Embryology 9

GU. Female repro. 90 min


ADPKD vs ARPKD!
 Autosomal dominant
polycystic kidney
disease
 Often presents in adults
FA19p592
 Autosomal recessive
polycystic kidney
disease
Often presents in infancy
 100% mortality
 Treatment?
Autosomal recessive polycystic kidney disease
Cystic dilation of collecting ducts.
Always bilateral.
Associated with congenital hepatic fibrosis
(portal hypertension).

Significant oliguric renal failure in utero

can lead to Potter sequence.

+ Cysts of the liver, pancreas?

FA19p592
FA19p592
 FA19p588

Don’t confuse with Neuroblastoma!

Wilms tumor
Duplex collecting system
Strongly associated with vesicoureteral reflux
and/or ureteral obstruction,  risk for UTIs.
FA19p567
FA19p567
Urachus vs Vitelline duct FA19p604
 FA19p357
NUTCRACKER SYNDROME
Compression of left renal vein
between superior mesenteric
artery and aorta. Characterized
by

Clinical findings:
Abdominal (flank) pain
Gross hematuria (from rupture of
thin-walled renal varicosities)
Left testicular pain in men
Left lower quadrant pain in women
Varicocele
Exstrophy of the bladder
The urinary bladder is exposed to the
exterior.

It is caused by a failure of the anterior

abdominal wall and anterior wall of the
bladder to develop properly.

It is associated with urine drainage to

the exterior and epispadias.
Home work
 Draw the Vitilline duct vs Allantois
 Draw aortic arches derivatives
Female reproductive system
Q
 A structure found within the adult female
pelvis formed from the gubernaculum is the

(a) broad ligament

(b) suspensory ligament of the ovary
(c) round ligament of the uterus
(d) medial umbilical ligament
(e) median umbilical ligament
WEEKS 1–6 FA19p598
Embryo remains in a sexually indifferent

WEEK 7
Phenotypic sexual differentiation

WEEK 12, 10
female or male characteristics of the external
genitalia can be recognized
FA19 p598
 Week 1  Week 4
Implantation, hCG secretion Heart begins to beat.
Upper and lower limb buds begin to form.
 Week 2 4 weeks = 4 limbs and 4 heart chambers.
Bilaminar disc (epiblast, hypoblast)  Week 6
Fetal cardiac activity visible by transvaginal
 Week 3 ultrasound.
Gastrulation forms trilaminar embryonic  Week 8
disc. Epiblast invaginate > primitive streak Ž Fetal movements start
endoderm, mesoderm, ectoderm Gait at week 8
 Week 10
 Weeks 3–8 Genitalia have male/female characteristics
(embryonic period) (Tenitalia)
Organogenesis (Extremely susceptible to
teratogens).
Phenotypic sexual differentiation is determined by the
Sry gene located on the short arm of the Y
chromosome (encodes for a protein called testes-
determining factor (TDF)).

No Sry gene – no male!

THE OVARY
The intermediate mesoderm forms a
longitudinal elevation along the dorsal body
wall called the urogenital ridge, which later
forms the gonadal ridge.
RELATIVE DESCENT OF THE OVARIES
 The ovaries originally develop within the
abdomen, but later undergo a relative
descent into the pelvis.

 The gubernaculum may also play a role.

Gubernaculum
 Extends from the medial pole of the ovary to
the uterus at the junction of the uterine tubes,
forming the ovarian ligament.
 Then continues into the labia majora,
forming the round ligament of the uterus.
Gubernaculum
Clinical consideration
 Round ligament pain – by stretching.
 Sharp pain often felt in the lower belly or groin
area on one or both sides. most often felt
during the second trimester.
 Symptoms: pain
 Pubic/groin
 Bladder
 Uterus
 Lower back
 Exercise may cause the pain, as will rapid
movements.
PARAMESONEPHRIC (MÜLLERIAN) DUCTS
Gives rise to:

Uterine tubes
Uterus
Cervix
Superior 1/3 vagina.
The paramesonephric ducts project into
the dorsal wall of the cloaca and induce
the formation of the sinovaginal bulbs.
The sinovaginal bulbs fuse to form the
solid vaginal plate, which canalizes
and develops into the inferior two-
thirds of the vagina
Müllerian agenesis
Müllerian agenesis (Mayer-Rokitansky-Küster-Hauser
syndrome)—may present as 1° amenorrhea (due to a lack
of uterine development) in females with fully developed
2° sexual characteristics (functional ovaries).
Low yield?
 Vestigial remnants of the
paramesonephric duct may be
found in the adult female and
are called the hydatid of
Morgagni (1).
Higher yield
 Vestigial remnants of the
mesonephric ducts/tubules may
be found in the adult female,
called the appendix vesiculosa
and
 Gartner’s duct (5)
 Epoophoron (3)
 Paroophoron (4)
 Hydatid of Morgagni
 Gartner’s duct
 Epoophoron
 Paroophoron

 All can form cysts!

 Why?
External genitalia
 A proliferation of mesoderm around
the cloacal membrane causes the
overlying ectoderm to rise up so that
three structures are visible externally:
Genital tubercle (Phallus)
Urogenital folds
Labioscrotal swellings
The genital tubercle forms clitoris (glans clitoris, corpora
cavernosa clitoris, and vestibular bulbs).
FA19p609
Diethylstilbestrol (DES) related anomalies
For a female offspring
exposed to DES in utero,
an increased incidence of :

Vaginal and cervical

adenocarcinoma

T-shaped uterus
Hymen variations include the following:

1. Crescentic hymen
2. Annular hymen
3. Redundant hymen
4. Imperforate hymen
5. Cribriform hymen
6. Microperforate hymen
7. Septate hymen