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‫الرحيم‬ ‫الرحمن‬ ‫هللا‬ ‫بسم‬
Dr. Ahmed Esawy
MBBS M.Sc
MD
Dr/AHMED ESAWY
FETAL
URINARY TRACT
Dr/AHMED ESAWY
• Normal kidneys at 13 weeks gestation. Coronal transvaginal US
image of the fetal abdomen shows normal kidneys (cursors). Fluid is
seen in the renal pelves (arrowheads).
Dr/AHMED ESAWY
Normal Renal Echogenicity
• The normal echogenicity of the fetal kidney is
equal to that of the liver (Figs. A and B). Since
this is a subjective assessment, it may have
significant intra and interobserver variation.
Generally, fetal renal echogenicity refers to the
appearance of the entire kidney rather than to
an isolated component of the kidney, i.e. the
cortex, medulla or pyramids.
Dr/AHMED ESAWY
• NORMAL
ECHOGENICITY
• 38 WEEKS NORMAL
ECHOGENICITY
Dr/AHMED ESAWY
Physiologic Echogenic Kidneys
• Bilateral echogenic kidneys in normal fetus
Dr/AHMED ESAWY
Echogenic fetal kidneys.
• Physiologic variation
• Intrinsic renal disease
– Autosomal dominant polycystic renal disease
– Autosomal recessive polycystic renal disease
• Obstructive uropathy
• Aneuploidy
– Trisomy 13
– Trisomy 18
• Infection
– Cytomegalovirus
– Candida
Dr/AHMED ESAWY
Echogenic fetal kidneys.
• Overgrowth Syndromes
– Beckwith-Wiedemann
– Perlman
– Simpson-Golabi-Behmael
• Meckel-Gruber
• Bardet-Biedl syndrome
• Renal vein thrombosis
• Congenital nephrotic syndrom
Dr/AHMED ESAWY
Causes of Fetal Hydronephrosis
Physiologic:
• Urine production 4-6x greater before delivery
• Increased compliance of fetal ureter
• Partial/transient obstructions associated with
development
Dr/AHMED ESAWY
HYDRONEPHROSIS
The most common causes are:
UPJ obstruction
Duplicated collecting system with obstruction of upper pole
Bladder outlet obstruction
Male
PUV (thick bladder wall)
Prune-belly syndrome (normal bladder wall)
Females and males
Caudal regression syndrome
Megacystis-microcolon-intestinal hypoperistalsis
Ureteral agenesis
Maternal drugs
Ectopic ureterocele
Dr/AHMED ESAWY
Grades of Fetal Hydronephrosis
Dr/AHMED ESAWY
Urinary
Obstruction
• Assess bladder filling /
emptying
– AFI
– Direct visualization
• Physiological Reflux
– Nml 2nd and 3rd trimester
Dr/AHMED ESAWY
CLASSIFICATION OF FETAL
HYDRONEPHROSIS
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Dr/AHMED ESAWY
DD .Prominent extra-renal pelvis
Dr/AHMED ESAWY
left renal pelvicalyceal dilatation (left image) and hyperechogenicity of
both kidneys without corticomedullary differentiation
Dr/AHMED ESAWY
• Axial renal images, 24
weeks GA showing
pelvicalyceal dilatation on
the left.
• Coronal section at 27
weeks GA with right renal
measurement (bottom of
image) and left dilated
renal pelvis measurement
Dr/AHMED ESAWY
Parasagittal images at 27 weeks with
measurements (left kidney is on the left image).
Parasagittal images at 29 weeks GA; left
kidney is on the left image.
Dr/AHMED ESAWY
Mild bil.pyelectasis
Not more than 4mm
Dr/AHMED ESAWY
Measuring Renal Dilatation
Anteroposterior diameter (APD): Size of the
renal pelvis measured in the anterior
→posterior direction on a transverse view
through the abdomen.
•For a diagnosis of hydronephrosis, APD must
be greater than:
6mm at < 20 weeks GA
8 mm at 20-30 weeks GA
10mm at > 30 weeks GA
Dr/AHMED ESAWY
• These ultrasound images reveal
unilateral hydronephrosis of
the left fetal kidney.
• RPD (renal pelvic A-P diameter)
is more than 10mm (after 20
weeks gestational age). If less
than 20 weeks, more than
6mm. is abnormal.
Dr/AHMED ESAWY
Protocol for post natal follow up of
fetal renal pyelectasis
• Less than 5mm ---- Normal –no follow up
• From (5-10) mild and need post natal scan after two weeks ,if
normal ,the imaging either stop or one additional scan
between three and six months of age.
• More than 10 significant pyelectasis . follow up done before
discharge (day 1or 2 of life).
• if scan looked normal follow up done at 3,6 months and one
year if scan remain normal one additional scan at 5 year.Dr/AHMED ESAWY
• if abnormalities detected voiding
cystourethrography should be done at 48 hours in
suspected outflow obstruction and surgery must
performed before discharge .
• Antibiotic prophylaxis should be given and
continued until reflux ruled out especially in case of
unilateral hydronephrosis. .
• Other cases of obstructive uropathy can be closely
followed in the 1st two years every 3 months using
US and Reno gram .surgery indicated only if renal
function deteriorated -hydronephrosis increased or
in febrile infection .
Dr/AHMED ESAWY
anteroposterior measurement of renal cortical thinning due to
pelvicalyceal dilatation.
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Left kidney, sagittal view
Right kidney, sagittal view
mild hydronephrosis on the
right with central dilatation
of 8-9 mm and severe
hydronephrosis on the left
with a large extrarenal
pelvis measuring 2.8 cm
Dr/AHMED ESAWY
Hydronephrosis
Dr/AHMED ESAWY
Ureteropelvic junction obstruction
Dr/AHMED ESAWY
• Difference between obstruction and reflux is
thick trabeculated UB wall in obstruction
Dr/AHMED ESAWY
Vesicoureteric reflux
Dr/AHMED ESAWY
Renal Cystic Disease
• Cystic Renal Dysplasia
– Posterior urethral valves
– Hydronephrosis
– Increased parenchymal echogenicity
– Cysts Vary Size
• Multicystic Dysplastic Kidney
– Multiple non-communicating cysts Vary in size
- Multiple or single echo- free spaces.
• - Identification should start after (22ws).
• - If bilateral (bladder not demonstrated- amniotic
• fluid vol decreased or absent).
• - Meckel’s syndrome ( encephalocele- cleft palate-
• polydacty + cong. Ht disease), may be associated.
– Non-functioning renal tissue
– If bilateral
• Oligohydraminos
• Not compatible w/ life
Dr/AHMED ESAWY
Renal Cystic Disease
• ARPCKDz / Infantile PCKDz
– Massively big kidneys
bilaterally
– Echogenic
– Sonolucent rim
– Discrete cysts are not seen
– Cysts ( Microscepic to
macroscepic +
oligohydramnios)
• ADPCKDz
– Enlarged kidneys
– Non-sonolucent rim
– Discrete cysts +/- seen
– Cysts + normal
amniotic fluid.
– Symptoms silent till 5
th decade
Dr/AHMED ESAWY
Differential Diagnosis of Bilateral
Multiple Cysts
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Dr/AHMED ESAWY
MULTICYSTIC DYSPLASTIC KIDNEY
DISEASE (POTTER TYPE II)
Dr/AHMED ESAWY
MCDK
Dr/AHMED ESAWY
Multicystic dysplastic kidney
several cysts
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Multicystic Dysplastic kidney
Dr/AHMED ESAWY
Multicystic
Dyplastic Kidney
Dr/AHMED ESAWY
multicystic dysplastic kidney
Dr/AHMED ESAWY
Multicystic
Dyplastic
Kidney with
a large cyst,
increasing in
size
Dr/AHMED ESAWY
Bilateral Multicystic Dyplastic Kidneys
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Multicystic Dyplasia
with multiple non-
communicating
cysts of varying
sizes Dr/AHMED ESAWY
• normal kidney
(calipers) with typical
corticomedullary
differentiation.
Dr/AHMED ESAWY
• left kidney with clear corticomedullary
differentiation.
Dr/AHMED ESAWY
Dr/AHMED ESAWY
• autosomal recessive polycystic kidney disease.
• markedly enlarged (8 cm)
• hyperechoic
• no corticomedullary differentiation.
• Oligohydramnios is present.
• Ch = fetal chest Dr/AHMED ESAWY
• autosomal recessive polycystic kidney disease
Dr/AHMED ESAWY
A small, slightly echogenic kidney (arrows) seen with the normal contra lateral one
Polycystic echogenic
Dr/AHMED ESAWY
Dr/AHMED ESAWY
• Sonography of the fetal abdomen in this 2nd trimester fetus reveals-
• 1) bilateral grossly enlarged fetal kidneys, almost filling the entire abdomen
• 2) markedly hyperechoic fetal kidneys with minute anechoic areas within them.
• 3) oligohydramnios
• 4) poorly distended urinary bladder.
• This ultrasound image is diagnostic of fetal polycystic kidney disease (ARPKD). This image
was taken by Dr. Durr-e-Sabih, Pakistan, using a Toshiba Nemio-30 color doppler machine.
Dr/AHMED ESAWY
• These ultrasound images (another
case) also show grossly enlarged,
echogenic fetal kidneys with
minute cystic lesions (1 to 2 mm.)
within them. (typical rosette like
pattern)
• is typical of ARPCKD.
Dr/AHMED ESAWY
• Recurrence of glomerulocystic
kidney disease.
• striking hyperechogenicity of
cortex and no corticomedullary
differentiation. Renal length is
30 mm. CH = fetal chest
Dr/AHMED ESAWY
Adult polycystic kidney disease (APKD)
enlarged and hyperechogenic with or without multiple cysts. Unlike infantile polycystic
kidneys, where there is a loss of the corticomedullary junction, in APKD there is
accentuation of this junction. The amniotic fluid volume is either normal or reduced
The kidney size is usually smaller than that of the infant polycystic kidneys
Dr/AHMED ESAWY
Differential diagnosis of Congenital
Cystic Disease of Kidney
• Isolated renal cysts : (DD: PUJO.)
– isolated + not communicating pelvis.
• Multicystic kidney (sequlae of
obstructive uropthy).
Dr/AHMED ESAWY
Dr/AHMED ESAWY
simple renal cyst (left kidney).
Dr/AHMED ESAWY
Large unilocular cystic mass (arrow) in a 36 weeks
mature fetus. It proved to be left hydronephrotic
kidney due to UPJ obstruction
Dr/AHMED ESAWY
Renal Agenesis (potter syndrome)
• Absent renal echoes and absent ur bladder
(identification of bladder exclude diagnosis
of polycystic kid. & renal agenesis).
• Oligohydramnios.
• Reduced foetal growth rate (BPD & A.C).
Dr/AHMED ESAWY
• The Potter sequence is a constellation of findings demonstrated
postnatally as a consequence of severe,
prolonged oligohydramnios in utero.
• It consists of :
• pulmonary hypoplasia: often severe and incompatible with life
• growth restriction (IUGR)
• abnormal facies (Potter facies): including
– low set ears
– flattened nose
– wrinkled skin
– micrognathia
• limb abnormalities: including club feet and contractures
Dr/AHMED ESAWY
Potter sequence
Potter sequence
• Although originally associated with renal causes of
olighydramnios, the Potter sequence may be seen with severe
oligohydramnios of essentially any cause.
• Common aetiologies include :
• fetal renal insufficiency
– bilateral renal agenesis: traditionally known as the classic Potter
syndrome
– autosomal recessive polycystic kidney disease
– posterior urethral valves, and other causes of antenatal renal tract
obstruction
• premature rupture of membranes (PROM)
• twin-twin transfusion
Dr/AHMED ESAWY
Potter sequence, oligohydramnios sequence
Renal agenesis
Common Findings
• Oligohydramnios, usually of early onset (by
16–18 weeks’ gestation)
• Absent kidneys (although the adrenal glands
may mimic kidneys; this is a pitfall in the early
diagnosis of this condition),
• Narrow chest (secondary to compression by
the oligohydramnios), appearing later in
gestation , Pulmonary hypoplasia
Dr/AHMED ESAWY
Potter sequence, oligohydramnios
sequence Renal agenesis
Occasional Findings
• Vertebral defects
• Complex cardiac defects (14%)
• Skeletal anomalies (40%), such as sirenomelia,
absent radius, and vertebral anomalies
• CNS anomalies (11%), such as ventriculomegaly
and neural tube defects
• Other anomalies, such as imperforate anus,
duodenal atresia, tracheoesophageal fistula, and
single umbilical artery
Dr/AHMED ESAWY
Syndromes and Conditions Associated
with Bilateral Renal Agenesis
• Caudal regression syndrome
• Fraser syndrome
• MURCS (müllerian duct aplasia, renal aplasia,
cervicothoracic somite dysplasia) association
• Sirenomelia
• VATER (vertebral defects, anal atresia,
tracheoesophageal fistula, esophageal atresia,
and radial and renal dysplasias) syndrome
Dr/AHMED ESAWY
Autosomal recessice polycystic kidney
disease ARPCKD POTTER type 1
• Massively
enlarged
echogenic
kidneys
Dr/AHMED ESAWY
POTTER Type II
Type II
IIA
IIB
Multicystic renal dysplasia.
Multilocular cystic nephroma.
Kidneys of normal / increased size.
Kidneys reduced in size.
Dr/AHMED ESAWY
• Bilateral
Multicystic renal
dysplasia
Dr/AHMED ESAWY
Multicystic Renal Dysplasia
(MCRD) or Potter Type II
Dr/AHMED ESAWY
• Longitudinal cut
of large renal
silhouettes with
multiple cystic
images
Multicystic Renal Dysplasia
(MCRD) or Potter Type II
Dr/AHMED ESAWY
Axial cut, at the abdominal level in the
posterior paravertebral sector, cystic formations
of variable size and anarchic distribution, but
mainly peripherical, are visualized.
• Color Doppler ultrasound showing the
presence of both renal and iliac arteries.
Dr/AHMED ESAWY
• Left kidney of large size
with cystic
structures,presented as
paraspinal mass.
Dr/AHMED ESAWY
Adult polycystic kidney disease
POTTER Type III
Type III Adult polycystic disease of the
kidneys.
Tuberous sclerosis.
Medullary sponge kidney.
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Adult polycystic kidney disease
• Prenatal diagnosis by ultrasonography is
confined to a few case reports and the kidneys
have been described as enlarged and hyper-
echogenic with or without multiple cysts
• accentuation of this junction
• suspected when cystic enlarged kidneys are
detected in association with a normal amount
of amniotic fluid
Dr/AHMED ESAWY
Dr/AHMED ESAWY
This 3rd trimester fetus has brightly echogenic kidneys with accentuation of the cortico-
medullary junction and a small bladder with decreased fluid.
• the echogenic kidneys but normal bladder and
amniotic fluid.
Dr/AHMED ESAWY
Type IV Small cortical cysts / cystic
renal dysplasia secondary
toureteropelvic junction obstruction.
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Dr/AHMED ESAWY
• both the renal fossae are
not occupied by kidneys,
and are occupied by the
adrenal glands producing
lying adrenal sign.
• Postnatal radiograph
shows bell-shaped thorax,
suggestive of pulmonary
hypoplasia
Dr/AHMED ESAWY
Antenatal ultrasound and MRI findings
of Pena–Shokeir syndrome
T2W MR coronal
(a) and oblique (b) images
demonstrate persistent flexion
of bilateral wrist and elbow
joints with extension of the
fingers, partial flexion at knee
joint and bilateral club foot Dr/AHMED ESAWY
(b) reveals scoliosis,
bilateral flexion at the
elbows, wrists, and knees.
No underlying bony
abnormality was seen
Dr/AHMED ESAWY
Antenatally detected ovarian cyst. a simple
cyst (4.5x3.8 cm) located in the left side of
the abdomen, bulging through the anterior
abdominal wall.
Dr/AHMED ESAWY
Overgrowth Syndromes
• Beckwith-Wiedemann syndrome is
characterized by visceromegaly,
macroglossia, polyhydramnios and
enlarged echogenic kidneys with a
loss of corticomedullary
differentiation . This overgrowth
syndrome also has a variety of
possible congenital anomalies,
including omphalocele,
hemihypertrophy and cardiac
anomalies.
Dr/AHMED ESAWY
ENLARGED ECHOGENIC KIDNEYS WITH
BECKWITH-WIEDEMANN SYNDROME
• Bardet-Biedl syndrome.
• marked enlargement (49
mm between calipers)
without corticomedullary
differentiation.
• B, Ultrasound scan
shows postaxial
polydactyly. six digits.
Dr/AHMED ESAWY
• Joubert syndrome.
• kidneys appear hyperechoic
• without corticomedullary differentiation
• cysts at corticomedullary junction.Dr/AHMED ESAWY
Meckel-Gruber Syndrome
• This is an autosomal
recessive disorder
characterized by an
occipital encephalocele
, postaxial polydactyly ,
and echogenic
significantly enlarged
kidneys. Of this triad of
anomalies
Dr/AHMED ESAWY
Occipital encephalocele
Post axial polydactyl
• Meckel-Grüber syndrome.
• kidney is enlarged.
• Corticomedullary differentiation is unexpectedly evident at this early stage
• mottled cystic appearance. Dr/AHMED ESAWY
Congenital Nephrotic Syndrome
• Congenital nephrotic syndrome of the Finnish type is
an autosomal recessive disease with severe
proteinuria and secondary hypoalbuminemia. The
reported incidence is 1/10,000 livebirths. The kidneys
are enlarged and contain diffuse punctuate
echogenicities consistent with microcysts due to
dilatation of the proximal convoluted tubules. The
placenta is always enlarged, weighing > 25% of the
birthweight. The in-utero proteinuria can result in non-
immune hydrops
• May be secondary to congenital infection
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Transvaginal US findings of urinary bladder and kidneys of a 12-week fetus.
The coronal (A) and axial (B) images show small round bladder (arrow) in the
center of pelvic cavity and both kidneys (small arrows).
Dr/AHMED ESAWY
US and MRI findings of normal
urinary bladder and kidneys of a 30-
week fetus.
(A) The bladder wall is thin and the
internal content is anechoic fluid. (B)
CDUS reveals bilateral umbilical
arteries (arrows) running around the
urinary bladder. (C & D) Fetal MRI
shows well-defined fetal urinary
bladder (arrows) and surrounding
structures.
• It is also useful to visualize the perivesical arteries to
delineate the bladder.
• Even in severe IUGR the bladder will usually contain a
little urine
• When the bladder is not identified in the presence of
normal liquor volume a diagnosis of bladder exstrophy.
should be considered.
Dr/AHMED ESAWY
• The use of fetal perivesical arteries and renal
artery Doppler can be of use .
• Failure to identify the renal arteries with
color Doppler implies a diagnosis of renal
agenesis .
Dr/AHMED ESAWY
Invisible urinary bladder
Dr/AHMED ESAWY
Dr/AHMED ESAWY
When one fails persistently to demonstrate the fetal
urinary bladder during an examination lasting 30
minutes or longer, urinary tract pathology should be
highly suspected.
Invisible fetal urinary bladder with normal amniotic
fluid is common condition due to fetal urination.
In most cases the bladder is filled within 30 minutes.
Invisible urinary bladder during
2nd & 3rd trimester
Dr/AHMED ESAWY
Invisible bladder during the 2nd & 3rd trimester usually needs careful radiological
evaluation of the fetus and estimation of amniotic fluid volume.
Invisible bladder with severely decreased amniotic fluid means impairment of urine
production caused by
bilateral renal agenesis,
bilateral MCDK,
autosomal recessive
polycystic kidney,
severe intrauterine growth restriction
syndromes such as sirenomelia and Meckel-Gruber syndrome.
Invisible bladder with normal amniotic fluid may be associated with bladder exstrophy
Sirenomelia
Dr/AHMED ESAWY
is a rare and invariably lethal congenital anomaly characterized
by fusion of the lower extremities and the presence of other
severe anomalies, such as bilateral renal agenesis.
Fusion of the lower extremities in sirenomelia ranges from
membranous
fusion of the soft tissue to total fusion of the lower legs with one
midline femur.
Dr/AHMED ESAWY
Sirenomelia in a 20-week fetus. (A) The coronal CDUS image shows no renal
arterial flow. Both kidneys and bladder are invisible. There is no amniotic fluid around th
fetus. (B) Fetal MRI shows single extended femur (arrow). The bladder is not visualized
and there is no amniotic fluid around the fetus. (C) Postmortem radiograph shows a
single lower extremity and hypoplastic pelvic bone.
Meckel-Gruber Syndrome
Dr/AHMED ESAWY
• Rare, lethal, ciliopathic, genetic disorder, characterized by renal cystic
dysplasia, central nervous system malformations, polydactyly, hepatic
developmental defects, and pulmonary hypoplasia due to oligohydramnios
US findings of Meckel-Gruber Syndrome. (A) Coronal image of a fetal abdomen
shows multiple cysts in both kidneys, suggesting multicystic dysplasia. The amount of
amniotic fluid is markedly decreased. (B) Axial image of fetal head shows a protruding
cyst (arrow) in the occipital area with disruption of occipital bone, suggesting occipital
encephalocele.
Dr/AHMED ESAWY
US findings of bilateral renal agenesis. (A) The axial image of the fetal abdomen
shows anhydramnios. Both kidneys are invisible and the bowel loops fills the renal
fossa (arrow).
(B) The coronal image shows lying-down adrenal gland at the paraspinal area
(small arrow). The urinary bladder was invisible during the US exam.
Dr/AHMED ESAWY
Both kidneys (arrows) are enlarged with increased
parenchymal echo and collapsed renal pelvis. There is
no amniotic fluid around fetus
Dr/AHMED ESAWY
US findings of bilateral MCDK. (A) There are multiple variable size cysts in both
kidneys without normal parenchymal echo. The amount of amniotic fluid is markedly
decreased. (B) On the power Doppler US, there is no renal arterial flow from the aorta.
Dr/AHMED ESAWY
US findings of unilateral renal agenesis. (A) The left kidney is not visualized. The
left adrenal gland (small arrow) is located in the paraspinal area. (B) The left renal artery
flow is not visualized on the power Doppler US. (C) Urinary bladder is normal. There is a
small round cyst (arrow) in the left lower portion, which is confirmed seminal vesicle
cyst.(D) Postnatal US reveals small cystic lesion at the left posterior portion of the
bladder,suggesting seminal vesicle cyst
MCDK DILATED URETER
Dr/AHMED ESAWY
Obstructive Uropathy
3 levels:-
1) Pelviureteric “PUJO” (most favorable):
- Intermittent adequate amniotic fluid +
bladder fill and empty.
- Enlarged renal pelvis (bilat).
- Cortical thickness (induce labor)
2)Urethral agenesis or stenosis:
- Absene of amniotic fluid.
- Small kidneys & dysplastic (Fatal)Dr/AHMED ESAWY
3)Inbetween these two extremes:
Post ureth Valves
intermittent ureth Obstruction + distended bladder.
• Oligohydramnios.
• Male foetuses (pulm.hypoplasia, Neonatal death).
• Fetal therapy ( intermittent catheterization)
• vesico amniotic shent
Obstructive Uropathy
Dr/AHMED ESAWY
Dr/AHMED ESAWY
Post ureth Valves
Upper tract dilation seen in association with ureteric dilation should
suggest Vesico-ureteric junction obstruction or reflux ,
Dr/AHMED ESAWY
outflow obstruction
Dr/AHMED ESAWY
• Difference between obstruction and reflux is
thick trabeculated UB wall in obstruction
Dr/AHMED ESAWY
Right sided ureterectasis (arrow) and hydronephrosis
due to primary reflux in a fetus of 28 weeks maturity
Dr/AHMED ESAWY
Abnormalities of the fetal
bladder
Dr/AHMED ESAWY
Fetal megacystis
• refers to the presence of an unusually large
bladder in a fetus. It is generally defined as a
• bladder diameter >7 mm in the first
trimester reference required
• bladder diameter >30 mm in the second
trimester reference required
• bladder diameter >60 mm in the third
trimester reference required
Dr/AHMED ESAWY
Megacystis
• In the chromosomally normal group, if the bladder length was
greater than 15 mm, the condition was invariably associated
with progressive obstructive uropathy
• However, if the bladder length was 7–15 mm, there was
spontaneous resolution of the megacystis by 20 weeks in 90%
of the cases.
• In 10%, there was enlargement of the megacystis and/or the
development of echogenic kidneys. Follow-up US is necessary
to correctly interpret the significance of megacystis detected
in the first trimester
Dr/AHMED ESAWY
large urinary bladder during 2nd
& 3rd trimester
Dr/AHMED ESAWY
usually associated with bladder outlet obstruction such as
Posterior urethral valve
Urethral atresia
Cloacal malformation .
Dr/AHMED ESAWY
US findings of posterior urethral valve of a 30-week fetus. (A) The coronal image
shows dilated bladder and posterior urethra (arrow), forming "key-hole" appearance.
The amniotic fluid is markedly decreased. (B) The bladder wall is thick with multiple
trabeculation. Pelvis and calyces of both kidneys are dilated
Large UB common associated
anomalies
• posterior urethral valves
• chromosomal anomalies
– on a first trimester scan (10-14 weeks)
• if the longitudinal bladder diameter of 7-15 mm there is a risk of a
chromosomal defects is estimated at ~25%
• if the bladder diameter is >15 mm the risk of chromosomal defects is
estimated at ~10%
• oligohydramnios
• megacystis microcolon intestinal hypoperistalsis (MMIH)
syndrome (Berdon syndrome)
• megacystis megaureter syndrome
• prune belly syndrome
Dr/AHMED ESAWY
MEGACYSTIS
Dr/AHMED ESAWY
Megacystis–
microcolon–
intestinal
hypoperistalsis
syndrome
(MMIHS)
Dr/AHMED ESAWY
• Megacystis at 12 weeks gestation. Sagittal transabdominal US image
of a male fetus shows a distended thick-walled bladder (arrow),
which is 13 mm long. It did not empty during a 30-minute
examination. There was no hydronephrosis. At subsequent US
examinations, the bladder remained large, but it was seen to empty
partially on several occasions when the observation was prolonged
(up to 1 hour). Dr/AHMED ESAWY
The normal size of the urinary bladder During
the 1st trimester is smaller than 7mm
Dr/AHMED ESAWY
Megacystis during the 1st trimester
usually associated with bladder outlet
obstruction
such as posterior urethral valve (A.)
and cloacal malformation (Figure B).
• Posterior urethral valve occurs in the
male only.
• Almost cloacal malformation occurs
in female.
Dr/AHMED ESAWY
US findings of the posterior urethral valve in a fetus at 11th and 13th weeks. (A
& B) US at 11 week shows markedly dilated fetal urinary bladder. (C) The follow-up US
at 13 week demonstrates more dilated urinary bladder. (D) The echogenecity of both
kidneys (arrows) is increased and the renal pelvis are dilated, suggesting cystic
dysplasia of both kidneys
• Prenatal ultrasound of a male patient with
posterior urethral valves. The thick-walled
bladder and dilated posterior urethra (keyhole
sign) are clearly visible.
Dr/AHMED ESAWY
1) keyhole urinary bladder (an overdistended
bladder with the dilated prostatic urethra
forming a keyhole like appearance). 2)
hydronephrotic kidneys (bilateral) with
thinning of the renal cortex. These ultrasound
images suggest fetal lower urinary tract
obstruction PUV
Dr/AHMED ESAWY
Posterior urethral valves
Dr/AHMED ESAWY
Dr/AHMED ESAWY
US findings of the posterior urethral
valve in a fetus at 11th and 13th
weeks. (A & B) US at 11 week
shows markedly dilated fetal urinary
bladder. (C) The follow-up US
at 13 week demonstrates more
dilated urinary bladder.
(D) The echogenecity of both
kidneys (arrows) is increased and
the renal pelvis are dilated,
suggesting cystic dysplasia
of both kidneys.
Urethral atresia Ultrasound at sixteen
weeks A shows a distended thick walled
urinary bladder with oligohydramnios
Ultrasound at 24 weeks
shows a distended urinary bladder entirely
filling the fetal abdomen
Dr/AHMED ESAWY
Posterior urethral valves
dilated thick walled urinary bladder in
a male fetus Suprapubic sonogram
the same case showing dilated
posterior urethra white arrow
Dr/AHMED ESAWY
• Gray-scale ultrasound images of
a 34-week male fetus with
posterior urethral valves at rest
(a) and during micturition
• (b). The posterior urethra is
seen ballooned out during
micturition (arrows). The
urinary bladder (BL) and the
urinary stream (arrowhead) are
indicated.
Dr/AHMED ESAWY
• Gray-scale ultrasound images of a
male fetus with posterior urethral
valves at 25 weeks resting (a) and
during micturition (b).
The ballooning out of the
posterior urethra (arrow) during
micturition is evident. The
arrowhead points to the dilated
lower ureter connected to a
ureterocele. (
Dr/AHMED ESAWY
Dr/AHMED ESAWY
US and photography findings of the cloacal malformation of a fetus at 12th
weeks. (A & B) Coronal and axial image show a large round cyst in the fetal pelvis
abdomen. (C) CDUS reveals the umbilical cord connected to the anterior part of the
cystic mass, suggesting this cystic mass is the dilated bladder.
Dr/AHMED ESAWY
US Findings of cloacal malformation of a 35-week fetus. (A) Coronal image
shows markedly dilated two cystic lesions with apparent "fold" (arrow), suggesting
communication of bladder and female genital tract. (B) Renal pelvis are dilated and
the amniotic fluid is markedly decreased.
Cloacal exstrophy at 14 weeks gestation.Sagittal transabdominal US image of the fetal lower
abdomen shows an irregular mass (arrows) arising from the anterior abdominal wall. The
bladder is not seen. There is mild hydronephrosis (H).
Dr/AHMED ESAWY
Dr/AHMED ESAWY
US findings of bladder exstrophy of a 23-week fetus. (A) Prenatal US of lower
abdomen shows protruding mass (arrow) in the lower anterior abdomen instead of
normal urinary bladder. The amniotic fluid volume is normal. (B) Coronal image of fetal
abdomen reveals a normal kidney.
Urethral obstruction
Dr/AHMED ESAWY
Dr/AHMED ESAWY
(A) Coronal image of the fetal abdomen shows multiple cystic changes of both
kidneys and perirenal urine collection (arrow) in the left side. Both ureters are dilated
(small arrows). Amniotic fluid is invisible. (B) Sagittal image shows markedly dilated
urinary bladder. There is beaking of the upper urethra (small arrow), suggesting
urethral atresia.
urethral atresia.
Cloacal exstrophy at 14 weeks gestation.Sagittal transabdominal US image of the fetal lower
abdomen shows an irregular mass (arrows) arising from the anterior abdominal wall. The
bladder is not seen. There is mild hydronephrosis (H).
Dr/AHMED ESAWY
Cloacal exstrophy
Dr/AHMED ESAWY
US findings of bladder exstrophy of a 23-week fetus. (A) Prenatal US of lower
abdomen shows protruding mass (arrow) in the lower anterior abdomen instead of
normal urinary bladder. The amniotic fluid volume is normal. (B) Coronal image of fetal
abdomen reveals a normal kidney.
bladder exstrophy
• Sonography of the fetal
urinary bladder, shows a
sac like structure (arrow)
in the region of the distal
end of the fetal left
ureter.. Ureterocele
Dr/AHMED ESAWY
Dr/AHMED ESAWY
US Findings of ureterocele with renal duplication of a 27-week fetus. (A)
Coronal image of fetal pelvic cavity shows a small cyst in the urinary bladder,
suggesting ureterocele (small arrow). (B) Coronal image of the right kidney shows
renal duplication and dilatation of the upper pole pelvis. The upper moiety ureter is also
dilated (arrow).
Dr/AHMED ESAWY
• a) Midline sagittal gray-scale ultrasound
image, in a 33-week male fetus with a
normal urethra, through the fetal pelvis,
perineum and penis during micturition,
showing the urinary bladder (BL), the
slightly fluid-distended posterior and
anterior urethra (arrows) and the
urinary stream from the tip of the penis
(arrowhead).
• (b) Color Doppler ultrasound image of the
same fetus showing the urinary stream
• from the tip of the penis (arrow).
Dr/AHMED ESAWY
• Gray-scale (a) and
color Doppler (b)
ultrasound images
of micturition in a
27-week female
fetus, showing the
urinary bladder (BL),
the urethra (arrow)
and the urinary
stream (arrowhead).
Dr/AHMED ESAWY
• Color Doppler
ultrasound image of a
31-week male fetus
with hypospadias
showing the flow in
the urethra (arrow)
and the ventral jet of
urinary stream
(arrowhead).
Dr/AHMED ESAWY
Complete duplication of urinary bladder and urethra
prenatal sonographic features
Dr/AHMED ESAWY
Serial coronal ultrasound images of the fetal pelvis
showing two urinary bladders. (a) Both bladders are visible.
(b) 5 min after (a) showing partially contracted left bladder. (c) 16 s
after (b) showing that left bladder has completely emptied and right
bladder is even more distended.
Dr/AHMED ESAWY
Postnatal transverse ultrasound
image of the pelvis of the
newborn showing two bladders
(BL1, BL2) and two uteri (U1, U2)
posterior to them.
Dr/AHMED ESAWY
Prenatal ultrasound image of the fetal perineum showing the two vulvae (arrows).
Dr/AHMED ESAWY
US findings of ovarian cyst of a 32-week fetus. (A) Sagittal image shows a large cystic
lesion at the superior portion of the urinary bladder (UB). The internal fluid
is anechoic. (B) The cyst is located in the left side and confirmed as left ovarian cyst
after birth.
Cyst around urinary bladder
Dr/AHMED ESAWY
US findings of hemorrhagic ovarian cyst of a 32-week fetus. (A) Axial image of
the fetal abdomen shows a heterogeneous echogenic mass (arrow) at the left posterior
portion of the bladder. (B) Postnatal US image reveals a cyst with echogenic debris,
confirmed as hemorrhagic ovarian cyst.
Dr/AHMED ESAWY
Prenatal US findings of pelvic kidney with multiple cystic dysplasia. (A) On the
coronal image the right kidney is not visualized and the right adrenal gland (small
arrow) is "lying down" in the right paraspinal area. (B) There are multiple variable
sized cysts at the superior portion of urinary bladder (arrow) suggesting multicystic
dysplastic change of ectopic right kidney.

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10-Fetal urinary tract Dr Ahmed Esawy

  • 2. Dr. Ahmed Esawy MBBS M.Sc MD Dr/AHMED ESAWY
  • 4. • Normal kidneys at 13 weeks gestation. Coronal transvaginal US image of the fetal abdomen shows normal kidneys (cursors). Fluid is seen in the renal pelves (arrowheads). Dr/AHMED ESAWY
  • 5. Normal Renal Echogenicity • The normal echogenicity of the fetal kidney is equal to that of the liver (Figs. A and B). Since this is a subjective assessment, it may have significant intra and interobserver variation. Generally, fetal renal echogenicity refers to the appearance of the entire kidney rather than to an isolated component of the kidney, i.e. the cortex, medulla or pyramids. Dr/AHMED ESAWY
  • 6. • NORMAL ECHOGENICITY • 38 WEEKS NORMAL ECHOGENICITY Dr/AHMED ESAWY
  • 7. Physiologic Echogenic Kidneys • Bilateral echogenic kidneys in normal fetus Dr/AHMED ESAWY
  • 8. Echogenic fetal kidneys. • Physiologic variation • Intrinsic renal disease – Autosomal dominant polycystic renal disease – Autosomal recessive polycystic renal disease • Obstructive uropathy • Aneuploidy – Trisomy 13 – Trisomy 18 • Infection – Cytomegalovirus – Candida Dr/AHMED ESAWY
  • 9. Echogenic fetal kidneys. • Overgrowth Syndromes – Beckwith-Wiedemann – Perlman – Simpson-Golabi-Behmael • Meckel-Gruber • Bardet-Biedl syndrome • Renal vein thrombosis • Congenital nephrotic syndrom Dr/AHMED ESAWY
  • 10. Causes of Fetal Hydronephrosis Physiologic: • Urine production 4-6x greater before delivery • Increased compliance of fetal ureter • Partial/transient obstructions associated with development Dr/AHMED ESAWY
  • 11. HYDRONEPHROSIS The most common causes are: UPJ obstruction Duplicated collecting system with obstruction of upper pole Bladder outlet obstruction Male PUV (thick bladder wall) Prune-belly syndrome (normal bladder wall) Females and males Caudal regression syndrome Megacystis-microcolon-intestinal hypoperistalsis Ureteral agenesis Maternal drugs Ectopic ureterocele Dr/AHMED ESAWY
  • 12. Grades of Fetal Hydronephrosis Dr/AHMED ESAWY
  • 13. Urinary Obstruction • Assess bladder filling / emptying – AFI – Direct visualization • Physiological Reflux – Nml 2nd and 3rd trimester Dr/AHMED ESAWY
  • 18. DD .Prominent extra-renal pelvis Dr/AHMED ESAWY
  • 19. left renal pelvicalyceal dilatation (left image) and hyperechogenicity of both kidneys without corticomedullary differentiation Dr/AHMED ESAWY
  • 20. • Axial renal images, 24 weeks GA showing pelvicalyceal dilatation on the left. • Coronal section at 27 weeks GA with right renal measurement (bottom of image) and left dilated renal pelvis measurement Dr/AHMED ESAWY
  • 21. Parasagittal images at 27 weeks with measurements (left kidney is on the left image). Parasagittal images at 29 weeks GA; left kidney is on the left image. Dr/AHMED ESAWY
  • 22. Mild bil.pyelectasis Not more than 4mm Dr/AHMED ESAWY
  • 23. Measuring Renal Dilatation Anteroposterior diameter (APD): Size of the renal pelvis measured in the anterior →posterior direction on a transverse view through the abdomen. •For a diagnosis of hydronephrosis, APD must be greater than: 6mm at < 20 weeks GA 8 mm at 20-30 weeks GA 10mm at > 30 weeks GA Dr/AHMED ESAWY
  • 24. • These ultrasound images reveal unilateral hydronephrosis of the left fetal kidney. • RPD (renal pelvic A-P diameter) is more than 10mm (after 20 weeks gestational age). If less than 20 weeks, more than 6mm. is abnormal. Dr/AHMED ESAWY
  • 25. Protocol for post natal follow up of fetal renal pyelectasis • Less than 5mm ---- Normal –no follow up • From (5-10) mild and need post natal scan after two weeks ,if normal ,the imaging either stop or one additional scan between three and six months of age. • More than 10 significant pyelectasis . follow up done before discharge (day 1or 2 of life). • if scan looked normal follow up done at 3,6 months and one year if scan remain normal one additional scan at 5 year.Dr/AHMED ESAWY
  • 26. • if abnormalities detected voiding cystourethrography should be done at 48 hours in suspected outflow obstruction and surgery must performed before discharge . • Antibiotic prophylaxis should be given and continued until reflux ruled out especially in case of unilateral hydronephrosis. . • Other cases of obstructive uropathy can be closely followed in the 1st two years every 3 months using US and Reno gram .surgery indicated only if renal function deteriorated -hydronephrosis increased or in febrile infection . Dr/AHMED ESAWY
  • 27. anteroposterior measurement of renal cortical thinning due to pelvicalyceal dilatation. Dr/AHMED ESAWY
  • 29. Left kidney, sagittal view Right kidney, sagittal view mild hydronephrosis on the right with central dilatation of 8-9 mm and severe hydronephrosis on the left with a large extrarenal pelvis measuring 2.8 cm Dr/AHMED ESAWY
  • 32. • Difference between obstruction and reflux is thick trabeculated UB wall in obstruction Dr/AHMED ESAWY
  • 34. Renal Cystic Disease • Cystic Renal Dysplasia – Posterior urethral valves – Hydronephrosis – Increased parenchymal echogenicity – Cysts Vary Size • Multicystic Dysplastic Kidney – Multiple non-communicating cysts Vary in size - Multiple or single echo- free spaces. • - Identification should start after (22ws). • - If bilateral (bladder not demonstrated- amniotic • fluid vol decreased or absent). • - Meckel’s syndrome ( encephalocele- cleft palate- • polydacty + cong. Ht disease), may be associated. – Non-functioning renal tissue – If bilateral • Oligohydraminos • Not compatible w/ life Dr/AHMED ESAWY
  • 35. Renal Cystic Disease • ARPCKDz / Infantile PCKDz – Massively big kidneys bilaterally – Echogenic – Sonolucent rim – Discrete cysts are not seen – Cysts ( Microscepic to macroscepic + oligohydramnios) • ADPCKDz – Enlarged kidneys – Non-sonolucent rim – Discrete cysts +/- seen – Cysts + normal amniotic fluid. – Symptoms silent till 5 th decade Dr/AHMED ESAWY
  • 36. Differential Diagnosis of Bilateral Multiple Cysts Dr/AHMED ESAWY
  • 39. MULTICYSTIC DYSPLASTIC KIDNEY DISEASE (POTTER TYPE II) Dr/AHMED ESAWY
  • 46. Multicystic Dyplastic Kidney with a large cyst, increasing in size Dr/AHMED ESAWY
  • 47. Bilateral Multicystic Dyplastic Kidneys Dr/AHMED ESAWY
  • 49. Multicystic Dyplasia with multiple non- communicating cysts of varying sizes Dr/AHMED ESAWY
  • 50. • normal kidney (calipers) with typical corticomedullary differentiation. Dr/AHMED ESAWY
  • 51. • left kidney with clear corticomedullary differentiation. Dr/AHMED ESAWY
  • 53. • autosomal recessive polycystic kidney disease. • markedly enlarged (8 cm) • hyperechoic • no corticomedullary differentiation. • Oligohydramnios is present. • Ch = fetal chest Dr/AHMED ESAWY
  • 54. • autosomal recessive polycystic kidney disease Dr/AHMED ESAWY
  • 55. A small, slightly echogenic kidney (arrows) seen with the normal contra lateral one Polycystic echogenic Dr/AHMED ESAWY
  • 57. • Sonography of the fetal abdomen in this 2nd trimester fetus reveals- • 1) bilateral grossly enlarged fetal kidneys, almost filling the entire abdomen • 2) markedly hyperechoic fetal kidneys with minute anechoic areas within them. • 3) oligohydramnios • 4) poorly distended urinary bladder. • This ultrasound image is diagnostic of fetal polycystic kidney disease (ARPKD). This image was taken by Dr. Durr-e-Sabih, Pakistan, using a Toshiba Nemio-30 color doppler machine. Dr/AHMED ESAWY
  • 58. • These ultrasound images (another case) also show grossly enlarged, echogenic fetal kidneys with minute cystic lesions (1 to 2 mm.) within them. (typical rosette like pattern) • is typical of ARPCKD. Dr/AHMED ESAWY
  • 59. • Recurrence of glomerulocystic kidney disease. • striking hyperechogenicity of cortex and no corticomedullary differentiation. Renal length is 30 mm. CH = fetal chest Dr/AHMED ESAWY
  • 60. Adult polycystic kidney disease (APKD) enlarged and hyperechogenic with or without multiple cysts. Unlike infantile polycystic kidneys, where there is a loss of the corticomedullary junction, in APKD there is accentuation of this junction. The amniotic fluid volume is either normal or reduced The kidney size is usually smaller than that of the infant polycystic kidneys Dr/AHMED ESAWY
  • 61. Differential diagnosis of Congenital Cystic Disease of Kidney • Isolated renal cysts : (DD: PUJO.) – isolated + not communicating pelvis. • Multicystic kidney (sequlae of obstructive uropthy). Dr/AHMED ESAWY
  • 63. simple renal cyst (left kidney). Dr/AHMED ESAWY
  • 64. Large unilocular cystic mass (arrow) in a 36 weeks mature fetus. It proved to be left hydronephrotic kidney due to UPJ obstruction Dr/AHMED ESAWY
  • 65. Renal Agenesis (potter syndrome) • Absent renal echoes and absent ur bladder (identification of bladder exclude diagnosis of polycystic kid. & renal agenesis). • Oligohydramnios. • Reduced foetal growth rate (BPD & A.C). Dr/AHMED ESAWY
  • 66. • The Potter sequence is a constellation of findings demonstrated postnatally as a consequence of severe, prolonged oligohydramnios in utero. • It consists of : • pulmonary hypoplasia: often severe and incompatible with life • growth restriction (IUGR) • abnormal facies (Potter facies): including – low set ears – flattened nose – wrinkled skin – micrognathia • limb abnormalities: including club feet and contractures Dr/AHMED ESAWY Potter sequence
  • 67. Potter sequence • Although originally associated with renal causes of olighydramnios, the Potter sequence may be seen with severe oligohydramnios of essentially any cause. • Common aetiologies include : • fetal renal insufficiency – bilateral renal agenesis: traditionally known as the classic Potter syndrome – autosomal recessive polycystic kidney disease – posterior urethral valves, and other causes of antenatal renal tract obstruction • premature rupture of membranes (PROM) • twin-twin transfusion Dr/AHMED ESAWY
  • 68. Potter sequence, oligohydramnios sequence Renal agenesis Common Findings • Oligohydramnios, usually of early onset (by 16–18 weeks’ gestation) • Absent kidneys (although the adrenal glands may mimic kidneys; this is a pitfall in the early diagnosis of this condition), • Narrow chest (secondary to compression by the oligohydramnios), appearing later in gestation , Pulmonary hypoplasia Dr/AHMED ESAWY
  • 69. Potter sequence, oligohydramnios sequence Renal agenesis Occasional Findings • Vertebral defects • Complex cardiac defects (14%) • Skeletal anomalies (40%), such as sirenomelia, absent radius, and vertebral anomalies • CNS anomalies (11%), such as ventriculomegaly and neural tube defects • Other anomalies, such as imperforate anus, duodenal atresia, tracheoesophageal fistula, and single umbilical artery Dr/AHMED ESAWY
  • 70. Syndromes and Conditions Associated with Bilateral Renal Agenesis • Caudal regression syndrome • Fraser syndrome • MURCS (müllerian duct aplasia, renal aplasia, cervicothoracic somite dysplasia) association • Sirenomelia • VATER (vertebral defects, anal atresia, tracheoesophageal fistula, esophageal atresia, and radial and renal dysplasias) syndrome Dr/AHMED ESAWY
  • 71. Autosomal recessice polycystic kidney disease ARPCKD POTTER type 1 • Massively enlarged echogenic kidneys Dr/AHMED ESAWY
  • 72. POTTER Type II Type II IIA IIB Multicystic renal dysplasia. Multilocular cystic nephroma. Kidneys of normal / increased size. Kidneys reduced in size. Dr/AHMED ESAWY
  • 74. Multicystic Renal Dysplasia (MCRD) or Potter Type II Dr/AHMED ESAWY • Longitudinal cut of large renal silhouettes with multiple cystic images
  • 75. Multicystic Renal Dysplasia (MCRD) or Potter Type II Dr/AHMED ESAWY Axial cut, at the abdominal level in the posterior paravertebral sector, cystic formations of variable size and anarchic distribution, but mainly peripherical, are visualized.
  • 76. • Color Doppler ultrasound showing the presence of both renal and iliac arteries. Dr/AHMED ESAWY
  • 77. • Left kidney of large size with cystic structures,presented as paraspinal mass. Dr/AHMED ESAWY
  • 78. Adult polycystic kidney disease POTTER Type III Type III Adult polycystic disease of the kidneys. Tuberous sclerosis. Medullary sponge kidney. Dr/AHMED ESAWY
  • 80. Adult polycystic kidney disease • Prenatal diagnosis by ultrasonography is confined to a few case reports and the kidneys have been described as enlarged and hyper- echogenic with or without multiple cysts • accentuation of this junction • suspected when cystic enlarged kidneys are detected in association with a normal amount of amniotic fluid Dr/AHMED ESAWY
  • 81. Dr/AHMED ESAWY This 3rd trimester fetus has brightly echogenic kidneys with accentuation of the cortico- medullary junction and a small bladder with decreased fluid.
  • 82. • the echogenic kidneys but normal bladder and amniotic fluid. Dr/AHMED ESAWY
  • 83. Type IV Small cortical cysts / cystic renal dysplasia secondary toureteropelvic junction obstruction. Dr/AHMED ESAWY
  • 86. • both the renal fossae are not occupied by kidneys, and are occupied by the adrenal glands producing lying adrenal sign. • Postnatal radiograph shows bell-shaped thorax, suggestive of pulmonary hypoplasia Dr/AHMED ESAWY
  • 87. Antenatal ultrasound and MRI findings of Pena–Shokeir syndrome T2W MR coronal (a) and oblique (b) images demonstrate persistent flexion of bilateral wrist and elbow joints with extension of the fingers, partial flexion at knee joint and bilateral club foot Dr/AHMED ESAWY
  • 88. (b) reveals scoliosis, bilateral flexion at the elbows, wrists, and knees. No underlying bony abnormality was seen Dr/AHMED ESAWY
  • 89. Antenatally detected ovarian cyst. a simple cyst (4.5x3.8 cm) located in the left side of the abdomen, bulging through the anterior abdominal wall. Dr/AHMED ESAWY
  • 90. Overgrowth Syndromes • Beckwith-Wiedemann syndrome is characterized by visceromegaly, macroglossia, polyhydramnios and enlarged echogenic kidneys with a loss of corticomedullary differentiation . This overgrowth syndrome also has a variety of possible congenital anomalies, including omphalocele, hemihypertrophy and cardiac anomalies. Dr/AHMED ESAWY ENLARGED ECHOGENIC KIDNEYS WITH BECKWITH-WIEDEMANN SYNDROME
  • 91. • Bardet-Biedl syndrome. • marked enlargement (49 mm between calipers) without corticomedullary differentiation. • B, Ultrasound scan shows postaxial polydactyly. six digits. Dr/AHMED ESAWY
  • 92. • Joubert syndrome. • kidneys appear hyperechoic • without corticomedullary differentiation • cysts at corticomedullary junction.Dr/AHMED ESAWY
  • 93. Meckel-Gruber Syndrome • This is an autosomal recessive disorder characterized by an occipital encephalocele , postaxial polydactyly , and echogenic significantly enlarged kidneys. Of this triad of anomalies Dr/AHMED ESAWY Occipital encephalocele Post axial polydactyl
  • 94. • Meckel-Grüber syndrome. • kidney is enlarged. • Corticomedullary differentiation is unexpectedly evident at this early stage • mottled cystic appearance. Dr/AHMED ESAWY
  • 95. Congenital Nephrotic Syndrome • Congenital nephrotic syndrome of the Finnish type is an autosomal recessive disease with severe proteinuria and secondary hypoalbuminemia. The reported incidence is 1/10,000 livebirths. The kidneys are enlarged and contain diffuse punctuate echogenicities consistent with microcysts due to dilatation of the proximal convoluted tubules. The placenta is always enlarged, weighing > 25% of the birthweight. The in-utero proteinuria can result in non- immune hydrops • May be secondary to congenital infection Dr/AHMED ESAWY
  • 96. Dr/AHMED ESAWY Transvaginal US findings of urinary bladder and kidneys of a 12-week fetus. The coronal (A) and axial (B) images show small round bladder (arrow) in the center of pelvic cavity and both kidneys (small arrows).
  • 97. Dr/AHMED ESAWY US and MRI findings of normal urinary bladder and kidneys of a 30- week fetus. (A) The bladder wall is thin and the internal content is anechoic fluid. (B) CDUS reveals bilateral umbilical arteries (arrows) running around the urinary bladder. (C & D) Fetal MRI shows well-defined fetal urinary bladder (arrows) and surrounding structures.
  • 98. • It is also useful to visualize the perivesical arteries to delineate the bladder. • Even in severe IUGR the bladder will usually contain a little urine • When the bladder is not identified in the presence of normal liquor volume a diagnosis of bladder exstrophy. should be considered. Dr/AHMED ESAWY
  • 99. • The use of fetal perivesical arteries and renal artery Doppler can be of use . • Failure to identify the renal arteries with color Doppler implies a diagnosis of renal agenesis . Dr/AHMED ESAWY
  • 101. Dr/AHMED ESAWY When one fails persistently to demonstrate the fetal urinary bladder during an examination lasting 30 minutes or longer, urinary tract pathology should be highly suspected. Invisible fetal urinary bladder with normal amniotic fluid is common condition due to fetal urination. In most cases the bladder is filled within 30 minutes.
  • 102. Invisible urinary bladder during 2nd & 3rd trimester Dr/AHMED ESAWY Invisible bladder during the 2nd & 3rd trimester usually needs careful radiological evaluation of the fetus and estimation of amniotic fluid volume. Invisible bladder with severely decreased amniotic fluid means impairment of urine production caused by bilateral renal agenesis, bilateral MCDK, autosomal recessive polycystic kidney, severe intrauterine growth restriction syndromes such as sirenomelia and Meckel-Gruber syndrome. Invisible bladder with normal amniotic fluid may be associated with bladder exstrophy
  • 103. Sirenomelia Dr/AHMED ESAWY is a rare and invariably lethal congenital anomaly characterized by fusion of the lower extremities and the presence of other severe anomalies, such as bilateral renal agenesis. Fusion of the lower extremities in sirenomelia ranges from membranous fusion of the soft tissue to total fusion of the lower legs with one midline femur.
  • 104. Dr/AHMED ESAWY Sirenomelia in a 20-week fetus. (A) The coronal CDUS image shows no renal arterial flow. Both kidneys and bladder are invisible. There is no amniotic fluid around th fetus. (B) Fetal MRI shows single extended femur (arrow). The bladder is not visualized and there is no amniotic fluid around the fetus. (C) Postmortem radiograph shows a single lower extremity and hypoplastic pelvic bone.
  • 105. Meckel-Gruber Syndrome Dr/AHMED ESAWY • Rare, lethal, ciliopathic, genetic disorder, characterized by renal cystic dysplasia, central nervous system malformations, polydactyly, hepatic developmental defects, and pulmonary hypoplasia due to oligohydramnios US findings of Meckel-Gruber Syndrome. (A) Coronal image of a fetal abdomen shows multiple cysts in both kidneys, suggesting multicystic dysplasia. The amount of amniotic fluid is markedly decreased. (B) Axial image of fetal head shows a protruding cyst (arrow) in the occipital area with disruption of occipital bone, suggesting occipital encephalocele.
  • 106. Dr/AHMED ESAWY US findings of bilateral renal agenesis. (A) The axial image of the fetal abdomen shows anhydramnios. Both kidneys are invisible and the bowel loops fills the renal fossa (arrow). (B) The coronal image shows lying-down adrenal gland at the paraspinal area (small arrow). The urinary bladder was invisible during the US exam.
  • 107. Dr/AHMED ESAWY Both kidneys (arrows) are enlarged with increased parenchymal echo and collapsed renal pelvis. There is no amniotic fluid around fetus
  • 108. Dr/AHMED ESAWY US findings of bilateral MCDK. (A) There are multiple variable size cysts in both kidneys without normal parenchymal echo. The amount of amniotic fluid is markedly decreased. (B) On the power Doppler US, there is no renal arterial flow from the aorta.
  • 109. Dr/AHMED ESAWY US findings of unilateral renal agenesis. (A) The left kidney is not visualized. The left adrenal gland (small arrow) is located in the paraspinal area. (B) The left renal artery flow is not visualized on the power Doppler US. (C) Urinary bladder is normal. There is a small round cyst (arrow) in the left lower portion, which is confirmed seminal vesicle cyst.(D) Postnatal US reveals small cystic lesion at the left posterior portion of the bladder,suggesting seminal vesicle cyst
  • 111. Obstructive Uropathy 3 levels:- 1) Pelviureteric “PUJO” (most favorable): - Intermittent adequate amniotic fluid + bladder fill and empty. - Enlarged renal pelvis (bilat). - Cortical thickness (induce labor) 2)Urethral agenesis or stenosis: - Absene of amniotic fluid. - Small kidneys & dysplastic (Fatal)Dr/AHMED ESAWY
  • 112. 3)Inbetween these two extremes: Post ureth Valves intermittent ureth Obstruction + distended bladder. • Oligohydramnios. • Male foetuses (pulm.hypoplasia, Neonatal death). • Fetal therapy ( intermittent catheterization) • vesico amniotic shent Obstructive Uropathy Dr/AHMED ESAWY
  • 114. Upper tract dilation seen in association with ureteric dilation should suggest Vesico-ureteric junction obstruction or reflux , Dr/AHMED ESAWY
  • 116. • Difference between obstruction and reflux is thick trabeculated UB wall in obstruction Dr/AHMED ESAWY
  • 117. Right sided ureterectasis (arrow) and hydronephrosis due to primary reflux in a fetus of 28 weeks maturity Dr/AHMED ESAWY
  • 118. Abnormalities of the fetal bladder Dr/AHMED ESAWY
  • 119. Fetal megacystis • refers to the presence of an unusually large bladder in a fetus. It is generally defined as a • bladder diameter >7 mm in the first trimester reference required • bladder diameter >30 mm in the second trimester reference required • bladder diameter >60 mm in the third trimester reference required Dr/AHMED ESAWY
  • 120. Megacystis • In the chromosomally normal group, if the bladder length was greater than 15 mm, the condition was invariably associated with progressive obstructive uropathy • However, if the bladder length was 7–15 mm, there was spontaneous resolution of the megacystis by 20 weeks in 90% of the cases. • In 10%, there was enlargement of the megacystis and/or the development of echogenic kidneys. Follow-up US is necessary to correctly interpret the significance of megacystis detected in the first trimester Dr/AHMED ESAWY
  • 121. large urinary bladder during 2nd & 3rd trimester Dr/AHMED ESAWY usually associated with bladder outlet obstruction such as Posterior urethral valve Urethral atresia Cloacal malformation .
  • 122. Dr/AHMED ESAWY US findings of posterior urethral valve of a 30-week fetus. (A) The coronal image shows dilated bladder and posterior urethra (arrow), forming "key-hole" appearance. The amniotic fluid is markedly decreased. (B) The bladder wall is thick with multiple trabeculation. Pelvis and calyces of both kidneys are dilated
  • 123. Large UB common associated anomalies • posterior urethral valves • chromosomal anomalies – on a first trimester scan (10-14 weeks) • if the longitudinal bladder diameter of 7-15 mm there is a risk of a chromosomal defects is estimated at ~25% • if the bladder diameter is >15 mm the risk of chromosomal defects is estimated at ~10% • oligohydramnios • megacystis microcolon intestinal hypoperistalsis (MMIH) syndrome (Berdon syndrome) • megacystis megaureter syndrome • prune belly syndrome Dr/AHMED ESAWY
  • 126. • Megacystis at 12 weeks gestation. Sagittal transabdominal US image of a male fetus shows a distended thick-walled bladder (arrow), which is 13 mm long. It did not empty during a 30-minute examination. There was no hydronephrosis. At subsequent US examinations, the bladder remained large, but it was seen to empty partially on several occasions when the observation was prolonged (up to 1 hour). Dr/AHMED ESAWY
  • 127. The normal size of the urinary bladder During the 1st trimester is smaller than 7mm Dr/AHMED ESAWY Megacystis during the 1st trimester usually associated with bladder outlet obstruction such as posterior urethral valve (A.) and cloacal malformation (Figure B). • Posterior urethral valve occurs in the male only. • Almost cloacal malformation occurs in female.
  • 128. Dr/AHMED ESAWY US findings of the posterior urethral valve in a fetus at 11th and 13th weeks. (A & B) US at 11 week shows markedly dilated fetal urinary bladder. (C) The follow-up US at 13 week demonstrates more dilated urinary bladder. (D) The echogenecity of both kidneys (arrows) is increased and the renal pelvis are dilated, suggesting cystic dysplasia of both kidneys
  • 129. • Prenatal ultrasound of a male patient with posterior urethral valves. The thick-walled bladder and dilated posterior urethra (keyhole sign) are clearly visible. Dr/AHMED ESAWY
  • 130. 1) keyhole urinary bladder (an overdistended bladder with the dilated prostatic urethra forming a keyhole like appearance). 2) hydronephrotic kidneys (bilateral) with thinning of the renal cortex. These ultrasound images suggest fetal lower urinary tract obstruction PUV Dr/AHMED ESAWY
  • 132. Dr/AHMED ESAWY US findings of the posterior urethral valve in a fetus at 11th and 13th weeks. (A & B) US at 11 week shows markedly dilated fetal urinary bladder. (C) The follow-up US at 13 week demonstrates more dilated urinary bladder. (D) The echogenecity of both kidneys (arrows) is increased and the renal pelvis are dilated, suggesting cystic dysplasia of both kidneys.
  • 133. Urethral atresia Ultrasound at sixteen weeks A shows a distended thick walled urinary bladder with oligohydramnios Ultrasound at 24 weeks shows a distended urinary bladder entirely filling the fetal abdomen Dr/AHMED ESAWY
  • 134. Posterior urethral valves dilated thick walled urinary bladder in a male fetus Suprapubic sonogram the same case showing dilated posterior urethra white arrow Dr/AHMED ESAWY
  • 135. • Gray-scale ultrasound images of a 34-week male fetus with posterior urethral valves at rest (a) and during micturition • (b). The posterior urethra is seen ballooned out during micturition (arrows). The urinary bladder (BL) and the urinary stream (arrowhead) are indicated. Dr/AHMED ESAWY
  • 136. • Gray-scale ultrasound images of a male fetus with posterior urethral valves at 25 weeks resting (a) and during micturition (b). The ballooning out of the posterior urethra (arrow) during micturition is evident. The arrowhead points to the dilated lower ureter connected to a ureterocele. ( Dr/AHMED ESAWY
  • 137. Dr/AHMED ESAWY US and photography findings of the cloacal malformation of a fetus at 12th weeks. (A & B) Coronal and axial image show a large round cyst in the fetal pelvis abdomen. (C) CDUS reveals the umbilical cord connected to the anterior part of the cystic mass, suggesting this cystic mass is the dilated bladder.
  • 138. Dr/AHMED ESAWY US Findings of cloacal malformation of a 35-week fetus. (A) Coronal image shows markedly dilated two cystic lesions with apparent "fold" (arrow), suggesting communication of bladder and female genital tract. (B) Renal pelvis are dilated and the amniotic fluid is markedly decreased.
  • 139. Cloacal exstrophy at 14 weeks gestation.Sagittal transabdominal US image of the fetal lower abdomen shows an irregular mass (arrows) arising from the anterior abdominal wall. The bladder is not seen. There is mild hydronephrosis (H). Dr/AHMED ESAWY
  • 140. Dr/AHMED ESAWY US findings of bladder exstrophy of a 23-week fetus. (A) Prenatal US of lower abdomen shows protruding mass (arrow) in the lower anterior abdomen instead of normal urinary bladder. The amniotic fluid volume is normal. (B) Coronal image of fetal abdomen reveals a normal kidney.
  • 142. Dr/AHMED ESAWY (A) Coronal image of the fetal abdomen shows multiple cystic changes of both kidneys and perirenal urine collection (arrow) in the left side. Both ureters are dilated (small arrows). Amniotic fluid is invisible. (B) Sagittal image shows markedly dilated urinary bladder. There is beaking of the upper urethra (small arrow), suggesting urethral atresia. urethral atresia.
  • 143. Cloacal exstrophy at 14 weeks gestation.Sagittal transabdominal US image of the fetal lower abdomen shows an irregular mass (arrows) arising from the anterior abdominal wall. The bladder is not seen. There is mild hydronephrosis (H). Dr/AHMED ESAWY Cloacal exstrophy
  • 144. Dr/AHMED ESAWY US findings of bladder exstrophy of a 23-week fetus. (A) Prenatal US of lower abdomen shows protruding mass (arrow) in the lower anterior abdomen instead of normal urinary bladder. The amniotic fluid volume is normal. (B) Coronal image of fetal abdomen reveals a normal kidney. bladder exstrophy
  • 145. • Sonography of the fetal urinary bladder, shows a sac like structure (arrow) in the region of the distal end of the fetal left ureter.. Ureterocele Dr/AHMED ESAWY
  • 146. Dr/AHMED ESAWY US Findings of ureterocele with renal duplication of a 27-week fetus. (A) Coronal image of fetal pelvic cavity shows a small cyst in the urinary bladder, suggesting ureterocele (small arrow). (B) Coronal image of the right kidney shows renal duplication and dilatation of the upper pole pelvis. The upper moiety ureter is also dilated (arrow).
  • 148. • a) Midline sagittal gray-scale ultrasound image, in a 33-week male fetus with a normal urethra, through the fetal pelvis, perineum and penis during micturition, showing the urinary bladder (BL), the slightly fluid-distended posterior and anterior urethra (arrows) and the urinary stream from the tip of the penis (arrowhead). • (b) Color Doppler ultrasound image of the same fetus showing the urinary stream • from the tip of the penis (arrow). Dr/AHMED ESAWY
  • 149. • Gray-scale (a) and color Doppler (b) ultrasound images of micturition in a 27-week female fetus, showing the urinary bladder (BL), the urethra (arrow) and the urinary stream (arrowhead). Dr/AHMED ESAWY
  • 150. • Color Doppler ultrasound image of a 31-week male fetus with hypospadias showing the flow in the urethra (arrow) and the ventral jet of urinary stream (arrowhead). Dr/AHMED ESAWY
  • 151. Complete duplication of urinary bladder and urethra prenatal sonographic features Dr/AHMED ESAWY Serial coronal ultrasound images of the fetal pelvis showing two urinary bladders. (a) Both bladders are visible. (b) 5 min after (a) showing partially contracted left bladder. (c) 16 s after (b) showing that left bladder has completely emptied and right bladder is even more distended.
  • 152. Dr/AHMED ESAWY Postnatal transverse ultrasound image of the pelvis of the newborn showing two bladders (BL1, BL2) and two uteri (U1, U2) posterior to them.
  • 153. Dr/AHMED ESAWY Prenatal ultrasound image of the fetal perineum showing the two vulvae (arrows).
  • 154. Dr/AHMED ESAWY US findings of ovarian cyst of a 32-week fetus. (A) Sagittal image shows a large cystic lesion at the superior portion of the urinary bladder (UB). The internal fluid is anechoic. (B) The cyst is located in the left side and confirmed as left ovarian cyst after birth. Cyst around urinary bladder
  • 155. Dr/AHMED ESAWY US findings of hemorrhagic ovarian cyst of a 32-week fetus. (A) Axial image of the fetal abdomen shows a heterogeneous echogenic mass (arrow) at the left posterior portion of the bladder. (B) Postnatal US image reveals a cyst with echogenic debris, confirmed as hemorrhagic ovarian cyst.
  • 156. Dr/AHMED ESAWY Prenatal US findings of pelvic kidney with multiple cystic dysplasia. (A) On the coronal image the right kidney is not visualized and the right adrenal gland (small arrow) is "lying down" in the right paraspinal area. (B) There are multiple variable sized cysts at the superior portion of urinary bladder (arrow) suggesting multicystic dysplastic change of ectopic right kidney.