Congenital Mullerian abnormalities

Question 1

what are the four steps of mullerian development?

Answer 1

elongation
fusion
canalization
septal resorption

Question 2

just to be annoying lets do each step of mullerian development in a little bit of detail.

1) elongation:

Answer 2

37 days after fertilization, mullerian ducts appear lat to wolffian ducts

elongation of mullerian duct is medial and caudal

Question 3

2) fusion

Answer 3

mullerian ducts fuse in midline and fuse with orugenital sinus at muller tubercle

wolffian ducts regress!!

Question 4

3) canalization

Answer 4

mullerian duct starts as a solid structure, but will canalize and become tubes

Question 5

4) septal resorption

Answer 5

resorption of the septum that is separating the uterus into two parts

uterus joins urogenital sinus to form lower vagina

sinovaginal bulb elongates and develops into full vagina

Question 6

name three vaginal obstructions and one common presentation of all

Answer 6

1) imperforate hymen
2) transverse vaginal septum
3) vaginal atresia

Presents with cyclical pain

Question 7

imperf hymen

Answer 7

failure of caudal end of sinovaginal bulbs to canalize

• cyclical pain and build up of blood upon mensturatoin EW GROSS
• tx= open the god dman hymen

Question 8

transverse vaginal septum

Answer 8

failed canalization of vaginal plate (where mullerian ducts meet UG sinus)
-vag ends in pnk tissue

Question 9

vaginal atresia

Answer 9

failure of ug sinus to canalize below the vaginal plate

tx= vaginal dilators, surgical vaginoplasty

Question 10

Mullerian Agenesis or Hypoplasia: Class I

Answer 10

FAILURE OF ELONGATION (step 1)

• most severe
• presents with amenorrhea because vagina ends in blind pouch
• develp 2 sex characteristics because have ovaries

Question 11

Unicornuate uterus: Class II

Answer 11

FAILURE OF ELONGATION ON ONE SIDE

-may never discover bc period is normal

Question 12

Uterine didelphys: Class III

Answer 12

FAILURE TO FUSE IN MIDLINE

• 2 endometrial cavities, 2 cervices, one fallopain tube on each
• everything is duplicated*

Question 13

Bicornuate: Class IV

Answer 13

INCOMPLETE FUSION IN MIDLINE

• leads to complete or partial separation
• *****high risk for preterm delivery

Question 14

Septate: Class V

Answer 14

FAILURE OF SEPTUM RESORPTION

• usu asymptomatic
• **most common type (55%)

Question 15

Arcuate: Class VI

Answer 15

variant of normal

• near complete resorption of uterovaginal septum, some remnant
• asymptomatic
• no adverse repro outcomes

Question 16

DES related: Class VII

Answer 16

rare now because we don’t use the drug

• 69% of women with exposure had anomalies
• T shaped uterus most common
• caused miscarriage, preterm delivery, malpresentation, associated renal anomalies