13. Benign Disorders of the Lower Genital Tract Flashcards
Congenital Anomalies of the Vulva and Vagina (5)
- Labial Fusion
- Imperforate Hymen
- Transverse Vaginal Septum
- Vaginal Atresia
- Vaginal Agenesis
Labial Fusion
- Association?
- Etiology?
- Next step?
Associated with excess androgens
- Most common etiology: exogenous androgen exposure
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Secondary etiology: enzymatic deficiency = 21-hydroxylase deficiency –> CAH
- Phenotype: neonate with ambiguous genitalia, hyperandrogenism with salt wasting, hypotension, hyperkalemia, and hypoglycemia
- Neonate often presents in adrenal crisis with salt wasting seen approximately 75% of the time
- Dx: elevated 17 alpha-hydroxyprogesterone
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Secondary etiology: enzymatic deficiency = 21-hydroxylase deficiency –> CAH
- Labial fusion and other forms of ambiguous genitalia –> reconstructive surgery
Imperforate Hymen
Results in an obstruction to the outflow tract of the reproductive system –> buildup of secretions in the vagina behind the hymen (hydrocolpos or mucocolpos)
Transverse Vaginal Septum
Pathogenesis
Clinical Presentation
Vagina is formed as the Mullerian system from above joins the sinovaginal bulb-derived system from below. This takes place at the Mullerian tubercle. The Mullerian tubercle must be canalized for a normal vagina to form. If this does not occur, the tissue may be left as a transverse vaginal septum.
Clinical presentation: primary amenorrhea and cyclic pelvic pain accompanied by menstrual symptoms
Vaginal Atresia (agenesis of the lower vagina)
Pathogenesis
Occurs when lower vagina fails to develop and is replaced by fibrous tissue
The ovaries, uterus, and upper vagina are all normal.
Vaginal Agenesis
(Mayer-Rokitansky-Kuster-Hauser syndrome)
Complete absence of vagina or hypoplasia of all or part of the cervix, uterus, and fallopian tubes
Benign Epithelial Disorders of the Vulva and Vagina
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Lichen sclerosis:
- Inflammatory dermatosis that is found on the vulva of women of all ages (but significance in postmenopausal women –> 3-4% risk of vulvar skin cancer)
- Tx: clobetasol
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Lichen planus:
- Uncommon inflammatory skin condition that can affect the nails, scalp, and skin mucosa
- Ass: vaginal adhesions –> occurs in women in their 50s and 60s and is associated with 3-4% risk of vulvar skin cancer
- Tx: clobetasol
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Vulvar psoriasis
- Red most lesions, sometimes scaly
- Tx: clobetasol, UV light
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Lichen simplex chronicus
- Thickened skin
- Intense pruritis
Most common tumor found on vulva?
Epidermal inclusion cyst (benign)
Usually result from occlusion of pilosebaceous duct or blocked hair follicle
How is the diagnosis of vulvar lesions made?
By palpation, visualization, magnified vulvoscopy, and biopsy
Cancer should always be excluded by biopsy
What kind of cysts can arise on the vulva and vagina?
Occlusion of pilosebaceous ducts, sebaceous ducts, and apocrine sweat glands
Where are Bartholin’s cysts and abscesses located? Presentation? Treatment?
4-o’ clock and 8-o’ clock positions on the labia majora
Cysts are usually asymptomatic and resolve on their own… however, large symptomatic ones should be drained along with placement of Word catheter/marsupialization… abx are generally indicated
Cervical stenosis may be congenital or idiopathic and may result from scarring from infection or surgical manipulation.
If symptomatic, tx?
Gentle dilation of cervical canal
26 yo G0 pt comes in with a problem visit for complaint of an intermittent painless mass on her vulva near the introitus. It seems to be aggravated by intercourse but usually goes away on its own. She’s had two lifetime sexual partners and has been with her last partner for 5 years. She has always had normal periods and Pap smears and has never had an STI. You examine her and find a 3 cm nontender mass in the area described.
What type of abnormality is this most likely to be?
a. Skene’s gland cyst
b. Gartner’s duct cyst
c. Bartholin’s duct cyst
d. Cystocele
e. Epidermal inclusion cyst
c. Bartholin’s duct cyst
Classic location of Bartholin’s glands. These glands, located at 4-o’clock and 8-o’clock positions near the introitus provides lubrication of the vagina. The ducts of Bartholin’s glands can become blocked resulting in a cyst formation.
Skene’s glands can be ID’ed as small openings on either side and just below the urethral meatus.
Gartner’s duct cysts are remnants of wolffian system. They are found in the upper 1/3 of the vagina on the anterior vaginal wall.
Cystocele = prolapse of bladder into vagina –> typically appears as midline protrusion of anterior vaginal wall into vagina
Treatment for recurrent Bartholin’s cyst
a. Expectant mgmt
b. Word catheterization
c. I&D
d. Marsupialization
e. Excision
b. Word catheterization
Word catheter is placed to relieve the obstruction. Leaving the Word catheter in place for several weeks gives the new tract time to reepithelize hopefully resulting in a means of long-terms drainage. Marsupialization is typically reserved for patients in whom the Word catheter has failed.
While on call, you are paged to ED to see a 16 yo G0 adolescent girl with cyclic pelvic pain. She has never had a menstrual cycle. She denies any hx of intercourse. She is afebrile and her vital signs are stable. Her pregnancy test is negative. On physical exam, she has age-appropriate breast and pubic hair development and normal external genitalia. However, when attempting the pelvic exam, you are unable to locate a vaginal introitus. You obtain a transabdominal U/S, which reveals hematocolpos and hematometra.
What is the most likely dx?
a. Transverse vaginal septum
b. Vertical vaginal septum
c. Imperforate hymen
d. Vaginal agenesis
e. Bicornuate uterus
d. Vaginal agenesis (MRKH)
Congenital absence of the vagina/ unable to locate vaginal introitus
