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
11. Collecting part
5th 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.
2nd ,3rd ,4th order absorbed –minor calyces.
All branches from 5th and subsequent
orders form collecting tubules. Stalk –
ureter, distal dilated end-pelvis
12. Excretory part
Develops from metanephric blastema
1st 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 9th week metanephric kidneys start
functioning.
13.
14.
15.
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
Foetal lobulation:
5%
Enitre kidney or just middle and lower third
Frequently bilateral
No Clinical significance
Can be confused with scars of Reflux
Nephropathy
19.
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:
22. (A) Longitudinal renal ultrasound scan
showing a prominent column of Bertin (arrow).
(B) Transverse scan showing the same feature
(arrow).
23.
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-absence of 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
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.
32. Horse shoe kidney
Common.1/400 live births M:F=2:1
Due to in utero contact of metanephric tissue
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 anteriorly and 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, accesory renal arterie
and PUJ obstruction.
2.Other Associations include anomalies of
Cardiovascular and GIT anomalies
increased risk of Wilm’s tumor,
Medullary sponge kidney,
Ellis-van Creveld syndrome
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
35.
36. Plain radiograph of the abdomen shows calcific opacities in
the region of left lower renal pole.
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
41.
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
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.
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
52.
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
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
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
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
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!
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
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
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
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. 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.
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
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
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
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.
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
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.
123.
124.
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
127.
128.
129.
130.
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.
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-
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 appearance of 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.
140.
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
o Phallic 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
145.
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.
148.
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.
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
4th month IUL-iliac fossa
7th month-deep inguinal ring
7th-8th 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
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
160.
161.
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.
164.
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.
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.