Congenital Gynecologic defects and Mullerian anomalies Flashcards
Cardinal steps in mullerian development (devel of ducts)
- Elongation
- Fusion
- Canalization
- Septal resorption
*2-4 happen simultaneously
3 types of vaginal obstruction
- Imperforate Hymen
- Transverse vaginal septum
- Vaginal atresia
Imperforate hymens present
Failure of caudel end of sino-vaginal bulbs to canalize
How do imperforate hymens present
obstructive symptoms
Hymen becomes perforate (blodd accum behind hymen) or patent in the vast majority of women in fetal life
- Cyclic pain,
- growing abdomino-pelvic mass
- Urinary retension
If paramesonephric duct does not develop from top ____ develops
mullerian agenesis or
Unicornate uterus
(no uterus or 1 sided uterus)
failed cardinal step of elongation
If two mullerian (paramesonephric) ducts do not fuse ____ develops
Uterine didelphys
- double uterus
- 2 cervix
What do you get if septal reabsorption fails?
Septate uterus
- midline uterine septum
(most common uterine anomaly seen and accounts for 55% of all mullerian anomalies)
Fibroids (leiomyoma uteri)
extremely common
- esp in AA
- assoc. w/ hysterectomy
The Mullerian system (cephalad) has to develop, but it also has to unite with what?
Urogenital sinus (caudad) and the resulting plane of fusion must resorb.
How do the mesonephric and para-mesonephric ducts develop?
In tandem.
So many of the embryologic defects of the Mullerian system, (esp the lateral fusion defects), co-evolve with the renal system.
Abnl differentiation of the mesonephric and paramesonephric ducts may also be predictive of abnl renal development.
What arises from the mesonephric ducts?
Ureteral buds –>
- Ureters
- Renal Calyces
- Collecting tubules
Failure of elongation of the ducts can be Bilateral or Unilateral. What are the resulting conditions of each?
Bilateral elongation –>
Mullerian Agenesis
(no uterus)
Unilateral –>
Unicornuate Uterus
(1 sided uterus)
*1st step failure in cardinal mullerian development
Failure in fusion of ducts can result in what conditions?
(ie upper Mullerian system to the caudal urogenital sinus)
Uterine didelphys
Transverse vaginal septum
*2nd step failure in cardinal mullerian development
Failure of canalization can result in what condition?
Vaginal agenesis
*3rd step failure in cardinal mullerian development
Failure of septal resorption results in what condition?
Septate uterus
*4th step failure in cardinal mullerian development
What develops into the vagina?
Upper 2/3: from Mullerian system
Lower 1/3: urogenital sinus
Transverse vaginal septum classical presentation + tx
young women with no menses and lower abdominal pain (hematocolpos)
*obstructive due to vaginal septal formation leading to blind vaginal pouch
Tx: skin graft if septum >2cm in order to prevent stricture of vaginal and allow for good mucosal healing.
Most common alternate dx to consider when evaluatin pt with a transverse vaginal septum
Imperforate hymen.
also presents with obstructive sx even though its not a mullerian defect
Unicornuate uterus
- what is it
- how does this affect renal development?
Unilateral mullerian duct elongation failure
- one duct elongates correctly and other does not
= 1 sided uterus
Renal agenesis on the same side as missing duct occurs in 40% of cases
- poor vascularity
Poor reproductive potential (spontaneous abortion 50%)
Lateral fusion defects (during mullerian development) can be complete or partial. Complete failure of fusion results in what?
- Didelpys: two uterine horns not communicating at all
- Good reproductive outcome (unlike unicornuate uterus)
or
A longitudinal vaginal septum (not transverse that causes obstruction)
- 2 cervices
- occurs if fusion defect is lower in the vagina
- note that
1. These defects especially lateral fusion defects co-develop with the renal system
Which one has communicating endometrial cavities, didelphic uterus or bicornuate uterus?
Bicornuate "heart shaped uterus" - upper pt of mullerian sys fails to fuse (caudal nl) - 1 uterus w/ 2 horns - both horns are separated by septum
(reproductive outcomes ok ~30% compared to unicornuate uterus)
Most common uterine anomaly seen.
- reproductive potential
septate uterus: failure of septal resorption btwn ducts
- very high first trimester pregnancy loss
DES uterine malformation
T shaped uterus due to fetal exposure to diethylstilbestrol (DES)
- intereres with mesenchyme of reproductive tract
