Benign Disorders of the Lower Genital Tract

Question 1

What are congenital anomalies of the female genital tract associated with?

Answer 1

Concomitant anomalies in the upper repro tract and GU tract

• unilateral renal agenesis
• pelvic or horseshoe kidney
• irregularities in collecting system

Question 2

What is the most common etiology of labial fusion?

Answer 2

Exogenous androgen exposure

Question 3

Labial fusion can also be caused by enzymatic errors. What enzyme is this and what condition does it cause? (include symptoms)

Answer 3

21-hydroxylase deficiency causing congenital adrenal hyperplasia.

• ambiguous genitalia
• hyperandrogenism
• salt wasting, hypotension, hyperK, hypoglycemia

Question 4

How is 21-OH deficiency inherited and diagnosed?

Answer 4

autosomal recessive diagnosed with elevated 17alpha-hydroxyprogesterone or urine 17-ketosteroid, decreased serum cortisol

Can cause adrenal crisis in neonates 75% of the time

Question 5

What is the treatment for congenital adrenal hyperplasia?

Answer 5

Exogenous cortisol - feedback inhibition to decrease ACTH and stimulation of adrenal gland that is shunting steroids into androgen pathway

Give mineralocorticoid (fludrocortisone acetate) for salt wasting

Reconstructive surgery for labial fusion

Question 6

What is the pathogenesis behind imperforate hymen?

Answer 6

Failure of epithelial cell degeneration in the central portion of the hymenal membrane

Question 7

What are the signs and symptoms of imperforate hymen?

Answer 7

Build-up of secretions leading to primary amenorrhea and cyclic pelvic pain

• Hematocolpos and hematometra (buildup of menstrual flow in vagina and uterus)
• absence of vaginal lumen
• tense bulging hymen
• increasing abdominal girth

Question 8

What is the treatment for imperforate hymen?

Answer 8

Surgery to excise extra tissue

Question 9

What is the embryonic formation of the vagina and uterus?

Answer 9

1. Paramesonephric (Mullerian) ducts elongate and meet in midline
2. Internal portion of each duct canalizes and septum dissolves
3. Caudal portion becomes uterus and upper vagina
4. Lower vagina forms as urogenital sinus evaginates to form the vaginal bulbs/plate
5. The central portion of the vaginal plate degenerates to form lumen of lower vagina (canalization or vacuolization)

Question 10

How do you differentiate between imperforate hymen and transverse vaginal septum?

Answer 10

Primary amenorrhea and cyclic pelvic pain appear in both

Difference is that with transverse vaginal septum, there is normal external female genitalia and a short vagina that ends in a blind pouch

Septum is typically 1 cm thick

Question 11

How do you diagnose and treat transverse vaginal septum

Answer 11

Ultrasound and MRI characterize thickness and other repro tract parts

Surgical correction is needed

Question 12

Define vaginal atresia. How does it occur? What parts of the repro tract are normal?

Answer 12

Lower vagina fails to develop and is replaced by fibrous tissue

Due to failure of urogenital sinus to contribute to lower vagina

Ovaries, uterus, cervix. and upper vagina are normal

Question 13

How does vaginal atresia present?

How is it treated?

Answer 13

Same as imperforate hymen and transverse vaginal septum

Treated with surgery to dissect the fibrous tissue, then pull the upper vaginal mucosa down = vaginal pull-through procedure

Question 14

What is vaginal agenesis?

Answer 14

Also known as Mater-Rokitansky-Kuster-Hauser syndrome (HRKH)

Congenital absence of vagina AND the absence of hypoplasia of the all or parts of the cervix, uterus, and fallopian tubes

Normal external genitalia, ovaries, and other secondary sexual characteristics

Question 15

How is vaginal atresia diagnosed and treated?

Answer 15

Diagnosed - US and MRI

Treatment - psychosocial support, nonsurgical , and surgical correction

• serial vaginal dilators
• McIndoe procedure to create neovagina

Question 16

What is the significance of lichen sclerosis

Answer 16

Inflammatory dermatosis of vulva (white patches)

In post-menopausal women, associated with 3-4% risk of vulvar skin cancer

Resulting atrophy can cause labial fusion, occlusion of clitoris, contracture of vaginal introitus, thinning of vulva, and skin fragility

Question 17

What is the presentation of lichen planus on vulva?

What is it associated with?

Answer 17

Chronic eruption of shiny purple papules with white striae on the vulva

Associated with vaginal adhesions and erosive vaginitis

3-4% risk of vulvar skin cancer

Also affects nails, scalp and oral mucous membranes

Question 18

What is silver-red scaly patches on the vulva?

Answer 18

Vulvar psoriasis

scaly patches over elbows and knees usually occur

Question 19

What is lichen simplex chronicus?

Answer 19

thickened skin with accentuated skin markings with chronic itching and scratching

Question 20

What are the definite indications for biopsy of epithelial disorders of the vulva and vagina?

Answer 20

Ulceration

Unifocal lesions

Uncertain suspicion of lichen sclerosus

Unidentifiable lesions

Lesions or symptoms that recur after conventional treatment

Question 21

What is the treament for epithelial disease of vulva/vagina

Answer 21

Healthy vulvar and vaginal hygiene practices

Wear loose-fitting clothing and underwear

Use unscented detergents and soaps

High potency topical steroids, clobetasol, can treat lichen sclerosis and severe simplex chronicus

Low-to- medium potency can treat mild dermatoses

No role for topical estrogen or testosterone

Question 22

Where can cysts arise from?

Answer 22

pilosebaceous ducts

sebaceous ducts

apocrine sweat glands

Question 23

What are the most common tumors found on the vulva?

What are its characteristics?

Answer 23

Epidermal inclusion cysts

Occlusion of pilosebaceous duct or hair follicle

lined with squamous epithelium

can become superinfected and develop into abscesses requiring I/D or excision

Question 24

What are the diseases of apocrine sweat glands of the vulva/vagina

Answer 24

Fox-Fordyce disease = chronic pruritic papular eruption where apocrine glands are found (around mons pubis and labia majora)

Hidradenitis suppurativa = underarms, breasts, inner thighs, groin, buttocks, can form abscesses

Question 25

What is the treatment for Bartholin’s duct cyst?

Answer 25

Found in the 4 and 8 ‘oclock positions

if small (<2 cm), will resolve on own or with sitz baths

If arising in women older than 40, need biopsy to rule out Bartholin’s gland carcinoma

Large cysts can cause pain and become infected = abscess requiring wound catheter (simple I/D can lead to recurrence) or marsupialization

Question 26

When is adjunct antibiotic therapy needed post-drainage of bartholin’s gland abscess?

Answer 26

When it cultures n. gonorrhoeae (10% of time)

Concomitant cellulitis or refractory to surgical treatment - need to cover staph aureus

Question 27

What are Gartner’s duct cysts?

Answer 27

remnants of the mesonephric ducts in the Wolffian system

Usually in the anterior lateral aspect of the upper part of the vagina

Usually asymptomatic

Treated by excision

Needs IVP and cystocopy pre-op

R/o urethral diverticuli, ectopic ureters, and vaginal and cervical cancer

Question 28

What may be needed during excision of Gartner duct cysts?

Answer 28

Vasopressin given potential for significant bleeding

Question 29

DDX of benign solid tumors of vagina/vulva.

Answer 29

lipoma

hemangioma

urethral caruncles

typically do not require surgery

Question 30

What medication is associated with abnormalities of the cervix?

What are the cervical abnormalities?

Answer 30

Diethylstilbestrol (DES)

cervical hypoplasia, cervical collars, cock’s comb cervix, and pseudopolyps

increased risk of cervical insufficiencyt in pregnancy

increased risk of clear cell adenocarcinoma

Question 31

What are the etiologies of cervical cysts?

Answer 31

Most are nabothian cysts = dilated retention cysts from blockage of endocervical gland

mesonephric cyst

Endometriosis - red/purple cysts with pelvic pain

Question 32

Describe cervical polyps

Answer 32

Benign, often asymptomatic

may cause intermenstrual or postcoital spotting

typically removed to prevent masking of other irregular bleeding

Question 33

Symptoms of cervical fibroids?

Answer 33

Leiomyomas = benign

can cause intermenstrual bleeding, dyspareunia, pressure

can cause problems in pregnancy

symptomatic = surgically removed

Question 34

What causes cervical stenosis?

Answer 34

Congenital, infection, atrophy, scarring, obstruction with cancer

typically asymptomatic and does not affect menstruation or fertility

can cause oligomenorrhea, amenorrhea, dysmenorrhea

can cause cervical dystocia during labor

treated by gently dilating the cervix