13: Lower GT disorders

Question 1

Labial fusion may be the result of

Answer 1

excess androgen exposure or an enzymatic deficiency, most commonly 21-hydroxylase deficiency leading to congenital adrenal hyperplasia (CAH) and ambiguous external genitalia.

Question 2

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH)

Answer 2

congenital absence of the vagina and the absence or hypoplasia of all or part of the cervix, uterus, and fallopian tubes. These patients typically have normal external genitalia, normal secondary sexual characteristics (breast development, axillary, and pubic hair), and normal ovarian function.

Question 3

A variety of cysts can arise on the vulva and vagina from occlusion of what glands?

Answer 3

pilosebaceous ducts, sebaceous ducts, and apocrine sweat glands.

Question 4

Bartholin’s cysts and abscesses are located ?

management?

Answer 4

at 4-o’clock and 8-o’clock positions on the labia majora.

• cysts are usually asymptomatic and resolve on their own.
• if appears for first time in woman older than 40 years, the cyst wall should be biopsied to rule out the rare possibility of Bartholin’s gland carcinoma
• large symptomatic Bartholin’s cysts and Bartholin’s abscesses should be appropriately drained along with placement of a Word catheter or marsupialization, no abx needed

Question 5

congenital adrenal hyperplasia s/s
how to diagnose?
treatment?

Answer 5

salt wasting, hypotension, hyperkalemia, and hypoglycemia, adrenal crisis

dx: elevated 17α-hydroxyprogesterone or urine 17-ketosteroid with decreased serum cortisol
tx: exogenous cortisol (decr. ACTH) +/- a mineralcorticoid if salt wasting (fludrocortisone acetate), may require reconstructive sx

Question 6

signs of imperforate hymen

Answer 6

primary amenorrhea and cyclic pelvic pain
buildup of secretions in the vagina behind the hymen (hydrocolpos or mucocolpos)
accumulation of menstrual flow behind the hymen in the vagina (hematocolpos) and uterus (hematometra)

Question 7

lower genital tract origins

Answer 7

upper vagina and uterus from caudal paramesonephric (mullerian) ducts
lower vagina from urogenital sinus

Question 8

if the Mullerian tubercle fails to canalize, may form ?

Answer 8

a transverse vaginal septum
s/s: primary amenorrhea and cyclic pelvic pain (like imp. hymen), blind pouch
-visualize with US, MRI
tx: sx correction

Question 9

vaginal atresia s/s

diagnosis, treatment

Answer 9

lower vagina fails to form, primary amenorrhea, cyclic pelvic pain
absence of introitus, presence of vaginal dimple
pelvic US, MRI may show large hematocolpos
tx: sx correction wi vaginal pull-through procedure

Question 10

vaginal agenesis
aka ?
dx

Answer 10

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH), congenital absence of the vagina and the absence or hypoplasia of all or part of the cervix, uterus, and fallopian tubes
pheno/geno F
dx with pelvic US/MRI
tx: support, sx/nonsx correction

Question 11

vaginal agenesis corrections

Answer 11

serial vaginal dilators (can take 4 mos to yrs)

creation of a neovagina: McIndoe procedure

Question 12

nonneoplastic epithelial disorders of the vulva

Answer 12

lichen sclerosis (postmenopausal), lichen planus (purple papules with white striae, vaginal adhesions), lichen simplex chronicus (itchy, thick skin), and vulvar psoriasis
-need histo exam to ddx from neoplasia

Question 13

indications for definite biopsy of vulvar lesions

Answer 13

ulceration, unifocal lesions, uncertain suspicion of lichen sclerosus, unidentifiable lesions, and lesions or symptoms that recur or persist after conventional therapy
-aid biopsy with colposcope

Question 14

ddx of vulvar/vaginal lesions

Answer 14

benign: aphthous ulcers, Behçet syndrome, Crohn disease, erythema multiforme, bullous pemphigoid, and plasma cell vulvitis.
malignant: squamous cell, basal cell, melanoma, sarcoma, and Paget disease of the vulva

Question 15

tx of vulvar/vaginal lesions

Answer 15

hygiene
topical steriods (high potency i.e. clobetasol for lichen sclerosis/planus)
-hormones not useful except topical estrogen for vulvovaginal atrophy
-sx correction for adhesion/stenosis in lichen planus

Question 16

the most common tumor found on the vulva

Answer 16

epidermal inclusion cysts

Question 17

Lichen sclerosis

Answer 17

Symmetric white, thinned skin on labia, perineum, and perianal region; shrinkage and agglutination of labia minora
s/s: Usually pruritus or dyspareunia, often asymptomatic
tx: High-potency topical steroids (clobetasol or halobetasol 0.05%) 1–2×/d for 6–12 wk, then a maintenance schedule of topical steroid

Question 18

Lichen planus

Answer 18

Multiple shiny, flat, red-purple papules, usually on the inner aspects of the labia minora and vestibule with lacy white changes; often erosive
s/s: Pruritus with mild inflammation to severe erosions
tx: High-potency topical steroids (clobetasol or halobetasol 0.05%) 1–2×/d for 6–12 wk, then a maintenance schedule of topical steroid

Question 19

Lichen simplex chronicus

Answer 19

Localized thickening of the vulvar skin, slight scaling

s/s: Chronic pruritus tx: Medium- to high-potency topical steroid 2-3x/d for 6 or more weeks

Question 20

Vulvar psoriasis

Answer 20

Red moist lesions, sometimes scaly s/s: Asymptomatic or sometimes pruritus tx: Topical steroids, UV light

Question 21

Fox-Fordyce disease

Answer 21

infrequently occurring chronic pruritic papular eruption that localizes to areas where apocrine glands are found

Question 22

Hidradenitis suppurativa

Answer 22

skin disease that most commonly affects areas bearing apocrine sweat glands or sebaceous glands, such as the underarms, breasts, inner thighs, groin, and buttocks

Question 23

paraurethral glands aka ? glands can also become dilated with duct obstruction

Answer 23

Skene’s glands

Question 24

Gartner’s duct cysts

Answer 24

remnants of the mesonephric ducts of the Wolffian system, anterior lateral aspects of the upper part of the vagina
-asymptomatic typically, may have dysparenuia
tx: excision, IVP, cystoscopy, use vasopressin
rule out: urethral diverticula, ectopic ureters, and vaginal and cervical cancer

Question 25

benign solid tumors of vulva/vagina

Answer 25

lipomas, hemangiomas, and urethral caruncles (vulvovaginal atrophy)

Question 26

symptoms from DES exposure in utero

Answer 26

cervical hypoplasia, cervical collars, cervical hoods, cock’s comb cervix, and pseudopolyps, cervical insufficiency, clear cell adenocarcinoma of cervix or vagina

Question 27

Most cervical cysts are dilated retention cysts called ?
caused by ?
other cysts on cervix?

Answer 27

nabothian cysts

• intermittent blockage of an endocervical gland and usually expand to no more than 1 cm in diameter, typ. no tx
• mesonephric cysts, implants of endometriosis

Question 28

why are cervical polyps removed?

Answer 28

to decrease the likelihood of masking irregular bleeding from another source such as cervical cancer, fibroids, adenomyosis, endometrial polyps, endometrial hyperplasia, and endometrial cancer

Question 29

cervical fibroids

Answer 29

may cause problems in pregnancy and may lead to hemorrhage, poor dilation of the cervix, malpresentation, or obstruction of the birth canal; dysparunia, pelvic pressure, intermenstrual bleeding

• need to rule out cervical cancer
• may remove with myomectomy or hysterectomy

Question 30

Cervical stenosis etiologies?
symptomatic?
rare complications?
treatment?

Answer 30

congenital, a product of infection, atrophy, or scarring (cervical surgical manipulation or radiotherapy); obstruction with a neoplasm, polyp, or fibroid.

• typically asymptomatic and does not affect menstruation or fertility
• oligomenorrhea, amenorrhea, dysmenorrhea, or an enlarged uterus, block endometrial/cervical canal, cervical dystocia during labor
• tx: gently dilating the cervix