Congenital Pediatric Urinary Disorders
Congenital
Pediatric
Urinary
Disorders
Dr.
Hamdan
ALHAZMI
Associated
Professor
and Consultant Pediatric
Urologist
L
e
a
r
n
i
n
g
O
b
j
e
c
t
i
v
e
s
:
Identify the
common
congenital
anomalies.
How
to
detect this
anomaly
on radiological
investigations.
Important
steps
in
management.
C
o
n
g
e
n
i
t
a
l
U
r
i
n
a
r
y
D
i
s
o
r
d
e
r
s
Anomalies of the Upper Urinary
Tract
Kidney
Ureter
Anomalies of the Lower Urinary
Tract
Urinary
Bladder
Urethra
A
n
o
m
a
l
i
e
s
o
f
t
h
e
k
i
d
n
e
y
Anomalies
of:
Number
Ascent
Form and
Fusion
Rotation
1 in
1100
births.
Male
:
Female
of 1.8 :
1
The
left
side is absent
more
frequently
.
The
ipsilateral
ureter
is
completely
absent in
50%.
1
-
U
n
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
U
n
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
…
Associated
anomalies:
Anomalies
of other
organ
systems are found frequently in
affected
individuals
CVS,GIT,MSC
Müllerian
duct
abnormalities
25%
to
50%
of
females
10%
to
15%
of
males
Approximately
one
fourth
to
one
third
of
women
with
Mullerian
duct
anomalies are
found
to
have
URA.
U
n
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
…
.
P
r
e
s
e
n
t
a
t
i
o
n
Prenatal
US
Incidentally
Abdominal
US
Abdominal
CT
U
n
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
…
D
i
a
g
n
o
s
i
s
Confirmed
Nuclear
study
(DMSA
)
U
n
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
…
2
-
B
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
B
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
…
B
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
…
B
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
Ureters
are
almost
always
absent
.
Bladder
is
either
absent or
hypoplastic
.
Adrenal glands
are usually positioned
normally
.
Müllerian duct
anomalies
are
commonly
observed.
Prognosis
40% are
stillborn.
Do
not
survive beyond
48
hours due to
respiratory
distress associated
with
pulmonary
hypoplasia.
B
i
l
a
t
e
r
a
l
R
e
n
a
l
A
g
e
n
e
s
i
s
…
Definitive
accessory
organ
with
its
own
collecting system,
blood
supply,
and distinct
encapsulated
parenchyma.
Either
completely
separate or
loosely
attached
to the kidney
on
the
ipsilateral
side.
The ureteral
inter-relationships
on
the side of the supernumerary
kidney can be
variable.
3
-
S
u
p
e
r
n
u
m
e
r
a
r
y
K
i
d
n
e
y
4
-
S
i
m
p
l
e
R
e
n
a
l
E
c
t
o
p
i
a
4
-
S
i
m
p
l
e
R
e
n
a
l
E
c
t
o
p
i
a
Left
more
than
the
right.
1 of 2100 to 3000
autopsies.
Most ectopic kidneys
are
clinically
asymptomatic
.
4
-
S
i
m
p
l
e
R
e
n
a
l
E
c
t
o
p
i
a
…
Associated
Anomalies:
50% have a
hydronephrosis:
Obstruction: UPJO
and
UVJO
Reflux (VUR): grade
III
or
greater
Malrotation
Genital
anomalies
in the
patient with ectopia is
about
15%.
5
-
C
r
o
s
s
e
d
R
e
n
a
l
E
c
t
o
p
i
a
C
r
o
s
s
e
d
e
c
t
o
p
i
a
:
k
i
d
n
e
y
i
s
l
o
c
a
t
e
d
o
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t
h
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i
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e
o
p
p
o
s
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f
r
o
m
t
h
a
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i
n
w
h
i
c
h
i
t
s
u
r
e
t
e
r
i
n
s
e
r
t
s
i
n
t
o
t
h
e
b
l
a
d
d
e
r
.
The
ureter
from
each
kidney
is
usually
orthotopic.
w
i
t
h
o
u
t
F
u
s
i
o
n
w
i
t
h
F
u
s
i
o
n
C
r
o
s
s
e
d
R
e
n
a
l
E
c
t
o
p
i
a
…
90%
are
fused
the
superior
pole
of
the
ectopic
kidney
usually
joins
with
the
inferior
aspect
of
the
normal
kidney.
6
-
H
o
r
s
e
s
h
o
e
K
i
d
n
e
y
The
isthmus
is
bulky and
consists
of
parenchymatous
tissue.
Occurs 1 in
400
persons.
The
calyces:
normal in
number
atypical
in
orientation.
pelvis
remains
in the vertical or obliquely
lateral
plane
Horseshoe kidney is frequently found in association with other
congenital
anomalies.
UPJ obstruction in
one
third.
60
%
asymptomatic.
H
o
r
s
e
s
h
o
e
K
i
d
n
e
y
The kidney and renal pelvis
normally
rotate 90 degrees
ventromedially
during
ascent
the calyces point
laterally.
the pelvis faces
medially
.
When this
alignment
is
not
exact,
the condition is
known
as
malrotation.
Frequently associated with
Turner
syndrome.
7
-
A
n
o
m
a
l
i
e
s
o
f
R
o
t
a
t
i
o
n
8
-
U
r
e
t
e
r
o
p
e
l
v
i
c
j
u
n
c
t
i
o
n
(
U
P
J
)
o
b
s
t
r
u
c
t
i
o
n
P
r
e
s
e
n
t
a
t
i
o
n
:
Prenatal
US
Incidental
in
Neonates/Children
Symptomatic:
UTI
Pain
Mass
Hematuria
Stone
U
P
J
…
U
P
J
O
…
D
y
n
a
m
i
c
r
e
n
o
g
r
a
m
U
P
J
O
…
D
i
s
m
e
m
b
e
r
e
d
P
y
e
l
o
p
l
a
s
t
y
U
P
J
O
…
9
-
U
r
e
t
e
r
o
v
e
s
i
c
a
l
j
u
n
c
t
i
o
n
(
U
V
J
)
o
b
s
t
r
u
c
t
i
o
n
(
M
e
g
a
u
r
e
t
e
r
s
)
An ectopic ureter is any
ureter,
single or duplex, that
doesn't
enter the trigonal area of the
bladder
.
1
1
-
E
c
t
o
p
i
c
U
r
e
t
e
r
In
females
the ectopic ureter
may
enter anywhere from the bladder
neck to the perineum
and into
the vagina, uterus,and even
rectum.
One
of the classic
symptoms
is
continuous
wetting.
E
c
t
o
p
i
c
U
r
e
t
e
r
…
In
males
the ectopic ureter
always
enters the urogenital system
above the external sphincter or
pelvic
floor,
and usually into the
wolffian
structures including vas
deferens,
seminal
vesicles, or
ejaculatory
duct.
E
c
t
o
p
i
c
U
r
e
t
e
r
A
cystic dilation
of the
distal
aspect of the
ureter
Located
either
within the
bladder
or spanning the bladder neck
and
urethra.
P
r
e
s
e
n
t
a
t
i
o
n
:
Antenatal
(U/S)
Urine
retention
Infection
Calculus
formation
1
2
-
U
r
e
t
e
r
o
c
e
l
e
U
r
e
t
e
r
o
c
e
l
e
…
1
3
-
V
e
s
i
c
o
u
r
e
t
e
r
a
l
R
e
f
l
u
x
(
V
U
R
)
N
o
r
m
a
l
a
n
t
i
-
r
e
f
l
u
x
m
e
c
h
a
n
i
s
m
“
F
l
a
p
v
a
l
v
e
”
1.
Oblique
course
as
it
enters
the
bladder.
2.
Proper
muscular
attachments
to
provide
fixation.
3.
Posterior
support
to
enable
its
occlusion.
4.
Adequate
submucosal
length.
V
e
s
i
c
o
u
r
e
t
e
r
a
l
R
e
f
l
u
x
(
V
U
R
)
…
Presentation
Asymptomatic
Prenatal
Fluctuated
dilatation
Febrile
UTIs
V
U
R
…
D
i
a
g
n
o
s
i
s
:
M
C
U
G
(
V
C
U
G
)
V
U
R
…
Management:
Prophylactic
antibiotic
Surgical
treatment
Endoscopic
treatment
Ureteral
reimplantation
V
U
R
…
Urachal
anomalies
are
usually
detected postnatally
due
to
umbilical
drainage.
Imaging possibilities
include
ultrasound,
CT,
and
VCUG.
1
4
-
U
r
a
c
h
a
l
a
b
n
o
r
m
a
l
i
t
i
e
s
Conservative
treatment
with
observation is
justified
in
asymptomatic
cases
due
to
possible spontaneous
resolution
Infected urachal
remnants
are
initially treated
with drainage
and
antibiotics, followed by
surgical
excision.
Nonresolved urachal
remnants
should be excised
due
to
theincreased risk of
later
adenocarcinoma
formation
1
4
-
U
r
a
c
h
a
l
a
b
n
o
r
m
a
l
i
t
i
e
s
…
1
5
-
B
l
a
d
d
e
r
D
i
v
e
r
t
i
c
u
l
u
m
Bladder
diverticula
can be
detected on prenatal
ultrasound,
but
the gold standard
remains
VCUG, which will reveal
possible
accompanying
VUR.
Types:
Primary
diverticula
arise
as
a localized herniation of
bladder
mucosa
at the ureteral
hiatus
and are
most
likely caused by a
congenitally
deficient bladder
wall.
Secondary para-ureteral
diverticula
are acquired and develop due to existing infra- vesical
obstruction.
Symptomatic diverticula,
especially in
conjunction
with VUR, should
be
treated
surgically.
B
l
a
d
d
e
r
D
i
v
e
r
t
i
c
u
l
u
m
…
Often associated with
duplication
anomalies
of the external
genitalia
and lower
gastrointestinal
tract.
Initial treatment
is
directed
toward
renal
preservation.
prevention of
infections.
1
6
-
B
l
a
d
d
e
r
D
u
p
l
i
c
a
t
i
o
n
B
l
a
d
d
e
r
D
u
p
l
i
c
a
t
i
o
n
…
Long-term goals include achieving continence
and
reconstructing
the internal and external
genitalia.
Due
to the rarity of the disease and the
large
variety
of
presentations, the
surgeries must
be
individualized
1
7
-
B
l
a
d
d
e
r
E
x
s
t
r
o
p
h
y
The incidence of bladder exstrophy
has been
estimated
as
between 1 in 10,000 and 1 in
50,000.
B
l
a
d
d
e
r
E
x
s
t
r
o
p
h
y
…
1
8
-
P
o
s
t
e
r
i
o
r
U
r
e
t
h
r
a
l
V
a
l
v
e
s
(
P
U
V
)
The bladder and the kidneys
developed under high
pressure
and
resistance.
1 in
8000
to
25,000
live
births.
Make
up
10%
of urinary
obstructions diagnosed in
utero.
Most
common
cause of
urine
retention in
male
infants.
50%
have renal
impairment.
P
U
V
…
P
U
V
…
•
Associated
findings:
1.
Oligohydramnios
2.
Bilateral renal dilatation
3.
VUR:
40%
4.
Valve
bladder
5.
Renal
impairment
Presentation:
1.
Antenatal
2.
Urine
retention
3.
UTI
4.
Poor urinary
stream
5.
Urinary
incontinence
6.
CRF
(ESRD)
P
U
V
…
Initial
treatment
Feeding
tube
insertion
Start
antibiotic
prophylactic
Ultrasound
MCUG
P
U
V
…
P
U
V
…
Endoscopic
valve
ablation
Cutaneous
vesicostomy
Abnormal position of the
EUM
on the ventral
surface.
Types:
Distal
hypospadias.
Proximal
hypospadias.
NO
Circumcision
6 to 9
months
repair.
1
9
-
H
y
p
o
s
p
a
d
i
a
s
F
e
m
a
l
e
M
a
l
e
2
0
-
E
p
i
s
p
a
d
i
a
s
2
1
-
C
l
o
a
c
a
l
E
x
s
t
r
o
p
h
y
The incidence
:1
in29,000 to 1
in
40,000
live
births
The three
major
findings
are
deficiency of the
abdominal
musculature,
bilateral intra-abdominal
testes,
anomalous urinary
tract
Other
names
Triad
syndrome
Eagle-Barrett
syndrome
abdominal
musculation
syndrome
2
2
-
P
r
u
n
e
-
B
e
l
l
y
S
y
n
d
r
o
m
e
The
most common
cause of
neurogenic bladder dysfunction
in
children is
abnormal
development
of the spinal canal
and
internecine
spinal
cord.
2
3
-
N
E
U
R
O
S
P
I
N
A
L
D
Y
S
R
A
P
H
I
S
M
S
Cutaneous lesions occur in
90%
of children with various occult
dysraphicstates.
These lesions vary
from
small
lipomeningocele
hair
patch
dermal
vascular
malformation
sacral
dimple
abnormal
gluteal
cleft.
N
E
U
R
O
S
P
I
N
A
L
D
Y
S
R
A
P
H
I
S
M
S
…
A
n
t
e
n
a
t
a
l
H
y
d
r
o
n
e
p
h
r
o
s
i
s
(
A
N
H
)
A
n
t
e
n
a
t
a
l
H
y
d
r
o
n
e
p
h
r
o
s
i
s
(
A
N
H
)
C
a
u
s
e
s
:
Pelviureteric
junction
obstruction
(41%)
Ureterovesical
junction
obstruction
(23%)
Vesicoureteric reflux(7%)
Duplication
anomalies
(13%)
Posterior
urethral
valves
(10
%)
MCDK
Others
(6%)
This content delves into congenital urinary disorders in children, covering anomalies of the upper and lower urinary tracts, focusing on kidney and ureter abnormalities like renal agenesis. Details on diagnosis, management, and associated anomalies are discussed.
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Presentation Transcript
Congenital Pediatric Urinary Disorders Dr. HamdanALHAZMI Associated Professor and Consultant PediatricUrologist drhamdan@ksu.edu.sa
LearningObjectives: Identify the common congenitalanomalies. How to detect this anomaly on radiologicalinvestigations. Important steps in management.
Congenital UrinaryDisorders Anomalies of the Upper UrinaryTract Kidney Ureter Anomalies of the Lower UrinaryTract Urinary Bladder Urethra
Anomalies of thekidney Anomaliesof: Number Ascent Form and Fusion Rotation
1- Unilateral RenalAgenesis 1 in 1100births. Male: Female of 1.8 : 1 The left side is absentmore frequently . The ipsilateral ureteris completely absent in 50%.
Unilateral RenalAgenesis. Associatedanomalies: Anomalies of other organ systems are found frequently in affected individuals CVS,GIT,MSC M llerian ductabnormalities 25% to 50% of females 10% to 15% of males Approximatelyonefourth to onethird of women with Mullerian duct anomalies are found to haveURA.
Unilateral RenalAgenesis Presentation PrenatalUS Incidentally AbdominalUS AbdominalCT
Unilateral RenalAgenesis Diagnosis Confirmed Nuclear study(DMSA)
Bilateral RenalAgenesis Ureters are almost always absent. Bladder is either absent or hypoplastic. Adrenal glands are usually positionednormally. M llerian duct anomalies are commonlyobserved.
Bilateral RenalAgenesis Prognosis 40% are stillborn. Do not survive beyond 48 hours due to respiratory distress with hypoplasia. associated pulmonary
3- SupernumeraryKidney Definitive accessory organ with its own collecting system, blood supply, and distinct encapsulated parenchyma. Either completely separate or loosely attached to the kidneyon the ipsilateral side. The ureteral inter-relationshipson the side of the supernumerary kidney can be variable.
4- Simple RenalEctopia Left more than the right. 1 of 2100 to 3000autopsies. Most ectopic kidneysare clinicallyasymptomatic.
4- Simple RenalEctopia AssociatedAnomalies: 50% have a hydronephrosis: Obstruction: UPJO and UVJO Reflux (VUR): gradeIII or greater Malrotation Genital anomalies in the patient with ectopia isabout 15%.
5- Crossed RenalEctopia Crossed ectopia: kidney is located on the side opposite from that in which its ureter insertsinto the bladder. The ureter from each kidney is usuallyorthotopic.
Crossed RenalEctopia withFusion withoutFusion 90% are fused the superior pole of the ectopic kidney usuallyjoins with the inferior aspect of the normalkidney.
6- HorseshoeKidney The isthmus is bulky and consists of parenchymatous tissue.
HorseshoeKidney Occurs 1 in 400 persons. The calyces: normal in number atypical in orientation. pelvis remains in the vertical or obliquely lateralplane Horseshoe kidney is frequently found in association with othercongenital anomalies. UPJ obstruction in one third. 60 % asymptomatic.
7- Anomalies ofRotation The kidney and renal pelvis normally rotate 90 degrees ventromedially duringascent the calyces point laterally. the pelvis faces medially. When this alignment is notexact, the condition is known as malrotation. Frequently associated withTurner syndrome.
UPJ Presentation: PrenatalUS Incidental inNeonates/Children Symptomatic: UTI Pain Mass Hematuria Stone
UPJ O Dynamicrenogram
UPJ O DismemberedPyeloplasty
9-Ureterovesical junction (UVJ) obstruction (Megaureters)
11-EctopicUreter An ectopic ureter is anyureter, single or duplex, that doesn't enter the trigonal area of the bladder.
EctopicUreter In females the ectopic ureter may enter anywhere from the bladder neck to the perineum and the vagina, uterus,and rectum. One of the classic symptomsis continuous wetting. into even
EctopicUreter In males the ectopic ureteralways enters the urogenital system above the external sphincter or pelvic floor, and usually into the wolffian structures including vas deferens, seminal vesicles, or ejaculatory duct.
12-Ureterocele A cystic dilation of thedistal aspect of the ureter Located either within the bladder or spanning the bladder neck and urethra. Presentation: Antenatal(U/S) Urineretention Infection Calculusformation
Vesicoureteral Reflux(VUR) Normal anti-reflux mechanism Flapvalve 1. Oblique course as it entersthe bladder. 2. Proper muscularattachments to providefixation. 3. Posterior support to enableits occlusion. 4. Adequate submucosallength.
VUR Presentation Asymptomatic Prenatal Fluctuateddilatation FebrileUTIs
VUR Diagnosis: MCUG(VCUG)
VUR Management: Prophylacticantibiotic Surgicaltreatment Endoscopictreatment Ureteral reimplantation
14-Urachalabnormalities Urachal anomalies areusually detected postnatally due to umbilical drainage. Imaging possibilitiesinclude ultrasound, CT, andVCUG.
14-Urachalabnormalities Conservative treatment with observation is justified in asymptomatic cases due to possible spontaneousresolution Infected urachal remnants are initially treated with drainageand antibiotics, followed by surgical excision. Nonresolved urachalremnants should be excised due to theincreased risk of later adenocarcinoma formation
15-BladderDiverticulum Bladder diverticula can be detected on prenatalultrasound, but the gold standard remains VCUG, which will reveal possible accompanyingVUR.
BladderDiverticulum Types: Primary diverticula arise as a localized herniation of bladder mucosa at the ureteral hiatus and are most likely caused by a congenitally deficient bladder wall. Secondary para-ureteral diverticula are acquired and develop due to existing infra- vesical obstruction. Symptomatic diverticula, especially in conjunction with VUR, shouldbe treated surgically.
16-BladderDuplication Often associated withduplication anomalies of the external genitalia and lower gastrointestinal tract. Initial treatment is directed toward renal preservation. prevention of infections.
BladderDuplication Long-term goals include achieving continenceand reconstructing the internal and externalgenitalia. Due to the rarity of the disease and the large varietyof presentations, the surgeries must beindividualized
17-BladderExstrophy The incidence of bladder exstrophy has been estimated as between 1 in 10,000 and 1 in 50,000.
18-Posterior Urethral Valves(PUV) The bladder and the kidneys developed under high pressure and resistance.
PUV 1 in 8000 to 25,000 livebirths. Make up 10% of urinary obstructions diagnosed inutero. Most common cause of urine retention in male infants. 50% have renalimpairment.
