Vulva and vagina

Question 1

What is the anatomy of the vagina and vulva?

Answer 1

• The vulva is the area of skin that stretches from the labia majora laterally to the mons pubis anteriorly and perineum posteriorly- it overlaps the vestibule (area between labia minora and hymen), which surrounds the urethral and vaginal orifices
• The vagina is 7-10cm long, it is lined with squamous epithelium
o Anterior- bladder and urethra
o Posterior to the upper third- pouch of Douglas
o Lower posterior wall- close to rectum
• Most lymph drainage occurs via the inguinal lymph nodes, which drain to the femoral and then the external iliac nodes of the pelvis, this is a route for metastatic spread of vulval carcinoma

Question 2

What are vulval symptoms?

Answer 2

o Pruritis
o Soreness
o Burning
o Superficial dyspareunia
• Symptoms can be due to local problems- including infection, dermatological disease, malignant and premalignant disease and the vulval pain syndromes
• Skin diseases affect the vulva, but rarely in isolation- systemic diseases can also predispose to certain vulval conditions – eg. candidiasis with DM

Question 3

What are the causes of pruritus vulvae?

Answer 3

Infections: 
	Candidiasis (± vaginal discharge)
	Vulval warts (condylomata acuminate)
	Pubic lice, scabies
Dermatological disease:
	Eczema
	Psoriasis
	Lichen simplex
	Lichen sclerosus
	Lichen planus
	Contact dermatitis
Neoplasia:
	Carcinoma
	Premalignant disease- VIN

Question 4

What is lichen simplex (chronic vulval dermatitis)?

Answer 4

• Common in women with sensitive skin, dermatitis or eczema, this presents with severe intractable pruritus, especially at night
• Typically the labia major is affected- it can become inflamed and thickened with hyper- and hypopigmentation
• The symptoms can be exacerbated by chemical or contact dermatitis- sometimes linked to stress or low body iron stores
• Vulval biopsy is indicated if the diagnosis is in doubt
• Emollients, moderately potent steroid creams and anti-histamines are used to treat

Question 5

What is lichen planus?

Answer 5

• A common disease which can affect any area of the body, particularly mucosal surfaces, such as mouth and genital region
• Presents with flat, popular, purplish lesions- in the mouth and genital region it can be erosive and is more commonly associated with pain than pruritus
• The aetiology is unknown, but may be autoimmune related- it can affect all aged and is not linked to hormonal status
• Treatment is with high-potency steroid creams- surgery should be avoided

Question 6

What is lichen sclerosus?

Answer 6

vuval epithelium is thin with loss of collagen
this may have an autoimmune basis and thyroid disease & vitiligo may coexist
~40% of women have or go on to develop another autoimmune condition
• Typically affects post-menopausal women, but much younger women can occasionally be affected

Question 7

How does lichen sclerosus present?

Answer 7

• It causes severe pruritus, which may be worse at night- uncontrollable scratching may cause trauma with bleeding and skin splitting, symptoms of discomfomort, pain and dyspareunia
• The skins appearance is pink-white papules, which coalesce to form parchment-like skin with fissures-
inflammatory adhesions can form, potentially causing fusion of the labia and narrowing of the introitus
• Vulval CA can develop in 5% of cases- biopsy is important to exclude carcinoma and to confirm diagnosis
• Treatment is with ultra-potent topical steroids

Question 8

What is vulvar dysaesthesia (vulvodynia)?

Answer 8

Diagnosis of exclusion- no evidence of organic vulval disease
they are divided into provoked or spontaneous vulvar dysaesthesia, then subdivided into local (vestibular) or generalised
Associated with
o A history of genital tract infections
o Former use of oral contraceptives
o Psychosexual disorders
• Spontaneous generalised vulvar dysaesthesia describes burning pain, more common in older patients
• Vulvar dysaethesia of the vestibule causes superficial dyspareunia or pain using tampons- more common in younger women, in whome introital damage must be excluded
• For both conditions, topical agents are seldom helpful, oral drugs (gabapentin or amitriptyline) are sometimes used

Question 9

What are the infections of the vulva?

Answer 9

o	Herpes simplex
o	Vulval warts
o	Syphilis
o	Donovanosis
Candidiasis (more common in diabetes, obesity, pregnancy, immunocompromised or when antibiotics are used)

Question 10

What are baertholin’s gland cysts and abscesses?

Answer 10

• The two glands behind the labia minora secrete lubricating mucus for coitus- blockage of the duct causes cyst formation
• If infection occurs an abscess forms- commonly caused by Staphlococcus or E.Coli
• This is acutely painful- a large tender red swelling is evident
• Treatment is with incision and drainage- marsupialisation may also be used – where the incision is sutured open to reduce risk of reformation

Question 11

What is introital damage?

Answer 11

• Commonly follows childbirth- caused by overtightening, incorrect apposition at perineal repair or extensive scar tissue, commonly presents with superficial dyspareunia, symptoms often resolve with time
• If the introitus is too tight- vaginal dilators or surgery (Fenton’s repair) are used

Question 12

What are vaginal cysts?

Answer 12

• Congenital cysts commonly arise in the vagina- they have a smooth white appearance and can be as large as a golf ball, often mistaken for a prolapse
• Do not often cause symptoms, but if there is dyspareunia they should be excised

Question 13

What is vaginal adenosis?

Answer 13

• Vaginal adenosis is when columnar epithelium is found in the vagina, which is normally squamous epithelium
• Commonly occurs in women whose mothers received diethylstilboestrol (DES) in pregnancy, when it is associated with genital tract abnormalities, women with DES exposure in utero are screened annually by colposcopy
• Spontaneous resolution is usual- can very occasionally turn malignant (clear cell CA of the vagina), it may also occur secondarily to trauma

Question 14

What is usual type vulval intraepithelial neoplasia (VIN)?

Answer 14

• Nearly all VIN is usual type, can be warty, basaloid or mixed, more common in women aged 35-55
• It is associated with
o HPV (16)
o CIN
o Cigarette smoking
o Chronic immunosuppression
• May be multifocal, but appearance can vary widely- red, white or pigmented, plaques, papules or patches, erosions, nodules, wart or hyperkeratosis, associated with warty or basaloid SCC

Question 15

What is differentiated type VIN?

Answer 15

• Rarer than usual type- can be associated with lichen sclerosis, commoner in older women
• The lesion is usually unifocal- ulcer or plaque formation, linked to keratinising SCC of the vulva
• The risk of progression to cancer is high than for usual type VIN

Question 16

What is the management for VIN?

Answer 16

• Pruritus or pain is common- emollients or a mild topical steroid may help
• Gold standard is local surgical excision to relieve symptoms, confirm histology and exclude invasive disease-15% of women having excision have unrecognised invasive disease, if conservative or medical treatment is used, adequate biopsies must be taken

Question 17

What is carcinoma of the vulva?

Answer 17

accounts for 5% of genital tract cancers, up to 1200 new cases each year in the UK and 400 deaths- most common >60yrs
• 95% of vulval malignancies are SCC, melanomas, BCCs, adenocarcinomas and others (sarcomas) account for the rest
Associated with:
o Lichen sclerosis
o Immunosuppression
o Smoking
o Paget’s disease of the vulva

Question 18

How does carcinoma of the vulva present?

Answer 18

• Presents with pruritus, bleeding or discharge- often presents late as lesions go unnoticed or cause embarrassment
• Examination will reveal an ulcer or mass- most common on the labia majora or clitoris, inguinal LN may be enlarged, hard and immobile
• 50% of patients present with stage 1 disease- spreads locally and via lymph drainage, spread is to superficial and then deep inguinal nodes, then to femoral and external iliacl nodes

Question 19

What is the management for vulval carcinoma?

Answer 19

• Stage 1a: wide local excision without inguinal lymphadenectomy, risk of spread is negligible
• Other stages: for women with unifocal squamous cancers of <4cm, with no clinical or radiological suspicion of LN metastasis a sentinel lymph node biopsy (SLNB) may be used- a radioactive isotope +/- blue dye is injected around the tumour site and any sentinel node is biopsied for metastasis
if no sentinel node is found- inguinofemoral lymphadenectomy is considered

Question 20

What is the management for vuval cancer if SLNB is +ve?

Answer 20

wide local excision and groin lymphadenectomy is performed eg. triple incision radical vulvectomy
• Radiotherapy may be used to shrink large tumours prior to surgery, post-operatively if groin lymph +ve or palliatively to treat severe symptoms
• Reconstructive surgery involving plastics is considered where a major resection is planned
• Many of these patients die from other diseases related to their age: 5yr survival in stage 1 in >90% and stage 3-4 is 40%

Question 21

What are the complications of a wide local excision and groin lymphadenectomy?

Answer 21

o	Wound breakdown
o	Infection
o	Leg lymphoedema
o	Lymphocyst formation
o	Sexual & body image problems

Question 22

What are the malignancies of the vagina?

Answer 22

• Secondary vaginal carcinoma- common and arises from local infiltration from cervix, endometrium, vulva or metastatic spread from cervix, endometrium or gastrointestinal tumours
• Primary carcinoma of the vagina: 2% of genital tract malignancies, older women usually SCC. bleeding, discharge and a mass/ulcer treatment: intravaginal radiotherapy or radical surgery. average 5yr survival is 50%
• Clear cell adenocarcinoma of the vagina: normally rare complication affecting the daughters of women prescribed DES during pregnancy during 1950-70s
radical surgery and radiotherapy- survival rates are good