CONGENITAL GU ABNORMALITIES Flashcards
Urogenital system develops from what 2 structures
- mesoderm of intermediate cell mass (genital ridge)
- urogenital sinus (ant part of cloaca)
mesoderm of intermediate cell mass (genital ridge) will develop into 3 parts and will develop into what female organs
medial - ovaries
middle - atrophy in female
lateral - FT, uterus, upper 3/4 vagina
Urogenital system will develop into
lower 1/4 of vagina and vulva
arises from persistent remnant of mesonephric duct
Located in the proximal anterolateralwall of the vagina
Usually asymptomatic
GARTNER DUCT CYST
genital ridge will develop into what organ
ovary
primordial cells will develop into
ova
sex cords will develop into
granulosa cells
Gubernaculum will develop into what organ
uteroovarian and round ligaments
mesonephric tubules will develop into
epoophoron and paroophoron
mesonephric ducts will develop into
gartner duct
paramesophric ducts
uterus, FT, upper vagina
urogenital sinus will develop into
vagina bladder urethra paraurethral glands greater bartholin and lesser vestibular glands
genital tubercle will develop into
clitoris
urogenital folds will develop into
labia minora
labioscrotal swellings will develop into
labia majora
ovary analogous in male
testis
ova analogous in male
spermatozoa
granulosa cells analogous in male
seminiferous tubules, sertoli cells
uteroovarian and round ligaments analogous in male
gubernaculum testis
epoophoron, paraoophoron analogous in male
efferent ductules, paradidymis
gartner duct analogous in male
epididymis, ductus deferens, ejaculatory duct
uterus
FT
upper vagina
analogous in male
prostatic utricle
appendix of testis
vagina analogous in male
prostatic utricle
paraurethral glands analogous in male
prostate glands
greater bartholin and lesser vestibular glands analogous in male
bulbourethral glands
clitoris analogous in male
glans penis
labia minora analogous in male
floor of penile urethra
labia majora analogous in male
scrotum
- heralded by differentiation of primordial gonad into a testis or an ovary
- if with Y chromosome, gonad develops into a testis
- TDF (testis determining factor) encoded by SRY gene on the short arm of Y chromosome
Gonadal Gender
sexual differentiation that begins at 8 weeks AOG
Phenotypic Gender
development of internal and external genitalia of male phenotype is dependent on what
testicular function
-produced by sertolicells of the seminiferous tubules
–Causes mullerianduct regression
–Prevents development of uterus , fallopian tube and vagina;
MIS (MULLERIAN INHIBITING SUBSTANCE)/ Antimullerian hormone (AMH)
- Streaked gonads
- Absent breast development
- Short stature (<60 in)
- Webbed neck
- Cubitus valgus
- Short 4th metacarpal
- Cardiac/renal abnormalities, hypothyroidism
Turner’s Syndrome
Testicular feminization (AIS- Androgen Insensitivity Syndrome) karyotype
46 XY
46 XY Testes produce estrogen Feminine, sparse pubic hair Shortened vagina Undescended testes-inguinal/abdominal Gonadectomy, ffdby Hormonal therapy; Vaginoplasty/ dilators
Testicular feminization (AIS- Androgen Insensitivity Syndrome) karyotype
upper vaginal agenesis, uterine hypoplasia or agenesis
MRKH (mayer-rokitansky-kuster-hauser) syndrome
Caused by mullerian hypoplasia or agenesis
Mullerian Agenesis
Mullerian Agenesis affects what 3 organ system
renal
skeletal
auditory
Mullerian Agenesis karyotype
46 XX
Tx of Mullerian Agenesis
–Creation of a neovagina
–Ovum retrieval via IVF: surrogate mother
Class I
Mullerian Agenesis
Maybe discovered during infertility evaluation, pelvic examination, cesarean delivery and tubal sterilization
Diagnostics: Hysterosalpingography, Laparoscopy, MRI, Hysteroscopy
Uterine Anomalies
Class II
Unicornuate uterus
- Underdeveloped or rudimentary horn of the uterus is present
- May or may not communicate with the other horn
- Dx: HSG, MRI
- Complications: ectopic gestation, miscarriage, preterm labor, Growth restriction
- Tx; Excision of horn that has a cavity (non pregnant)
Unicornuate uterus
Class III
Uterine Didelphys
- Complete lack of fusion
- 2 uterus, 2 cervix, 2 vagina
- OHVIRA (obstructed hemivagina, with ipsilateralrenal agenesis)
- DX: PE-identification of longitudinal septum, 2 cervix
- HSG: 2 endocervicalcanal
- Imaging: 3D ultrasound, MRI
- Complications: miscarriage, preterm birth
- TX: metroplasty–resection of intervening myometrium
Uterine Didelphys
Class IV
Bicornuate Uterus
- Fusion anomaly resulting in 2 hemiuteri
- Central myometrium runs partially or completely into the cervix
- Longitudinal septum not uncommon
- Dx: HSG, 3D USG, MRI
- Complications: miscarriage, preterm birth
- TX: metroplasty–resection of intervening myometrium
Bicornuate Uterus
Class V
Septate Uterus
- Resorptiondefect
- Complete or partial longitudinal uterine septum
- DX: 3D USG, MRI
- Complication: malpresentation, miscarriage, PTB
- TX: Hysteroscopic septal resection
Septate Uterus
Class VI
Arcuate uterus
- Mild derivation from normal uterus
* No increased adverse obstetrical outcomes
Arcuate uterus
Class VII
DES
- Transverse septum, circumferential ridge, cervical collar, uterus has smaller cavity, T-shaped
- Vaginal clear cell ca, CIN, small cell cervical Ca, vaginal adenosis
- > 50 years since discontinued
DES