Bladder Outlet Obstruction

Presenter : Dr PASHI
Moderator : Prof Munkonge
Introduction
BOO describes blockage of urine outflow from the bladder and
through the urethra
Obstruction may be partial or complete
Childhood Urethral obstruction are mostly congenital
Only posterior urethral valves commonly give rise to secondary
changes in the upper renal tracts
Compared with the adult kidney, the developing kidney is highly
susceptible to injury from obstruction to urine flow
The earlier the obstruction develops the worse the impact on the
upper tracts
Incidence
Various causes of BOO and vary in incidence
In Africa, reports on the incidence are scanty, probably
Voiding dysfunction describe abnormalities in either the filling and/or
emptying of the bladder
Constitutes 40% of paediatric urology clinic visits.
The Urinary system
The urinary tract includes two kidneys, two
ureters, a bladder, and a urethra.
Urine flows from the kidneys through the
ureters to the bladder which stores urine until
releasing it through the urethra by urination
Lower urinary tract bladder and urethra
Obstruction may occur anywhere
More common in males
Embryology
Lower urinary tract development is closely interrelated with genital
tract and the hindgut.
3rd week of gestation, the cloaca meets the ectoderm of body wall at
cloacal membrane
5th week, cloaca divided by urorectal septum to form the primitive
rectum posteriorly and urogenital sinus anteriorly
Allantois, bladder, pelvic, and phallic portions of the urogenital system
are recognisable in the 6th week.
Embryology
Embryology
7th week gestation, the mesonephric ducts (vas deferens) shift
further caudal in the sinus and come to lie close to each other
Metanephric buds (ureters) arise from mesonephric ducts
root of the mesonephric duct exstrophies into the posterior wall of
the developing bladder
This process brings the openings of the ureteric buds into the bladder
wall
mesonephric duct is carried inferiorly to the level of the pelvic urethra
The triangular region of exstrophied mesonephric duct incorporated
into the posterior bladder wall forms the trigone of the bladder
Embryology
Embryology
9th week of gestation, the bladder cavity expands, urachus elongates
and continues with the allantoic stalk at the umbilicus.
12th week gestation, extra embryonic allantois degenerates, urachus
closes forming the median umbilical ligament.
BOO manifest in some foetuses with closure of urachus, but delayed
closure may protect others.
Embryology
The early bladder epithelium initially consists of a single cell layer, but
later become transitional
Bladder epithelium or Chwallas membrane temporarily covers and
occludes the ureteral orifice
Bladder muscle arises from mesenchyme as a longitudinal layer on
the dorsal surface and spreads cephalad to the intrarenal collecting
system
16th week of gestation, the bladder is completely muscularized and
the urachus is closed
Developmental Anomalies
Bladder Agenesis : rare, is due to failure of allantoic stalk to develop
Urachal anomalies (e.g., patent urachus) result from delayed closure
of the urachus, that arise from lower urinary tract obstruction at less
than 12 weeks gestation
Some anomalies result from a general mesodermal failure (the
urachal diverticulum of the Prune belly syndrome).
Intravesical (simple) ureterocoeles are thought to be due to the
persistence of Chwallas membrane beyond the time when urine flow
begins
 Bladder outlet obstruction caused by the urethral obstruction of
posterior urethral valves or urethral hypoplasia may be detected from
4 weeks gestation concurrent with the absorption of the mesonephric
duct and the resorption of the urogenital membrane.
Abnormal dilatation of Cowpers glands may give rise to an
obstructive urethral syringocele
Bladder Innervation
Autonomic nervous system
Parasympathetic (S2S4)
Sympathetic (T11L2)
Inhibit parasympathetic to allow detrusor relaxation and expansion
Constrict internal sphincter
Somatic nervous system (pudendal nerve(S1S4)
Control external sphincter
Physiology of voiding
Normal voiding cycle is a two-phased process, consisting of
low-pressure and adequate volume bladder filling
complete evacuation of the bladder.
Bladder filling is passive determined by
Vesicoelastic properties of the bladder wall
Inhibition of bladder contraction
Increased urinary sphincter
Urinary continence
maintained by contraction of sphincter complex which keeps
urethral pressure above normal bladder pressure
Physiology of voiding
Normal voiding is a spinal reflex modulated by the CNS (brain, pontine
micturition centre, and spinal cord), which coordinates the functions
of the bladder and urinary sphincter
As the bladder fills, the pudendal nerve becomes excited, resulting in
contraction of the external urethral sphincter
As the bladder approaches fullness, tension receptors in the bladder
wall trigger afferent nerves that send signals of the need to void.
Voluntary voiding involves inhibition of sphincter contractions and
coordinated contraction of the bladder smooth muscle.
Physiology of voiding
Bladder filling in neonates is followed by reflexive emptying
This micturition cycle occurs hourly under sacral spinal cord control
After 6 months of age, the urinary volumes increase, the frequency of
voiding decreases as the control of voiding shifts from the sacral cord
to the pontine voiding center
Brain maturation and development, leads to bladder and urinary
sphincter control to inhibit involuntary voiding
At 2 years the child develops conscious sensation of bladder fullness
and has urge incontinence
Physiology of voiding
Between 2 and 4 years of age acquires voluntary control of bowel and
bladder function according to the following sequence:
1. nocturnal bowel control
2. daytime bowel control
3. daytime bladder control
4. nocturnal bladder control.
By 4 years of age, most children demonstrate adult voiding pattern.
The timing of this sequence is influenced by ethnic, cultural,
economic, and individual family differences.
Pathophysiology of BOO
BOO results in an elevation of intravesical storage pressure
Detrusor muscles generate excessive force against outlet resistance
Massive hypertrophy of the detrusor muscles results
Formation of sacculation, trabeculation, and ultimately diverticula
The antireflux mechanism of the ureterovesical junction becomes
incompetent
Ureteral peristalsis is overcome
increased hydrostatic pressures are transmitted directly to the nephron
Results in impairment of renal development and function
Pathophysiology of BOO
Pathophysiology of BOO
GFR falls as pressure in the proximal tubule and bowman space increase
After 1224 hours of complete obstruction, intratubular pressure
decreases to preobstruction levels
If not relieved, a depressed GFR is maintained by decreases in renal blood
flow mediated by thromboxane A2 and angiotensin II
With continued obstruction, there is a progressive fall in renal blood flow,
ischaemia, and nephron damage.
GFR falls, but tubular function is particularly severely affected, with a high
water and sodium loss resulting in the high output renal failure of
obstructive nephropathy.
Pathophysiology of BOO
In the foetus, the placenta functions as the primary excretory organ in
place of the kidney throughout gestation.
Hence, in the new-born with BOO, the renal function is usually similar
to the normal maternal levels initially because of the placental
function.
The kidneys commence gradual glomerular filtration by the 11th to
12th weeks of gestation.
About 90% of amniotic fluid is produced in the kidneys, and only 10%
comes from the GIT, lungs, and skin.
Pathophysiology of BOO
Inadequate foetal urine production leads to oligohydramnios
lung movement is therefore restricted
Poor acinar growth and decreased surfactant production due to
decreased fluid in the bronchial tree
The newborn may have compressed limbs, Potters facies, and
pulmonary hypoplasia presenting with respiratory distress and
pneumothorax
Pathophysiology of BOO
The pathophysiology of this process is complex and incompletely
understood.
Long-term bladder dysfunction may plague these patients in their
daily lives despite relieving the obstruction.
Obstruction impacts renal concentrating ability, glomerular ltration
rate, and renal morphology.
Classification
Primary causes :
Anatomical ( congenital or acquired)
functional (congenital or acquired)
Anatomical causes
Intraluminal
Intramural
extrinsic.
Functional causes
Neurogenic
Nonneurogenic
Aetiolgy
Congenital urinary anomalies
Male Female
Posterior urethral valves Vaginal obstruction
Anterior urethral valves Urogenital sinus
Posterior urethral polyps Cloacal abnormalities
Urethral diverticulum, atresia, duplication, stricture
Ureterocele
Prune belly syndrome
Acquired urogenital anomalies
Bladder neck fibrosis
Trauma
Urethral strictures
Tumors
Clinical Presentation
History Elicit prenatal health, birth and development, perinatal
complication, and bowel and bladder habits.
Many are asymptomatic for a long time or present with constitutional
features that often are misleading to an unsuspecting practitioner
Acute or chronic urinary retention, overflow incontinence, UTI, or
renal failure.
Neonate
Presentation is characterised by
abdominal distention
palpable suprapubic or flank masses (bladder and the kidneys);
And/or urachal cyst, fistula, or abscess.
Neonatal sepsis and respiratory distress may also be present.
Parents may give a history of urine dribble, poor stream, and failure
to thrive in the young infant
Infants and children
Enuresis, weight loss, vomiting, and diarrhoea.
Constipation may be present in cases of dysfunctional voiding, faecal
impaction, Hirschsprungs disease, or compression from pelvic
masses.
Other features include urine dribbling, incontinence, straining,
frequency, and intermittency or staccato stream.
Physical Examination
In the African setting, many children present with complications , due
to late presentation and lack of prenatal diagnosis
small stature for age,
anaemia,
gross pitting pedal oedema,
ascites, and
abdominal masses
Physical examination
Other features depend on primary or secondary causes.
SPINE examine for defects, and the lower extremities for reflexes,
muscle mass and strength, sensation, and gait.
PERINEUM check for gluteal fold symmetry, natal cleft depth, absent
coccyx, perineal sensation, tone and reflexes.
Digital Rectal Examination check anal sphincteric tone, faecal
impaction, distended rectum, and presacral and pelvic masses.
Investigations
Depend on availability and condition
A variety of tests may be required to confirm the diagnosis and
determine the extent of damage
Imaging may be sufficient to define:
site and extent of an obstructing pathology;
extent of urinary tract reaction to the BOO
split and total renal function and scarring;
presence and degree of reflux; and
associated anomalies, where present.
Investigations
Haemogram to quantify anaemia. The platelet count may also drop.
Serum biochemistry to assess the electrolytes, urea, creatinine, and
acid-base balance
Investigations
Plain radiography
can identify radio-opaque stones.
May suggest an enlarged or poorly emptying bladder,
displacement of the bowel shadow from the hypochondrium by a
large fluid-filled kidney, and/or outline associated vertebral and
other bony anomalies.
Investigations
Ultrasonography
Demonstrate filling, dilated, or narrow posterior urethra or the
presence of intraluminal lesions.
Size and shape of the kidneys
bladder as well as dilated pelvicaliceal system and ureter.
US should be performed with a moderately filled urinary bladder,
and if an anomaly is found, US should be repeated with the
bladder empty.
It can be used to estimate the post void residual volume of urine,
and a spinal ultrasound in children younger than 3 months of age
is useful for spinal lesions (tethering, defects, or masses)
Investigations
CT scan/MRI
Done with or without contrast enhancement
Provide precise anatomical details of the lesion
Extent of damage
Good-quality ultrasound with Doppler is a good substitute.
Investigations
Micturating cystourethrography
Essential for demonstration of bladder shape and capacity,
including diverticulum and vesicoureteric reflux and grading,
posterior urethral dilatation, and possible filling defects and
urinary incontinence.
A filling phase is important to identify ureterocoele
Investigations
Scintigraphy
Dimercaptosuccinic acid (DMSA) scintigraphy is a radionuclide
technique that provides a functional cortical map of the kidney,
quantifying renal tubular cell mass.
Useful for identifying scars of reflux nephropathy and estimating
differential function, provided there is no obstruction.
Diethylenetriamine penta-acetic acid (DTPA) scintigram, the DTPA is
filtered by the glomerulus and gives a dynamic study similar to an IVU.
It identifies dilatation and obstruction, and in the latter stages of the
study it can give information concerning reflux.
Differential renal function can also be quantified.
Investigations
Intravenous urogram
An IVU study shows morphology of the urinary tract, provided the
kidney is working well enough.
Is disappointing and sometimes dangerous in infants
In the neonate, due to the low GFR, the urinary tract does not
opacify well.
The x-ray exposure for IVU is relatively high, and US scans are
performed where possible
Investigations
Urodynamic studies
Urodynamic studies of the physiologic function of the bladder
mechanics during filling and voiding
used to ascertain the aetiology and epidemiology of
nonneurogenic bladder sphincter dysfunction (NNBSD) with the
aid of x-ray screening
Egs of such studies include uroflowmetry and cystometrogram.
Investigations
Cystourethroscopy
Approach of choice for identification and treatment of structural
abnormalities.
Posterior urethral valves, ureterocoeles may be treated, and
Polyps May Be biopsied or resected.
In the African setting, however, appropriate scopes may not be
readily available or accessible for either adults or paediatric
surgical services
Management
BOO is a potentially curable form of lower urinary tract and renal
disease
Determinants of bladder dysfunction - Degree and duration of
obstruction
Early recognition and treatment are key to preventrenal function loss
Definitive treatment of mechanical causes of BOO is mostly surgical
Posterior Urethral Valves (PUV)
History
First reported by Langenbeck in 1802 (Dewan et al, 1999)
Hugh Humpton Young developed Classification (Young et al, 1919)
Most common anatomic cause of BOO in pediatric population in
boys
PUVs are not valves, have no function, are not part of normal
development
PUV
PUV classification
Classification(Young)
Type I 95%
2 folds extend anteroinferiorly from caudal aspect of
verumontanum often fusing anteriorly at a lower level
Type II - now discounted as non-obstructive folds of supercial
muscle and mucosa, between the verumontanum and the bladder
neck
Type III 5%
Circular diaphragm with a central or eccentric narrow aperture in
membranous urethra
PUV classification
 Dewan and Ransleys challenged Youngs
classification; the types are secondary to urethral
instrumentation
Characteristics of the urethral valve vary but include:
attachment posteriorly to the distal part of the
verumontanum;
a small hole adjacent to the verumontanum
an oblique membrane with the distal attachment
lying anteriorly
paramedian parallel reinforcements;
Distal ballooning with suprapubic pressure;
traversing the urethral sphincter
PUV Incidence
Incidence is between 1 in 5000 - 8000 male births
Few familial cases recorded, including in siblings, but no established
genetic predisposition
Represent 10% of urological anomalies detected by prenatal U/S in Europe
Accounts for less than 1% of antenatally diagnosed hydronephrosis
Overall mortality is 2550%.
Many foetuses are lost antenatally, and 45% of survivors have renal failure
Other associated anomalies = chromosomal abnormalities, and some
deformations related to the oligohydramnios.
PUV symptoms
Severe obstruction leads to intrauterine renal failure and fetal death
in utero or soon after birth from potters syndrome.
Less severe obstruction allows the fetus to survive, but if not detected
early leads to
Septic complications from UTI and
metabolic abnormalities caused by renal failure
PUV Diagnosis - Prenatal
Fetus
Prenatal U/S detects 80% of cases, 50% between 16-20 weeks
Obstruction develop at 7 wks GA and are detectable by U/S by 14 wks
PUV Diagnosis Prenatal U/S features
Bilateral upper tract dilatation
Persistently distended full bladder
Predictors of poor functional outcome an early-onset renal failure
Detection before 24 weeks gestation
Bladder wall thickening
Echo-bright kidneys (renal dysplasia)
Oligohydramnios
PUV Diagnosis - Prenatal
PUV Treatment
Fetus treatment
Vesicoamniotic shunting
Intrauterine valve ablation
Fetal intervention still controversial because diagnosis is difficult and
benefits are difficult to ascertain
Fetal intervention carries high risk of mortality 43%
Early diagnosis ensures early treatment and minimises risk of
infection
.
Complications
Shunt blockage
Migration
Preterm labour
Intrauterine death
PUV postnatal
Neonate
Symptoms of BOO
Poor urinary stream
Palpable bladder
Kidneys may be palpable
Urinary ascites
Listlessness, poor feeding, irritability, failure to thrive
PUV Postnatal
Infant
May be asymptomatic
Urinary tract infections
Chronically impaired renal function manifest as poor growth
PUV postnatal
Older child
Urinary tact infections
Voiding symptoms
Growth retardation
Treatment
endoscopic ablation of the valves is the initial treatment
PUV postnatal diagnosis
U/S dilation of prostatic urethra and thickening of bladder wall amd
also hydronephrosis
VCUG dilated prostatic urethra, valve leaflets, detrusor hypertrophy,
bladder diverticula, bladder neck hypertrophy, narrow penile urethra
stream and probably incomplete emptying
Renal scan assess kidney function
PUV Treatment
Minimises risk of electrolyte disturbance and infection
Primary treatment
bladder drainage
Antibiotics
Correction of metabolic disturbances
Urethral or suprapubic drainage
Transurethral valve ablation definitive RX incision at 4 and 8 o clock
Post op catheterisation only when significant intra op bleeding but
may be left for 1 or 2 days
 Vesicostomy and delayed valve ablation
Indication
markedly impaired renal function, especially
deteriorating despite catheterisation,
gross bilateral vesicoureteric reflux.
Stoma created at the apex of the bladder to
minimise risk of prolapse
Closure done after valve ablation, by 618
months, depending on the
initial indication
the childs level of renal function
PUV treatment
Urinary leaks due to high intravesical pressure
Urinary ascites and perinephric collections or urinomas usually
respond to a short period of bladder drainage.
Large or persistent perirenal collections may require ultrasound-
guided percutaneous aspiration or open drainage (in combination
with open nephrostomy)
PUV treatment complications
Damage to sphincters
Urethral strictures
haemorrhage
Patent Urachus
Associated with drainage of urine from the umbilicus.
Suspect Clear drainage from the umbilicus
Investigate the urinary tract for bladder outlet obstruction in which the
urachus is functioning as a relief valve
A patent urachus may be approached either through the umbilicus or
through an infraumbilical incision.
It is important to identify all the umbilical structures for a definitive
diagnosis.
The patent urachus is ligated and transected at the level of the bladder;
broad-based connections are closed in two layers with absorbable sutures
Urethral Polyps
Urethral polyps are fibromuscular epithelial structures with
transitional epithelium covering the surface.
They may occur either in the posterior or anterior urethra.
In either position, they may cause hematuria, urgency, or obstructive
symptoms.
Bladder ultrasound may show the polyps in the posterior urethra but
VCUG and cystoscopy are diagnostic and excision through the
cystoscope is usually curative.
Polyps can present as congenital obstructing lesions with all of the
characteristics of bladder outlet obstruction.
References
Coran Pediatric Surgery 7th Edition
Essential of Pediatric Urology, 2nd Edition, Edited by David FM Thomas
Clinical Pediatric Nephrology, 2nd Edition, Edited by Kanwal K Kher
MD
Langmans medical embryology, 12th Edition