of boys
born with anorectal malformation will have a rectal fistula to the urinary tract ( Hong et al, 1992 ).
Since the initial diverting colostomy is completed during the neonatal period, the fistula tract is
usually seen on distal colostogram during subsequent workup prior to anorectoplasty ( Fig. 14119 ). Pediatric urologists are an integral part of any multidisciplinary team caring for children with
anorectal malformations. Because associated genitourinary anomalies range from 25% to 50%,
routine diagnostic imaging to determine the presence of renal anomalies and vesicoureteral reflux is
recommended ( Hoekstra et al, 1983 ). A recent analysis of 190 patients with anorectal
malformations found that 31 (16.3%) developed a febrile urinary tract infection; of these, 51.6%
had a diagnosis of vesicoureteral reflux. On multivariate analysis the presence of genitourinary
malformations was associated with a urinary tract infection, but the association did not reach
statistical significance ( Sanchez et al, 2014 ). When definitive surgical repair is planned in
association with general pediatric surgeons, a posterior sagittal approach is preferred to address the
rectourethral fistula concurrently. Care must be taken to properly identify the fistula tract and ensure
that the tract is excised adjacent to the urethra as well as the rectum, to ensure complete removal of
excess tissue that could become a diverticulum if not resected completely. A urologist is present at
the time of anorectoplasty and fistula repair, and the procedure begins with cystoscopy with an
attempt to pass an open-ended ureteral catheter through the fistula tract. A guidewire may also be
passed if the caliber of the fistula is small. A urethral catheter is then placed into the bladder, across
the urethra. Once the child is placed prone, and posterior sagittal anorectoplasty begins, the rectum
is adequately mobilized away from the urethra using the previously placed catheter as a guide to
location. The fistula tract is excised completely, and the urethral defect is approximated as close to
the urethra as possible, reducing the risk of a urethral diverticulum. The rectal defect may be closed
primarily, but if redundant intestinal tissue is available, then the fistulous portion of intestine is
excised. Healthy adjacent tissue is interposed and the catheter is left in place for at least 1 week to
ensure healing of the urethroplasty.