Embryology of genitourinary system

Embryology of genitourinary system by Dr. Bom BC

1. Dr Bom B. C.
1
st
Year Resident
MD Radiodianosis
Development of Urinary System
and Important Congenital Lesions

2. Urogenitalsystem develops from
intermediate cell mass of mesoderm.

3. Development of kidneys and
ureters
Intermediate cell mass
forms
pronephros,mesonephros
and metanephros in
craniocaudal direction.

4. Pronephros
Situated in cervical and upper
thoracic region.
Consists of 7-8 segmentally
arranged pronephric tubules
(segmented part of intermediate cell
masss form nephrotomes, distal
unsegmented part forms
nephrogenic cord)

5. Parts of pronepros
1.Pronephric duct:extends caudally
along the dorsal surface of
nephrogenic cord and finally opens
into ventral part of cloaca.
2.Pronephric tubule:horizontal,connects
pronephric duct to intraembryonic
coelom.

6. Fate of pronephros
All pronephric tubules and cephalic
part of pronephric duct disappears.
Lower part of pronephric duct
persists as mesonephric duct.

7. Mesonephros
Lower thoracic and lumbar region
70-80 nonsegmental mesonephric
tubules open into remaining part of
pronephric duct---duct is now called
mesonephric duct(wolffian duct)

8. Fate of mesonephros
Male :
Efferent ductules of testes from
tubules.
Canal of epididymis ,vas deferens
,seminal vesicle,ejaculatory duct
from duct.
Female:
Most of tubules disappear.
Some persist in rudimentary forms
as tubules of epoophoron and

10. Metanephros
Lumbosacral segment
Persists as permanent kidney.
Parts:
1.Collecting part
2.Excretory part

11. Collecting part
5
th
week ,ureteric bud grows out from
caudal part of mesonephric duct. It runs
dorsally and cranially to invade the caudal
end of nephrogenic cord.
Nephrogenic cord forms a cap like
investment –metanephric blastema.
Distal end of ureteric bud divides into
cranial and caudal end –future major
calyces—divides repeatedly 13 or more
generations.
2
nd
,3
rd
,4
th
order absorbed –minor calyces.
All branches from 5
th
and subsequent
orders form collecting tubules. Stalk –
ureter, distal dilated end-pelvis

12. Excretory part
Develops from metanephric blastema
1
st
order ureteric bud –capped with bilaminar
metanephric blasatema. On further
subdivision –the cap breaks to form clusters
of cells on each side of tubule-renal vesicle-
one end abut CT, other dilated end forms
glomerulus.
Intermediate portion-PCT, DCT,loop of Henle
Renal vesicle and tubule coalesce connecting
nephrons with collecting tubules.
By 9
th
week metanephric kidneys start
functioning.

16. Ascent of kidney
1.Pelvic cavity-median sacral artery
2.Iliac fossa-CIA,IIA
3.Undersurface of diaphragm-
lowest suprarenal artery

17. Rotation of kidney
During ascent ,hilum vertical .when
reaches in permanent position
undergoes medial rotation around
vertical axis –hilum turns medially

18. Developmental abnormalities
and varients
Foetallobulation:
5%
Enitrekidney or just middle and lower third
Frequently bilateral
No Clinical significance
Can be confused with scars of Reflux
Nephropathy

20. Renal Pseudotumors
Prominient areas of normal renal tissue which
may appear as mass lesion
No clinical significancce but may be
misdiagnosed as Neoplastic masses
Column of Bertin-Due to prominient column of
normal renal parenchyma usually at the junction
of upper and ,iddle thirds of kidney
Dromedary or Splenic Hump:

21. Hypertrophied column of Bertin
(HCB)
7. Similar color flow to surrounding parenchyma

22. (A) Longitudinal renal ultrasound scan
showing a prominent column of Bertin (arrow).
(B) Transverse scan showing the same feature
(arrow).

24. Agenesis of kidney:
Failure of the ureteric bud to reach the metanephros
Ipsilateral ureter and hemitrigone also fail to develop
10%-ipsilateral adrenal agenesis, rest hypertrophy
Associations-absence of vas deferens,unicornuate
uterus,absence or cyst of seminal vesicle
Classically ass.with VATER
Unilateral-0.1% of live births. M:F=3:1
Assymptomtic.but increased incidence of anomalies
of contralateral kidney eg
malrotation,ectopia.contralateral kidney usually
hypertrophy

25. Plain film-absence of renal outline,medial
displacement of splenic or hepatic
flexure into the renal bed
Usg and IVU-absenceof kidney on one
side and hypertrophy on contralateral
side
CT and radionuclide study-absence of
renel tissue definite
D/D-ectopia,nephrectomy,severly
atrophic kidney after insult
B/L-1/3000 live births-oligohydramnios
with potter’s syndrome.incompatible to

26. Renal ectopia
Failure of complete ascent of kidney to level
of L2
Kidney lies anywhere from pelvis upwards
Relatively common, Often small size.
Over ascent rare, usually limited by
diaphragm but there can be true intrathoracic
kidney
Usually anomalous blood supply
Pelvic kidney-1/1000 live births .M:F=1.5:1
increased risk of trauma/VUR/calculus
formation.
Increased incidence of anomaly of

28. Pelvic kidney

29. Crossed fused ectopia

31. Crossed nonfused ectopia

32. Horse shoe kidney
Common.1/400 live births M:F=2:1
Due to in uterocontact of metanephrictissue
with midline connection of lower poles.
The connecting band may be fibrous to block
of renal tissue.
Due to fusion Kidney is malrotated, Renal
Pelvis anteriorlyand Lower calyces more
medially
Low position of Kidneys due to as ascent
prevented by renal tissue encountering the
inf. Mesenteric artery at midline

33. Association and Complications
1.Associated malrotation, accesoryrenal arterie
and PUJ obstruction.
2.Other Associations include anomalies of
Cardiovascular and GIT anomalies
increased risk of Wilm’stumor,
Medullarysponge kidney,
Ellis-van Creveldsyndrome
Turner’s Syndrome etc
3.Increased incidence of renal infection and
calculi & injury due to low position

34. Plain film-renal outline low lying and
lower poles medially rotated
IVU-best seen in nephrogram phase-low
lying kidneys with upper poles pointing
superolaterally and lower poles
inferomedially.uper poles usually at the
same level
PCS point anteriorly and show fullness
USG/CT/MRI

36. Plain radiograph of the abdomen shows calcific opacities in
the region of left lower renal pole.

37. USG

39. CT

40. Renal vascular anomalies
As kidneys ascend they lose arteries from iliac
arteries and acquire arteries from aorta.
Failure of involution of these arteries –
common developmental variant (25%)-most
commonly small lower pole artery.
Very important while doing partial
nephrectomy, pyeloplasty, renal
transplantation, embolization etc.
Common in horse shoe and crossed fused
ectopic kidney.
Accessory renal veins-1/8 live births

42. Duplication abnormalities
Common.10% of population
Most minor form is bifid
pelvis(normal variant)
Otherwise 2 ureters and renal pelvis
and even upto 6 have been
reported-duplex ureter may be
complete or incomplete.

43. Incomplete-no clinical
significance.sometimes yo-yo
reflux-loin pain on micturition and
UTI
Completely duplicated ureters –
potential problems eg PUJ
obstructon
Lower moety drains in normal
position,upper moety drains distal to
the lower moety

44. In male never below ext.sphncture so no
incontinence.
In females insertion into vagina or
urethra may occur and lead to
continuous incontinence and ascending
infections.
Stenosis of orifice.ureterocele
common.VUR much less common in
upper moety
Uncomplicated duplex kidney enlarged
on all imaging modalities.

45. IVU : -2 collecting systems identified
USG-sinus fat surrounding the collecting
systems of two moeties and renal
parenchyma separting them.
Analogous appearance in CT n MRI.
Signs of reflux nephropathy(cortical
scarring and clubbed calyces) may be
seen,most often affecting the lower
moety.

46. If the upper moety is severely
obstructed,it becomes hydronephrotic
with diffuse cortical loss.
Upper moety may not opacify or may
opacify late and hydronephrotic pelvis
may displace the lower moety inferiorly
and giving rise to the so called drooping
lily sign
Incomplete duplex-assymptomatic.yo-yo
reflux

47. Excretory urography in a woman shows complete ureteral duplication on
the right. The upper moiety ureter empties below and medial to the
ureter of the lower moiety.

48. Excretory urography in a man shows a blind-ending ureteric bud on the left
arising from the bladder.

49. Excretory urography in an adult patient with bilateral
complete ureteral duplication.

50. Ureterocele F:M =4:1
Submucosal dilation of the intramural distal ureter.
They often project into the bladder lumen
May become large and may obstruct the contralateral
ureter of the duplex system or even the urethral
orrifice, giving rise to B/L hydronephrosis.
Most are associated with the upper moety ureter of
duplex system n therefore ectopic.
Have a strong tendency to obstruction sometimes
severe hydroureter and hydronephrosis
Those not asso. with duplex system though
congenital present, in adulthood are often as
incidental finding

51. They tend to be small and may be associted with UTI
and calculus
No obstruction until cmplicated by calculus formation
IVU-contrast filled structure with a smooth
radioluscent wall surrounded by contrast filled
bladder-cobra’s head appearance
If the ureterocele is obstructed and associated kidney
nonfunctioning,it appears as well defined radiolucent
mass within the opacified bladder
USG-it apears as a thin walled purely cystic str
projecting into the bladder lumen at the site of ureteric
insertion
Ass.ureteric calculus and dilation as well as
hydronephrosis may be seen

53. Transverse sonogram of the pelvis depicts the bladder with a ureterocele.
The cyst within a cyst is a pathognomonic radiologic sign of ureterocele.

54. Longitudinal sonogram of the
pelvis depicts the submucosal
course of the dilated ureter within
the bladder. The dilated ureter
ends in the ureterocele.

55. Ureterocele. Intravenous
urogram shows the bladder
filled with contrast material and
duplicated ureters inserting
into the bladder. The rim of
radiolucency surrounding the
insertion of the left ureter into
the bladder is diagnostic of
ureterocele. This also is called
the cobra-head deformity

56. Ureterocele. Delayed CT
scan obtained after the
administration of contrast
material shows bilateral
ureteroceles filled with
contrast agent within the
bladder.

57. Multicystic kidney
Relatively common condition.
Failure of the ureteric bud to connect with the
nephrons in the metanephric blastema in utero
Ureter fails to develop and is atretic, kidney non
functional.
USG/CT-non communicating cysts of variable size
Hydronephrotic multicystic kidney-a variant, only
upper ureter is atretic and cysts are arranged aroud a
large central cyst,with which they may communicate.
Ass. With an increased risk of contralateral PUJ
obstruction

60. Polycystic kidneys
Polystic kidney disease
Tuberous sclerosis
Von hippel lindau syndrome

61. Polycystic Kidney Disease
Two major types: Autosomal Dominant
Polycystic Kidney Disease (Adult Polycystic
Kidney Disease [APKD]
Autosomal Recessive Polycystic Kidney Disease
(Infantile Polycystic Kidney Disease)

62. Autosomal Dominant
Polycystic Kidney Disease
Is a slowly progressive disease with
nearly 100% penetrance
Cause: gene located on short arm of
chromosome 16 (in 90%
Spontaneous mutation in 10%
Incidence:1:1,000 people carry the
mutant gene

63. One of the common causes of chronic renal
failure
Histo: abnormal rate of tubule divisions (Potter
Type III) with hypoplasia of portions of tubules
left behind as the ureteral bud advances;
cystic dilatation of Bowman capsule, loop of
Henle, proximal convoluted tubule, coexisting
with normal tissue

64. Mean age at diagnosis: 43 years (neonatal /
infantile onset has been reported)
M:F = 1:1
Onset of cyst formation:
54% in 1st decade
72% in 2nd decade
86% in 3rd decade

65. Associated with: Cysts in: liver (50%),
pancreas (10 %)
Aneurysm: saccular "berry" aneurysm of
cerebral arteries (15%)
Mitral valve prolapse
Hypertension (50-70%)
Azotemia

66. Swiss cheese" nephrogram= multiple lesions of
varying size with smooth margins
Polycystic kidneys shrink after beginning of renal
failure, after renal transplantation, or on chronic
hemodialysis
NUC: poor renal function on Tc-99m DTPA scan

67. USG-Multiple cysts in cortical
region (usually not seen prior to
teens)
Diffusely echogenic when cysts
small (children)
Renal contour poorly demarcated

68. OB-US-Large echogenic kidneys similar to
infantile PCKD (usually in 3rd trimester,
earliest sonographic diagnosis at 14 weeks),
can be unilateral
Macroscopic cysts (rare)
Normal amount of amniotic fluid /
oligohydramnios (renal function usually not
impaired

69. Complications-
Death from uremia / cerebral
hemorrhage (secondary to hypertension
or ruptured aneurysm
cardiac complications (mean age 50
years)
Renal calculi
Urinary tract infection
Cyst rupture
Hemorrhage
Renal cell carcinoma (increased risk)

70. Differential Diagnosis
Multiple simple cysts (less diffuse, no family
history)
von Hippel-Lindau disease (cerebellar
hemangioblastoma, retinal hemangiomas,
occasionally pheochromocytomas
Acquired uremic cystic disease (kidneys small, no
renal function, transplant)

71. Due to this,some cysts become
thick walled,septated,calcified and
contain echogenic material
CT n MRI-cysts of varying size
Sometimes a coexistent malignancy
is extremely difficult to diagnose n
serial imaging is required

75. Autosomal dominant polycystic kidney
disease: IVU shows enlarged kidneys with
pelvicalyceal distortion consistent with

77. Autosomal Recessive
Polycystic Kidney Disease
Incidence:1: 6,000 to 1:50,000 livebirths
F > M; carrier frequency of 1:112
Pathology
-Kidney: abnormal proliferation +
dilatation of collecting tubules resulting
in multiple 1-to 2-mm cysts
Liver: periportal fibrosis often with
abnormal proliferation + dilatation of bile
ducts
Pancreas: pancreatic fibrosis

78. ANTENATAL FORM (most common)
90% of tubules show cystic changes
Onset of renal failure in utero
Potter sequence
Oligohydramnios and dystocia (large
abdominal mass)
Prognosis: death from renal failure /
respiratory insufficiency (pulmonary
hypoplasia) within 24 hours in 75%, within 1
year in 93%; uniformly fatal

79. NEONATAL FORM -60% of tubules show
ectasia + minimal hepatic fibrosis + bile duct
proliferation
Onset of renal failure within 1st month of life
Prognosis: death from renal failure / hypertension
/ left ventricular failure within 1st year of life

80. INFANTILE FORM-20% of renal tubules
involved + mild / moderate periportal fibrosis
Disease appears by 3-6 months of age
Prognosis: death from chronic renal failure /
systemic arterial hypertension / portal
hypertension

81. JUVENILE FORM-10% of tubules involved +
gross hepatic fibrosis + bile duct proliferation
Disease appears at 1-5 years of age
Prognosis: death from portal hypertension The
less severe the renal findings, the more severe
the hepatic findings!

82. Lung
Severe pulmonary hypoplasia
Pneumothorax / pneumomediastinum

83. Liver· Portal venous
hypertension·Tubular cystic dilatation of
small intrahepatic bile ducts·Increase in liver
echogenicity (from congenital hepatic fibrosis

84. Kidneys
· Bilateral gross renal enlargement
· Faint nephrogram + blotchy opacification
on initial images
· Increasingly dense nephrogram
· Poor visualization of collecting system
· "Sunburst nephrogram" = striated
nephrogram with persistent radiating opaque
streaks (collecting ducts) on Delayed images
· Prominent fetal lobulation

85.  CT
Prolonged corticomedullary phase

86. US
· Hyperechoic enlarged kidneys (unresolved 1-to
2-mm cystic / ectatic dilatation of renal tubules
increase number of acoustic interfaces)
· Increased renal through-transmission (high fluid
content of cysts)
· Loss of corticomedullary differentiation, poor
visualization of renal sinus + renal borders
· Occasionally discrete macroscopic cysts <1 cm
· Compressed / minimally dilated collecting system

87. OB-US (diagnostic as early as 17 weeks GA):
· Progressive renal enlargement with renal
circumference : abdominal circumference ratio >0.30
· Hyperechoic renal parenchyma
· Nonvisualization of urine in fetal bladder (in
severe cases)
· Oligohydramnios (33%)
· Small fetal thorax

88. OB management
· Chromosome studies to determine if other
malformations present (e.g., trisomy 13 / 18)
· Option of pregnancy termination <24
weeks
· Nonintervention for fetal distress >24
weeks if severe oligohydramnios present
· Risk of recurrence:25%
· DDx: Meckel-Gruber syndrome, adult
polycystic kidney disease

89. ARPKD

90. Infantile pkd

91. Von Hippel Lindau Syndrome
Autosomal dominant NCS of incomplete
penetrance and variable expressivity.
High incidence of multiple cysts in various
organs
Kidney-75%,pancreas-50%
Liver n spleen etc
Tumors in different organ system-RCC(25-
40%,75% multifocal),cerebellar
hemangioblastoma,retinal angioma,pancreatic
adeno ca and adenoma,endocrine
tumors(phaeochromocytoma and islet cell
tumor

92. Medullary cystic disease
Characterized by medulary cysts
Progressive tubular atrophy with
salt wasting nephropathy and renal
failure

93. CT image of the kidney obtained immediately after the intravenous administration of
contrast agent. The nonenhanced medullary cysts are seen clearly against the
background of enhancing renal cortex.

94. Medullary sponge kidney
Common condition
Fusiform or cystic ectasia of collecting ducts
within renal pyramids
1/200 IVUS
Generally bilateral but may be unilateral and
segmental
Usually a benign incidental finding but there is
weak ass. With some tumors eg wilm’s tumor
and phaeochromocytoma,developmental
lesions eg horse shoe kidney,distal RTA,and
hemihypertrophy,caroli’s and ehlers danlos

95. IVU-multiple linear,sometimes saccular contrast
collections within the medulla
-should be differentiated from papillary blush
Assymptomatic and no USG features
Small calculi may form within the ectatic tubules-
subtle or nephrocalcinosis

96. Contrast medium collecting in ectatic
tubules in large medullary sponge
kidneys.

97. Control image from an intravenous urographic series in a 35-year-old
woman who presented with painless hematuria. Plain radiograph shows
bilateral tiny rounded calcifications corresponding to the renal pyramids
suggestive of medullary sponge kidney.

98. Image from an intravenous urographic series in a 35-year-old woman who
presented with painless hematuria (same patient as in Image 1). Image
obtained at 15 minutes after the intravenous injection of contrast material
shows collection of the contrast agent in the ectatic renal collecting tubules

99. Image from an intravenous urographic series in a 50-year-old woman with
abdominal pain (same patient as in Image 3). Compression view of the kidneys
obtained at 15 minutes after shows linear striations of contrast material that
opacifies the collecting tubules, giving a paint brush like appearance that is typical
of medullary sponge kidney.

100. Image from an intravenous urographic series in a 40-year-old man
obtained after a 15-minute delay shows contrast agent accumulation in the
collecting tubules

101. Sonogram of a kidney in a patient with medullary sponge kidney shows a
hyperechoic medulla associated with echogenic foci, some of which are casting
shadows. A hyperechoic medulla can also be seen in conditions causing
hyperuricemia and hypokalemia.

102. Axial CT scan of the kidneys in a 21-year-old woman with medullary sponge kidney.
Delayed contrast-enhanced scan shows persistence of the contrast enhancement in
the renal collecting tubules, a finding typical of medullary sponge kidney.

103. Delayed contrast-enhanced image of the kidney in an adult man (same patient as in
Images 11-15). Note the gradual pooling of contrast agent in the cystlike collecting
tubules.

104. Vesicoureteric reflux

105. I: reflux into ureter alone
Ia: part of ureter only Ib: whole of the ureter Ic: with
dilatation
II: reflux into ureter and pelvis
Incomplete opacification IIa: without and IIb: with
focal dilatation, IIc: complete opacification
III: reflux into ureter and pelvis with mild dilatation
IIIa: Fornices preserved IIIb: mildly blunted

106. IV: reflux into ureter and pelvis with moderate
dilatation and preservation of papillae
IVa partial obliteration and IVb complete obliteration
V: reflux into ureter and pelvis with obliteration of
papillae
Va: subtotal papillary obliteration, total paillary
obliteration with severe Vb and extreme Vc
pelviureteric dilatation
Ass. anomalies:
Duplex kidneys, COPUM (cong. Obstructive
posterior urethral membrane), bulbar urethral
obstruction, ureteral partial obstruction

108. Vur grd III

110. Adult reflux nephropathy
After surgical procedures
Ileal conduit
ureterosigmoidostomy

111. Posterior urethral valve
{> 6 mm dilatation}
PUV represents a spectrum of severity, ranging
from disease incompatible with postnatal life to
that which is minimal and may not manifest until
later in life.
Pathophysiology: During embryogenesis, the
most caudal end of the mesonephric duct is
absorbed into the primitive cloaca at the site of
the future verumontanum in the posterior urethra.

112. In healthy males, the remnants of this process
are the posterior urethral folds called plicae
colliculi.
Abnormally high insertion and fusion of these
primitive folds are believed to be the origin of
95% of PUVs, which are called type I PUV.

113. Although Young described a type II PUV, most
pediatric urologists believe that these are not
obstructing valves but simply hypertrophy of
the plicae colliculi {from distal ureteric orifice
to veromontenum}in response to a more
distal obstruction as observed in types I
and/or III PUV
Also described by Young, type III PUV
constitutes a septum at the junction of the
posterior and anterior urethra {slit like orifice
in membranous urethra} k/a Cobb’s collar
instead of a sail-like valve. Type III is believed
to originate from incomplete dissolution of the
urogenital membrane.

114. Approximately 10-15% of children undergoing renal
transplant have PUV as the cause of renal
insufficiency, and approximately one third of patients
born with PUV progress to end-stage renal disease
(ESRD
Moreover, children with PUV develop thickened
bladders because of increased collagen deposition
and muscle hypertrophy within the bladder wall.
Hypertrophy and hyperplasia of the detrusor muscle
and increases in connective tissue decrease bladder
compliance during filling. Bladder emptying occurs
with high intravesical pressures, which can be
transmitted to the ureters and kidneys. These patients
are susceptible to incontinence, infection, and
progressive renal damage

115. Frequency:
In the US: PUV is the most common cause of
lower urinary tract obstruction in male
neonates; reported incidence is 1 per 10,000
live births.
a/w Downs syndrome, craniospinal defects,
bowel atresia.
Mortality/Morbidity: Approximately 10-15%
of children undergoing renal transplant have
PUV as the cause of renal insufficiency, and
approximately one third of patients born with
PUV progress to ESRD.

116. Sex: PUV occurs exclusively in males. The
homolog to the male verumontanum from
which the valves originate is the female
hymen.
Age: Diagnosis usually is made before or at
birth when a boy is evaluated for antenatal
hydronephrosis. Before the era of prenatal
sonography, PUV was discovered during
evaluation of urinary tract infection (UTI),
voiding dysfunction, or renal failure. While
rare, adult presentation of PUV has been
described in case reports, with symptoms
varying from obstructive voiding symptoms to
postejaculatory dysuria. In the presonography
era, late presentation of PUV was considered
a good prognostic sign suggestive of a lesser
degree of obstruction.

117. Imaging Studies:
Renal and bladder sonography
Every child with antenatal hydronephrosis requires
renal and bladder sonography performed in the
immediate postnatal period.
Because newborns commonly have relative
hypovolemia during the first few days of life, obtain
a repeat sonogram after the first week of life if
findings on previous sonography were normal in a
child with previously diagnosed antenatal
hydronephrosis before making a final determination
that the hydronephrosis has resolved

118. Voiding cystourethrography
The key to the workup of any child with antenatal
hydronephrosis is voiding cystourethrography
(VCUG). Perform VCUG during voiding and under
fluoroscopy, with imaging of the posterior urethra.
The diagnosis of PUV is indicated by visualization
of the valve leaflets. Other clues to the diagnosis
are a thickened trabeculated bladder, a dilated (6
mm) or elongated posterior urethra, and a
hypertrophied bladder neck.

119. Renal scintigraphy
Although not necessary in every child, renal
scintigraphy may be helpful in some cases. If renal
dysplasia is suspected, nuclear imaging can
determine relative renal function. Some children
may have secondary ureterovesical junction
obstruction from bladder hypertrophy.
Tc-dimercaptosuccinic acid (DMSA),
glucoheptonate, and/or mercaptoacetyl triglycine
(MAG-3) renal scintigraphy are cortical imaging
studies that provide information about relative renal
function (each kidney relative to the other) and
intrarenal function (eg, photopenic areas within the
kidney indicate scarring or dysplasia). Additionally,
the MAG-3 renal scan with furosemide (Lasix)
provides information about renal drainage and
possible obstruction.
Other Tests:

120. Urodynamic studies
Urodynamic evaluation provides information about
bladder storage and emptying. The mature bladder
should store urine at a low pressure and then empty
completely at appropriate pressures.
The term "valve bladder" is used to describe
patients with PUV and a fibrotic noncompliant
bladder. These patients are at risk of developing
hydroureteronephrosis, progressive renal
deterioration, recurrent infections, and urinary
incontinence that persists in school-aged children.
Patients with PUV require periodic urodynamic
testing throughout childhood because bladder
compliance may deteriorate over time.

121. Procedures:
Cystoscopy serves both diagnostic and
therapeutic functions in these infants.
Appropriately infant-sized cystoscopes (<8F)
are needed to avoid injury to the urethra.
Diagnostic cystoscopy: Confirmation with
cystoscopy is required in every child in whom PUV
is suggested after VCUG. In some, the filling defect
observed on VCUG may represent only external
sphincter contraction during voiding. In others, the
valve leaflets are confirmed.
Therapeutic cystoscopy (ie, transurethral incision of
the PUVs): Currently, valves are disrupted under
direct vision by cystoscopy using an endoscopic
loop, Bugbee electrocauterization, or laser
fulguration. The objective is to relieve the
obstruction by cutting the valves at the 12-, 5-, and
7-o'clock positions. Perform this in the least
traumatic fashion to avoid secondary urethral

122. Vesicostomy: In some patients, the urethra
may be too small for the available cystoscopic
instrumentation. Fortunately, because of
continued advancements in pediatric
endoscopic equipment, this is an uncommon
occurrence. When this situation arises, a
temporary vesicostomy is performed.

125. Retrocaval ureter
Also known as “circumcaval ureter”
Abnormality in embryogenesis of IVC
Results from abnormal persistence of right
subcardinal vein positioned ventral to ureter in the
definitive IVC
Developing right ureter courses behind and medial to
the IVC
Incidence
0.07%
Male to female ratio of 3:1
Clinical findings
Symptoms of right ureteral obstruction

126. Imaging findings
Normal course of ureters
About the width of your thumb lateral to the lumbar vertebral
pedicles
About the width of two fingers medial to pelvic brim in true
pelvis
With retrocaval ureter
Right ureter’s course swings medially over pedicle of L3/4
Passes behind IVC
Then exits anteriorly between IVC and aorta returning to its
normal position
Produces varying degrees of proximal hydroureteronephrosis

131. Prune belly syndrome
Definition: Prune-belly syndrome is a rare
congenital disorder, more common in males,
consisting of deficiency of abdominal wall
muscles (absent or hypoplastic), cryptorchidism,
and genitourinary malformations.

132. Prune belly syndrome

133. Diagnosis:
The diagnosis should be suspected in fetuses
with very large abdominal masses. These are,
most typically, bladder obstruction caused by
urethral valves, urethral agenesis, but also
other abdominal masses such as ovarian cyst.
Possible results are oligohydramnios with
consequent pulmonary hypoplasia and urinary
ascites. The oligohydramnios, usually, makes
impossible the detection of cryptorchidism.
Other findings could be: Potter face,
pulmonary hypoplasia, gastric dilatation, short
bowel, micro ileum, microcolon, malrotation of
the intestines imperforate anus,
arthrogryposis and clubfoot. Additional
information can be gained by addition three-
dimensional ultrasonography scanning and
could be useful for more-efficient counseling

134. A massively
distended bladder as
in this case of
urethral agenesis is
the main cause of
Prune-belly
syndrome.

135. Bladder extrophy
(Ectopia vesicae)
Male predilection (M:F=3:1)
Cloacal membrane defect = eversion of bladder
mucosa –protrude out as mass like lesion
Infra umblical wall defect (large soft tissue mass
protruding through it) –low lying cord insertion
and absence of UB
Pubic diastases (Manta Ray sign–manta ray
swimming towards you)
Hurley stick appearanceof distal ureter =>
abnormal upward and lateral curvature of ureter.

136. Bladder
extrophy
Ass. With
Epispadiasis
cryptoorchidism
OEIS complex
Omphalocele
Bladder extrosphy
Imperforate anus
Spinal anomalies: kyphosis and hemivertebrae
females:
Clitoris cleft
Vaginal duplication

137. Floating kidney
Kidney may be suspended by a fold of peritoneum
from post abdominal wall.
Upper pole may be tilted downward -twisting of
renal vessels and pelvis -manifested by sudden,
severe pain in loin with suppression of urine –
Dietl’s crisis.

138. UROGENITAL SINUS
Hind gut divided by allatoic diverticulum into two
parts-preallantoic and postallantoic.
Postallantoic part is dilated and is called
endodermal cloaca.
Endodermal cloaca-

139. Development of genital fold, genital tubercle and
infraumbilical part of anterior abdominal wall from
the primitive streak.

141. Formation of urogenital sinus
Urorectal septum divides endodermal cloaca dorsal
primitive rectum and ventral primitive urogenital
sinus.
Urogenital sinus
Cephalic vesicourethral part
Caudal-pelvic part
oPhallic part

142. Development of urinary bladder
Development of mucous membrane
1.All mucosa except trigone-from endoderm of
vesico urethral part of urogenital sinus
2.Trigone-developed from mesoderm by
incorporation of caudal parts of mesonephric
ducts
3.Apex of bladder-From absorption of proximal
part of allantoic diverticulum

143. Musculature of UB is formed from splanchnic
layer of lateral plate mesoderm which surrounds
the cloaca.

144. Developmental anomalies
Congenital recto vaginal fistula-d/t incomplete
dev.of urorectal septum and perst.of cloacal duct
Urachal cyst:sometimes allantoic diverticulum is
obliterated at proximal and distal part but remains
patent in middle.
Urachal fistula
Ectopia vesicae

146. Dev.of male urethra
Prostatic part-Above ejaculatory duct
1.Dosal wall-from mesoderm by incorporation of
2 mesonephric duct
2.ventral part-from endoderm of vesicourethral
part of cloaca
Below ejaculatory part:-From endoderm of
pelvic part of urogenital sinus
Membranous part:derived from pelvic part of
urogenital sinus

147. Spongy part
Upto base of glans penis: develops from phallus,
primary urethral groove and urethral plate derived
from phallic part of urogenital sinus.
Parts of urethra within glans penis-developed
from ectodermal groove which appears
longitudinally on undersurface of glans penis.

149. Female urtethra
Mostly developed from vesico urethral part of
endodermal cloaca.
Corresponds in dev.with prostatic urethra above
the level of ejaculatory duct.

150. Dev.anomalies
Hypospadias
1.Balanic type-m/c,d/t failure of dev.of ectodermal
groove
2.Penile type-d/t failure of fusion of ant.part of
genital folds
3.Complete type-complete failure of fusion of genital
folds after rupture of urogenital membrane

151. Epispadias-
Ectopia vesicae-d/t failure of ventral migration of
mesodermal cells from primitive streak along
genital folds on each side of urogenital
membrane.

152. Genital system
Gonads
External genitalia
Genital ducts

153. Gonads
Dev.of testes
Developed from 3 sources-
1.Seminiferous tubules ,rete testes ,interstitial
cells,fibrous septa and intrinsic coverings of
testes –from medulla of genital
ridge(elongated structure formed medial to
mesonephric ridge by proliferation of
coelomic mesothelium)
2.Efferent tubules from 12-15 mesonephric
tubules
3.Canal of epididymis and vas deferens from
mesonephric duct.

154. Descent of testes
Each testes—retroperitoneally in dorsal
abd.wall,descends downwards gradually--just
before or after birth reaches scrotum
4
th
month IUL-iliac fossa
7
th
month-deep inguinal ring
7
th
-8
th
month-traverses inguinal canal
At or aftr birth-reaches scrotum

155. Factors for descent of testes
Gubernaculum testes
Increased intraabdominal pressure
Intraabdominal temperature
Uncurling of foetal curve
Secretion of testicular hormone

156. Anomalies of descent
Anorchism
Monorchism
Partially descended testis
Ectopia testis:in perineum,above the penis and in
front of symphysis pubis,at saphenous opening of
thigh,rarely in ASIS
Persistence of processus vaginalis
Encysted hydrocele of spermatic cord

157. Ovaries
Each ovary –developed from cortex of
undifferentiated genital ridge.
Descent of ovary: primitive ovary lies
retroperitoneally in dorsal abdominal wall and
later on it descends to its normal position

158. 

159. Genital ducts
Primitive genital duct
Mesonephric-Male-retained as duct system of
testes
Female –mostly regresses
Paramesonephric duct-Male-regresses
Female-develops into reproductive organs

162. DEV OF UTERINE TUBES
Develops from cephalic vertical and most of the
horizontal part of corresponding paramesonephric
duct.

163. DEV OF UTERUS
Caudal part of two PMD –fuse–uterovaginal
canal–uterus
Fundus of uterus develops from the distal portion
of horizontal part of PMD.

165. ANOMALIES OF UTERUS
Didelphys-complete failure of fusion of two PMD.
Two uterus, two cervix, two vagina.
Bicornis bicollis-two uterus, two cervix, single
vagina. Occurs due to partial regression of
partition from the caudal part of uterovaginal
canal.

166. Septate uterus-septum divides uterus into two
parts otherwise uterus is normal.
Arcuate uterus-fundus is concave otherwise
uterus is normal
Unicornuate uterus-unilateral supression of PMD.

167. Uterine anomalies classification

168. DEV OF VAGINA
Upper 4/5 above the hymen
Mucosa–endoderm of canalised sinovaginal bulb
which develops from evagination of mullerian duct
by endodermal cells of urogenital sinus.
Musculature-from mesoderm of united lower
vertical part of two PMD.
Lower 1/5 below hymen
From endoderm of urogenital sinus.
External vaginal orifice
From ectoderm of genital folds after rupture of
urogenital membrane.

169. Development of
Labia majora from genital swelling
Labia minora from genital folds
Scrotum in male from genital swelling.