Normal Pelvic Anatomy and Mullerian Anomalies Flashcards
What is the cervix?
The inferior most part of the uterus
What is the corpus?
The body of the uterus - between the cervix and the fundus
What is the fundus?
The superior most part of the uterus
What is the cornua?
Part of the uterus where the Fallopian tubes enter the uterine cavity
What do internal organs develop from?
Paramesonephritic (Mullerian) Ducts and Mesonephritic (Wolffian) Ducts
What do both Wolffian and Mullerian ducts lead to?
Formation of a cloaca in a urogenital sinus
What determines if Wolffian or Mullerian ducts develop?
Hormones
What determines the development/progression of Wolffian ducts?
Testosterone and DHT
What do Sertoli Cells produce that causes regression of Mullerian Ducts?
AMH - Anti Mullerian Hormone
What produces AMH?
Sertoli Cells
What leads to the development of external femal genitalia?
Nothing - absence of androgen/AMH all that is required
What develops from the Mullerian ducts?
Tubes, Uterus, Cervix, Upper 2/3 of the Vagina
What develops from the urogenital sinus?
Lower 1/3 of the Vagina
Where is the hymen located?
At the junction of the Upper 2/3 of the Vagina from the Mullerian Ducts and the Lower 1/3 of the Vagina from the Urogenital Sinus
When does fusion of the Mullerian Ducts begin?
8-9 weeks post gestation
What follows fusion?
Canalization
When is Canalization Complete?
20 weeks
What happens to any anomalies of canalization at 20 weeks?
They stay but aren’t easily seen on U/S
What forms male internal genitalia and parts of the external genitalia?
Wolffian duct
What does the Umbilical vein become in adults?
Falciform Ligament
What does the Umbilical Artery become in adults?
Lateral suspensory ligaments of bladder
Where do you insert the laparoscope?
Umbilicus
What do you inflate the abdominal wall with?
CO2
What do you inflate the abdominal wall with CO2?
CO2 will dissolve, and be exhaled
- Room air –> Air embolism
What can be seen during a laparoscopy?
- Fundus of uterus, ovaries, and tubes
- Retroperitoneal vessels
- Utero-ovarian ligament (contains a vessel)
- Uterine Ligaments
- Infundibulopelvic ligaments (AKA Suspensory Lifgament of ovary) at pelvic brim
- Other abdominal Organs
What do the pelvic organs look like during a laparoscopy?
Upside down as you are looking at them from above instead of from below
What does the infundibulopelvic ligament (suspensory ligament of the ovary) contain?
Ovarian Artery, Veins, and Nerves
What can PID lead to?
Fitz-Hugh-Curtis Syndrome
- Adhesion of liver to diaphragm
What is endometriosis?
Discolorization of all pelvic organs due to intra-pelvic bleeds (rust-colored)
What is a hysteroscopy?
Used to look inside the uterus and to operate inside the uterus
What do you fill uterus with during hysteroscopy?
Saline
- Allows operations
- NOT CO2
What is the main etiology of Mullerian anomalies?
Environment + Genetics + Epigenetics
What happens when you karyotype a woman with an Mullerian anomaly?
Karyotype is normal
What are the classifications of Mullerian Anomalies?
- Hypoplasia/Agenesis
- Unicornuate
- Didelphus
- Bicornuate
- Septate
- Arcuate
- DES Drug Related
Subclasses of Hypoplasia/Agenesis
a. Vaginal
b. Cervical
c. Fundal (aka uterine)
d. Tubal
e. Combined
What is agenesis?
No fusion of Mullarian duct –> no development of female tract
Presentation of Hypoplasia/Agenesis?
Primary amenorrhea w/ cyclic pain , normal secondary sex characteristics
Possible anomalies in hypoplasia/agenesis?
No uterus
No uterus + no tubes
No uterus + no tubes + no cervix/upper vagina
What is the etiology of unicornuate uterus?
Failure of one of the Mullerian Ducts to develop/elongate
Subclasses of unicornuate uterus?
Communicating
Non-communicating
No Cavity
True Unicornuate Uterus
What is a communicating unicornuate uterus?
Rudimentary horn w/ a cavity and its communicating with uncornuate cavity
What is a non-communicating unicornuate uterus?
Rudimentary horn w/ a cavity but its not communicating with uncornuate cavity
What is a No cavity unicornuate uterus?
Rudimentary horn w/o a cavity
What is a true unicornuate uterus?
No rudimentary horn –> Very rare
Etiology of Uterine didelphys?
Failure of Mullerian ducts to fuse –> two seperate uterine horns –> each with endometrial cavity and its own cervix
Subclasses of bicornuate uterus?
a. Complete Septum
b. Partial Septum
Etiology of bicornuate?
Incomplete fusion
- Walls of Mullerian ducts don’t fuse properly, so you end up with a kind of Y-shaped uterus
How many cervices do you have in a bicornuate uterus?
One Cervix with two separate but communicating endometrial cavities
What happens during pregnancy with a bicornuate uterus?
Increased incidence of preterm delivery
Subclasses of Septate uterus?
a. Complete Septate Uterus
b. Partial septate (subseptate) uterus
Etiology of Septate uterus?
Incomplete canalization
Where in the uterus are bicornuate and septate uterus similar? different?
Inside the uterus
Outside the uterus
What does the outside of the uterus look like in a septate uterus?
Normal
What are the risk with septate uterus?
Infertility and early pregnancy loss
How do you differentiate between a bicornuate uterus and a septate uterus?
Measuring depth of outside indentation
- > 1 cm = bicornuate
- <1 cm = septate
How do you distinguish between arcuate uterus and septate uterus?
Measuring length of inside indentation
- > 1 cm = septate
- <1 cm = arcuate
What does DES make the uterus look like?
T-Shaped Uterine Cavity
DDx of agenesis?
- Meyer-Rokitansky-Kuster-Hauser (MRKH) Syndrome
2. Androgen insensitivity (AI) Syndrome
How do you tell MRKH from AI?
Karyotype
- MRKH = 46 XX
- AI = 46 XY
Genetics of MRKH?
46 XX
Phenotype of MRKH?
Female
What is absence in MRKH?
Vagina, Cervix, Uterus
Do pts with MRKH have ovaries?
Yes - leads to normal pubetal secondary sex characteristic development
Genetics of AI?
46 XY
Phenotype of AI?
Female
Why do pts with AI present as females?
Androgen receptors do not respond to androgen leading to no Wolffian duct development so they default to female external genetalia
Do pts with AI produce AMH?
yes
The production of AMH in pts with AI leads to what?
The regression of the Mullerian Ducts –> No vagina, uterus, cervix, fallopian tubes
How to pts with AI go thru female puberty?
High levels of androgen get converted to estrogen via aromtase
Do pts with AI have testes?
Yes