Reproductive: Congenital Flashcards
Imaging appearance of the neonatal uterus
- Larger uterus than you would think for a baby (due to maternal/placental hormones)
- Cervix often larger than the fundus
Note: The fundus doesn’t become larger than the cervix until puberty.
When does the endometrium become visible on imaging?
Puberty
Imaging appearance of uterus/ovaries in Turner syndrome
- Streaky ovaries
- Pre-puberty uterus (cervix equal in size to the fundus)
What do the mullerian ducts become?
- Fallopian tubes
- Uterus
- Upper 2/3 vagina
What embryological structure becomes the lower 1/3 of the vagina?
The urogenital sinus
Note: The upper 2/3 is made by the Müllerian ducts.
What do the wolffish ducts become?
- Vas deferens
- Seminal vesicles
- Epididymus
What does the urogenital sinus become
- Prostate (males)
- Lower 1/3 vagina (females)
In what direction does uterine cleavage occur embryologically?
From bottom to top
Note: This is why a uterine septum always extends distally from the fundus.
What are the 3 major categories of embryology mistakes that result in congenital uterine anomalies?
- Failure to form (e.g. unicornuate uterus +/- rudimentary horn)
- Failure to fuse (Uterine didelphys/bicornuate uterus)
- Failure to cleave (e.g. septet uterus)
What are the features of mullerian agenesis?
- Vaginal atresia
- Absent or rudimentary uterus
- Normal ovaries
Note: About 50% of these pts have renal issues (agenesis, ectopia, etc).
What are the 4 major variants of a unicornuate uterus?
- Isolated unnicornuate (most common)
- Unicornuate + noncavitary rudimentary horn
- Unicornuate + non-communicating cavitary rudimentary horn
- Unicornuate + communicating cavitary rudimentary horn
Why is it important to identify whether there is a rudimentary horn in the setting of a unicornuate uterus?
If the rudimentary horn has endometrial tissue (communicating or not), it can proceed a lot of problems (dysmenorrhea, hematometra, hematosalpinx, miscarriages, rudimentary horn pregnancy/rupture, etc.)
Is it possible to have a pregnancy of a rudimentary horn?
Yes, as long as there is endometrial tissue (even if the rudimentary horn is not communicating, it can contain a gestation)
Note: This is dangerous because a rudimentary horn pregnancy will often cause rupture and life threatening bleeding (especially if the horn is non-communicating)
Does having an endometrial rudimentary horn increase risk for miscarriage?
Yes, if the rudimentary horn contains endometrial tissue
What is the most common renal anomaly associated with a unicornuate uterus?
Renal agenesis (ipsilateral to the rudimentary horn)
Name that congenital anomaly
Uterine didelphys (complete uterine duplication due to a failure to fuse)
Note: 75% of these pts will also have a vaginal septum.
Name that congenital anomaly
Arcuate uterus (mild concavity of the endometrium at the fundus with normal uterine contour)
Note: This is more of a normal variant than an anomaly.
Name that congenital anomaly
Unicornuate uterus without a rudimentary horn (most common type of unicornuate uterus)
Name that congenital anomaly
Unicornuate uterus with noncavitary rudimentary horn
Name that congenital anomaly
Unicornuate uterus with cavitary non-communicating rudimentary horn
Name that congenital anomaly
Unicornuate uterus with cavitary communicating rudimentary horn
Name that congenital anomaly
Partial bicornuate uterus
Name that congenital anomaly
Complete bicornuate uterus
Name that congenital anomaly
Partial septate uterus
Name that congenital anomaly
Complete septate uterus
Unicollis vs bicollis bicornuate uterus
Unicollis refers to “one cervix”
Bicollis refers to “two cervices”
Is a vaginal septum common in pts with a bicornuate uterus?
Yes, 25% of pts with a bicornuate uterus will also have a vaginal septum
T-shaped uterus
Note: This is related to maternal DES (diethylstilbestrol) exposure.
Complications of maternal diethylstilbestrol exposure
- Vaginal clear cell carcinoma
- Uterine anomalies (classically, the T-shaped uterus)
What are the two major types of septate uterus?
- Fibrous septum
- Muscular septum
Note: You can tell the difference on MRI and this changes the surgical approach to management.
What is the most common Müllerian duct anomaly associated with miscarriage?
Septate uterus
Note: Resection of the septum decreases risk of miscarriage.
Why is a uterine septum more likely to cause a miscarriage than a bicornuate uterus?
To support a fetus, you need adequate blood supply. A uterine septum has poor blood supply and if the embryo implants on the septum, there is a high rate of miscarriage. A bicornuate uterus is also associated with miscarriages, but not nearly as much as a septate uterus due to better blood supply to both uterine cavities.
Does an arcuate uterus increase the risk of miscarriage?
No, an arcuate uterus is NOT associated with reduced fertility or obstetric complications
Note: It is considered more of a normal variant than a true anomaly.
How can you differentiate a septate uterus from a bicornuate uterus?
The fundal contour:
A septate uterus has a normal fundal contour (contour is >5 mm above the tubal ostia)
A bicornuate uterus has an indentation in the fundal contour (indentation is < 5mm above the tubal ostia)
When is a hysterosalpingogram best performed?
During the proliferative phase (day 7-12 of the menstrual cycle), when the endometrium is thinnest (and pregnancy is least likely)
What are the major phases of the menstrual cycle?
- Days 0-7 (menses)
- Days 7-14 (proliferative phase)
- Day 14 (ovulation)
- Days 14-28 (secretory)
Contraindications for hysterosalpingogram
- Pregnancy
- Active pelvic infection
- Active bleeding (e.g. menses)
- Contrast allergy
What is the most common cause of irregular filling defects on a hysterosalpingogram?
Synechiae/scarring/adhesions (usually secondary to prior curettage)
Note: Endometrial infections can also appear this way.
Infertility
Think Asherman syndrome (due to uterine synechiae/scarrin/adhesions)
Note: It’s only called Asherman is its symptomatic (e.g. infertility).
Think endometrial polyp or intrauterine pedunculated fibroid
Note: If mobile/transient, then think gas bubble.
Most common cause of uterine tubal occlusion
Infection/PID
Treatment for uterine tubal occlusions in the setting of infertility
Tubal catheterization (only for proximal obstruction, not distal obstruction/hydrosalpinx)