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CHAPTER 13 Benign Diseases of the Female Reproductive Tract. Novak ’ s Gynecology page 399~412 OBGY R1 Lee eun suk . Vulvar Conditions. Neonatal Various developmental & congenital abnormalities Etiology Chromosomal abnormalities
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CHAPTER 13 Benign Diseases of the Female Reproductive Tract Novak’s Gynecology page 399~412 OBGY R1 Lee eun suk
Vulvar Conditions • Neonatal • Various developmental & congenital abnormalities • Etiology • Chromosomal abnormalities • Enzyme deficiencies (including 17- or 21- hydroxylase deficiency as causes of congenital adrenal hyperplasia) • Prenatal masculinization resulting from maternal androgen-secreting ovarian tumors • Drug exposures
Vulvar Conditions • Neonatal • Ambiguous genitalia represents a social & potential medical emergency that is best handled by a team of specialists, which include urologists, neonatologists, endocrinologists & pediatric gynecologists • The issues of gender assignment & timing of surgical therapy are controversial and should be managed by clinicians with extensive experience in the field
Vulvar Conditions • Neonatal • Careful inspection of the external genitalia of all female infants with gentle probing of the introitus & anus to determine the patency of the hymen or a possible imperforate anus • Congenital vulvar tumors • Strawberry hemangiomas : relatively superficial vascular lesions • Large cavernous hemangiomas
Vulvar Conditions • Childhood • Vulvovaginitis is most common ginechological problem • Prepubertally, the vulva, vestibule, vagina are anatomically & histologically vulnerable to infection with bacteria • The phygical proximity of the vagina & vestibule to anus can result in bacterial overgrowth → primary vulvitis & secondary vaginitis
Vulvar Conditions • Childhood - Lichen sclerosus • The cause is not well established • Cigarette paper appearance in a keyhole distribution (around vulva & anus) • Spontaneous regression through adrenarche & menarche • Medication • Progesterone in oil (400mg in 4 OZ. Of Aquaphor) • Betamethasone valerate (0.1% ointment, Valisone) • High-petency topical corticosteroids • Temovate(0.05%) cream
Vulvar Conditions • Childhood - Labial agglutination • Result of chronic vulva inflammation from any cause • Treatment • Brief course (2 to 4 weeks) of estrogen cream • Agglutination (adhesion) will become thin as the result • Separation can often be perfomed with topical anesthetic
Vulvar Conditions • Childhood • Urethral prolapse • Acute pain or bleeding • The presence of a mass may be noted • Vulvovaginal complaints of any sort in a young child should prompt the consideration of possible sexual abuse • Sexually transmitted infections may occur in prepubertal children • Sensitive but, direct questioning of the parent or caretaker & the child
Vulvar Conditions • Adolescence • Various development abnormalities • Vaginal agenesis, imperforate hymen, transverse & longitudinal vaginal septa, vaginal and uterine duplications, hymenal bands & septa • A tight hymenal ring may discovered because of concerns about the ability to use tampons or initiate intercourse • Manual dilation : small relaxing incisions at 6 o’clock & 8 o’clock • This can be done in the office using local anesthesia but may require general anesthesia in operating room • The possibility of sexual abuse, incest, or involuntary intercourse should be considered for vulvovaginal complaints, STDs, or pregnancy
Vulvar Conditions • Reproductive age women • Most related to a primary vaginits and a secondary vulvitis • Vaginal discharge → vulvar irritative symptoms or candida vulvutis • Vulvar symptoms : itching, pain, discharge, discomfort, burning, external dysuria, soreness, pain with intercourse or sexual activity • Itching is a very commom vulvar symptom • A variety of vulva conditions and lesions can present with pruritus • Burning with urination from noninfection causes may difficult to distinguish from a urinary tract infection • Some women can distinguish pain when the urine hits the vulvar area (an external dysuria) from burning pain (often suprapubic in locaion) • Urine culture
Vulvar Conditions • Reproductive age women • Vulvar conditions classified by infectious or noninfectious causes • The diagnosis of some of these conditions is apparent form inspection alone (e.g., a skin tag) • Any lesions that appear atypical or in which the diagnosis is not clear should be analyzed by biopsy
Vulvar Conditions • Reproductive age women • Pigmented vulvar lesions : benign nevi, lentigines, melanosis, seborrheic keratosis, & some vulvar intraepithelial neoplasias • 10% of white women have a pigmented vulvar lesion, some of lesions may be malignant or have the potential progression • The behavior of some nevocellular lesions (representing about 2% of nevi) is not well established but has been linked to melanoma • Multiple hyperpigmented lesions of typical lentigo simplex and melanosis are common, and any areas with irregular borders should be evaluated by biopsy
Vulvar Conditions • Vulvar biopsy • A vulvar biopsy is essential in distinguishing benign from pre-malignant or malignant vulvar lesions lesions, especially because many types of lesions may have a somewhat similar appearance • Epidermal inclusion cyst, lentigo, bartholin duct obstruction, CIS, melanocytic nevi, acrochordon, mucous cyst, hemangioma, post inflammatory hyperpigmentation, seborreic keratosis, varicosities, hidradenomas, verruca, basal cell ca, neurofibroma, ectopic tissue, syringomas, & abscess
Vulvar Conditions • Vulvar biopsy • Vulvar biopsies should be performed liberally to ensure these lesions are diagnosed and treated appropriately • Biopsy is easily performed in the office using a local anesthetic (1% lidocaine) • Punch biopsy Instruments, forceps, scissors, & a scalpel • Smaller biopsies unnecessary to place a suture • The biopsy sites will be painful for several days after procedure → 2% lidocaine jelly to be applied periodically and before urination
Other Vulvar Conditions • Pseudofolliculitis • Occur in women who follow the increasingly popular practice of shving pubic hair to confirm to swimsuit • Inflmmatory reaction surrounding an ingrown hair • Comomly among individual with curly hair, particularly African Americans
Other Vulvar Conditions • Fox-Fordyce diseassse • A chronic, pruritic eruption of small papules or cysts formed by keratin-plugged apocrine glands • Commonly present over the lower abdomen, mons pubis, labia majora, & inner portions of the thighs • Hidradenitis suppurativa • Chronic condition involving the apocrine glands with the formation of multiple deep nodules, scar, pits, and sinuses in the axilla, vulva, & perineum • Treatment : antibiotics, estrogens or antiadrogen therapy, isotreinoin & steroids, surgical therapy with wide local excision
Other Vulvar Conditions • Acanthosis nigricans • Wide spread velvety pigmentation in skin folds • Particularly the axillae, neck, thighs, submammary area, vulva and surrounding skin • Association with hyperandrogenism & PCOS as such • Obesity, chronic anovulation, acne, glucose intolerance, and cardiovascular disease
Vulvar Conditions • Intraepithelial neoplasia • Extramammary Paget’s disease of the vulva is an intraepithelial neoplasia containing vacuolated Paget’s cells • Moist, oozing ulcerations, eczematoid lesion with scaling and crusting • Biopsy to confirm the diagnosis is mandatory • Vulvar intraepithelial neoplasia • Associated with human pillomavirus infection • Increasing in frequency, particularly among young women • Suspicious vulvar lesions that are pigmented or discolored → biopsy
Vulvar Conditions • Vulva tumors, cysts, and masses • Condyloma accuminata • Very commom vulvar lesions • Treated with topical therapies such as tri-and bichloroacetic acid • Other sexually transmitted organisms, such as the virus responsible for molluscum contagiosum, the lesions of syphilis, and condyloma lata , may occasionally be mistaken
Vulvar Conditions • Vulva tumors, cysts, and masses • Bartholin duct cysts • Common vulvar lesions • Occlusion of the duct with accumulation of mucus • Frequently asymtomatic • Infection of the gland → accumulation of purulent material → formation of a rapidly enlarging, painful, inflammatory mass • Inflatable bulb-tipped catheter described by Word is inserted through a stab wound into the abscess →The ballon of the catheter is inflated with 2 to 3 ml of saline →The catheter remains in place for 4 to 6 weeks →Epithelization of the tract & creation of a permanent gland opening
Vulvar Conditions • Vulva tumors, cysts, and masses • Skene duct cysts • Cystic dilations of the Skene glands • Typically located adjacent tourethral meatus within the vulvar vestibule • Most small and asymtomatic • May enlarge & cause urinary obstruction → excision
Vulvar Conditions • Vulva tumors, cysts, and masses • The symptom of painful intercourse (dyspareunia) may be caused by many different vulvovaginal conditions • A careful sexual history & examination of the vulvar area is essential • Vulvodynia • Unexplained vulva pain, sexual dysfunction, & psychological disability • Vulva vestibulitis • Pain during intercourse, primarily during entry • Tender areas surrounding the vulvar vestibule and hymenal ring • Recent studies have failed to demonstrate a consistent relationship with any genital infectious organism, cllmydia, donorrhea, Trichomonas, mycoplasma, Ureaplasma, Gardnerella, candida, human papillomavirus
Vulvar Conditions • Vulvar ulcers • Caused by a number of STDs : herpes simplex virus, syphilis, lymphogranuloma venereum & granuloma inguinale • Chron’s disease • Abscesses, fistulae, sinus tracts, fenestrations, and other scarring • Behcet’s disease • Genital and oral ulcerations with ocular inflammation • The cause & the most effective therapy are not well established • Lichen planus • Oral & genital ulcerations • Typically desquamative vaginitis with vestibule erosion • Topical and systemic steroids
Vulvar Conditions • Post menopausal women - vulvar dystrophies • Vulvar epithelial growth that produce a number of nonspecific gross changes • Malignant potential is less than 5% • At particular risk is the patient cellular atypia on initial • Squamous hyperplasia • Most common in postmenopausal women • Pruritus is the most symptoms • Lesion appears thickened, hyperkeratotic & there may be exocoriation • Biopsy → evaluate the presence of atypia & exclude malignancy • fluorinated corticosteroid ointment 2 times s day for 6 weeks • New lesions recur → biopsy & additional 6 weeks of treatment
Vulvar Conditions • Post menopausal women - vulvar dystrophies • Lichen sclerosis • Most common white lesion of the vulva • Can occur at any age, although it is most common among post menopause and prepubertal girls • Symptoms : pruritus, dyspareunia, & burning • Decreased subcutaneous fat→ vulvar atrophy, with small or abscent labia minora, thin labia majora, & sometimes phimosis of the prepuce • Pale surface with a shiny, crinkled pattern, with fissures & excoriation • The lesions is symmetric and extends to the perineal & perianal area • The diagnosis is confirmed by biopsy & invasive cancer is rare • Treatment : ultrapotent topical steroid ( 0.05% clobestasol)
Vulvar Conditions • Post menopausal women – urethral lesions • Urethral caruncles and prolapse of the urethral mucosa • Can be treated with topical or systemic estrogen preparations
Vaginal Conditions • Vaginal discharge • One of the most common vaginal symptoms • Vaginal candidiasis, chlamydia cervicitis, bacterial vaginosis, & cervical carcinoma cause vaginal discharge • Other noninfectious causes of discharge • Retained foreign body-tampon, pessary • Ulcerations-tampon induced, lichen planus • Malignancy-cervical, vaginal • Postmenopausal atropic vaginitis, postradiation vulvovaginitis
Vaginal Conditions • Pediatric • Sexual abuse should always be considered in prepubertal children with vaginal discharge • Routine STD cultures in girls with a history of sexual abuse Vaginal culture for gonorrhea and chlamydia should be performed • Vulvovaginitis • Usually caused by multiple organisms in perineal area • Single organism, streptococcus or Shigella, may be causative • Treatment → Hygienic and cleansing measures → Short-term (<4 weeks) course of topical estrogens & broad-spectrum antibiotics
Vaginal Conditions • Adolescence and older • Toxic shock syndrome (TSS) • Associated with tampon use & vaginal Staphylococcus aureus-produced exotoxins • Fever, hypotension, a diffuse erythroderma with desquamation of the palms & soles, plus involvement of at least three major organ systems • Vagina : mucous membrane inflammations
Vaginal Conditions • Adolescence and older • Fibroepithelial polyps • Polypoid folds of connective tissue, capillaries, and stroma covered by vaginal epithelium • Excision • Cysts of embryonic origin • From mesonephric, paramesonephric & urogenital sinus epithelium • Gartner’s duct cysts • Mesonephric origin • Usually present on lateral vaginal wall
Vaginal Conditions • Adolescence and older • Vaginal adenosis • The presence of epithelial-lined glands within the vagina • Associated with in utero exposure to diethylstilbestrol • No therapy is necessary • Bulging lesion of the vagina & vulvar area • Associated with symptoms of pressure or discomfort • Most common cause : cystocele, rectocele, or urethrocele