Mullerian Abnormalities CPC Flashcards
How is the vagina formed?
Sinovaginal bulbs fuse to make vaginal plate.
Inner cells of the plate degenerate to form the lumen of the vagina.
What separates the vagina from the urogenital sinus cavity?
The hymen - it should canalize.
Do mullerian defects affect the ovaries?
Nope.
What are examples of verticle fusion defects?
Transverse vaginal septum (high, mid, or low).
Inperforate hymen*
Cervical agenesis/dysgenesis.
Mullerian Agenesis (MRKH)
*not actually considered a Mullerian anomaly
Lateral fusion defects?
2 uteri, bicornate, septate uterus.
Blind uteri, connected or not.
Obstructed hemi-vagina.
All sorts of stuff can happen.
2 major things on DDx if girl is missing vagina and uterus?
Mullerian agenesis (MRKH). Androgen insensitivity syndrome (she's actually XY).
Anomalies associated with mullerian agenesis?
Kidney problems, skeletal problems.
Is mullerian agenesis a problem with anti-mullerian hormone?
Nope.
might have to do with Wnt4, Hox genes, others. But there’s not good MZ concordance
Can women with mullerian agenesis have children?
Yes, through IVF and surrogacy. Recall that mullerian defects don’t affect the ovaries.
2 ways to make a vagina?
Non-surgical… just keeping pushing. (I’m amazed that this works)
A bunch of different surgical techniques.
How does imperforate hymen often present? Treatment?
Amennorhea, but lots of pain, swelling abdomen.
Open it up and drain it. (wear a face mask)
Why do blind uteri cause problems? Treament?
Like with imperforate hymen, menses just build up. Symptoms from stretching and compressing of structures.
Also can lead to endometriosis.
Remove the blind uterine horn and fallopian tube.
(keep the ovary!)
Why are bicornuate / septate uteri bad?
They decrease space in uterus, predisposing to spontaneous abortion / pre-term delivery.
Also can lead to endometriosis due to blackflow.
