13: Lower GT disorders Flashcards
Labial fusion may be the result of
excess androgen exposure or an enzymatic deficiency, most commonly 21-hydroxylase deficiency leading to congenital adrenal hyperplasia (CAH) and ambiguous external genitalia.
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. These patients typically have normal external genitalia, normal secondary sexual characteristics (breast development, axillary, and pubic hair), and normal ovarian function.
A variety of cysts can arise on the vulva and vagina from occlusion of what glands?
pilosebaceous ducts, sebaceous ducts, and apocrine sweat glands.
Bartholin’s cysts and abscesses are located ?
management?
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
congenital adrenal hyperplasia s/s
how to diagnose?
treatment?
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
signs of imperforate hymen
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)
lower genital tract origins
upper vagina and uterus from caudal paramesonephric (mullerian) ducts
lower vagina from urogenital sinus
if the Mullerian tubercle fails to canalize, may form ?
a transverse vaginal septum
s/s: primary amenorrhea and cyclic pelvic pain (like imp. hymen), blind pouch
-visualize with US, MRI
tx: sx correction
vaginal atresia s/s
diagnosis, treatment
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
vaginal agenesis
aka ?
dx
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
vaginal agenesis corrections
serial vaginal dilators (can take 4 mos to yrs)
creation of a neovagina: McIndoe procedure
nonneoplastic epithelial disorders of the vulva
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
indications for definite biopsy of vulvar lesions
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
ddx of vulvar/vaginal lesions
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
tx of vulvar/vaginal lesions
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
the most common tumor found on the vulva
epidermal inclusion cysts
Lichen sclerosis
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
Lichen planus
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
Lichen simplex chronicus
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
Vulvar psoriasis
Red moist lesions, sometimes scaly s/s: Asymptomatic or sometimes pruritus tx: Topical steroids, UV light
Fox-Fordyce disease
infrequently occurring chronic pruritic papular eruption that localizes to areas where apocrine glands are found
Hidradenitis suppurativa
skin disease that most commonly affects areas bearing apocrine sweat glands or sebaceous glands, such as the underarms, breasts, inner thighs, groin, and buttocks
paraurethral glands aka ? glands can also become dilated with duct obstruction
Skene’s glands
Gartner’s duct cysts
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
benign solid tumors of vulva/vagina
lipomas, hemangiomas, and urethral caruncles (vulvovaginal atrophy)
symptoms from DES exposure in utero
cervical hypoplasia, cervical collars, cervical hoods, cock’s comb cervix, and pseudopolyps, cervical insufficiency, clear cell adenocarcinoma of cervix or vagina
Most cervical cysts are dilated retention cysts called ?
caused by ?
other cysts on cervix?
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
why are cervical polyps removed?
to decrease the likelihood of masking irregular bleeding from another source such as cervical cancer, fibroids, adenomyosis, endometrial polyps, endometrial hyperplasia, and endometrial cancer
cervical fibroids
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
Cervical stenosis etiologies?
symptomatic?
rare complications?
treatment?
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
