Benign Disorders of the Lower Genital Tract Flashcards
What are congenital anomalies of the female genital tract associated with?
Concomitant anomalies in the upper repro tract and GU tract
- unilateral renal agenesis
- pelvic or horseshoe kidney
- irregularities in collecting system
What is the most common etiology of labial fusion?
Exogenous androgen exposure
Labial fusion can also be caused by enzymatic errors. What enzyme is this and what condition does it cause? (include symptoms)
21-hydroxylase deficiency causing congenital adrenal hyperplasia.
- ambiguous genitalia
- hyperandrogenism
- salt wasting, hypotension, hyperK, hypoglycemia
How is 21-OH deficiency inherited and diagnosed?
autosomal recessive diagnosed with elevated 17alpha-hydroxyprogesterone or urine 17-ketosteroid, decreased serum cortisol
Can cause adrenal crisis in neonates 75% of the time
What is the treatment for congenital adrenal hyperplasia?
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
What is the pathogenesis behind imperforate hymen?
Failure of epithelial cell degeneration in the central portion of the hymenal membrane
What are the signs and symptoms of imperforate hymen?
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
What is the treatment for imperforate hymen?
Surgery to excise extra tissue
What is the embryonic formation of the vagina and uterus?
- Paramesonephric (Mullerian) ducts elongate and meet in midline
- Internal portion of each duct canalizes and septum dissolves
- Caudal portion becomes uterus and upper vagina
- Lower vagina forms as urogenital sinus evaginates to form the vaginal bulbs/plate
- The central portion of the vaginal plate degenerates to form lumen of lower vagina (canalization or vacuolization)
How do you differentiate between imperforate hymen and transverse vaginal septum?
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
How do you diagnose and treat transverse vaginal septum
Ultrasound and MRI characterize thickness and other repro tract parts
Surgical correction is needed
Define vaginal atresia. How does it occur? What parts of the repro tract are normal?
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
How does vaginal atresia present?
How is it treated?
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
What is vaginal agenesis?
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
How is vaginal atresia diagnosed and treated?
Diagnosed - US and MRI
Treatment - psychosocial support, nonsurgical , and surgical correction
- serial vaginal dilators
- McIndoe procedure to create neovagina
What is the significance of lichen sclerosis
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
What is the presentation of lichen planus on vulva?
What is it associated with?
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
What is silver-red scaly patches on the vulva?
Vulvar psoriasis
scaly patches over elbows and knees usually occur
What is lichen simplex chronicus?
thickened skin with accentuated skin markings with chronic itching and scratching
What are the definite indications for biopsy of epithelial disorders of the vulva and vagina?
Ulceration
Unifocal lesions
Uncertain suspicion of lichen sclerosus
Unidentifiable lesions
Lesions or symptoms that recur after conventional treatment
What is the treament for epithelial disease of vulva/vagina
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
Where can cysts arise from?
pilosebaceous ducts
sebaceous ducts
apocrine sweat glands
What are the most common tumors found on the vulva?
What are its characteristics?
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
What are the diseases of apocrine sweat glands of the vulva/vagina
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
