Gynecology Review Blueprints

Front 1

Labial fusion

1. causes

2. treatment

Back 1

1. This most commonly arises from excess exogenous androgen exposure, but it can also occur from increased androgen production from an enzymatic error, such as 21-hydroxylase deficiency, which leads to congenital adrenal hyperplasia.

2. reconstructive surgery

Front 2

21 hydroxylase deficiency

Back 2

Enzymatic deficiency which leads to congenital adrenal hyperplasia.

• Presents with ambiguous genitalia, hyperandrogenism with salt wasting, hypotension, hyperkalemia, and hypoglycemia
• neonates often present in adrenal crisis with salt wasting in 75% of cases
• autosomal recessive (1 in 40,000-50,000)
• Dx: elevated 17 alpha hydroxyprogesterone or urine 17-ketosteroid with decreased urine cortisol
  • tx: exogenous cortisol- negatively feeds back to the pituitary to decrease the release of ACTH, which inhibits the stimulation of the adrenal gland that is hunting all the steroid precursors into androgens. If there is salt wasting, than fludrocortisone should be given

Front 3

Imperforate hymen - cause

Back 3

The hymen is located at the junction between the urogenital sinus and sinovaginal bulbs. Before birth, the epithelial cells in the central portion of the hymenal membrane degenerate, leaving a thin rim of mucous membrane at the vaginal introitus, known at the hymenal ring. If this degeneration fails to occur then there is an imperorate hymen (1 in 1000 women)

Front 4

Imperforate hymen- presentation and problems

Back 4

- obstruction of the outflow tract of the reproductive system, which can lead to buildup of secretions in the vagina behind the hymen

- if not diagnosed at birth, this condition is usually diagnosed at puberty when there is amenorrhea and cyclic pelvic pain. This is due to accumulation of blood in the vagina (hematocolpos) and in the uterus (hematometra)

* treatment is surgical

Front 5

Transverse vaginal septum

Back 5

- failure for the upper and lower portions of the vagina to fuse during congenital development, resulting in a septum that divides the two portions.

- this usually occurs between the lower 2/3 and the upper 1/3

- It often presents as primary amenorrhea with cyclic pelvic pain and menstrual symptoms during puberty- like imperforate hymen, however on exam there is normal external female genitalia with a short vagina that appears to end in a blind pouch.

Front 6

Vaginal atresia

Back 6

- when the lower vagina fails to develop and is replaced by fibrous tissue

- the ovaries, uterus, cervix and upper vagina all develop normally

- presents during puberty as primary amenorrhea and cyclic pelvic pain

- physical exam reveals the absence of an introitus and the presence of a vaginal dimple

- tx: vaginal pull-through surgical procedure

Front 7

Vaginal agenesis

Back 7

- congenital absence of the vagina and the absence or hypoplasia of all or part of the cervix, uterus, and fallopian tubes

- these patients generally have normal external genitalia and normal secondary sexual characteristics (breast development, axillary and pubic hair)

- patients are phenotypically and genotypically female with normal 46, XX karyotype

- Tx- non-surgical approach uses vaginal dilators pressed into the perineal body, while the surgical approach involves using a skin graft to create a neo-vagina. With the surgical approach vaginal dilators still need to be used in order to keep it patent

Front 8

What are the non-neoplastic epithelial disorders of the vulva

Back 8

1. lichen sclerosis
2. lichen planus
3. lichen simplex chronicus
4. vulvar psoriasis

* These lesions often require histological examination of the tissue in order to differentiate between them , neoplasia and cancer

Front 9

Lichen sclerosis

Back 9

- inflammatory dermatosis that can be found on the vulva of women of all age groups, but has major significance in postmenopausal women, where it is associated with a 3-4% risk of vulvar skin cancer

- the atrophy that results from this condition can lead to resorption of the labia minora, labial fusion, occlusion of the clitoris, contracture of the vaginal introitus, thinning of the vulvar skin and skin fragility

- There are ivory white plaques, "cigarette paper" appearance of the skin in a butterfly/figure 8 pattern

Front 10

Lichen planus

Back 10

- uncommon inflammatory condition that affects the nails, scalp, and skin mucosa

- when it affects the vulva, there are papular or erosive lesions of the vulva that may also involve the vagina

- the inflammatory process results in the chronic eruption of shiny purple papules with white striae on the vulva, and often the flexor surfaces, mucous membranes of the oral cavity

- may be associated with vaginal adhesions and erosive vaginitis

- normally occurs in women in their 50s and 60s and is associated with a 3-4% risk of vulvar cancer

Front 11

Vulvar psoriasis

Back 11

- this may occur in combination with generalized psoriasis (which affects 2% of the population), or may occur in isolation.

- it appears as silvery-red patches of skin that often occurs on the elbows and knees, but may also affect the scalp and nails

Front 12

Lichen simplex chronicus

Back 12

- thickened skin with accentuated skin markings and excoriations due to chronic itching and scratching

- the intense pruritus that leads to this may be from atopic dermatitis, psoriasism, neuropathic pain, or psychological disorders

- there is a scratch-itch cycle. The scratching leads to thickened skin, which then scratches more.

Front 13

Indications for definite vulvar skin biopsy

Back 13

• ulceration
• unifocal lesions
• uncertain suspicion of lichen sclerosis
• unidentifiable lesions
  • lesions or symptoms that persist despite treatment or recur after tx

Front 14

Differential diagnosis for vulvar skin lesions

Back 14

• lichen sclerosis
• lichen planus
• lichen simplex chronicus
• vulvar psoriasis
• apthous ulcers
• behcet syndrome
• crohn disease
• erythema multiforme
• bullous pemiphigoid
• plasma cell vulvitis
• squamous or basal cell carcinoma
• melanoma
• sarcoma
  • paget disease of the vulva

Front 15

Treatment for benign epithelial disorders of the vulva or vagina

Back 15

• vulvar and vaginal hygeine
• patients should avoid tightfitting clothes, pantyhose, panty liners, scented soaps and detergents, bubble baths, washcloths, feminine sprays douches, and powders
• patients should wear loose-fitting clothing and take morning and evening tub baths without additives
• lichen sclerosis, lichen planus and severe lichen simplex chronicus may require high-potency steroids, such as clobetasol

Front 16

What is the most common tumor of the vulva?

Back 16

Epidermal inclusion cyst- usually result from the occlusion of pilosebaceous duct or a blocked hair follicle

- they are lines with squamous epithelium and contain tissue that would normally be exfoliated

- these are solitary and usually asymptomatic, however if they become superinfected and develop into abscesses - I&D or complete excision in required

Front 17

Sebaceous cysts

Back 17

- these occur when the duct of subaceous gland becomes blocked trapping the sebum

- these can become superinfected with local flora and require I&D

Front 18

Apocrine sweat gland cysts

Back 18

If a sweat gland becomes blocked this can form a cyst

Front 19

Fox-Fordyce disease

Back 19

- uncommon chronic pruritic papular eruption that localizes to areas where apocrine glands are found

Front 20

Hidradentis suppurativa

Back 20

- skin disease that most commonly affects areas bearing apocrine sweat glands or sebacous glands, such as the underms, breasts, inner thighs, groin and buttocks

- these cysts can become infected and form multiple abcesses

- treatment is with I&D or excision. Antibiotics are needed if there is an overlying cellulitis

Front 21

Skene's/ paraurethral glands

Back 21

These are located next to the urethral meatus and can become blocked, leading to cystic dilation

Front 22

Bartholin's duct cysts and abscesses

Back 22

- the batholin's glands are found bilaterally at the 4 and 8 o'clock positions on the posterior lateral aspect of the vaginal orifice. These are mucus-secreting glands with ducts that open just external to the hymenal ring.

- obstruction of these ducts forms cysts that can be left alone if small (1-2 cm)-- sitz baths may resolve them

- if the bartholin duct cyst occurs for the first time in a woman over 40 then a biopsy should be done to rule out cancer

- if these cysts enlarge they can become quite painful, resulting in dyspareunia and difficulty walking

- if they do not resolve they can become infected and form abscesses which are usually polymicrobial in nature, but can at times be associated with STDs

- these should be treated with I&D, however simple I&D usually leads to recurrence, so word catheter placement or marsupialization should be done

Front 23

Word catheter placement

Back 23

- this is commonly preferred method for treating abscesses of the Bartholin's gland in the emergency setting or office

- a small incision is made to drain and irrigate the abscess, the a catheter with a balloon tip is inserted. The balloon is expanded to fill the space and left in place for 4-6 weeks, being serially reduced in size, while epithelialization of the cyst and tract occurs

- adjunct antibiotic treatment is only required when the drainage is culture positive for n. gonorrhea or there is concomittant cellulitis

Front 24

Marsupialization

Back 24

- treatment for recurrent Bartholin's cysts or abscesses.

- the entire lesion is incised and the cyst wall is sutured to the vaginal mucosa to prevent reformation

- adjunct antibiotic treatment is only required when the drainage is culture positive for n. gonorrhea or there is concomittant cellulitis

Front 25

Gartner's duct cysts

Back 25

- gartner's ducts are remnants of the mesonephric ducts of the wolffian system and are most commonly found in the anterior lateral aspects of the upper part of the vagina.

- these cysts are usually asymptomatic, however patients may present during adolscence with dyspareunia or pain with inserting a tampon

- treatment is usually with excision (IVY and cystoscopy should be done pre-op to locate the bladder and ureters relative to the cyst and rule out other possible causes of the symptoms such as urethral diverticula, cancer etc)

Front 26

Lipomas

Back 26

- soft pedunculated or sessile tumors composed of mature fat cells and fibrous strands

- these usually do not require removal unless they become large and symptomatic

Front 27

cherry hemangiomas

Back 27

- elevated soft red papules that contain an abnormal proliferation of blood vessels

Front 28

urethral caruncles and urethral prolapse

Back 28

- the present as small, red, fleshy tumors found in the distal urethral meatus

- they occur almost exclusively in postmenopausal women as a result of vulvovaginal atrophy

- when there is bloody spotting then a short course of vaginal estrogen is needed

- rarely, surgical excision may be required

Front 29

DES exposure in utero

Back 29

- 25% of these females will have cervical abnormalities including cervical hypoplasia, cervical collars, cervical hoods, cock's comb cervix, and pseudopolyps

- these women are at increased risk for cervical insufficiency during pregnancy

- these women are also at risk for a very rare clear cell adenocarcinoma or the cervix and vagina, which is seen in young women under 20 and only 0.1% of women with DES exposure

Front 30

Nabothian cysts

Back 30

- cervical cyst that are dilated retention cysts caused by intermittent blockage of an endocervical gland

- they usually expand to no more than 1 cm

- most commonly found in menstruating women and are usually asymptomatic

Front 31

Mesonephric cysts

Back 31

- cervical cysts that are remnants of the wolffian ducts

- they tend to lie deeper than nabothian cysts and on the external surface of the cervix

Front 32

Cervical endometriosis

Back 32

- endometriosis can implant on the cervix

- these cysts tent to be red or purple

- the patient usually has associated cyclic pelvic pain and dyspareunia

Front 33

Cervical polyps

Back 33

- benign growths that may be pedunculated or broad-based

- often asymptomatic, but if there are symptoms, it is usually intermenstrual spotting, NOT pain

- they are not considered pre-cancerous but are usually removed to prevent masking of irregular bleeding from other causes

Front 34

Cervical fibroids

Back 34

- Leiomyomas (myomas or fibroids) are common within the uterine body, however they can also occur in the cervix or prolapse into the cervical canal

- usually cause intermenstrual bleeding, but can also cause dyspareunia and bladder or rectal pressure and may lead to hemorrhage, poor dilation of the cervix, malpresentation, or obstruction of the birth canal

- rule out cancer, if symptomatic surgical excision (myomectomy or hysterectomy can be performed)

Front 35

Cervical stenosis

Back 35

- this may be congenital, a product of infection, atrophy, or scarring

- it can also occur from obstruction with a neoplasm, polyp, or fibroid

- typically asymptomatic and does not affect menstruation or fertility- and in these settings no treatment is needed

- if there are symptoms or there needs to be access to the endocervical canal or glands then gentle dilation can be performed-- a catheter can be left in place to prolong patency

Front 36

What is the most common non-drug related congenital mullerian anomaly?

Back 36

• septate uterus due to malfusion of the paramesonephric ducts.
  
• Uterine septae are nads of collagen fiber positioned vertically within the uterus. Because these fibers do not have good blood supply they cannot support placental implantation and 25% of women with this condition will suffer from recurrent pregnancy loss in the first trimester.
  
• there is an increased risk of renal anomalies associated with ANY mullerian anomaly and these should be evaluated for
• tx: surgical excision if symptomatic

Front 37

bicornuate uterus

Back 37

uterine anomaly involving a "heart-shaped" uterus with two horns. Pregnancy/implantation can occur in either horn. If IUDs are used for contraception that an IUD is needed in both horns.

There is increased risk for second trimester pregnancy loss, preterm labor, malpresentation, and deformity.

Front 38

What is the most common indication for surgery in women in the US?

Back 38

Uterine fibroids (approximately 1/3 of all hysterectomies are performed for fibroids)

Front 39

Describe the hormone responsivity of fibroids

Back 39

• fibroids are responsive to both estrogen and progesterone, however the type of response is not always consistent
• in women of reproductive age, fibroids can go and shrink at different times and rates in the same woman
• During menopause, the tumors usually stop growing and may atrophy in response to the naturally lower levels of estrogen
• low dose oral contraceptives may be protective against the development of fibroids, but may stimulate the growth of existing fibroids

Front 40

Classification of uterine fibroids

Back 40

Uterine fibroids are classified by their location within the uterus. This includes:

1. submucosal (beneath the endometrium)

2. intramurual (in the muscular wall of the uterus)- most common type

3. subserousal (beneath the uterine serosa)

4. pedunculated (on a stalk with it's own blood supply)

Front 41

What is the most common type of uterine fibroid?

Back 41

intramural (in the muscular wall of the uterus)

Front 42

What are the symptoms associated with uterine fibroids? What is most common?

Back 42

F- Frequency and retention of urine, hydronephrosis

I- Iron deficiency anemia

B- bleeding abnormalities (menorrhagia, metrorhagia, menometrorrhagia, postcoital spotting) Bloating - most common if symptoms exist however 50-65% of woman are asx

R- Reproductive difficulties (dysfunctional labor, premature labor/delivery, fetal malpresentation, increased need for c-section)

O- obstipation, constipation, rectal pressure

I- Infertility

D- Dysmenorrha, dyspareunia

S- Symptomless - most common

Front 43

What is the relationship between uterine fibroids and uterine cancer?

Back 43

• This is currently unclear, although fibroids are considered benign tumors.
  
• Leiomyosarcomas are thought to represent separate new entities rather than degeneration of previously benign fibroid.
  

Front 44

Epidemiology and risk factors of uterine fibroids

Back 44

• lifetime risk is 70% in whites and greater than 80% for blacks. Blacks are more likely to be younger at the time of diagnosis, have larger fibroids, a greater number of fibroids, heavier bleeding, and more severe anemia.
• nonsmoking
• early menarche
• nulliparity
• perimenopause
• increased alcohol use
• hypertension

Front 45

Pelvic pain and uterine fibroids

Back 45

• Usually fibroids do not cause pelvic pain unless there is vascular compromise, which is most common in subserousal pedunculated fibroids
• pain is often due to secondary dysmenorrhea with menses
• there may be pressure related symptoms depending on the number, size and location of the tumors

Front 46

Uterine fibroids and pregnancy

Back 46

• submucosal fibroids can impact implantation, placentation, and ongoing pregnancy
• resection of submucosal fibroids in patients with infertility does increase conception rates
• intramural (most common type) and subserosal fibroids are unlikely to affect conception or pregnancy loss except when multiple fibroids are present
• The vast majority of women with fibroids are able to conceive without difficulty
• when multiple large fibroids are present or are located behind the placenta there is increased risk for preterm labor/delivery, malpresentation, dysfunctional labor, c-section

Front 47

Diagnostic evaluation of uterine fibroids

Back 47

1. pelvic ultrasound is the most common means of diagnosis

2. sonohysterogram, HSG, and hysteroscopy may also be used

3. MRI is especially helpful in distinguishing between fibroids and adenomyosis and planning for surgery

4. on physical exam, there may be a non-tender, irregularly enlarged uterus with a lumpy-bumpy or cobblestone feel on palpation

Front 48

Treatment of uterine fibroids

Back 48

• most cases do NOT require treamtne as they are asymptomatic
• first other causes of pelvic masses should be ruled out
• when leiomyomas cause severe pain, heavy or irregular bleeding, infertility, or pressure symptoms then treatment should be considered
• non-hormonal options: NSAIDs and anti-fibrinolytics- for dysmenorrhea and heavy, prolonged bleeding
• hormonal options: combined OCPs, progestins, GnRH agonists (e.g. leuprolide)
• GnRH agonists have been found to shrink fibroids and decreased bleeding by decreasing circulating estrogen levels. They are usually used in women nearing menopause or to shrink fibroids prior to surgery
• Uterine artery embolization- becoming more popular but only for women who no longer wish to have children
• MRI guided high intensity ultrasound- rare and expensive
• myomectomy for women who wish to preserve fertility- however there is a 60% recurrence rate within 5 yrs
• hysterectomy is the definitive treatment. The ovaries are left in place in women younger than 45 if they appear normal
• no surgical intervention during pregnancy because of increased risk of hemorrhage

Front 49

Follow-up care for uterine fibroids

Back 49

• if a fibroid is growing it should be followed by US every 6 month
• rapid tumor growth in a postmenopausal woman may be a sign of leiomyosarcoma (very rare) and should be followed up urgently
• low dose OCPs and HRT do NOT appear to increase risk of recurrence