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Development and congenital anomalies
of urogenital system

Moderator : Dr. (Mrs) Nabanita Deka
Asst. Professor
Presenter : Dr. Jayeta Choudhury
Department of Radiology
Gauhati Medical College
Functionally, the urogenital system can be divided
into two entirely different components: the urinary
system and the genital system.

Embryologically and anatomically, however, they are
intimately interwoven.

Both develop from a common mesodermal ridge
(intermediate mesoderm) and open into a common
channel the cloaca.
“Ontogeny Recapitulates Phylogeny”
Ernst Haeckel 1860
 Ontogeny is the development of
the individual

 Phylogeny is the evolution of
the species
 So this is the idea that during
development an organism (or an
organ) goes through the same
stages as during their evolution.
The kidneys are a perfect example.
URINARY SYSTEM : Kidney Systems
• Three slightly overlapping kidney systems are formed in a cranial-tocaudal sequence during intrauterine life in humans: the pronephros,
mesonephros, and metanephros.
PRONEPHROS
• Beginning of the 4th wk (21days).

•The pronephros is represented by 7 to
10 solid cell groups in the cervical
region .
•Their excretory units: are called
nephrotomes.
•By the end of the fourth week, all
indications of the pronephric system
regress.
MESONEPHROS
•Appear late in the 4th wk
• From the thoracic and lumbar
seg of intermediate mesoderm.
•They function as interim
kidneys for approximately 4 wks.
• Excretory tubules form their
basic unit which open into
mesonephric ducts.
•The mesonephric ducts open
into the cloaca.
METANEPHROS: THE DEFINITIVE KIDNEY
•Appears in the 5th wk.
•Develops from sacral segments of the intermediate mesoderm .
•However, the development of the duct system differs from that of the other
kidney systems.

COLLECTING SYSTEM
Collecting ducts of the permanent kidney develop from the URETERIC BUD, an
outgrowth of the mesonephric duct close to its entrance to the cloaca .
The bud penetrates the metanephric tissue, which is moulded over its distal
end as a cap .
Subsequently, the bud dilates, forming the primitive renal pelvis, and splits and
subsequently divides and sub divides to finally give rise to approximately 1 to 3
million collecting tubules.
Hence, the kidney develops from two sources: (a) metanephric

excretory units and (b) the ureteric
bud, which gives rise to the collecting system. At birth, the
mesoderm, which provides

kidneys have a lobulated appearance, but the lobulation disappears
during infancy .
POSITIONAL CHANGES OF THE KIDNEYS

• Initially : close/ pelvic / anterior to the sacrum .
• Gradually come to lie in the abdomen and move farther apart .
• Attain their adult position by 9th wk. This relative ascent results
mainly from the growth of the embryo's body caudal to the kidneys .
• Initially the hilum : faces ventrally; however, as the kidney relocates
(ascends), it rotates medially almost 90 degrees.
• By the 9th week, the hilum is directed anteromedially .
Development of Bladder and Urethra
urogenital
sinus anteriorly

During the 4th to 7th weeks of development,
CLOACA

anal canal
posteriorly

TIP OF
UROGENITAL
SEPTUM

PERINEAL
BODY
UROGENITAL
SINUS

CRANIAL
VESICAL PART
MOST OF THE
BLADDER

MIDDLE
PELVIC PART

* THE BLADDER NECK
* THE PROSTATIC PART OF
URETHRA IN MALES
* THE ENTIRE URETHRA IN
FEMALES

CAUDAL
PHALLIC PART

REST OF THE
MALE URETHRA
Initially, the bladder is continuous with the
allantois, but when the lumen of the allantois
is obliterated, a thick fibrous cord, the
urachus, remains and connects the apex of
the bladder with the umbilicus . In the adult,
it forms the median umbilical ligament.
FORMATION
OF THE
TRIGONE OF
THE BLADDER

CAUDAL
PORTIONS OF
THE
MESONEPHRIC
DUCTS

ABSORBED
INTO THE WALL
OF THE
URINARY
BLADDER
GENITAL SYSTEM

•Sex differentiation is a complex process that involves
many genes.
•The key to sexual dimorphism is the Y chromosome,
which contains the testis-determining gene called
the SRY (sex-determining region on Y) gene in its
short arm (Yp11).
Indifferent embryo
• Weeks 1-6 sexually indifferent or undifferentiated
stage
• Week 7 begins phenotypic sexual differentiation.
• The sex of the fetus may be discerned by
ultrasound as early as 12 weeks gestation. However,
it can be determined with 95-100% accuracy only
at or after 20wks.
At day 8, cells of the inner cell mass of the blastocyst differentiate into :
hypoblast and epiblast layers.
Primordial germ cells originate in the epiblast, migrate through the
primitive streak, and by the 3rd week reside among endoderm cells in the
wall of the yolk sac close to the allantois.

During the 4th week, they migrate by ameboid movement along the dorsal
mesentery of the hindgut , to reach the primitive gonads in the genital
ridges.
The gonadal ridges appear as a pair of longitudinal ridges, on either side of
midline.
The primordial germ cells have an inductive influence on the
development of the gonad into ovary or testis.
Shortly before and during arrival of primordial
germ cells, the epithelium of the gonadal ridge
proliferates, and epithelial cells penetrate the
underlying mesenchyme. Here they form a
number of irregularly shaped cords, the
primitive sex cords .
These sex cords and the germ cells make up
the ovary and testis.
Genital Ducts in the Male
MESONEPHRIC
DUCTS

TOWARDS THE HILUM

ELONGATE AND
BECOME HIGHLY
CONVOLUTED

THE TESTIS CORDS
BREAK UP INTO A
NETWORK OF TUBULES

FORMS THE
(DUCTUS)
EPIDIDYMIS

FORMING THE RETE
TESTIS

JOINS THE EFFERENT
DUCTULES WHICH ARE
DERIVATIVES OF THE
MESONEPHRIC
TUBULES
Genital Ducts in the Female
• The paramesonephric duct arises as a longitudinal
invagination of the epithelium on the anterolateral
surface of the urogenital ridge .

• Cranially, the duct opens into the abdominal cavity
with a funnel-like structure. Caudally, it first runs
lateral to the mesonephric duct, then crosses it
anteriorly to fuse with the duct from the other side in
the midline.
• The two ducts are initially separated by a septum
but later fuse to form the uterine canal.
Fate of the mesonephric duct, mesonephric
ductules and parameso nephric duct

Mesonephric
duct

Epididymis, vas
defferens, seminal
vesicles and
ejaculatory duct in
males
Degenerates except
for some remnants
e.g. duct of
epoophoron, duct of
paroophoron and
Gartner's duct

Mesonephric
ductules

* Efferrent ductules
* Paradiddymis
*Appendix of
epididymis

Females : tubules
of epoophoron
and
paroophoron.

Paramesonephric
duct

-Cranial end
forms the
appendix testis.
- Caudal end
formis the
prostatic utricle.

Fallopian tubes,
uterus and 4/5ths
vagina
Formation of the broad ligament
Formation of the vagina

Thus, the vagina has a dual
origin :
with the upper portion (four
fifth) derived from the uterine
canal
the lower portion derived from
the urogenital sinus.
EXTERNAL GENITALIA : INDIFFERENT STAGE

In the 3rd wk, mesenchyme cells originating in the region of the
primitive streak migrate around the cloacal membrane to form a
pair of slightly elevated cloacal folds.
EXTERNAL GENITALIA IN THE MALE
EXTERNAL GENITALIA IN THE FEMALE
CONGENITAL ANOMALIES OF
THE KIDNEYS
ANOMALIES OF FORM

 Renal Agenesis
 Renal Hypoplasia
 Supernumerary kidney
RENAL AGENESIS

 Unilateral renal agenesis :
• 1 in 1000
• Males / left kidney
• Unilateral renal agenesis should be suspected in infants with a single
umbilical artery
• The term LYING DOWN ADRENAL SIGN as been ascribed to the
elongated appearance of the adrenal not normally molded by the
adjacent kidney

 Bilateral renal agenesis :
• 1 in 5000
• Results when the ureteric bud fails to develop or it fails to penetrate
the metanephrogenic blastema
• Is incompatible with postnatal life because of the associated
pulmonary hypoplasia.
• There is associated with oligohydramnios.
• These infants have a characteristic facies, club feet and other
anomalies called “ POTTER’S SEQUENCE”
RENAL HYPOPLASIA :

• Unusual renal anomaly in which the kidney is at least 50% smaller than
normal and typically contains fewer than the normal number of calyces.
• Usually unilateral, and the kidney functions normally for its size.
• There are varoius acquired causes of unilaterally small kidneys .
• Can be differentiated from hypoplasia :
* by the normal number of calyces
* and normal cupping of the calyces
• The ASH-UPMARK KIDNEY was prevoiusly thought by some to be a
variant of renal hypoplasia. However, it is currently thought to be caused by
scarring due to chronic pyelonephritis with cortical loss at its upper pole.
SUPERNUMERARY KIDNEY

• A supernumerary kidney is extremely rare.
• Cleavage of the metanephric blastema has
been suggested as the cause for this
abnormality.
•Most supernumerary kidneys are caudally
placed and are hypoplastic.
• They may be connected to the ipsilateral
dominant kidney either completely or by loose
areolar connective tissue.
ANOMALIES OF POSITION

• Malrotated kidneys
• Ectopic kidneys
MALROTATED KIDNEYS

If a kidney fails to rotate, the hilum faces
anteriorly, that is, the fetal kidney retains its
embryonic position . If the hilum faces
posteriorly, rotation of the kidney proceeded
too far; if it faces laterally, lateral instead of
medial rotation occurred. Abnormal rotation
of the kidneys is often associated with
ectopic kidneys.
RENAL ECTOPIA

• Renal ectopia results from failure of normal ascent of the embryonic
kidney.
• Ectopic kidneys lie outside the renal fossa and may be:
^pelvic ^lie opposite the sacrum ^ below the aortic bifurcation ^iliac ^
thoracic ^ crossed .
• They are often small, lobulated kidneys with abnormal rotation with
extrarenal pelvis and an irregular vascular supply.
•The most common problem associated with an ectopic kidney is vesicoureteric reflux (VUR) (85%), pelvi-ureteric junction (PUJ) obstruction (3352% ) and renal calculi .
ANOMALIES OF FUSION

• These anomalies are thought to result because of an
abnormally situated umbilical artery that prevents normal
cephalic migration from occurring.
• In all fused kidneys, the arterial supply and venous
drainage are grossly abnormal.
HORSESHOE KIDNEY
• 1 in 400
• Horseshoe kidney is the most common renal fusion
anomaly.(90%)

• It consists of two distinct functioning kidneys lying
vertically on either side of the midline, connected at
their lower poles by an isthmus of functioning renal
parenchyma or, rarely, fibrous tissue that crosses the
midline of the body .
• In rare instances, the upper poles may be the site of
fusion.
• The position of the isthmus and hence the kidneys are variable :
 40% : the isthmus lies at level of L4 just below the origin of the IMA
 Another 40% : the isthmus is located in a normal anatomical
position of the kidneys
 Remaining 20% :kidneys lie lower in the pelvis
• Ureters may have the so-called “FLOWER-VASE
APPEARANCE” , in which the upper ureters
diverge laterally over the isthmus and then
converge inferiorly.

• Because of their abnormal course, the ureters are
prone to be obstructed, precipitating
hydronephrosis, infection and stone formation.
• Some authours have reported HSK to be
associated with increased risk for renal neoplasms
such as Wilms tumors, renal carcinoids, and
transitional cell carcinoma.
CROSSED FUSED RENAL ECTOPIA
• Incidence : 1 in 1000

• 2 : 1 male
•The left kidney is most frequently ectopic, 3 : 1
• Origin : abnormal migration of ureteral bud.
• One kidney crosses over to opposite side, and the parenchyma
of the two kidneys fuse.
• Most commonly, the upper pole of the inferiorly positioned
crossed ectopic kidney is fused to the lower pole of the
superior, normally positioned kidney. The ureter of the ectopic
kidney crosses the midline and enters the bladder on the
opposite side .
McDonald and McClellan classified crossed ectopic kidney into
four types: crossed renal ectopia with fusion (85%), crossed renal
ectopia without fusion (10%), solitary crossed renal ectopia and
bilaterally crossed renal ectopia .

Six variations of crossed fusion have been described. In decreasing
order of frequency, they are
type 1: inferior crossed fused ectopia
type 2: sigmoid or S-shaped kidney
type 3 :unilateral lump kidney
type 4: unilateral disc kidney
type 5: L-shaped kidney
type 6: superior crossed fused ectopia
CAKE KIDNEY (COMPLETE RENAL FUSION)
• Pan Cake kidney is a rare congenital anomaly of the genitourinary
system,with fewer than 30 cases described in the literature .

•The term cake kidney or fused pelvic kidney was defined by Glenn as an
anomaly in which “the entire renal substance is fused into one mass,
lying in the pelvis, and giving rise to two separate ureters which enter the
bladder in normal relationship”
•In rare instances, such kidneys possess one ureter .
•The fused kidney occupies prevertebral or presacral space .
•The cake kidney may remain asymptomatic and be detected at autopsy.
•It may become infected or may cause local pain from dragging on the
renal vessels by the weight of the organ
CONGENITAL CYSTIC
RENAL DISEASES
CONGENITAL CYSTIC RENAL DISEASES
• Renal cysts detected in utero and after birth
has been a topic of considerable confusion.
• A standardized step-by-step sonographic
approach is most helpful in differentiating
renal cystic diseases.
• This approach must be combined with the
clinical data, familial history, and in some
cases, complementary examinations.
Once renal cystic disease is suspected, the first step is to rule out
Dysplasia associated with obstructive uropathy the features of
which include:
• Dilated PCS
• Hyperechoic kidneys
• Multiple cysts
• Can be normal, enlarged or small/ Unilateral or Bilateral
Next step is to consider Multicystic Dysplastic kidneys:
• Multiple cysts throughout the kidney without communication.
• No renal parenchyma in between
• Unilateral
• Atretic ureter
• Vascular pedicle may also be absent or atretic
Both obstructive dysplasia and MCDK
should be differentiated from complex
cysts, which include cystic tumors like
renal lymphangioma
If the foregoing diagnoses are unlikely, inherited renal cystic
disease should be considered.
If no familial history is present, cases are further split
 patients with renal findings limited to the kidney
 renal anomalies are associated with other organ malformations
Both these groups can be separated into
 those with typical ultrasound patterns suggestive of specific
diagnoses
 those with atypical ultrasound patterns.
The ARPKD pattern:
• Bilateral, very large (> 4 SD) kidneys
• Diffusely hyperechoic kidneys
• Loss of CMD with or without evident cysts
• Oligohydramnios is the rule
• Very large hyperechoic kidneys with hyperechoic medulla
(reversed CMD) is another typical pattern.
•The next step is to rule out Glomerular cysts (GCKD) (AD):

• It also includes hyperechoic and enlarged (+2 SD) kidneys without
CMD but there are charecteristic multiple subcapsular as well as
cortical cysts.
Third typical pattern is Nephronophthisis:
• autosomal recessive tubulointerstitial
ciliopathies
• urinary concentration defects and anemia
At ultrasound :
 the kidneys are hyperechoic, normal-sized or
small, and without CMD.
 renal cysts typically develop at the
corticomedullary junction
Multiple congenital syndromes are also
associated with renal cysts. These should also
be kept in mind while dealing with a case of
renal cysts in utero or in a child . This
includes :
•Bardet-Biedl syndrome (BBS)
•Meckel-Grüber syndrome
•Joubert syndrome
•Tuberous Sclerosis
ANOMALIES OF RENAL PELVIS
AND URETER

- Polycalicosis and Congenital Megacalyces
- Calyceal diverticula
- Microcalyx
- Aberrant (Ectopic) Papilla
- Ureteropelvic Junction Obstruction
- Congenital Megaureter
- Duplex Collecting Systems
CONGENITAL MEGACALYCES/ PUIGVERT'S DISEASE
Calyces are asymmetrically dilated. Renal pelvis is
normal. Some doubt its congenital nature.
CALYCEAL DIVERTICULUM

EXCRETORY PHASE
• 4.5 : 1000
• Focal extrinsic dilatation of a
renal calyx

• In 1/3 patients : stones can form
within these.

DELAYED IMAGES
URETEROPELVIC JUNCTION OBSTRUCTION

• Congenital obstruction of the UPJ is a common anomaly of the
urinary tract.
• The disorder produces caliectasis and marked pelviectasis as a result
of a functional narrowing of the UPJ.
• In 5% cases : extrinsic compression by an aberrant renal artery
• Males (2:1), Left sided
• Commonly presents as an abdominal mass in a neonate.

• In some cases, symptoms may present in the setting of a sustained
diuresis, a condition that has become known as "beer-drinker's

hydronephrosis."
On urography, a dilated renal pelvis and calyces will be demonstrated.
Because of the dilation, slow opacification of the affected side is the
rule and delayed radiographs are usually necessary.
DIFFERENTIATING UPJ FROM EXTRA RENAL PELVIS IS IMPORTANT.

Normal
calyces

Dilated
calyces

RGP

RGP
• USG and CT reveal similar findings.
• In patients with equivocal UPJ
obstruction, or in cases where there is a
discrepancy between the patient's
clinical symptoms and the radiologic
findings, diuresis renography or the
Whitaker procedure may be employed.
DUPLICATION OF THE URETER :

• Results from early splitting of the ureteric bud .
• Duplication can be variable. At one end of the spectrum there is
merely duplication of the renal pelvis, draining via a single ureter.
At the other extreme, two separate collecting systems drain

independently into the bladder or ectopically (see below)
• Duplex systems may be unilateral (more common) or bilateral.
•Partial or complete.

Duplication of a renal pelvis (1 in 40) :
Duplication of a renal pelvis is the most common
congenital abnormality of the upper renal tract .
.
The upper
pole
ureter

The lower
pole
moiety

• drains inferomedially
• can drain ectopically
• usually associated with an
ureterocele

• drains superolaterally
• is usually associated with VUR

• CT is able to delineate essentially all abnormalities, especially
when performed during the excretory phase. A duplicated renal
collecting system can be suspected by identifying the so
called faceless kidney .
Faceless Kidney Sign
Drooping Lily Sign
PRIMARY MEGAURETER :

• Megaureter is a generic term indicating the presence of an enlarged
ureter with or without concomitant megacalyces.
• The normal ureter in children rarely exceeds 5 mm in diameter . In
practice, a ureter with a diameter of 7 mm or more should be considered
a megaureter .
• There are three major categories of primary megaureter:
obstructed primary megaureter
refluxing primary megaureter
nonrefluxing unobstructed primary megaureter
•Secondary megaureter occurs as a result of some abnormality
involving the bladder or urethra (eg, urethral valves, strictures,
ureteroceles etc)
 Obstructed primary megaureter: a condition akin to achalasia and
Hirschsprung disease. The normal ureter proximal to the aperistaltic
segment dilates because of relative obstruction.
There is associated hydronephrosis, and active peristaltic waves can be
seen on ultrasound.

 Refluxing primary megaureter :is caused by a short or absent
intravesical ureter, congenital paraureteric diverticulum, or other :
derangement of the VUJ.
Vesicoureteric reflux can be demonstrated in this.

 Nonrefluxing unobstructed primary megaureter: there is neither
reflux nor stenosis of the vesicoureteral junction, but the ureter is
dilated beginning at a point just above the bladder .
Most primary megaureter in neonates fall into this category.
ANOMALOUS TERMINATION OF
THE URETER
- ECTOPIC URETER
- VUR
- URETEROCELE : ORTHOTOPIC OR ECTOPIC
ECTOPIC URETER

•Ectopic insertion of the ureter occurs due to abnormal ureteral bud
migration and usually results in caudal ectopia.
•70% are associated with complete ureteral duplication.
• In the female, it can insert urethra, vestibule, or vagina. More rarely,
it can empty into the uterus or Gartner duct or cyst.
• In males, it empties into the lower bladder, posterior urethra, seminal
vesicle, vas deferens, or ejaculatory duct. In very rare instances, it can
empty into the rectum.
VESICO URETERAL REFLUX

• VUR is the abnormal flow of urine from the bladder into the upper
urinary tract.
• Cause :

primary maturation abnormality of the VUJ

short distal ureteric submucosal tunnel

due to an associated congenital anomaly

• Reflux predisposes to renal infection (pyelonephritis).
• The majority of pediatric patients who develop renal scars after a
urinary tract infection have VUR.
• Detection of VUR in neonates and infants is particularly important
because these patients are more predisposed to the development of
renal scars and renal failure than are older children.
• Reflux is also the most common cause of antenatal
hydronephrosis (40%)
•The primary diagnostic procedure for evaluation of VUR is
VCUG, which should be performed after the first episode of
urinary tract infection.
• VCUG should be used to document the presence of VUR
and to determine the grade of reflux and whether reflux
occurs during micturation or during bladder filling.
Grades of VUR Based on Guidelines of the International Reflux Study
Committee

Echo-enhanced cystosonography has recently been
proposed as a promising new method for detecting
and grading VUR without exposing patients to
ionizing radiation.
URETEROCELES

• Ureteroceles represent cystic dilatation of the intravesical

segment of the ureter.
• The defect is in the obstruction of the meatus, and the
ureterocele is simply a hyperplastic response to this
obstruction with the bladdder and ureteral mucosa prolapsing
out.
• They may be
 Simple

25% - a ureterocoele that occurs at a VUJ in a

normal position
 Ectopic

75% - that which occurs at a VUJ whose site is

abnormal (duplex ureter)
IMAGING :

 At US: the ureterocele is identified as a
cystic intravesical mass, contiguous with a
dilated ureter.

 In IVP: demonstrates a collection of
contrast material within the ureterocele :
which produces the classic “cobra head”
appearance consisting of a round or oval
area of increased opacity surrounded by the
radiolucent halo of the wall of the
ureterocele.

 In VCUG: a ureterocele usually
manifests as a rounded filling defect within
the bladder.
PRUNE BELLY SYNDROME

• is also called Eagle-Barrett syndrome
• exclusively in males
• Renal anomalies include

Kidneys: normal
or dysplastic
Ureters: dilated :
segmental or total /
VUR
Bladder: large/ Patent
urachus (lacks
trabeculations)

Dilated posterior urethra :
PUV like picture
ABNORMALITIES OF THE BLADDER

- Exstrophy
- Bladder Duplication
- Bladder diveriticulum
- Bladder ears
VESICAL EXSTROPHY COMPLEX

•

•
•
•

Depending on the timing of the intrauterine insult, defect in lower anterior
abdominal wall defect and premature perforation of the cloacal membrane
results in a spectrum of diseases.
30% are epispadias, 60% are classic bladder exstrophy, and less than 10% are
cloacal exstrophies.
Incidence of bladder exostrophy : 0.25-0.5 per 10,000 births
Males ( 2:1)

Ante natal USG findings :
Lower abdominal wall defect with protruding
abdominal mass.
Absence of the normally filled urinary bladder.
No sign of oligohydramnios or other gross renal
abnormalities
Colour Doppler shows umbilical arteries alongside
the abdominal wall mass
External genitalia malformation, pubic diastasis etc
BLADDER DUPLICATION

• Can be complete or incomplete.
• In incomplete bladder duplication, two bladders
communicate with each other and drain into a
common urethra.
• Complete duplication is very rare : 40 cases in
surgical literature
• Duplication of the bladder may occur in the sagittal
or coronal plane. The most common form is sagittal
duplication with the bladders lying side by side.
• Each bladder receives the ureter of the ipsilateral
kidney and is drained by its own urethra.
BLADDER DIVERTICULA
• Congenital BD : occur without obstruction
• Male predominance
• Described as herniation of mucosa through bladder
musculature.
• Usually > 2cm, solitary
• Types : 90% paraureteral : associated with VUR :
“ Hutch Divericula”
10 % postero-lateral : not associated with VUR
URACHAL ABNORMALITIES

Patent urachus
Urachal Cyst
Urachal-umbilical sinus
Vesicourachal diverticulum
CONGENITAL URACHAL REMNANT ABNORMALITIES

• Refers to a series of potential anomalies that can occur in
association with the urachus.
• There are four types of congenital urachal remnant anomalies.
They are
•
•
•
•

Patent urachus : commonest ~ 50 %
Urachal Cyst : next commonest : ~ 30 %
Urachal-umbilical sinus : ~ 15 %
Vesicourachal diverticulum : ~ 5 %

Usually midline : may occasionally
deviate laterally to merge with one of
the obliterated umbilical arteries
Development and congenital anomalies of urogenital system
ABNORMALITIES IN THE
MALE URETHRA

• Diverticulae
• Absent phallus/ agenesis of urethra - Posterior urethral
- Anterior urethral
• Hypospadias
• Congenital megalourethra
• Congenital urethral duplication
• Posterior urethral polyp
• Congenital urethral fistulae
• Prostatic utricle
• Posterior urethral valve
• Congenital meatal stenosis
• Anterior urethral valve
• Congenital urethral stenosis
POSTERIOR URETHRAL VALVES

• M.C congenital obstructive lesion of urethra, occurring only in
phenotypic boys.
• Speculated to be a result of abnormal insertion of the
mesonephros into cloaca.
• It courses obliquely from the verumontanum to the most distal
portion of the prostatic urethra in a bicuspid or unicuspid
fashion.
• VUR with gross hydronephrosis, dysplastic kidneys, and urine
ascites are common findings in such a patient.
Development and congenital anomalies of urogenital system
HYPOSPADIAS

The urethral meatus is
located on the ventral
surface, anywhere from the
penile shaft to the
penoscrotal region
ANTERIOR URETHRAL VALVES

•These are rare congenital anomalies that cause lower
urinary tract obstruction in children.
•Anterior urethral valves may be found anywhere in the
anterior urethra.

•Most commonly in bulbar urethra.
CONGENITAL URETHRAL DUPLICATION

It is a rare anomaly. Commonly ,the duplication commonly occurs in the
sagittal plane. Effmann Class.

I : Blind ending
I I A: double meatus

I I: Patent assesory urethra
I I B: single meatus

III : Assesory urethra from duplicated bladder
MULLERIAN DUCT CYSTS AND PROSTATIC UTRICLE

MULLERIAN DUCT CYSTS
• Nonatrophy of the mullerian duct may produce cystic dilations along the
route of the vas deferens from the scrotum to the ejaculatory ducts .
• Mullerian duct cysts are rare, but most commonly occur in the midline just
above the prostate.

PROSTATIC UTRICLE

• The prostatic utricle is a small, blind-ending midline pouch arising from the
prostatic urethra at the level of the verumontanum.
• The normal prostatic utricle is 8 to 10 mm in length, narrow at its orifice (2
mm).
• It may be associated with urinary retention, stasis, and infection.
URETHRAL DIVERTICULUM

• Anterior urethral diverticula : obstruction
• Posterior urethral diverticula : complicated by urinary stasis,
infection, and calculi formation.
CONGENITAL MEGALOURETHRA

This is a rare congenital anomaly resulting from the faulty development of
the corpora cavernosa and corpus spongiosum.
Two types are described.
Milder form : scaphoid variety. More severe form : fusiform variety
MULLERIAN DUCT ANOMALIES
MULLERIAN DUCT ANOMALIES

Phase of Organogenesis : one or both ducts
may not develop completely leading to
complete agenesis / hypoplasia / unicornuate
anomalies.
Phase of lateral fusion : where the lower segments of the
Mullerian ducts fuse to form uterus, cervix and upper 4/5 ths
vagina. Failure of this could lead to Bicornuate or Didelphys
anomalies.
Vertical fusion is referred to ascending sinovaginal bulb
fusion to descending Mullerian system. Incomplete fusion
would produce transverse vaginal septum.

Septal resorption : After lower Mullerian
system fuses, septum which is present gets
resorbed naturally. Failure leads to septate
uterus anomaly.
• Developed by the American Fertility Society (AFS)
• Developmental anomalies of the renal system is common. Renal
agenesis is the most commonly reported anomaly, ( 67% ).
• Many of the anomalies are initially diagnosed at HSG and USG ;
however MR imaging is the study of choice because of its high
accuracy and detailed elaboration of uterovaginal anatomy.
• Three-dimensional ultrasound with multiplanar imaging has
been shown to be more accurate than plain TVS.

• Laparoscopy and hysteroscopy are reserved for women in whom
interventional therapy is likely to be undertaken.
CLASS I — AGENESIS/HYPOPLASIA (5-10% )

• Early developmental failure of the Mullerian ducts, results in
various degrees of agenesis or hypoplasia of the uterus, cervix
and upper 4/5 ths of the vagina.
• In agenesis a uterus is not identified or small amounts of
rudimentary tissue without differentiation may be identified.
• The most common form is the Mayer–Rokitansky– Kuster–
Hauser syndrome.
 90% cases : vaginal + uterine agenesis
 10% cases : isolated vaginal agenesis with an obstructed
uterus or small rudimentary uterus.
On US images, a normal uterus cannot be identified. The
ovaries often are normally situated.
On MR images, uterine agenesis and hypoplasia are best
characterized on sagittal images, while vaginal agenesis is best
demonstrated on transverse images. Agenesis results in no
identifiable uterus. Uterine hypoplasia demonstrates small uterus
with abnormal low-signal-intensity myometrium on T2-weighted
images and poorly delineated zonal anatomy.
CLASS II — UNICORNUATE (20%)

This anomaly results from complete or
near-complete arrested development of
one of the Mullerian ducts.

Four possible subtypes can
develop:
(i)absent horn
(ii) non-cavitary (non-functional)
rudimentary horn
(iii) cavitary communicating
rudimentary horn and
(iv) cavitary non-communicating
rudimentary horn.
The embryologic predominance of the unicornuate uterus to be on the
RIGHT has not been explained.
HSG :
• Fusiform shape : tapering
at the apex
• Draining into a solitary
fallopian tube
• The uterus is generally
shifted off of midline
USG :
On MR images:
• Unicornuate uterus appears curved and elongated
• The external uterine contour assumes a banana shape.
• Uterine volume is reduced.
•The endometrium may be uniformly narrow or may assume a bullet
shape, tapering at the apex.
• Normal myometrial zonal anatomy is maintained.
• The endometrial-to-myometrial width and ratio are reported to be
normal .
CLASS III — DIDELPHYS (5%)
• This anomaly results from complete non-fusion of both Mullerian ducts.

• The individual horns are fully developed and almost normal in size.
• Each mullerian duct develops its own hemiuterus and cervix and
demonstrates normal zonal anatomy with a minor degree of fusion at the
level of the cervices. NO COMMUNICATION IS PRESENT BETWEEN THE
DUPLICATED ENDOMETRIAL CAVITIES.
• A longitudinal vaginal septum is associated in 75% of these anomalies .
On USG :
• Separate divergent uterine horns are identified
• With a large fundal cleft
• Endometrial cavities are uniformly separate, with no evidence of
communication.
MR IMAGING
• Some patients with uterus didelphys present with a unilateral
hemivaginal septum, which may result in obstruction with
consequent hematometrocolpos .
• Majority of cases reported in the literature are associated with
renal agenesis on the same side as the obstructing transverse
hemivaginal septum. This syndrome has been referred to as

OBSTRUCTED HEMIVAGINA–IPSILATERAL RENAL
AGENESIS ANOMALY.
CLASS IV — BICORNUATE (10%)

• This class is characterised by partial non-fusion of the
Mullerian ducts.
• This results in a central myometrium separating the uterus
that may extend to the level of the internal cervical os
(BICORNUATE UNICOLLIS) or external os (BICORNUATE
BICOLLIS). The fundal cleft is usually > 1 cm deep.
• The horns of the bicornuate uteri are not as fully developed
and are smaller than those in the didelphys uteri.
Ultrasound demonstrates two uterine horns
(arrows) and a single cervix
The horns demonstrate normal uterine zonal
anatomy. The endometrial-to-myometrial ratio and
width are normal in appearance.
CLASS V — SEPTATE (55%)
• This class of anomaly occurs when the final fibrous septum between
the two Mullerian ducts fails to resorb.
• This results in the formation of a uterus that is completely or partially
divided into two cavities.
• The septum may be muscular or fibrous or a combination of both.
• This class is associated with the poorest obstetrical outcomes. It has
been theorized that the decreased connective tissue may result in
POOR DECIDUALIZATION AND IMPLANTATION, while increased
muscular tissue may result in INCREASED CONTRACTILITY of the
tissue, thereby predisposing the patient to spontaneous abortion.
• It is important to distinguish a fibrous septum from a muscular
septum, as the former can be repaired by a hysteroscopic approach,
whereas the latter may require a transabdominal surgical approach.
US: The echogenic endometrial cavities are separated at the
fundus by the intermediate echogenicity of the myometrium and
by a hypoechoic fibrous segment caudally.

COMPLETE
On MR imaging:
• The differentiation between a septate and bicornuate uterus is
important because they differ in their reproductive prognosis and
treatment.
Bicornuate
Features
Septate Uterus
Uterus
• Depth of
fundal cleft
• Fundal
contour
• Intercornual
angle
• Intercornual
distance
• Intercornual
tissue

• < 1 cm

• > 1 cm

• Convex/ flat

• Concave

• <75 degrees
• <4 cm
• Fibrous or
myometrial

• >105 degrees
• >4 cm
• Myometrial
CLASS VI — ARCUATE

• This group is characterised by mild indentation of the endometrium
at the uterine fundus .
• It is the result of near complete resorption of the uterovaginal
septum.
• Currently, No Definitive Depth Has Been Established To Differentiate
The Arcuate Configuration From The Septate.
• This class is highly controversial, as it remains unclear whether this
variant should be classified as a true anomaly or as an anatomic
variant of normal.
• Data regarding the reproductive outcome of patients in this category
are extremely limited and conflicting. Currently, it is generally thought
that an arcuate uterus is compatible with normal pregnancy and
delivery.
CLASS VII — DIETHYLSTILBESTROL RELATED

•Several million women were treated with diethylstilbestrol (DES),
a non-steroidal oestrogen, to prevent miscarriage between 1945
and 1970 .
•The drug was promptly removed from the market when it was
foundthat up to 15% of newborn girls who were exposed to DES
had UTERINE MALFORMATIONS and an increased risk of
VAGINAL CLEAR CELL CARCINOMA .
•The uterine abnormalities include hypoplasia and a T-shaped
uterine cavity .
• Patients may also have abnormal transverse ridges,
pseudopolyps and stenoses of the cervix.
MR Evaluation :
TRANSVERSE VAGINAL SEPTUM
• Defects of vertical fusion results in a transverse vaginal septum.

• It can occur anywhere along the vagina, although it occurs most
frequently at the junction of the upper and middle third.
•The septum is a membrane of fibrous connective tissue with
vascular and muscular components .
• There is resultant hematocolpos and hematometra, which is
usually less striking, secondary to decreased distensibility of the
more muscular myometrium.
UNDESCENDED AND ECTOPIC TESTES

•Undescended testis(UDT) is a common genitourinary anomaly in which
the testis fails to descend completely and is located along the normal
pathway of descent.
•Ectopia testis on the other hand is rare with an incidence of <1% of all
cases of undescended testes.

•Usg is the primary modality. However, if the testis cannot be localised
by USG MRI is used.
•The testicle is typically low signal on T1 and high signal on T2. The
normal testicle exhibits intense diffusion restriction, and these
sequences can aid confident identification.
DISORDERS OF SEXUAL
DEVELOPMENT
- Male pseudohermaphroditism
- Female pseudohermaphroditism
- True hermaphrodite
- Mixed gonadal dysgenesis
- Pure gonadal dysgenesis
- Persistent mullerian duct syndrome
AMBIGUOUS GENITALIA

INTERNAL MALE PSEUDO HERMAPHRODITE
CONCLUSION
Radiologic investigation continues to be one of
the most important sources of clinical information
in the evaluation of urinary and genital tract
disorders.

The role of diagnostic imaging is to help
determine as closely as possible the exact nature
of the abnormality.
But the need for radiologic examinations should
be carefully weighed to avoid inconveniencing the
patient, exposing the patient to unnecessary
radiation, or delaying surgical correction.
“In the field of scientific observation,
chance favours the prepared mind”
Dr. Louis Pasteur

Thank you

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Development and congenital anomalies of urogenital system

  • 1. Development and congenital anomalies of urogenital system Moderator : Dr. (Mrs) Nabanita Deka Asst. Professor Presenter : Dr. Jayeta Choudhury Department of Radiology Gauhati Medical College
  • 2. Functionally, the urogenital system can be divided into two entirely different components: the urinary system and the genital system. Embryologically and anatomically, however, they are intimately interwoven. Both develop from a common mesodermal ridge (intermediate mesoderm) and open into a common channel the cloaca.
  • 3. “Ontogeny Recapitulates Phylogeny” Ernst Haeckel 1860  Ontogeny is the development of the individual  Phylogeny is the evolution of the species  So this is the idea that during development an organism (or an organ) goes through the same stages as during their evolution. The kidneys are a perfect example.
  • 4. URINARY SYSTEM : Kidney Systems • Three slightly overlapping kidney systems are formed in a cranial-tocaudal sequence during intrauterine life in humans: the pronephros, mesonephros, and metanephros. PRONEPHROS • Beginning of the 4th wk (21days). •The pronephros is represented by 7 to 10 solid cell groups in the cervical region . •Their excretory units: are called nephrotomes. •By the end of the fourth week, all indications of the pronephric system regress.
  • 5. MESONEPHROS •Appear late in the 4th wk • From the thoracic and lumbar seg of intermediate mesoderm. •They function as interim kidneys for approximately 4 wks. • Excretory tubules form their basic unit which open into mesonephric ducts. •The mesonephric ducts open into the cloaca.
  • 6. METANEPHROS: THE DEFINITIVE KIDNEY •Appears in the 5th wk. •Develops from sacral segments of the intermediate mesoderm . •However, the development of the duct system differs from that of the other kidney systems. COLLECTING SYSTEM Collecting ducts of the permanent kidney develop from the URETERIC BUD, an outgrowth of the mesonephric duct close to its entrance to the cloaca . The bud penetrates the metanephric tissue, which is moulded over its distal end as a cap . Subsequently, the bud dilates, forming the primitive renal pelvis, and splits and subsequently divides and sub divides to finally give rise to approximately 1 to 3 million collecting tubules.
  • 7. Hence, the kidney develops from two sources: (a) metanephric excretory units and (b) the ureteric bud, which gives rise to the collecting system. At birth, the mesoderm, which provides kidneys have a lobulated appearance, but the lobulation disappears during infancy .
  • 8. POSITIONAL CHANGES OF THE KIDNEYS • Initially : close/ pelvic / anterior to the sacrum . • Gradually come to lie in the abdomen and move farther apart . • Attain their adult position by 9th wk. This relative ascent results mainly from the growth of the embryo's body caudal to the kidneys . • Initially the hilum : faces ventrally; however, as the kidney relocates (ascends), it rotates medially almost 90 degrees. • By the 9th week, the hilum is directed anteromedially .
  • 9. Development of Bladder and Urethra urogenital sinus anteriorly During the 4th to 7th weeks of development, CLOACA anal canal posteriorly TIP OF UROGENITAL SEPTUM PERINEAL BODY UROGENITAL SINUS CRANIAL VESICAL PART MOST OF THE BLADDER MIDDLE PELVIC PART * THE BLADDER NECK * THE PROSTATIC PART OF URETHRA IN MALES * THE ENTIRE URETHRA IN FEMALES CAUDAL PHALLIC PART REST OF THE MALE URETHRA
  • 10. Initially, the bladder is continuous with the allantois, but when the lumen of the allantois is obliterated, a thick fibrous cord, the urachus, remains and connects the apex of the bladder with the umbilicus . In the adult, it forms the median umbilical ligament.
  • 11. FORMATION OF THE TRIGONE OF THE BLADDER CAUDAL PORTIONS OF THE MESONEPHRIC DUCTS ABSORBED INTO THE WALL OF THE URINARY BLADDER
  • 12. GENITAL SYSTEM •Sex differentiation is a complex process that involves many genes. •The key to sexual dimorphism is the Y chromosome, which contains the testis-determining gene called the SRY (sex-determining region on Y) gene in its short arm (Yp11).
  • 13. Indifferent embryo • Weeks 1-6 sexually indifferent or undifferentiated stage • Week 7 begins phenotypic sexual differentiation. • The sex of the fetus may be discerned by ultrasound as early as 12 weeks gestation. However, it can be determined with 95-100% accuracy only at or after 20wks.
  • 14. At day 8, cells of the inner cell mass of the blastocyst differentiate into : hypoblast and epiblast layers. Primordial germ cells originate in the epiblast, migrate through the primitive streak, and by the 3rd week reside among endoderm cells in the wall of the yolk sac close to the allantois. During the 4th week, they migrate by ameboid movement along the dorsal mesentery of the hindgut , to reach the primitive gonads in the genital ridges. The gonadal ridges appear as a pair of longitudinal ridges, on either side of midline. The primordial germ cells have an inductive influence on the development of the gonad into ovary or testis.
  • 15. Shortly before and during arrival of primordial germ cells, the epithelium of the gonadal ridge proliferates, and epithelial cells penetrate the underlying mesenchyme. Here they form a number of irregularly shaped cords, the primitive sex cords . These sex cords and the germ cells make up the ovary and testis.
  • 16. Genital Ducts in the Male MESONEPHRIC DUCTS TOWARDS THE HILUM ELONGATE AND BECOME HIGHLY CONVOLUTED THE TESTIS CORDS BREAK UP INTO A NETWORK OF TUBULES FORMS THE (DUCTUS) EPIDIDYMIS FORMING THE RETE TESTIS JOINS THE EFFERENT DUCTULES WHICH ARE DERIVATIVES OF THE MESONEPHRIC TUBULES
  • 17. Genital Ducts in the Female • The paramesonephric duct arises as a longitudinal invagination of the epithelium on the anterolateral surface of the urogenital ridge . • Cranially, the duct opens into the abdominal cavity with a funnel-like structure. Caudally, it first runs lateral to the mesonephric duct, then crosses it anteriorly to fuse with the duct from the other side in the midline. • The two ducts are initially separated by a septum but later fuse to form the uterine canal.
  • 18. Fate of the mesonephric duct, mesonephric ductules and parameso nephric duct Mesonephric duct Epididymis, vas defferens, seminal vesicles and ejaculatory duct in males Degenerates except for some remnants e.g. duct of epoophoron, duct of paroophoron and Gartner's duct Mesonephric ductules * Efferrent ductules * Paradiddymis *Appendix of epididymis Females : tubules of epoophoron and paroophoron. Paramesonephric duct -Cranial end forms the appendix testis. - Caudal end formis the prostatic utricle. Fallopian tubes, uterus and 4/5ths vagina
  • 19. Formation of the broad ligament
  • 20. Formation of the vagina Thus, the vagina has a dual origin : with the upper portion (four fifth) derived from the uterine canal the lower portion derived from the urogenital sinus.
  • 21. EXTERNAL GENITALIA : INDIFFERENT STAGE In the 3rd wk, mesenchyme cells originating in the region of the primitive streak migrate around the cloacal membrane to form a pair of slightly elevated cloacal folds.
  • 23. EXTERNAL GENITALIA IN THE FEMALE
  • 25. ANOMALIES OF FORM  Renal Agenesis  Renal Hypoplasia  Supernumerary kidney
  • 26. RENAL AGENESIS  Unilateral renal agenesis : • 1 in 1000 • Males / left kidney • Unilateral renal agenesis should be suspected in infants with a single umbilical artery • The term LYING DOWN ADRENAL SIGN as been ascribed to the elongated appearance of the adrenal not normally molded by the adjacent kidney  Bilateral renal agenesis : • 1 in 5000 • Results when the ureteric bud fails to develop or it fails to penetrate the metanephrogenic blastema • Is incompatible with postnatal life because of the associated pulmonary hypoplasia. • There is associated with oligohydramnios. • These infants have a characteristic facies, club feet and other anomalies called “ POTTER’S SEQUENCE”
  • 27. RENAL HYPOPLASIA : • Unusual renal anomaly in which the kidney is at least 50% smaller than normal and typically contains fewer than the normal number of calyces. • Usually unilateral, and the kidney functions normally for its size. • There are varoius acquired causes of unilaterally small kidneys . • Can be differentiated from hypoplasia : * by the normal number of calyces * and normal cupping of the calyces • The ASH-UPMARK KIDNEY was prevoiusly thought by some to be a variant of renal hypoplasia. However, it is currently thought to be caused by scarring due to chronic pyelonephritis with cortical loss at its upper pole.
  • 28. SUPERNUMERARY KIDNEY • A supernumerary kidney is extremely rare. • Cleavage of the metanephric blastema has been suggested as the cause for this abnormality. •Most supernumerary kidneys are caudally placed and are hypoplastic. • They may be connected to the ipsilateral dominant kidney either completely or by loose areolar connective tissue.
  • 29. ANOMALIES OF POSITION • Malrotated kidneys • Ectopic kidneys
  • 30. MALROTATED KIDNEYS If a kidney fails to rotate, the hilum faces anteriorly, that is, the fetal kidney retains its embryonic position . If the hilum faces posteriorly, rotation of the kidney proceeded too far; if it faces laterally, lateral instead of medial rotation occurred. Abnormal rotation of the kidneys is often associated with ectopic kidneys.
  • 31. RENAL ECTOPIA • Renal ectopia results from failure of normal ascent of the embryonic kidney. • Ectopic kidneys lie outside the renal fossa and may be: ^pelvic ^lie opposite the sacrum ^ below the aortic bifurcation ^iliac ^ thoracic ^ crossed . • They are often small, lobulated kidneys with abnormal rotation with extrarenal pelvis and an irregular vascular supply. •The most common problem associated with an ectopic kidney is vesicoureteric reflux (VUR) (85%), pelvi-ureteric junction (PUJ) obstruction (3352% ) and renal calculi .
  • 32. ANOMALIES OF FUSION • These anomalies are thought to result because of an abnormally situated umbilical artery that prevents normal cephalic migration from occurring. • In all fused kidneys, the arterial supply and venous drainage are grossly abnormal.
  • 33. HORSESHOE KIDNEY • 1 in 400 • Horseshoe kidney is the most common renal fusion anomaly.(90%) • It consists of two distinct functioning kidneys lying vertically on either side of the midline, connected at their lower poles by an isthmus of functioning renal parenchyma or, rarely, fibrous tissue that crosses the midline of the body . • In rare instances, the upper poles may be the site of fusion.
  • 34. • The position of the isthmus and hence the kidneys are variable :  40% : the isthmus lies at level of L4 just below the origin of the IMA  Another 40% : the isthmus is located in a normal anatomical position of the kidneys  Remaining 20% :kidneys lie lower in the pelvis
  • 35. • Ureters may have the so-called “FLOWER-VASE APPEARANCE” , in which the upper ureters diverge laterally over the isthmus and then converge inferiorly. • Because of their abnormal course, the ureters are prone to be obstructed, precipitating hydronephrosis, infection and stone formation. • Some authours have reported HSK to be associated with increased risk for renal neoplasms such as Wilms tumors, renal carcinoids, and transitional cell carcinoma.
  • 36. CROSSED FUSED RENAL ECTOPIA • Incidence : 1 in 1000 • 2 : 1 male •The left kidney is most frequently ectopic, 3 : 1 • Origin : abnormal migration of ureteral bud. • One kidney crosses over to opposite side, and the parenchyma of the two kidneys fuse. • Most commonly, the upper pole of the inferiorly positioned crossed ectopic kidney is fused to the lower pole of the superior, normally positioned kidney. The ureter of the ectopic kidney crosses the midline and enters the bladder on the opposite side .
  • 37. McDonald and McClellan classified crossed ectopic kidney into four types: crossed renal ectopia with fusion (85%), crossed renal ectopia without fusion (10%), solitary crossed renal ectopia and bilaterally crossed renal ectopia . Six variations of crossed fusion have been described. In decreasing order of frequency, they are type 1: inferior crossed fused ectopia type 2: sigmoid or S-shaped kidney type 3 :unilateral lump kidney type 4: unilateral disc kidney type 5: L-shaped kidney type 6: superior crossed fused ectopia
  • 38. CAKE KIDNEY (COMPLETE RENAL FUSION) • Pan Cake kidney is a rare congenital anomaly of the genitourinary system,with fewer than 30 cases described in the literature . •The term cake kidney or fused pelvic kidney was defined by Glenn as an anomaly in which “the entire renal substance is fused into one mass, lying in the pelvis, and giving rise to two separate ureters which enter the bladder in normal relationship” •In rare instances, such kidneys possess one ureter . •The fused kidney occupies prevertebral or presacral space . •The cake kidney may remain asymptomatic and be detected at autopsy. •It may become infected or may cause local pain from dragging on the renal vessels by the weight of the organ
  • 40. CONGENITAL CYSTIC RENAL DISEASES • Renal cysts detected in utero and after birth has been a topic of considerable confusion. • A standardized step-by-step sonographic approach is most helpful in differentiating renal cystic diseases. • This approach must be combined with the clinical data, familial history, and in some cases, complementary examinations.
  • 41. Once renal cystic disease is suspected, the first step is to rule out Dysplasia associated with obstructive uropathy the features of which include: • Dilated PCS • Hyperechoic kidneys • Multiple cysts • Can be normal, enlarged or small/ Unilateral or Bilateral Next step is to consider Multicystic Dysplastic kidneys: • Multiple cysts throughout the kidney without communication. • No renal parenchyma in between • Unilateral • Atretic ureter • Vascular pedicle may also be absent or atretic
  • 42. Both obstructive dysplasia and MCDK should be differentiated from complex cysts, which include cystic tumors like renal lymphangioma
  • 43. If the foregoing diagnoses are unlikely, inherited renal cystic disease should be considered. If no familial history is present, cases are further split  patients with renal findings limited to the kidney  renal anomalies are associated with other organ malformations Both these groups can be separated into  those with typical ultrasound patterns suggestive of specific diagnoses  those with atypical ultrasound patterns.
  • 44. The ARPKD pattern: • Bilateral, very large (> 4 SD) kidneys • Diffusely hyperechoic kidneys • Loss of CMD with or without evident cysts • Oligohydramnios is the rule • Very large hyperechoic kidneys with hyperechoic medulla (reversed CMD) is another typical pattern.
  • 45. •The next step is to rule out Glomerular cysts (GCKD) (AD): • It also includes hyperechoic and enlarged (+2 SD) kidneys without CMD but there are charecteristic multiple subcapsular as well as cortical cysts.
  • 46. Third typical pattern is Nephronophthisis: • autosomal recessive tubulointerstitial ciliopathies • urinary concentration defects and anemia At ultrasound :  the kidneys are hyperechoic, normal-sized or small, and without CMD.  renal cysts typically develop at the corticomedullary junction
  • 47. Multiple congenital syndromes are also associated with renal cysts. These should also be kept in mind while dealing with a case of renal cysts in utero or in a child . This includes : •Bardet-Biedl syndrome (BBS) •Meckel-Grüber syndrome •Joubert syndrome •Tuberous Sclerosis
  • 48. ANOMALIES OF RENAL PELVIS AND URETER - Polycalicosis and Congenital Megacalyces - Calyceal diverticula - Microcalyx - Aberrant (Ectopic) Papilla - Ureteropelvic Junction Obstruction - Congenital Megaureter - Duplex Collecting Systems
  • 49. CONGENITAL MEGACALYCES/ PUIGVERT'S DISEASE Calyces are asymmetrically dilated. Renal pelvis is normal. Some doubt its congenital nature.
  • 50. CALYCEAL DIVERTICULUM EXCRETORY PHASE • 4.5 : 1000 • Focal extrinsic dilatation of a renal calyx • In 1/3 patients : stones can form within these. DELAYED IMAGES
  • 51. URETEROPELVIC JUNCTION OBSTRUCTION • Congenital obstruction of the UPJ is a common anomaly of the urinary tract. • The disorder produces caliectasis and marked pelviectasis as a result of a functional narrowing of the UPJ. • In 5% cases : extrinsic compression by an aberrant renal artery • Males (2:1), Left sided • Commonly presents as an abdominal mass in a neonate. • In some cases, symptoms may present in the setting of a sustained diuresis, a condition that has become known as "beer-drinker's hydronephrosis."
  • 52. On urography, a dilated renal pelvis and calyces will be demonstrated. Because of the dilation, slow opacification of the affected side is the rule and delayed radiographs are usually necessary. DIFFERENTIATING UPJ FROM EXTRA RENAL PELVIS IS IMPORTANT. Normal calyces Dilated calyces RGP RGP
  • 53. • USG and CT reveal similar findings. • In patients with equivocal UPJ obstruction, or in cases where there is a discrepancy between the patient's clinical symptoms and the radiologic findings, diuresis renography or the Whitaker procedure may be employed.
  • 54. DUPLICATION OF THE URETER : • Results from early splitting of the ureteric bud . • Duplication can be variable. At one end of the spectrum there is merely duplication of the renal pelvis, draining via a single ureter. At the other extreme, two separate collecting systems drain independently into the bladder or ectopically (see below) • Duplex systems may be unilateral (more common) or bilateral. •Partial or complete. Duplication of a renal pelvis (1 in 40) : Duplication of a renal pelvis is the most common congenital abnormality of the upper renal tract .
  • 55. . The upper pole ureter The lower pole moiety • drains inferomedially • can drain ectopically • usually associated with an ureterocele • drains superolaterally • is usually associated with VUR • CT is able to delineate essentially all abnormalities, especially when performed during the excretory phase. A duplicated renal collecting system can be suspected by identifying the so called faceless kidney .
  • 58. PRIMARY MEGAURETER : • Megaureter is a generic term indicating the presence of an enlarged ureter with or without concomitant megacalyces. • The normal ureter in children rarely exceeds 5 mm in diameter . In practice, a ureter with a diameter of 7 mm or more should be considered a megaureter . • There are three major categories of primary megaureter: obstructed primary megaureter refluxing primary megaureter nonrefluxing unobstructed primary megaureter •Secondary megaureter occurs as a result of some abnormality involving the bladder or urethra (eg, urethral valves, strictures, ureteroceles etc)
  • 59.  Obstructed primary megaureter: a condition akin to achalasia and Hirschsprung disease. The normal ureter proximal to the aperistaltic segment dilates because of relative obstruction. There is associated hydronephrosis, and active peristaltic waves can be seen on ultrasound.  Refluxing primary megaureter :is caused by a short or absent intravesical ureter, congenital paraureteric diverticulum, or other : derangement of the VUJ. Vesicoureteric reflux can be demonstrated in this.  Nonrefluxing unobstructed primary megaureter: there is neither reflux nor stenosis of the vesicoureteral junction, but the ureter is dilated beginning at a point just above the bladder . Most primary megaureter in neonates fall into this category.
  • 60. ANOMALOUS TERMINATION OF THE URETER - ECTOPIC URETER - VUR - URETEROCELE : ORTHOTOPIC OR ECTOPIC
  • 61. ECTOPIC URETER •Ectopic insertion of the ureter occurs due to abnormal ureteral bud migration and usually results in caudal ectopia. •70% are associated with complete ureteral duplication. • In the female, it can insert urethra, vestibule, or vagina. More rarely, it can empty into the uterus or Gartner duct or cyst. • In males, it empties into the lower bladder, posterior urethra, seminal vesicle, vas deferens, or ejaculatory duct. In very rare instances, it can empty into the rectum.
  • 62. VESICO URETERAL REFLUX • VUR is the abnormal flow of urine from the bladder into the upper urinary tract. • Cause :  primary maturation abnormality of the VUJ  short distal ureteric submucosal tunnel  due to an associated congenital anomaly • Reflux predisposes to renal infection (pyelonephritis). • The majority of pediatric patients who develop renal scars after a urinary tract infection have VUR. • Detection of VUR in neonates and infants is particularly important because these patients are more predisposed to the development of renal scars and renal failure than are older children.
  • 63. • Reflux is also the most common cause of antenatal hydronephrosis (40%) •The primary diagnostic procedure for evaluation of VUR is VCUG, which should be performed after the first episode of urinary tract infection. • VCUG should be used to document the presence of VUR and to determine the grade of reflux and whether reflux occurs during micturation or during bladder filling.
  • 64. Grades of VUR Based on Guidelines of the International Reflux Study Committee Echo-enhanced cystosonography has recently been proposed as a promising new method for detecting and grading VUR without exposing patients to ionizing radiation.
  • 65. URETEROCELES • Ureteroceles represent cystic dilatation of the intravesical segment of the ureter. • The defect is in the obstruction of the meatus, and the ureterocele is simply a hyperplastic response to this obstruction with the bladdder and ureteral mucosa prolapsing out. • They may be  Simple 25% - a ureterocoele that occurs at a VUJ in a normal position  Ectopic 75% - that which occurs at a VUJ whose site is abnormal (duplex ureter)
  • 66. IMAGING :  At US: the ureterocele is identified as a cystic intravesical mass, contiguous with a dilated ureter.  In IVP: demonstrates a collection of contrast material within the ureterocele : which produces the classic “cobra head” appearance consisting of a round or oval area of increased opacity surrounded by the radiolucent halo of the wall of the ureterocele.  In VCUG: a ureterocele usually manifests as a rounded filling defect within the bladder.
  • 67. PRUNE BELLY SYNDROME • is also called Eagle-Barrett syndrome • exclusively in males • Renal anomalies include Kidneys: normal or dysplastic Ureters: dilated : segmental or total / VUR Bladder: large/ Patent urachus (lacks trabeculations) Dilated posterior urethra : PUV like picture
  • 68. ABNORMALITIES OF THE BLADDER - Exstrophy - Bladder Duplication - Bladder diveriticulum - Bladder ears
  • 69. VESICAL EXSTROPHY COMPLEX • • • • Depending on the timing of the intrauterine insult, defect in lower anterior abdominal wall defect and premature perforation of the cloacal membrane results in a spectrum of diseases. 30% are epispadias, 60% are classic bladder exstrophy, and less than 10% are cloacal exstrophies. Incidence of bladder exostrophy : 0.25-0.5 per 10,000 births Males ( 2:1) Ante natal USG findings : Lower abdominal wall defect with protruding abdominal mass. Absence of the normally filled urinary bladder. No sign of oligohydramnios or other gross renal abnormalities Colour Doppler shows umbilical arteries alongside the abdominal wall mass External genitalia malformation, pubic diastasis etc
  • 70. BLADDER DUPLICATION • Can be complete or incomplete. • In incomplete bladder duplication, two bladders communicate with each other and drain into a common urethra. • Complete duplication is very rare : 40 cases in surgical literature • Duplication of the bladder may occur in the sagittal or coronal plane. The most common form is sagittal duplication with the bladders lying side by side. • Each bladder receives the ureter of the ipsilateral kidney and is drained by its own urethra.
  • 71. BLADDER DIVERTICULA • Congenital BD : occur without obstruction • Male predominance • Described as herniation of mucosa through bladder musculature. • Usually > 2cm, solitary • Types : 90% paraureteral : associated with VUR : “ Hutch Divericula” 10 % postero-lateral : not associated with VUR
  • 72. URACHAL ABNORMALITIES Patent urachus Urachal Cyst Urachal-umbilical sinus Vesicourachal diverticulum
  • 73. CONGENITAL URACHAL REMNANT ABNORMALITIES • Refers to a series of potential anomalies that can occur in association with the urachus. • There are four types of congenital urachal remnant anomalies. They are • • • • Patent urachus : commonest ~ 50 % Urachal Cyst : next commonest : ~ 30 % Urachal-umbilical sinus : ~ 15 % Vesicourachal diverticulum : ~ 5 % Usually midline : may occasionally deviate laterally to merge with one of the obliterated umbilical arteries
  • 75. ABNORMALITIES IN THE MALE URETHRA • Diverticulae • Absent phallus/ agenesis of urethra - Posterior urethral - Anterior urethral • Hypospadias • Congenital megalourethra • Congenital urethral duplication • Posterior urethral polyp • Congenital urethral fistulae • Prostatic utricle • Posterior urethral valve • Congenital meatal stenosis • Anterior urethral valve • Congenital urethral stenosis
  • 76. POSTERIOR URETHRAL VALVES • M.C congenital obstructive lesion of urethra, occurring only in phenotypic boys. • Speculated to be a result of abnormal insertion of the mesonephros into cloaca. • It courses obliquely from the verumontanum to the most distal portion of the prostatic urethra in a bicuspid or unicuspid fashion. • VUR with gross hydronephrosis, dysplastic kidneys, and urine ascites are common findings in such a patient.
  • 78. HYPOSPADIAS The urethral meatus is located on the ventral surface, anywhere from the penile shaft to the penoscrotal region
  • 79. ANTERIOR URETHRAL VALVES •These are rare congenital anomalies that cause lower urinary tract obstruction in children. •Anterior urethral valves may be found anywhere in the anterior urethra. •Most commonly in bulbar urethra.
  • 80. CONGENITAL URETHRAL DUPLICATION It is a rare anomaly. Commonly ,the duplication commonly occurs in the sagittal plane. Effmann Class. I : Blind ending I I A: double meatus I I: Patent assesory urethra I I B: single meatus III : Assesory urethra from duplicated bladder
  • 81. MULLERIAN DUCT CYSTS AND PROSTATIC UTRICLE MULLERIAN DUCT CYSTS • Nonatrophy of the mullerian duct may produce cystic dilations along the route of the vas deferens from the scrotum to the ejaculatory ducts . • Mullerian duct cysts are rare, but most commonly occur in the midline just above the prostate. PROSTATIC UTRICLE • The prostatic utricle is a small, blind-ending midline pouch arising from the prostatic urethra at the level of the verumontanum. • The normal prostatic utricle is 8 to 10 mm in length, narrow at its orifice (2 mm). • It may be associated with urinary retention, stasis, and infection.
  • 82. URETHRAL DIVERTICULUM • Anterior urethral diverticula : obstruction • Posterior urethral diverticula : complicated by urinary stasis, infection, and calculi formation.
  • 83. CONGENITAL MEGALOURETHRA This is a rare congenital anomaly resulting from the faulty development of the corpora cavernosa and corpus spongiosum. Two types are described. Milder form : scaphoid variety. More severe form : fusiform variety
  • 85. MULLERIAN DUCT ANOMALIES Phase of Organogenesis : one or both ducts may not develop completely leading to complete agenesis / hypoplasia / unicornuate anomalies. Phase of lateral fusion : where the lower segments of the Mullerian ducts fuse to form uterus, cervix and upper 4/5 ths vagina. Failure of this could lead to Bicornuate or Didelphys anomalies. Vertical fusion is referred to ascending sinovaginal bulb fusion to descending Mullerian system. Incomplete fusion would produce transverse vaginal septum. Septal resorption : After lower Mullerian system fuses, septum which is present gets resorbed naturally. Failure leads to septate uterus anomaly.
  • 86. • Developed by the American Fertility Society (AFS) • Developmental anomalies of the renal system is common. Renal agenesis is the most commonly reported anomaly, ( 67% ). • Many of the anomalies are initially diagnosed at HSG and USG ; however MR imaging is the study of choice because of its high accuracy and detailed elaboration of uterovaginal anatomy. • Three-dimensional ultrasound with multiplanar imaging has been shown to be more accurate than plain TVS. • Laparoscopy and hysteroscopy are reserved for women in whom interventional therapy is likely to be undertaken.
  • 87. CLASS I — AGENESIS/HYPOPLASIA (5-10% ) • Early developmental failure of the Mullerian ducts, results in various degrees of agenesis or hypoplasia of the uterus, cervix and upper 4/5 ths of the vagina. • In agenesis a uterus is not identified or small amounts of rudimentary tissue without differentiation may be identified. • The most common form is the Mayer–Rokitansky– Kuster– Hauser syndrome.  90% cases : vaginal + uterine agenesis  10% cases : isolated vaginal agenesis with an obstructed uterus or small rudimentary uterus. On US images, a normal uterus cannot be identified. The ovaries often are normally situated.
  • 88. On MR images, uterine agenesis and hypoplasia are best characterized on sagittal images, while vaginal agenesis is best demonstrated on transverse images. Agenesis results in no identifiable uterus. Uterine hypoplasia demonstrates small uterus with abnormal low-signal-intensity myometrium on T2-weighted images and poorly delineated zonal anatomy.
  • 89. CLASS II — UNICORNUATE (20%) This anomaly results from complete or near-complete arrested development of one of the Mullerian ducts. Four possible subtypes can develop: (i)absent horn (ii) non-cavitary (non-functional) rudimentary horn (iii) cavitary communicating rudimentary horn and (iv) cavitary non-communicating rudimentary horn. The embryologic predominance of the unicornuate uterus to be on the RIGHT has not been explained.
  • 90. HSG : • Fusiform shape : tapering at the apex • Draining into a solitary fallopian tube • The uterus is generally shifted off of midline
  • 91. USG :
  • 92. On MR images: • Unicornuate uterus appears curved and elongated • The external uterine contour assumes a banana shape. • Uterine volume is reduced. •The endometrium may be uniformly narrow or may assume a bullet shape, tapering at the apex. • Normal myometrial zonal anatomy is maintained. • The endometrial-to-myometrial width and ratio are reported to be normal .
  • 93. CLASS III — DIDELPHYS (5%) • This anomaly results from complete non-fusion of both Mullerian ducts. • The individual horns are fully developed and almost normal in size. • Each mullerian duct develops its own hemiuterus and cervix and demonstrates normal zonal anatomy with a minor degree of fusion at the level of the cervices. NO COMMUNICATION IS PRESENT BETWEEN THE DUPLICATED ENDOMETRIAL CAVITIES. • A longitudinal vaginal septum is associated in 75% of these anomalies .
  • 94. On USG : • Separate divergent uterine horns are identified • With a large fundal cleft • Endometrial cavities are uniformly separate, with no evidence of communication.
  • 96. • Some patients with uterus didelphys present with a unilateral hemivaginal septum, which may result in obstruction with consequent hematometrocolpos . • Majority of cases reported in the literature are associated with renal agenesis on the same side as the obstructing transverse hemivaginal septum. This syndrome has been referred to as OBSTRUCTED HEMIVAGINA–IPSILATERAL RENAL AGENESIS ANOMALY.
  • 97. CLASS IV — BICORNUATE (10%) • This class is characterised by partial non-fusion of the Mullerian ducts. • This results in a central myometrium separating the uterus that may extend to the level of the internal cervical os (BICORNUATE UNICOLLIS) or external os (BICORNUATE BICOLLIS). The fundal cleft is usually > 1 cm deep. • The horns of the bicornuate uteri are not as fully developed and are smaller than those in the didelphys uteri.
  • 98. Ultrasound demonstrates two uterine horns (arrows) and a single cervix
  • 99. The horns demonstrate normal uterine zonal anatomy. The endometrial-to-myometrial ratio and width are normal in appearance.
  • 100. CLASS V — SEPTATE (55%) • This class of anomaly occurs when the final fibrous septum between the two Mullerian ducts fails to resorb. • This results in the formation of a uterus that is completely or partially divided into two cavities. • The septum may be muscular or fibrous or a combination of both. • This class is associated with the poorest obstetrical outcomes. It has been theorized that the decreased connective tissue may result in POOR DECIDUALIZATION AND IMPLANTATION, while increased muscular tissue may result in INCREASED CONTRACTILITY of the tissue, thereby predisposing the patient to spontaneous abortion. • It is important to distinguish a fibrous septum from a muscular septum, as the former can be repaired by a hysteroscopic approach, whereas the latter may require a transabdominal surgical approach.
  • 101. US: The echogenic endometrial cavities are separated at the fundus by the intermediate echogenicity of the myometrium and by a hypoechoic fibrous segment caudally. COMPLETE
  • 103. • The differentiation between a septate and bicornuate uterus is important because they differ in their reproductive prognosis and treatment. Bicornuate Features Septate Uterus Uterus • Depth of fundal cleft • Fundal contour • Intercornual angle • Intercornual distance • Intercornual tissue • < 1 cm • > 1 cm • Convex/ flat • Concave • <75 degrees • <4 cm • Fibrous or myometrial • >105 degrees • >4 cm • Myometrial
  • 104. CLASS VI — ARCUATE • This group is characterised by mild indentation of the endometrium at the uterine fundus . • It is the result of near complete resorption of the uterovaginal septum. • Currently, No Definitive Depth Has Been Established To Differentiate The Arcuate Configuration From The Septate. • This class is highly controversial, as it remains unclear whether this variant should be classified as a true anomaly or as an anatomic variant of normal. • Data regarding the reproductive outcome of patients in this category are extremely limited and conflicting. Currently, it is generally thought that an arcuate uterus is compatible with normal pregnancy and delivery.
  • 105. CLASS VII — DIETHYLSTILBESTROL RELATED •Several million women were treated with diethylstilbestrol (DES), a non-steroidal oestrogen, to prevent miscarriage between 1945 and 1970 . •The drug was promptly removed from the market when it was foundthat up to 15% of newborn girls who were exposed to DES had UTERINE MALFORMATIONS and an increased risk of VAGINAL CLEAR CELL CARCINOMA . •The uterine abnormalities include hypoplasia and a T-shaped uterine cavity . • Patients may also have abnormal transverse ridges, pseudopolyps and stenoses of the cervix.
  • 107. TRANSVERSE VAGINAL SEPTUM • Defects of vertical fusion results in a transverse vaginal septum. • It can occur anywhere along the vagina, although it occurs most frequently at the junction of the upper and middle third. •The septum is a membrane of fibrous connective tissue with vascular and muscular components . • There is resultant hematocolpos and hematometra, which is usually less striking, secondary to decreased distensibility of the more muscular myometrium.
  • 108. UNDESCENDED AND ECTOPIC TESTES •Undescended testis(UDT) is a common genitourinary anomaly in which the testis fails to descend completely and is located along the normal pathway of descent. •Ectopia testis on the other hand is rare with an incidence of <1% of all cases of undescended testes. •Usg is the primary modality. However, if the testis cannot be localised by USG MRI is used. •The testicle is typically low signal on T1 and high signal on T2. The normal testicle exhibits intense diffusion restriction, and these sequences can aid confident identification.
  • 109. DISORDERS OF SEXUAL DEVELOPMENT - Male pseudohermaphroditism - Female pseudohermaphroditism - True hermaphrodite - Mixed gonadal dysgenesis - Pure gonadal dysgenesis - Persistent mullerian duct syndrome
  • 110. AMBIGUOUS GENITALIA INTERNAL MALE PSEUDO HERMAPHRODITE
  • 111. CONCLUSION Radiologic investigation continues to be one of the most important sources of clinical information in the evaluation of urinary and genital tract disorders. The role of diagnostic imaging is to help determine as closely as possible the exact nature of the abnormality. But the need for radiologic examinations should be carefully weighed to avoid inconveniencing the patient, exposing the patient to unnecessary radiation, or delaying surgical correction.
  • 112. “In the field of scientific observation, chance favours the prepared mind” Dr. Louis Pasteur Thank you