Disorders of Vulva & Vagina Flashcards
External vulvar anatomy
- boundaries
- structures included
- Boundaries extend from mons pubis to anus to labial cural folds
- Structures include: Labia majora, labia minora, paired Bartholin’s glands, Skene’s glands, urethra, anus, clitoris
Lymphatic drainage of vulva
- Lymphatics of vulva drain from posterior to anterior traversing thru the mons pubis and into superficial and deep inguinal nodes
- Tend to drain to ipsilateral side
What is the function of bartholin glands?
Secrete mucus for lubrication
What are the 3 tissue types and locations that make up bartholin glands?
- Proximal = glandular epithelium
- Distal = transitional epithelium
- Opening = squamous epithelium
What causes a bartholin gland cyst?
Orifice of gland may become obstructed leading to mucus accumulation and cystic dilatation.
Characteristics of bartholin gland cyst
- unilateral
- soft, painless mass
Which population should always have an excision of a bartholin gland cyst?
women over 40 to exclude carcinoma
How is a bartholin abscess different from a bartholin gland cyst?
-the cyst is now infected
Characteristics of bartholin gland abscess
- very painful
- fluctuant, swollen, red, warm, mass
Tx of bartholin gland abscess
I&D with culture, consider drain placement (Word catheter)
What is a word catheter?
- catheter that is placed into the area of the now empty abscess
- it is inflated and left in place for 4-6 weeks
- this promotes the formation of an epithelialized tract for drainage
Marsupialization of Bartholin Gland Abscess
- Abscess cavity is incised 1-2 cm in length and drained
- The edge of the cyst wall is grasped and everted open
- The opening is then sutured open creating a new larger duct for drainage
List the non-neoplatic disorders (4)
- Lichen Sclerosis
- Lichen Simplex Chronicus/Squamous Cell Hyperplasia
- Lichen Planus
- Vulvar Psoriasis
What is the most common vulvar derm disorder?
Lichen sclerosis
Etiology of lichen sclerosis
- Chronic, relapsing and remitting disorder (inflammatory)
- Peak Onset: Pre-pubertal and postmenopausal women
Appearance/sx of lichen sclerosis
- *Figure 8/hourglass appearance**
- Lesions appear as smooth, white plaques.
- Surface is smoothed and resembles parchment or wrinkled cigarette paper.
- Sx: intense pruritis, pain, dyspareunia
Etiology and anatomy of lichen sclerosis
Etiology is unknown:
- Autoimmune
- Genetic
- Hormonal
- labia minora, labia majora, clitoris, and perineum can all be involved
- vagina is spared
Dx/Tx of lichen sclerosis
Diagnosis made by punch biopsy.
-Repeat biopsy if new lesions/symptoms as these patients are at increased risk for vulvar cancer.
Treatment:
- Patient education
- Topical corticosteroids
- -Applied BID/daily until symptoms are controlled.
- -Then 1-3 x weekly for maintenance.
List the drugs for tx of lichen sclerosis
- Topical tacrolimus (Protopic 0.03 or 0.1%) or pimecrolimus (Elidel 1%) demonstrated effective but mainly use with taper.
- Recalcitrant: oral hydroxychloroquine or cyclosporin
Etiology of Lichen Simplex Chronicus
- AKA squamous cell hyperplasia – NOT a malignant precursor**
- Secondary to chronic rubbing/scratching.
- Associated with pruritis
- Mostly in premenopausal women
- Typically found on hair-bearing areas of labia majora
Describe clinical findings of lichen simplex chronicus
- Characterized by benign epithelial thickening and hyperkeratosis from chronic irritation
- Skin is thick with exaggerated skin markings
- Usually bilateral and symmetric
- *not a distinct entity but rather a description of morphologic alterations of vulva.
Dx/Tx of lichen simplex chronicus
Diagnosis is again made by biopsy.
Treatment:
- Patient education
- Identify and treat cause of itching (infection, contact dermatitis, etc.)
- Topical corticosteroids
Etiology of Lichen Planus
- Rare disorder believed to be autoimmune.
- Affects skin and mucous membranes
- Commonly affects the groin and the mouth**
- Leads to intense pruritus, burning, dysuria, dyspareunia, and post coital bleeding
- Mostly affects postmenopausal women
- Almost 70% of patients have vaginal involvement
What is lichen planus characterized by??
Lesions characterized by bright erythematous erosions with white striae or white border (Wickham’s striae) visible along the margins
Complications of lichen planus
- Recurrent exacerbations, slow healing and scarring are common
- Scarring can cause significant anatomic disruption, stenosis of vaginal opening and urethral opening
Tx of lichen planus
Can be difficult to treat:
- Topical corticosteroids
- Oral corticosteroids
- Vaginal dilators
- Recalcitrant : hydroxychloroquine, thalidomide, apremilast (Otezla), cyclosporine, methotrexate, mycophenolate mofetil
There is no role for surgery
Etiology of vulvar psoriasis
- Systemic skin disease characterized by silver-white scales.
- Vulvar lesions sharply demarcated erythematous absent of the silver-white scale.
- Moist and shiny
- Can affect elbows, knees, back, scalp, and vulva.
- It can be located just in one area, but it’s likely to be elsewhere on the body.
- Associated with pruritis.
What is koebner phenomenon in regards to vulvar psoriasis?
Koebner phenomenon – skin lesions on lines of trauma
Dx/Tx of vulvar psoriasis
Diagnosis is made by vulvar biopsy.
Treatment includes:
- Topical high potency steroids followed by maintenance therapy with lower potency steroids.
- Tar creams
List the vulvar neoplasms (5)
- Extramammary Paget Disease
- Vulvar Intraepithelial Neoplasia (VIN)
- Vulvar carcinoma
- Bartholin gland carcinoma
- Vulvar melanoma
Etiology of Extramammary Paget Disease
- Generally benign condition.
- Pruritic, erythematous, with well-demarcated eczematoid appearance.
- MC in postmenopausal, Caucasian women, 60s and 70s.
What nonvulvar locations will women have carcinoma in with extramammary paget disease?
- Breast
- Colon
- Urethra
- Bladder
Tx of extramammary paget disease
Treatment includes:
- Patient education
- Wide local excision
- Chemotherapy – 5-FU, imiquimod
- -These are typically used for widespread skin cancers: help to attract the bodies own immune system.
- Microscopically positive margins are common.
- Recurrence is common – 30-60%, 8-26% with MOHS.
What is vulvar intraepithelial neoplasia (VIN)?
- Precancerous/dysplastic lesion.
- Spectrum of disease ranging from mild dysplasia to vulvar carcinoma in-situ
Describe the lesions in vulvar intraepithelial neoplasia (VIN)
- Lesion appearance is highly variable but usually sharply demarcated.
- May be white, hyperkeratotic plaques, hyperpigmented lesions or areas of erythema
Patient presentation with VIN
Patients may be asymptomatic or have pruritus, bleeding, or pain.
Etiology and risk factors of VIN
- Incidence is increasing - particularly in younger women (related to HPV)
- Bimodal distribution of patients:
- -Younger, premenopausal women
- -Postmenopausal women
Risk Factors:
- HPV infection (90%)
- Smoking
- Immunodeficiency (HIV)
What is the importance of the “grades” of VIN?
- The degree of dysplasia has to do with the depth into the epidermis/dermis.
- Remember: carcinoma in-situ is full-thickness of the epidermis.
Describe mild dysplasia/VIN I
- dysplasia confined to the lower third of epithelium.
- now thought to be benign reactive changes or HPV effect by low-risk HPV types
Describe moderate dysplasia/VIN II
dysplasia confined to the lower two-thirds of epithelium
Describe severe dysplasia/VIN III
dysplasia extending to the upper third of epithelium, but not involving full thickness
Describe carcinoma in situ
full thickness involvement
When can you classify invasive disease or cancer?
Invasive disease or cancer is NOT present until abnormal cells extend beyond the basement membrane and into the stroma below***
Define usual type VIN (uVIN). What patient population is it seen in?
-most VIN 2 and 3.
-associated with oncogenic HPV infection.
HPV 16 is the most prevalent HPV type found in VIN and vulvar cancer***
-Tend to be multicentric.
-Younger women, multiple sex partners, STDs and smoking
Define differentiated type VIN (dVIN). What patient population is it seen in?
- Tend to be unifocal.
- Older, non-smoking, post-menopausal (60-70s).
- Oncogenic HPV uncommon.
- More likely to progress to SCC vs uVIN***
Define unclassified type VIN
- pagetoid types
- Extramammary paget’s disease
Dx/Tx of VIN
-Diagnosis is made by biopsy
Treatment options include:
- Wide local excision – both for symptom relief as well as r/o invasion
- Laser ablation
- Topical imiquimod cream
- Topical 5-fluorouracil cream
- Patients should be counseled regarding smoking cessation.
- Untreated lesions may persist, progress, or resolve.
- Recurs frequently.
Etiology of vulvular carcinoma
-Represents 5% of all gyn malignancies.
90% SCC
-< 20% are younger than 50 years: >50% in women older than 70
Vulvular carcinoma in older women
- Peak incidence in women ages 60–70 years
- Chronic inflammation - Lichen sclerosis OR genetic alterations
Vulvular carcinoma in younger women
- HPV-related- 50% cases.
* H/O smoking + H/O HPV genital warts have a 35–fold increased risk for developing vulvar cancer
Vulvular carcinoma dx/tx
- Diagnosis made by biopsy
- Refer to Gyn for vulcoscopy- helps direct biopsy site selection (similar to colposcopy).
- Biopsy via punch bx to allow evaluation for depth of invasion
Tx of vulvar cancer
Surgical excision with 2 cm margin
-Modified radical vulvectomy
Sentinel lymph node biopsy
-For prognosis only
Adjuvant therapy for vulvar cancer
- Chemoradiation
- Chemotherapy
- Immune therapy
- Targeted therapy
Etiology of Bartholin Gland Carcinoma
- 1% vulvar cancers
- Painless vulvar mass.
- Dyspareunia common first complaint
- Size range from 1 – 7 cm.
- Can get quite large before symptomatic.
- Most appear in postmenopausal women peak mid-60’s
REMEMBER: Bartholin gland enlargement in a woman older than 40 years and recurrent cysts or abscesses warrant a biopsy***
What cancer types are most prominent in Bartholin Gland Carcinoma?
- Squamous cell carcinoma (50%)
- Adenocarcinoma (40%)
- Adenoid cystic carcinoma
- Transitional cell carcinoma
- Adenosquamous carcinoma
What is the criteria for diagnosis of Bartholin gland carcinoma?
1) arise at site of Bartholin gland
2) consistent histologically with primary Bartholin neoplasm
3) not be metastatic
Explain the lymphatic spread of Bartholin gland carcinoma
Propensity for lymphatic spread into the inguinal and pelvic lymph nodes
Tx of Bartholin gland carcinoma
- Radical partial vulvectomy with inguinofemoral lymphadenectomy
- Postoperative chemoradiation has been shown to reduce the likelihood of local recurrence
Etiology of vulvar melanoma
- 5-10% of all vulvar cancers, second most common vulvar cancer.
- Postmenopausal**
- MC arises in the region of the labia minora and clitoris
Pigmented vs. nonpigmented vulvar melanoma
- Nonpigmented melanoma may closely resemble squamous cell carcinoma
- Pigmented vulvar neoplasia may be VIN, squamous cell carcinoma, Paget disease or melanoma
- *sampling is mandatory
- Staging and prognosis linked to depth of invasion.
What kind of biopsy should you do for vulvar melanoma?
-Excisional biopsy
-Punch biopsy
**NOT shave biopsy
In shave biopsy, you use flat edge razor blade and get a little scoop of tissue - you’re not getting down through the whole dermis this way
Tx of vulvar melanoma
- Excision is the single best definitive therapy
- May consider adjuvant alpha interferon (IFN-α)
List the vaginal neoplasms (2)
- Vaginal intraepithelial Neoplasia (VAIN)
2. Vaginal carcinoma
Etiology of vaginal intraepithelial neoplasia (VAIN)
- Pre-cancerous lesion/dysplasia.
- Less common than VIN or CIN.
Risk factors for VAIN
- HPV infection
- Immunosuppression
- Preceding or co-existing squamous cell carcinoma of the vulva, cervix, or anus.
- At least ½ - 2/3 with VAIN have been treated for similar disease in either the cervix or the vulva
Tx of VAIN
surgical excision or laser ablation
Etiology of vaginal carcinoma
- Rare, representing < 1% of malignancies of the female genital tract.
- Vaginal cancer is more likely metastatic dz
- *Mean age of diagnosis is 64 years
Sx of vaginal carcinoma
painless vaginal bleeding, especially postcoital, vaginal discharge, or dysuria.
Risk factors for vaginal carcinoma
- HPV infection
- Smoking
- H/o previous vulvar or cervical cancer
What tissue type is MC for vaginal carcinoma?
Majority of cases are squamous cell carcinoma.
What is considered a primary tumor of vaginal carcinoma?
- Located in vagina
- No evidence of cervix or vulvar involvement
**Most tumors are in the upper 1/3 vagina and posterior wall.
Tx of vaginal carcinoma
- surgical excision in lower stage cancers if negative surgical margins can be obtained. + hysterectomy
- Radiation therapy with chemotherapy
Define cystocele
Prolapse of the anterior vaginal wall causing by bladder collapsing on weak vaginal muscle.
Define rectocele
Rectum collapsing on weak vaginal muscle in the posterior wall.
Define apical prolapse
AKA uterine prolapse
-uterus collapsing from the top of the vaginal wall
What can cause pelvic organ prolapse?
Anterior and posterior vaginal relaxation, loss of pelvic floor muscle support, loss of connective attachments between the vaginal wall and the pelvic floor
Etiology of pelvic organ prolapse
- Large anterior vaginal prolapse (cystocele) is MC than posterior vaginal prolapse
- Third MC indication for hysterectomy
- Estimated that 30 to 65% of women presenting for routine gynecologic care have stage 2 prolapse
Pelvic organ prolapse risk factors
- Vaginal childbirth – especially if multiple
- Increasing parity
- Age
- Obesity
- Race - Black and Asian women show the lowest risk, whereas Hispanic and white women appear to have the highest risk
- Hysterectomy
- Repetitive increases in intraabdominal pressure (chronic constipation, COPD)
- Connective tissue disorders
Clinical Manifestations of pelvic organ prolapse
- Bulge/pressure
- “Something falling out”; “sitting on a ball”
- Urinary incontinence, frequency
- Back/pelvic pain
- Effects on sexual function
Pelvic organ prolapse management
- tx indicated for symptomatic patients
- conservative management:
- -Pelvic floor muscle exercises/PT- Kegel exercises
- -Pessary
- Surgical management
What are the 2 kinds of pessaries? How do they work?
**support and space-filling
Support pessaries rest in the posterior fornix behind the cervix and behind the pubic symphysis anteriorly.
Space filling crate suction between the pessary and vaginal walls, or by creating a larger diameter hiatus or both.
What causes atrophic vaginitis?
- Also called urogenital atrophy**
- Due to estrogen deficiency
What population is atrophic vaginitis seen in?
40% postmenopausal women, but only 20-25% symptomatic seek medical attn.
Explain how the vaginal epithelium responds to estrogen from birth to puberty
Vaginal epithelium responds to levels of circulating estrogen:
- Maternal estrogen – newborn epithelium rich in glycogen
- Puberty – thickens with copious amounts of glycogen
- Lactobacilli depend on glycogen
- Lactic acid produced by lactobacilli keep pH lower, essential for natural defense against vaginal and urinary infections
Atrophic Vaginitis
-signs and symptoms
- Long-term decrease in estrogen stimulation is generally required before symptoms
- Decrease in vaginal lubrication is an early hallmark hormone insufficiency**
Genital symptoms of atrophic vaginitis
Dryness, burning, dyspareunia, vaginal dryness, vulvar pruritus, feeling of pressure, itching and yellow malodorous discharge
Urinary symptoms of atrophic vaginitis
Urethral discomfort, frequency, hematuria, urinary tract infection, dysuria and stress incontinence may be later symptoms
What should be considered on your diff dx for atrophic vaginitis?
- *Do not assume a diagnosis of atrophic vaginitis (or solely AV) in the postmenopausal patient who presents with urogenital complaint
- Exogenous agents that may cause or further aggravate symptoms:
- Perfumes, powders, soaps, deodorants, panty liners, spermicides and lubricants often contain irritant compounds.
- In addition, tight-fitting clothing and long-term use of perineal pads or synthetic materials
- Candidiasis, BV, Trichomoniasis
Atrophic Vaginitis
-genital physical exam
- Pale, smooth and shiny vaginal epithelium
- Inflammation with patchy erythema, petechiae
- Loss of elasticity or turgor of skin
- Sparsity of pubic hair
- Dryness of labia
- Fusion of labia minora
- Introital stenosis
- Friable, unrugated epithelium
- Pelvic organ prolapse/Rectocele
- Vulvar dermatoses/lesions
Atrophic vaginitis
-urethral physical exam
- Urethral caruncle (benign fleshy outgrowth of posterior urethral meatus)
- Eversion of urethral mucosa
- Cystocele
- Urethral polyps
- Ecchymoses
- Minor lacerations at peri-introital
Diagnosis of atrophic vaginitis
- Dx is usually clinical but serum hormone levels and Pap smear, can confirm
- Elevated pH level (postmenopausal pH levels exceeding 5), pH strip in the vaginal vault
- Vaginal US of the uterine lining demonstrates thin endometrium measuring between 4 and 5 mm - signifies loss of adequate estrogenic stimulation
Tx of atrophic vaginitis
- Estrogen replacement therapy helps to restore normal pH levels, thickens/revascularizes epithelium
- -May alleviate existing symptoms or even prevent development of urogenital symptoms if initiated
- Systemic administration
- -Also helps with decrease in postmenopausal bone loss and alleviation of vasomotor dysfunction (hot flashes)
- -Higher dosages may be necessary to alleviate atrophic sxs
- -Up to 24 months of therapy may be necessary to totally eradicate dryness
Transvaginal delivery tx for atrophic vaginitis
-advantages
- creams, pessaries, hormone-releasing ring (Estring)
- Advantage may be decreased risk of endometrial carcinoma because a lower hormone amount is required**
Transvaginal delivery tx for atrophic vaginitis
-disadvantages
- patient dislike of vaginal manipulation
- less prevention of postmenopausal bone loss and vasomotor dysfunction
- decreased control of absorption with vaginal creams compared to oral and transdermal delivery
- irregular treatment intervals that may cause patients to forget to administer
What is the relationship between hormone concentration and transvaginal delivery for atrophic vaginitis?
- Transvaginal rings offer convenience, constancy of hormonal concentration in the blood stream and a therapeutic value equivalent to creams without the need for frequent application
- Atrophic vaginitis symptoms are relieved (dosage of 5 to 10 μg per 24 hours) without stimulation of endometrial proliferation, thereby eliminating need to add opposing progesterone
Atrophic vaginitis tx
-moisturizers and lubricants
- For those who choose no ERT or contraindicated BUT then should not use products with ginseng - may have estrogenic properties**
- Length of effectiveness - generally less than 24 hours
Sexual activity and atrophic vaginitis
- Shown to encourage vaginal elasticity and pliability, and the lubricative response
- Pts report fewer symptoms of atrophic vaginitis, less evidence of stenosis compared with sexually inactive women
