PAGS

Question 1

describe technique in Williams vaginoplasty

Answer 1

skin flaps from labia majora -> pouch

Question 2

describe Vechietti procedure

Answer 2

constant tension on olive-shaped ball into dimple of vagina -> neovagina

Question 3

describe sigmoid vaginoplasy

Answer 3

use sigmoid portion for vaginal pouch

Question 4

Describe mcindoe procedure

Answer 4

surgically insert skin flap through peritoneum -> neovagina

Question 5

Mayer Rokitansky Kuster hauser S/d a.k.a.

Answer 5

Mullerian agenesis

Question 6

Mullerian agenesis incidence

Answer 6

1/5000 Females

Question 7

Mullerian agenesis etiology

Answer 7

underdevelopment of Mullerian Duct (vagina, uterus)

ovaries normal b/c seprate embryologic source

Question 8

Mullerian agenesis karyotype

Answer 8

46XX

Question 9

Mullerian agenesis what is present?
ovaries?
uterus/vagina?
secondary sexual characteristics?

karyotype

Answer 9

ovaries: yes
uterus/vagina: no/remnant
secondary sexual caracteristics: yes (b/c ovaries)

46XX

Question 10

embryologic origin of urinary and genital system?

Answer 10

intermediate mesoderm

Question 11

when do mullerian ducts develop?

Answer 11

6wks

aka paramesonephric ducts

Question 12

embryologic origin of Bartholin glands

Answer 12

urogenital sinus

Question 13

embryologic origin of clitoris

Answer 13

genital tubercle

Question 14

embryologic origin of labia majora

Answer 14

genital swelling

Question 15

emryologic origin of labia minora

Answer 15

urogenital sinus

Question 16

gene known as testis-determining factor

Answer 16

SRY gene (on Y ch)

Question 17

AMH secreted by what cell?

timing

what does it do?

Answer 17

Sertoli cells

by 9-10w GA

causes regression of mullerian duct system, testis growth

Question 18

female differentiation timing and MoA

Answer 18

timing: ~10-12ish weeks
MoA: absence of SRY gene -> bipotential gonad develops into ovary

female = default mode

Question 19

mesonephric duct differentiates into:

if no testosterone, what happens? (timing)

Answer 19

epididymis
vas deferens
seminal vesicles

regress by 12w

Question 20

Turner S/D karyotype

Answer 20

45XO

Question 21

Turner S/D:
ovaries?
uterus/vagina?
secondary sexual characteristics?

Answer 21

ovaries: none / streak
uterus/vagina: yes
secondary sexual characteristics: no

capable of responding to exogenous hormones

Question 22

Klinefelter S/D karyotype

Answer 22

47XXY

Question 23

Swyer S/d a.k.a.

Answer 23

46XY gonadal dysgenesis

Question 24

46XY gonadal dysgenesis etiology / MoA

Answer 24

SRY (testis-determining) mutation -> gonads dont produce androgens, AMH

Question 25

46XY gonadal dysgenesis ovaries? uterus/vagina? secondary sex characteristics?

Answer 25

ovaries? no - streak gonads uterus/vagina? yes (no SRY -> no AMH -> normal mullerian system) secondary sex characteristics: prepubertal F

Question 26

Androgen Insensitivity S/D ovaries? uterus/vagina? secondary sex. characteristics?

Answer 26

ovaries? no - testes uterus/vagina? absent/short secondary sex. characteristics? breasts (androgen-> estrogen conversion)

Question 27

Androgen Insensitivity S/D karyotype

Answer 27

46XY

Question 28

Androgen Insensitivity S/D etiology / MoA

Answer 28

abnormal 5alpha-reductase | (impaired conversion testosterone -> DHT)

Question 29

Describe uterine didelphys (and Etiology)

Answer 29

=double uterus etiol: failure of Mullerian ducts to fuse

Question 30

Describe bicornuate uterus and etiol

Answer 30

fundus indented >1cm (2 endomerial cavities) etiol: partial fusion of Mullerian ducts

Question 31

Describe septate uterus

Answer 31

=normal external surface, 2 endometrial cavities etiol: defect in midline septum resorption

Question 32

AIS vs Mullerian Agenesis | describe phenotype

Answer 32

AIS = 46XY Mullerian Agenesis = 46XX | both have female phenotype + breast dev, no uterus/cervix

Question 33

(likely) mgmt imperforate hymen

Answer 33

cruciate incision drainage + intravaginal catheter 2wks (w/ abx) +/- estrogen cream

Question 34

Mgmt transverse vaginal septum

Answer 34

complete resection (after sono)