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34 Cards in this Set

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.Development of Female External Genitalia

primordial phallus gradually ceases and it becomes
clitoris
Development of Female External Genitalia
unfused parts of the urogenital folds form
labia minora.
..Development of Female External Genitalia
labia majora, which are homologous to the
.scrotum.
Clinical Considerations
A. Male anomalies :
Hypospadias
Epispadias
Agenesis of external genitalia
Bifid penis
Micropenis
Hypospadias :
urethral folds fail to fuse completely, resulting in the external urethral orifice opening onto the ventral surface

It is generally associated with a poorly developed penis that curves ventrally, known as
chordee
Chordee exists when there is a =

It can occur in the setting of . =
Chordee exists when there is a =

It can occur in the setting of . =
.ventral curvature of the penis

hypospadias
Chordee is a contraindication to
circumcision

circumcision can be done at the time of surgical correction.
Phynotype
Genotype
Looks like
Gentically is ...
Agenesis of external genitalia
Bifid penis
Micropenis
Failure of the genital tubercle to develop

Two genital tubercles develop.

Commonly associated with hypopituitarism
B. Female anomalies : 4ct
-Anomalies of uterus: = 4ct

-Absence of vagina and uterus
-Vaginal atresia
-Imperforate hymen
►Unicornuate uterus
►Double uterus
►Bicornuate uterus
►Septate uterus anomalies
1.Unicornuate uterus anomalies:
occur when One
=
paramesonephric duct fails to
develop or incompletely
develops.

►Women with the condition may be asymptomatic and unaware of having a uniconuate uterus.

►Pregnancy: ?Normal =
Yes
Yes
1.Unicornuate  Risks:  

 % of patients delivered a live baby. 
ectopic pregnancy %, =
miscarriage%, =
preterm delivery %, =
1.Unicornuate Risks:

 % of patients delivered a live baby.
ectopic pregnancy %, =
miscarriage%, =
preterm delivery %, =
50 %

2.7%,

34 %,

20 %
Aromatase does what =
Changes Testosterone to Estrogen
patients with a unicornuate uterus are at a higher risk for =
pregnancy loss and obstetrical complications.
rudimentary horn that does not communicate with the main part of the uterus may cause =
pain during the menstrual period because of distention of the horn by blood.
Most anomalies resulting from incomplete fusion of the paramesonephric ducts do not
cause clinical problems;


Because most rudimentary uterine horns are thicker than uterine tubes, a rudimentary horn pregnancy is likely to
rupture much later than a tubal pregnancy
Double uterus:
occur when there is a complete
lack of fusion

of the =
paramesonephric ducts.
Complications
Women with a double uterus have normal sex lives, pregnancies and deliveries.

sometimes a double uterus leads to = 2ct
infertility
miscarriage
premature birth
unusual positions
baby in the uterus (breech presentation).
Bicornuate uterus:
occur when there is =
partial fusion of the upper part of paramesonephric ducts

. As a result the caudal part of the uterus is normal while the cranial part is =
bifurcated
bifurcated
__________ __________
occur when there is partial fusion of the upper part of paramesonephric ducts. As a result the caudal part of the uterus is normal while the cranial part is bifurcated.
Bicornuate uterus:
Bicornuate uterus:
occur when there is partial fusion of the upper part of __________ ducts. As a result the caudal part of the _______ is normal while the cranial part is bifurcated.
paramesonephric

uterus
AKA: Heart-shaped uterus, where two "horns" form at the upper part of the uterus.

► Pregnancies in a bicornuate uterus are usually considered
high-risk and require extra monitoring because of association with poor reproduction potential.
.
Septate uterus:
 occur when the medial 
walls of the caudal portion 
of the =
Septate uterus:
occur when the medial
walls of the caudal portion
of the =
paramesonephric
ducts partially or
completely fail to resorb.

A septum can be resected
with surgery
Septate uterus is associated with a higher risk for =
miscarriage
premature birth
malpresentation
According to the classical study by Buttram there is a 60% risk of a spontaneous abortion, this being more common in the second than in the first trimester.However, there is no agreement on this number and other studies show a lower risk. Woelfer found that the miscarriage risk is more pronounced in the first trimester.

The condition is also associated with abnormalities of the renal system. Further, skeletal abnormalities have been linked to the condition.
.
disorder is caused by defective or absent androgen receptors.

=
Androgen insensitivity syndrome (AIS)
Androgen insensitivity syndrome (AIS)

Testosterone and estrogen levels measured in a patient’s blood are similar to male levels.

With end organ insensitivity to the testosterone, the primordial tissues develop in a =
female pattern and the patient is a =
phenotypical female.
Androgen insensitivity syndrome (AIS)

Because the patient still has testes
Location of testes =
abdominal

____ ______ is present and prevents the development of internal female structures such as the uterus, cervix, upper vagina, and fallopian tubes.
antimüllerian hormone
Androgen insensitivity syndrome (AIS)

Clinically, patients appear as a female, with normal breast development but scant axillary and pubic hair and a =
4ct
uterus
cervix
upper vagina
fallopian tubes.
Androgen insensitivity syndrome (AIS)

The disorder is caused by defective or absent =
androgen receptors.
Clinically, patients appear as a female, with normal breast development but scant axillary and pubic hair and a blind
vaginal pouch with absent uterus and cervix.
Breast tissue develops as a result of peripheral
conversion of testosterone to estrogen.
Patients with androgen insensitivity are often diagnosed after a workup for =
primary amenorrhea.