Reproductive: Congenital

Question 1

Imaging appearance of the neonatal uterus

Answer 1

• 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.

Question 2

When does the endometrium become visible on imaging?

Answer 2

Puberty

Question 3

Imaging appearance of uterus/ovaries in Turner syndrome

Answer 3

• Streaky ovaries
• Pre-puberty uterus (cervix equal in size to the fundus)

Question 4

What do the mullerian ducts become?

Answer 4

• Fallopian tubes
• Uterus
• Upper 2/3 vagina

Question 5

What embryological structure becomes the lower 1/3 of the vagina?

Answer 5

The urogenital sinus

Note: The upper 2/3 is made by the Müllerian ducts.

Question 6

What do the wolffish ducts become?

Answer 6

• Vas deferens
• Seminal vesicles
• Epididymus

Question 7

What does the urogenital sinus become

Answer 7

• Prostate (males)
• Lower 1/3 vagina (females)

Question 8

In what direction does uterine cleavage occur embryologically?

Answer 8

From bottom to top

Note: This is why a uterine septum always extends distally from the fundus.

Question 9

What are the 3 major categories of embryology mistakes that result in congenital uterine anomalies?

Answer 9

• Failure to form (e.g. unicornuate uterus +/- rudimentary horn)
• Failure to fuse (Uterine didelphys/bicornuate uterus)
• Failure to cleave (e.g. septet uterus)

Question 10

What are the features of mullerian agenesis?

Answer 10

• Vaginal atresia
• Absent or rudimentary uterus
• Normal ovaries

Note: About 50% of these pts have renal issues (agenesis, ectopia, etc).

Question 11

What are the 4 major variants of a unicornuate uterus?

Answer 11

• Isolated unnicornuate (most common)
• Unicornuate + noncavitary rudimentary horn
• Unicornuate + non-communicating cavitary rudimentary horn
• Unicornuate + communicating cavitary rudimentary horn

Question 12

Why is it important to identify whether there is a rudimentary horn in the setting of a unicornuate uterus?

Answer 12

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.)

Question 13

Is it possible to have a pregnancy of a rudimentary horn?

Answer 13

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)

Question 14

Does having an endometrial rudimentary horn increase risk for miscarriage?

Answer 14

Yes, if the rudimentary horn contains endometrial tissue

Question 15

What is the most common renal anomaly associated with a unicornuate uterus?

Answer 15

Renal agenesis (ipsilateral to the rudimentary horn)

Question 16

Name that congenital anomaly

Answer 16

Uterine didelphys (complete uterine duplication due to a failure to fuse)

Note: 75% of these pts will also have a vaginal septum.

Question 17

Name that congenital anomaly

Answer 17

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.

Question 18

Name that congenital anomaly

Answer 18

Unicornuate uterus without a rudimentary horn (most common type of unicornuate uterus)

Question 19

Name that congenital anomaly

Answer 19

Unicornuate uterus with noncavitary rudimentary horn

Question 20

Name that congenital anomaly

Answer 20

Unicornuate uterus with cavitary non-communicating rudimentary horn

Question 21

Name that congenital anomaly

Answer 21

Unicornuate uterus with cavitary communicating rudimentary horn

Question 22

Name that congenital anomaly

Answer 22

Partial bicornuate uterus

Question 23

Name that congenital anomaly

Answer 23

Complete bicornuate uterus

Question 24

Name that congenital anomaly

Answer 24

Partial septate uterus

Question 25

Name that congenital anomaly

Answer 25

Complete septate uterus

Question 26

Unicollis vs bicollis bicornuate uterus

Answer 26

Unicollis refers to “one cervix”

Bicollis refers to “two cervices”

Question 27

Is a vaginal septum common in pts with a bicornuate uterus?

Answer 27

Yes, 25% of pts with a bicornuate uterus will also have a vaginal septum

Question 28

Answer 28

T-shaped uterus

Note: This is related to maternal DES (diethylstilbestrol) exposure.

Question 29

Complications of maternal diethylstilbestrol exposure

Answer 29

• Vaginal clear cell carcinoma
• Uterine anomalies (classically, the T-shaped uterus)

Question 30

What are the two major types of septate uterus?

Answer 30

• Fibrous septum
• Muscular septum

Note: You can tell the difference on MRI and this changes the surgical approach to management.

Question 31

What is the most common Müllerian duct anomaly associated with miscarriage?

Answer 31

Septate uterus

Note: Resection of the septum decreases risk of miscarriage.

Question 32

Why is a uterine septum more likely to cause a miscarriage than a bicornuate uterus?

Answer 32

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.

Question 33

Does an arcuate uterus increase the risk of miscarriage?

Answer 33

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.

Question 34

How can you differentiate a septate uterus from a bicornuate uterus?

Answer 34

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)

Question 35

When is a hysterosalpingogram best performed?

Answer 35

During the proliferative phase (day 7-12 of the menstrual cycle), when the endometrium is thinnest (and pregnancy is least likely)

Question 36

What are the major phases of the menstrual cycle?

Answer 36

• Days 0-7 (menses)
• Days 7-14 (proliferative phase)
• Day 14 (ovulation)
• Days 14-28 (secretory)

Question 37

Contraindications for hysterosalpingogram

Answer 37

• Pregnancy
• Active pelvic infection
• Active bleeding (e.g. menses)
• Contrast allergy

Question 38

What is the most common cause of irregular filling defects on a hysterosalpingogram?

Answer 38

Synechiae/scarring/adhesions (usually secondary to prior curettage)

Note: Endometrial infections can also appear this way.

Question 39

Infertility

Answer 39

Think Asherman syndrome (due to uterine synechiae/scarrin/adhesions)

Note: It’s only called Asherman is its symptomatic (e.g. infertility).

Question 40

Answer 40

Think endometrial polyp or intrauterine pedunculated fibroid

Note: If mobile/transient, then think gas bubble.

Question 41

Most common cause of uterine tubal occlusion

Answer 41

Infection/PID

Question 42

Treatment for uterine tubal occlusions in the setting of infertility

Answer 42

Tubal catheterization (only for proximal obstruction, not distal obstruction/hydrosalpinx)