Blueprints Flashcards
labial fusion is due to what?
excess androgens
what is most common etiology of labial fusion?
EXOGENOUS androgen exposure
What is most common form of enzymatic deficiency –> labial fusion + ____?
21 hydroxylase deficiency which can cause labial fusion and CONGENITAL ADRENAL HYPERPLAISA
Clinical appearance of labial fusion and congenital adrenal hyperplasia?
ambiguous genitalia, hyperandrogenism, salt wasting, hypOtension, hyperkalemia, and hypOglycemia
How to diagnose 21 hydroxylase deficiency?
Either:
- elevated 17a-hydroxyprogesterone
- elevated urine 17-ketosteroid w/ dec serum cortisol
Treatment of 21 hydroxylase deficiency (–> labial fusion and CAH)?
- Exogenous cortisol (helps normalize level + neg feedback to inhib ACTH)
- mineralcorticoid for salt-wasting
- reconstructive surgery for ambiguous genitalia
What is imperforate hymen?
When the epithelial cells in the central portion of the hymenal membrane do NOT degenerate before birth as they are supposed to –> obstruction of outflow tract
What is hydrocolpos/mucocolpos?
Accumulation of fluid due to congenital vaginal obstruction often caused by an imperforate hymen or less commonly a transverse vaginal septum. The fluid often consists of cervical and endometrial mucus
At puberty, Primary amenorrhea + cyclic pelvic pain –> ?
imperforate hyman, transverse vaginal septum, OR vaginal atresia
hematocolpos?
accumulation of menstrual flow in vagina due to imperforate hymen
hematometra?
accumulation of menstrual flow in uterus due to imperforate hymen
Treatment of imperforate hymen?
surgery
Transverse vaginal septum arise bc?
mullerian tubercle does not canalize leaving the vaginal plate intact (supposed to degenerate) ==> the sino-vaginal bulb system and mullerian (paramesonephric) duct system separate (so upper and lower tracts not connected)
Treatment of transverse vaginal septum ?
Surgery
Physical exam of transverse vag septum?
normal external genitalia w/ short vagina that ends in blind pouch
symptoms of transverse vag septum, imperforate hymen, OR vaginal atresia?
primary amenorrhea and cyclic pelvic pain
vaginal atresia aka?
agenesis of the lower vagina
vaginal atresia occurs bc?
urogenital sinus fails to contribute the lower portion of the vegina –> lower vagina fails to develop and is replaced by fibrous tissue.
what is wrong and what is normal in vaginal atresia anatomically?
normal: upper vag, cervix, uterus, and ovaries.
abnormal: lower vag undev and replaced by fibrous tissue
phys exam of pt w/ vag atresia –?
absence of vag introitus w/ vag dimple
Treatment of vag atresia?
Surgery; vaginal pull-through procedure (normal upper vag mucosa brought down to introitus and sutured to hymenal ring)
Mayer-Rokitanksy-Kuster-Hauser syndrome = ?
Vaginal agenesis
Vaginal agenesis =?
congenital absence of the vagina and the absence or hypoplasia of all or part of the cervix, uterus, and fallopian tubes
Whats normal genotypically and phenotypically in pt w/ vag agenesis?
Normal external genitalia, secondary sexual characteristics and ovarian function.
Normal 46XX karyotype
Treatment of pt w/ vag agenesis?
nonsurg–> dilators (4mo-years)
surg –> most common McIndoe
McIndoe procedure?
For vag agenesis –> create neovagina using mold and skin graft. After mold removed use dialators for several months.
Post surgery for vag agenesis, pts can and can’t what?
Can have sex, cannot get pregnant (bc abnormal/no uterus fallopian tubes even post surgery) –> BUT CAN HARVEST EGGS
lichen sclerosis?
inflammatory dermatosis found on the vulva of women of all age groups (white + skin thinning) that can cause resorption of labia minoria, labial fusion, occlusion of clit, contracture of vag introitus, thinning vulvar skin, and skin fragility.
What age group is lichen sclerosis most significant and why?
sig in POSTmenopausal women bc associated w/ 3-4% risk of vulvar skin cancer
lichen sclerosis can –>?
resorption of labia minoria, labial fusion, occlusion of clit, contracture of vag introitus, thinning vulvar skin, and skin fragility.
Lichen planus?
uncommon inflammatory skin condition characterized by papular or erosive lesions of vulva and poss vag. Also effects nails, scalp, and skin mucosa
Clinical apperance of lichen planus?
chronic eruption of shiny purple papules w/ white striae on vulva.
Papules in lichen planus found elsewhere beyond vulva?
flexor surfaces and mucous membrane of oral cavity
Lichen planus associated w/?
vaginal adhesions and erosive vaginitis
Vulvar psoriasis ?
similar to gen psoriasis just including or only on genital skin. Silvery-red scaly patches. common
silvery-red scaly patches on genitals and flexor surfaces?
think vulvar psoriasis
shiny purple papules w/ white striae on vulva and flexor surfaces?
think lichen planus
Lichen simplex chronicus clinical apperance?
thickened skin w/ accentuated skin markings and excoriations due to chronic itching and scratching
Scratch-itch cycle is? and found in what disease?
something irritates –> scratch –> causes thickening of skin –> itchy –> scratch –> thick skin, etc
Found w/ LICHEN SIMPLEX CHRONICUS
indications for definite biopsy (5)?
- ulceration,
- unifocal lesions
- uncertain suspicion of lichen sclerosis
- unidentifiable lesion
- lesions/symp recur/persist after conventiaonl therapy
Evaluate vulvar and vag lesions w/?
colposcopy
Treatment of benign skin conditions of vulvar/vag?
- loose fitting clothes; avoid phys irritants
- morn and evening bathing
- unscented detergents and soaps
- sometimes topical steroids
Lichen sclerosis phys findings?
symmetrics white, thinned skin on labia, perineums and perianal region. Shrinkage and agglutination of labia minora
Lichen planus phys findings?
multiple shiny, flat, red-purple papules usually on the inner aspect of the labia minora and vestibule w/ lacy white changes; often erosive
Lichen simplex chronicus phys findings?
localized thickening of vulvar skin w/ slight scaling
vulvar psoriasis phys findings?
red moist lesions sometimes scaly
lichen sclerosis symptoms?
asymptomatic OR pruritus or dyspareunia
Treatment lichen sclerosis?
high potency topical steroids 1-2x/d for 6-12wks –> then a maintenance schedule of topical steroid
(ie clobetasol or halobetasol 0.05%)
Treatment lichen planus?
high potency topical steroids 1-2x/d for 6-12wks –> then a maintenance schedule of topical steroid
(ie clobetasol or halobetasol 0.05%)
Treatment of lichen simplex chronicus?
Medium to high potency topical steroid 2x/d for >=6 wks
Treatment vulvar psoriasis?
topical steroids or UV light
Most common tumor found on vulva =?
Epidermal inclusion cysts
Epidermal inclusion cysts arise bc?
occlusion of a pilosebaceous duct or a blocked hair follicle
Epidermal inclusion cyst treatment?
usually asymptomatic –> do nothing
if infected –> absecess –> I&D or complete excision needed
Sebaceous cyst arise from?
blocked sebaceous gland
treatment of sebaceous cyst?
usually asymptomatic –> do nothing
if infected –> may need I&D or complete excision needed
Fox-Fordyce disease
chronic pruritic papular eruption localized to areas w/ apocrine glands (blocked); often related to stress, humidity, heat.
Apperance of fox fordyce disease
appears as small water-filled blisters, skincoloured spots or darkened spots at the base of the hair follicles
Hidradenitis suppurativa
Rare, long-term skin condition that features small, painful lumps under the skin due to blocked apocrine or sebacous glands or hair follicles. They typically develop where the skin rubs together, such as the armpits, the groin, between the buttocks and under the breasts. The lumps may break open and smell or cause tunnels under the skin.
skene gland cysts occur where?
skene glands are paraurethral glands, meaning cysts would appear next to the urethra meatus
Bartholin duct cyst 1- 2cm, what do you do?
nothing, can beleft untreated and will often resolve on own or w/ sitz baths
What size bartholin cyst can be left alone to self resolve?
2cm or less
If bartholin duct cyst first appears in a woman >40yo what do you do?
biopsy to r/o bartholin gland carcinoma
bartholin gland carcinoma presents?
new appearant BDCyst in a woman >40yo
symptomatic Bartholin duct cysts should or should not be simply I&D?
NO simple I&D bc can often lead to recurrance
Treatment of Bartholin duct cyst or abscess (2)?
1) Word catheter placement - I&D + balloon catheter 4-6wks serially reduced
2) Marsupialization - abscess/cyst I&D + cyst wall sutured to vaginal mucosa to prevent reformation (for recurrent BDC/As)
Adjunct antibiotic therapy recommended post procedure treatment of BDcyst/abscess when?
1) drainage grows Neisseria gonorrhoeae
2) concomitant cellulitis
3) abscess refractory to simple surgical treatment
Gartner’s duct cysts?
cysts that develop from theremnants of the mesonephric ducts (wolffian system). Commonly found in anterior lateral aspect of the upper vag. usually asymptomatic.
Gartner duct cyst presentation clinically?
adolescent w/ dyspareunia or diff inserting tampon
Treatment of gartner duct cyst?
Excision! but first intravenous pylogram and cystoscopy should be performed preop to id bladder and ureters relative to cyst and r/o urethral diverticula and ectopic ureters.
Benign solid tumors of vulva =?
lipomas, cherry hemangiomas, urethral caruncles, urethral prolapse
Campbell De Morgan spots or senile angiomas = aka?
Cherry HEMANGIOMAS
hemangiomas =?
elevated soft red papules that contain an abn proliferation of bld vessels
urethral caruncle =?
benign fleshy outgrowth at the urethral meatus
urethral caruncle and urethral prolapse found most commonly in what population?
Postmenopausal due vulvovaginal atrophy.
Treatment of urethral caruncle or urethral prolapse?
Usually nothing bc asymp, if bloody spotting can do short course of topical estrogen. rare - excision
uterine didelphys =?
uterine malformation where there are 2 uterus’ (cervix, often vaginas too!) bc embryonic fusion of mullerian ducts fails.
bicollis=?
means double cervix, often seen w/ uterine didelphys
25% of women exposed to what in utero have associated abnormalities of the cervix?
Diethylstilbestrol (DES)
exposed in utero to Diethylstilbestrol (DES) inc risk of what (3)?
1) cerival abnormalities (ie cervical hypoplasia, cervical collar, etc)
2) cervical insufficiency in pregnancy
3) clear cell adenocarcinoma of cervix and/or vag
nabothian cyst and who found in?
cervical cyst due to intermittent blockage of endocervical gland usually seen in menstruating women, usually asymp so need no treatment
mesonephric cysts =?
cervical cysts dev from remnants of mesonephric (wolffian) duct
mesonephric cysts differ from nabothian how?
Theylie deeper to cervical stroma and on external surface of cervix
Which cysts dev from remnants of the mesonephric ducts?
Gartner's duct cysts (ant lateral upper vag) mesonephric cysts (cervix)
intermenstrual or postcoital spotting (no pain) what benign finding should you consider?
cervical polyp
cervical stenosis causes (4)?
1) congenital
2) infection
3) atrophy
4) scarring (from surgery, trauma, etc)
cervical stenosis usually clinically presents?
ASYMPTOMATIC and doesn’t affect menstruation or fertility (in which case do nothing)
if symptomatic or need access to uterus how do you treat a cervical stenosis?
gently dilate cervix
Origins of upper and lower repro organs?
1) ovaries - genital ridge
2) lower vagina - urogenital diaphragm
3) everything else - mullerian system
