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First_Aid_Step2. Hx/PE: The 1° cause of anterior pituitary insuffciency in adult females. The most common presenting syndrome is failure to lactate (due to ↓ prolactin levels). Other symptoms include weakness, lethargy, cold insensitivity, genital atrophy, and menstrual disorders. Dx: The diagnosis is established with provocative hormonal testing and MRI of the pituitary and hypothalamus to rule out tumor or other pathology. Tx: Treatment consists of the replacement of all defcient hormones. However, some patients may recover TSH and even gonadotropin function after cortisol replacement alone. During pregnancy, ↑ estrogen and progesterone result in breast hypertrophy and inhibition of prolactin release. After delivery of the placenta, hormone levels ↓ markedly and prolactin is released, stimulating milk production. Periodic infant suckling leads to further release of prolactin and oxytocin, which stimulate myoepithelial cell contraction and milk ejection (“let-down ref ex”).


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First_Aid_Step2. Colostrum (“early breast milk”) contains protein, fat, secretory IgA, and minerals. Within one week postpartum, mature milk with protein, fat, lactose, and water is produced. High IgA levels in colostrum provide passive immunity for the infant and protect against enteric bacteria. Other benef ts include ↓ incidence of infant allergies, early upper respiratory tract infections, and GI infections; facilitation of mother-child bonding; and maternal weight loss. Contraindications to breastfeeding include HIV infection, active HBV and HCV infection, and use of certain medications (e.g., tetracycline, chloramphenicol, warfarin). Cellulitis of the periglandular tissue caused by nipple trauma from breastfeeding coupled with the introduction of bacteria, usually S. aureus, from the infant’s pharynx into the nipple ducts. Affects 2–3% of nursing women. Symptoms often begin 2–4 weeks postpartum.


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First_Aid_Step2. Symptoms often begin 2–4 weeks postpartum. Breast symptoms are usually unilateral and include breast tenderness, a palpable mass, erythema, edema, warmth, and possible purulent nipple drainage. Signifcant fever, chills, and malaise may also be seen. Differentiate from simple breast swelling. Infection is suggested by focal symptoms, a breast milk culture, an ↑ WBC count, and fever. ■ First step: Continued breastfeeding to prevent the accumulation of infected material (or use of a breast pump in patients who are no longer The treatment of mastitis breastfeeding) and PO antibiotics (dicloxacillin, cephalexin, amoxicillin/ clavulanate, azithromycin, clindamycin). ■ Next step: If abscess is present, treat with incision and drainage. continued breastfeeding. Menarche and Normal Female Development 359 Normal Menstrual Cycle 359 Menopause 360 Contraception 361 Sexual Assault 361 Abnormalities of the Menstrual Cycle 364


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First_Aid_Step2. Menarche and Normal Female Development 359 Normal Menstrual Cycle 359 Menopause 360 Contraception 361 Sexual Assault 361 Abnormalities of the Menstrual Cycle 364 Breast development (thelarche) precedes menarche and usually begins between the ages of 8 and 11. Menarche usually occurs between the ages of 10 and 16. Figure 2.12-1 graphically illustrates the stages of normal female development. The progression of a normal menstrual cycle is as follows (see also Figure 2.12-2): Follicular phase (days 1–13): Typically lasts about 13 days but may vary. ↑ FSH → growth of follicles → ↑ estrogen production. Results in the development of straight glands and thin secretions of the uterine lining (proliferative phase). Ovulation (day 14): LH and FSH spike results in rupture of the ovarian follicle and release of a mature ovum. Ruptured follicular cells involute and create the corpus luteum.


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First_Aid_Step2. Ovulation (day 14): LH and FSH spike results in rupture of the ovarian follicle and release of a mature ovum. Ruptured follicular cells involute and create the corpus luteum. Luteal phase (days 15–28): This phase is the length of time (14 days) that the corpus luteum can survive without further LH stimulation. HEIGHT SPURT GROWTH RATE AGE RANGE PEAK Height 3 in/y Weight 17.5 lb/y 11.5–16.5 yWeight 6 lb/y AGE RANGE 10-16.5 y MENARCHE Average Height 62.5 in (158.5 cm) Average Weight 106 lb (48 kg) RATING PUBIC HAIR Initial hair is straight and fine. AGE RANGE 8–14 years Pubic hair becomes coarse, darkens, and spreads. Hair looks like an adult’s but limited in area. Inverted triangular pattern is established. AGE RANGE 12.5–16.5 years F IGU R E 2.1 2-1. Normal female development. (Reproduced, with permission, from Hay WW Jr et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill, 2008: Fig. 3-4.)


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First_Aid_Step2. F IGU R E 2.1 2-1. Normal female development. (Reproduced, with permission, from Hay WW Jr et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill, 2008: Fig. 3-4.) Currently, HRT is not recommended as f rst-line treatment for menopausal symptoms. (Reproduced, with permission, from Fauci AS et al. Harrison’s Principles of Internal Medicine, 17th ed. New York: McGraw-Hill, 2008: Fig. 341-8.) The corpus luteum produces estrogen and progesterone, allowing the endometrial lining to develop thick endometrial glands with thick secretions (secretory phase). In the absence of implantation, the corpus luteum cannot be sustained, and the endometrial lining sloughs off. Cessation of menses for a minimum of 12 months as a result of cessation of follicular development. Average age of onset is 51. “Premature menopause” is defned as ovarian failure and menstrual cessation before age 40. The mnemonic HAVOC lists prominent features of menopause.


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First_Aid_Step2. The mnemonic HAVOC lists prominent features of menopause. Other symptoms include insomnia, anxiety/irritability, vaginal bleeding, poor concentration, mood changes, dyspareunia, and loss of libido. Labs f rst show ↑ FSH and then show ↑ LH. DEXA scan to follow bone density for osteoporosis. Lipid prof le (↑ total cholesterol, ↓ HDL). ■ Vasomotor symptoms: ■HRT (combination estrogen and progestin): HRT has been shown to ↑ cardiovascular morbidity and mortality and may ↑ the incidence of breast and endometrial cancers. For this reason, clinicians should thoroughly review the risks and benef ts of HRT before initiating treatment. Posthysterectomy patients do not need progestin. Unopposed estrogen in patients with a uterus predisposes to endometrial cancer. Contraindications to HRT include vaginal bleeding, suspected or known breast cancer, endometrial cancer, and a history of thromboembolism, chronic liver disease, or hypertriglyceridemia.


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First_Aid_Step2. Contraindications to HRT include vaginal bleeding, suspected or known breast cancer, endometrial cancer, and a history of thromboembolism, chronic liver disease, or hypertriglyceridemia. Non-HRT: Venlafaxine and, less commonly, clonidine can be given to ↓ the frequency of hot f ashes. Vaginal atrophy: Long term: Estradiol vaginal ring. Short term: Estrogen vaginal cream will relieve symptoms. Once a woman is Osteoporosis: Treat with daily calcium supplementation and exercise; pos postmenopausal, she should sibly bisphosphonates. osteoporosis. ■Of women who are sexually active and do not use a contraceptive method, 85% will become pregnant within one year. Absolute contraindications to various methods are as follows: Estrogen-containing hormonal methods (OCPs, NuvaRing, “the patch”): A history of CAD or DVT Mirena and Copper T IUDs: PID that is active (within three months) or recurrent A Pap smear with squamous intraepithelial lesions or two atypical Pap smears


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First_Aid_Step2. A history of CAD or DVT Mirena and Copper T IUDs: PID that is active (within three months) or recurrent A Pap smear with squamous intraepithelial lesions or two atypical Pap smears A history of heart valve replacement or artif cial joints Copper T alone: Copper intolerance (allergy to copper, Wilson’s disease); severe dysmenorrhea and/or menorrhagia. Mirena alone: Levonorgestrel allergy, breast cancer, acute liver disease or liver tumor, or a history of BRCA. Table 2.12-1 describes the effectiveness of contraceptive methods along with their relative advantages and disadvantages. Emergency contraception (EC) methods prevent pregnancy after unprotected sex or contraceptive failure. Table 2.12-2 describes the various methods of EC. More than one sexual partner and nulliparity are not absolute contraindications to IUD use. The most effective methods of contraception are longer-term methods that ↓ user error.


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First_Aid_Step2. More than one sexual partner and nulliparity are not absolute contraindications to IUD use. The most effective methods of contraception are longer-term methods that ↓ user error. Combined hormonal methods of contraception protect against endometrial, ovarian, and breast cancer. Sexual assault is the most frequently unreported crime in the United States. Physicians are often required to evaluate rape victims and collect evidence. Most rape victims are women; however, men may also be victims of rape. ■Take a full history, including contraceptive use, last time of coitus, condom use prior to the assault, drug or alcohol use, history of STDs, history of mental illness or defciency, description of the assailant, location and T AB LE 2.1 2-1. Contraceptive Methods


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First_Aid_Step2. Most effective: > 99% (1 in 100 women will become pregnant using these methods) Implanon (“the implant”): Progestin-only implant Lasts up to three years. Immediate fertility with removal. Safe with breastfeeding. Associated with weight gain, depression, irregular bleeding (no period; spotting; heavier, more frequent periods). Intrauterine device with progestin (Mirena): Inf ammation from foreign body; cervical thickening and endometrial decidualization with progesterone Effective for up to f ve years. Lighter periods; less cramping. Immediate fertility with removal. Safe with breastfeeding. Spotting (up to six months). Associated with acne. Risk of uterine puncture (1/1000). Intrauterine device— ParaGard (Copper T): Infammation from foreign body; copper exerts a spermicidal effect Effective for up to 10 years. Immediate fertility with removal. Safe with breastfeeding. ↑ cramping and bleeding (5–10%). Risk of uterine puncture (1/1000). Surgical sterilization (vasectomy, tubal ligation)


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First_Aid_Step2. for up to 10 years. Immediate fertility with removal. Safe with breastfeeding. ↑ cramping and bleeding (5–10%). Risk of uterine puncture (1/1000). Surgical sterilization (vasectomy, tubal ligation) Permanently effective. Safe with breastfeeding. Tubal ligation: Irreversible; ↑ ectopic pregnancy. Vasectomy: Most failures are due to not waiting for two semen samples. Very effective: 90–99% (1–10 in 100 women will become pregnant using these methods) Depo-Provera (medroxy-progesterone): IM injection every three months Lighter or no periods. Each shot works for three months. Safe with breastfeeding. Associated with irregular bleeding and weight gain. Decreases in bone mineral density (reversible). Delayed fertility after discontinuation (one shot can last 10 months). Ortho Evra (“the patch”): Combined weekly estrogen and progestin dermal patch Can make periods more regular. Not administered daily. Risk of thromboembolism (especially for smokers and those > 35 years of age). NuvaRing


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First_Aid_Step2. Combined weekly estrogen and progestin dermal patch Can make periods more regular. Not administered daily. Risk of thromboembolism (especially for smokers and those > 35 years of age). NuvaRing (“the ring”): Combined low-dose progestin and an estrogen vaginal ring Can make periods more regular. Can improve acne. Used continuously for three weeks; then one week without the ring. Safe to use continuously to skip periods. May ↑ vaginal discharge. Spotting occurs in the frst 1–2 months.


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First_Aid_Step2. T AB LE 2.1 2-1. Contraceptive Methods (continued)


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First_Aid_Step2. Oral contraceptive pills (combination estrogen and progestin): Inhibit FSH/LH, suppressing ovulation; thicken cervical mucus; decidualize endometrium ↓ risk of ovarian and endometrial cancers.a Predictable, lighter, less painful menses. Often improve acne. Immediate fertility upon cessation. Require daily compliance. Breakthrough bleeding (10–30%). Risk of thromboembolism (especially for smokers and those > 35 years of age). Progestin-only “minipills”: Thicken cervical mucus Safe with breastfeeding. Requires strict compliance (taking pill at the same time each day). Moderately effective: 75–90% (up to 10–25 women in 100 will become pregnant using these methods) Male condoms: A latex sheath covers the penis The only method that effectively protects against pregnancy and STDs, including HIV. Possible allergy to latex or spermicides. Diaphragm with spermicide Some protection against STDs. Must be ftted by provider. Female condom Some protection against STDs. Can be clumsy and diff cult.


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First_Aid_Step2. Possible allergy to latex or spermicides. Diaphragm with spermicide Some protection against STDs. Must be ftted by provider. Female condom Some protection against STDs. Can be clumsy and diff cult. Fertility awareness methods No side effects. Often used by women who have a cultural, religious, or personal preference for “natural” contraception. Requires partner’s participation. No STD/HIV protection. Less effective: 68–74% (26–32 women in 100 will become pregnant using these methods) Withdrawal No side effects. No STD/HIV protection. Spermicide Frequent use may protect against HIV. May be used as a 2° method. Not recommended as a 1° method. a Other combined hormonal methods (e.g., patch, ring) may also protect against endometrial, breast, and ovarian cancer; however, data are still lacking given their relatively recent introduction.


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First_Aid_Step2. time of the assault, circumstances of the assault (e.g., penile penetration, use of condoms, extragenital acts, use or display of weapons), and the patient’s actions since the assault (e.g., douching, bathing, brushing teeth, urination/defecation, changing clothes). ■Conduct a complete physical exam, making note of any signs of trauma, along with a detailed pelvic exam, including a survey of the external genitals, vagina, cervix, and anus. Saline prep for sperm. Gonorrhea and chlamydia smear/culture (including rectal if appropriate).


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First_Aid_Step2. Saline prep for sperm. Gonorrhea and chlamydia smear/culture (including rectal if appropriate). T AB LE 2.1 2-2. Emergency Contraceptive Methods “Morning-After Pill”—used within 120 hours of unprotected sex Combined estrogen/progestin (75% effective) Progestin only (80% effective) Available over the counter. Does not disrupt embryo postimplantation. Can be used as bridge contraception. Safe for all women. Same as above. Fewer nausea/vomiting side effects than combined EC. Nausea, vomiting, fatigue, headache, dizziness, breast tenderness. No protection against STDs. Same as above. Copper T IUD—used within 7 days of unprotected sex (99% effective) Can be used as EC and continued for up to 10 years of contraception. High initial cost of insertion. Must be inserted by provider. No protection against STDs. Serologic testing for HIV, syphilis, HSV, HBV, and CMV. Serum pregnancy test. Blood alcohol level; urine toxicology screen. STD treatment (ceftriaxone plus doxycycline).


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First_Aid_Step2. Serologic testing for HIV, syphilis, HSV, HBV, and CMV. Serum pregnancy test. Blood alcohol level; urine toxicology screen. STD treatment (ceftriaxone plus doxycycline). HIV risk assessment and possible postexposure prophylaxis. EC for pregnancy prevention. Refer for psychological counseling. Arrange for follow-up with the same physician or with another provider if more appropriate. Follow-up should include repeat screening for STDs, repeat screening for pregnancy, and a discussion of coping methods with appropriate referrals for psychiatric care if needed. Pediatric vaginal discharge is caused by a variety of factors and may be normal, but STDs resulting from sexual abuse must be ruled out. Etiologies of vaginal discharge in pediatric patients include the following: Infectious vulvovaginitis: May present with a malodorous, yellow-green, purulent discharge; most often caused by group A streptococcus.


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First_Aid_Step2. Infectious vulvovaginitis: May present with a malodorous, yellow-green, purulent discharge; most often caused by group A streptococcus. May also be caused by any STD resulting from sexual abuse (STDs must be ruled out and, if found, reported to child protective services). Foreign objects. Candidal infection: May be associated with diabetes; measure glucose and/or check for glycosuria. Sarcoma botryoides (rhabdomyosarcoma): A malignancy with lesions that have the appearance of “bunches of grapes” within the vagina. Onset of 2° sexual characteristics before the age of eight. Subtypes are as follows (see also Table 2.12-3): Central precocious puberty: Results from early activation of hypothalamic GnRH production. Most commonly idiopathic (also known as constitutional or true); may be related to obesity. May also be caused by CNS tumors. Peripheral precocious puberty: Also called pseudo-precocious puberty. Results from nonhypothalamic GnRH production.


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First_Aid_Step2. Peripheral precocious puberty: Also called pseudo-precocious puberty. Results from nonhypothalamic GnRH production. Signs of estrogen excess (breast development and possibly vaginal bleeding) point to ovarian cysts or tumors. Signs of androgen excess (pubic and/or axillary hair, enlarged clitoris, acne, and/or ↑ body odor) suggest adrenal tumors or congenital adrenal hyperplasia (CAH). First step: Obtain a radiograph of the wrist and hand to determine bone age. If bone age is within one year of chronological age, puberty has not started or has just recently begun. If bone age exceeds chronological age by > 2 years, puberty has been present for at least one year or is progressing rapidly. Next step: Conduct a GnRH agonist (leuprolide) stimulation test. Central precocious puberty: If LH response is , obtain a cranial MRI to look for CNS tumors. In girls 6–8 years of age with signs of precocious puberty, the incidence of CNS tumor is 2% in the absence of other CNS signs.


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First_Aid_Step2. In girls 6–8 years of age with signs of precocious puberty, the incidence of CNS tumor is 2% in the absence of other CNS signs. If CNS tumors are ruled out, constitutional precocious puberty is the likely etiology. Peripheral precocious puberty: If LH response is , order the following: Ultrasound of the ovaries and/or adrenals: To look for ovarian or adrenal cysts/tumors. Estradiol: Levels will be ↑ in ovarian cysts or tumors. T AB LE 2.1 2-3. Causes of Precocious Pubertal Development If onset of 2° sexual characteristics is seen by age eight, work up for precocious puberty by determining bone age and conducting a GnRH stimulation test to distinguish central from peripheral precocious puberty.


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First_Aid_Step2. Constitutional (idiopathic) Hypothalamic lesions (hamartomas, tumors, congenital malformations) Dysgerminomas Hydrocephalus CNS infections CNS trauma/irradiation Pineal tumors (rare) Neurofbromatosis with CNS involvement Tuberous sclerosis Congenital adrenal hyperplasia Adrenal tumors McCune-Albright syndrome (polyostotic f brous dysplasia) Gonadal tumors Exogenous estrogen, oral (OCPs) or topical Ovarian cysts (females) Surgery is not required for the treatment of ambiguous genitalia in congenital adrenal hyperplasia; it is always cosmetic and may be deferred. Androgen (DHEA, DHEAS): Especially critical in the setting of advanced bone age or signs of adrenarche. 17-OH progesterone: To screen for advanced bone age or adrenarche. Central precocious puberty: Leuprolide is frst-line therapy. With treatment, physical changes regress or cease to progress. Peripheral precocious puberty: Treat the cause.


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First_Aid_Step2. Central precocious puberty: Leuprolide is frst-line therapy. With treatment, physical changes regress or cease to progress. Peripheral precocious puberty: Treat the cause. Ovarian cysts: No intervention is necessary, as cysts will usually regress spontaneously. CAH: Treat with glucocorticoids. Surgery is not required for the treatment of ambiguous genitalia. Adrenal or ovarian tumors: Require surgical resection. McCune-Albright syndrome: Antiestrogens (tamoxifen) or estrogen synthesis blockers (ketoconazole or testolactone) may be effective. 1° amenorrhea is def ned as the absence of menses by age 16 with 2° sexual development present, or the absence of 2° sexual characteristics by age 14. Absence of 2° sexual characteristics (no estrogen production): Etiologies are as follows: Constitutional growth delay: The most common cause. 1° ovarian insuff ciency: Most commonly Turner’s syndrome. Look for a history of radiation and chemotherapy.


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First_Aid_Step2. Constitutional growth delay: The most common cause. 1° ovarian insuff ciency: Most commonly Turner’s syndrome. Look for a history of radiation and chemotherapy. Central hypogonadism: May be caused by a variety of factors, including the following: Undernourishment, stress, prolactinemia, or exercise. CNS tumor or cranial irradiation. Kallmann’s syndrome (isolated gonadotropin def ciency) associated with anosmia. Presence of 2° sexual characteristics (evidence of estrogen production but other anatomic or genetic problems): Etiologies include the following: Mlerian agenesis: Absence of two-thirds of the vagina; uterine abnormalities. Imperforate hymen: Presents with hematocolpos (blood in the vagina) that cannot escape, along with a bulging hymen. Requires surgical opening. Complete androgen insensitivity: Patients present with breast development (aromatization of testosterone to estrogen) but are amenorrheic and lack pubic hair. First step: Get a pregnancy test.


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First_Aid_Step2. Complete androgen insensitivity: Patients present with breast development (aromatization of testosterone to estrogen) but are amenorrheic and lack pubic hair. First step: Get a pregnancy test. Next step: Obtain a radiograph to determine if bone age is consistent with pubertal onset (> 12 years in girls). ■If the patient is of short stature (bone age < 12 years) with normal growth velocity, constitutional growth delay (the most common cause of 1° amenorrhea) is the probable cause. ■If bone age is > 12 years but there are no signs of puberty, obtain LH/ FSH and consider where the problem is on the HPA axis (see Figure 2.12-3). ■↓ GnRH, ↓ LH/FSH, ↓ estrogen/progesterone at prepuberty levels: Points to constitutional growth delay (puberty has not yet started). ■↓ GnRH, ↓ LH/FSH, ↓ estrogen/progesterone: Hypogonadotropic hypogonadism. Suggests a hypothalamic or pituitary problem.


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First_Aid_Step2. ■↓ GnRH, ↓ LH/FSH, ↓ estrogen/progesterone: Hypogonadotropic hypogonadism. Suggests a hypothalamic or pituitary problem. ■↑ GnRH, ↑ LH/FSH, ↓ estrogen/progesterone: Hypergonadotropic hypogonadism. Points to a condition in which the ovaries fail to produce estrogen. ■↑ GnRH, ↑ LH/FSH, high estrogen or testosterone: Suggests PCOS or a problem with estrogen receptors. ■Normal pubertal hormone levels: Indicates an anatomic problem (menstrual blood can’t get out). Constitutional growth delay: ↓ GnRH, ↓ LH/FSH, ↓ E/P at prepuberty levels (↓ GnRH, ↓ LH/FSH, ↓ E/P): (↓ GnRH, ↓ LH/FSH, ↓ E/P): Gonadotropin-releasing hormone (GnRH) Anorexia, excess exercise, weight loss, stress (GnRH deficiency) Sheehan’s syndrome Hypothalamus -Tumors, infection, trauma, Anorexia, excess exercise, Pituitary gland Hyperprolactinemia weight loss, stress Hypothyroidism


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First_Aid_Step2. Sheehan’s syndrome Hypothalamus -Tumors, infection, trauma, Anorexia, excess exercise, Pituitary gland Hyperprolactinemia weight loss, stress Hypothyroidism Luteinizing hormone (LH) Follicle-stimulating hormone (FSH) Adrenal—CAH, Cushing’s Adrenal syndrome, Addison’s disease Pancreas gland Thyroid Other endocrine glands (↑ GnRH, ↑ LH/FSH, ↓ E/P): (chemotherapy, radiation, idiopathic) (gonadotropin-resistant (↑ GnRH, ↑ LH/FSH, ↓ E/P): ovary syndrome) Premature ovarian failure; 40(↑ GnRH, ↑ LH/FSH): -Menopause-Androgen insensitivity Chemotherapy with (↑ testosterone, ↑ estrogen) alkylating agents-PCOS (↑ estrogen, androgen) hydroxylase or aromatase) Primary anatomic: Secondary anatomic: Anovulatory problem: Normal hormone levels Normal hormone levels PCOS -Transverse vaginal septum to endometritis, scarring -Vaginal and/or cervical after delivery, or D&C agenesis Cervical stenosis F IGU R E 2.1 2-3. Causes of primary and secondary amenorrhea.


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First_Aid_Step2. F IGU R E 2.1 2-3. Causes of primary and secondary amenorrhea. Ultrasound may be needed to evaluate the ovaries. Normal breast development and no uterus: Obtain a karyotype to evalu- The f rst step in the workup of 1° or 2° amenorrhea is a pregnancy test! ate for androgen insensitivity syndrome. Stigmata of Turner’s syndrome: Obtain a karyotype. Normal breast development and uterus: Measure prolactin and obtain a cranial MRI. Constitutional growth delay: No treatment is needed. Hypogonadism: Begin HRT with estrogen alone at the lowest dose. Twelve to eighteen months later, begin cyclic estrogen/progesterone therapy (if the uterus is present). Anatomic: Generally requires surgical intervention. Defned as the absence of menses for six consecutive months in women who have passed menarche. First step: Get a pregnancy test.


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First_Aid_Step2. Anatomic: Generally requires surgical intervention. Defned as the absence of menses for six consecutive months in women who have passed menarche. First step: Get a pregnancy test. Second step: β-hCG: Measure TSH and prolactin. ■↑ TSH: Indicates hypothyroidism. ■↑ prolactin (inhibits the release of LH and FSH): Points to a thy roid pathology. Order an MRI of the pituitary to rule out tumor. ■↑↑ prolactin: Suggests a prolactin-secreting pituitary adenoma. Normal β-hCG: Initiate a progestin challenge (10 days of progestin). progestin challenge (withdrawal bleed): Indicates anovulation that is likely due to noncyclic gonadotropin secretion, pointing to PCOS or idiopathic anovulation. Check LH levels, and if LH is moderately high, the etiology is likely PCOS. Marked elevation of LH can indicate premature menopause. progestin challenge (no bleed): Indicates uterine abnormality or estrogen defciency. Check FSH levels. ■↑ FSH: Indicates hypergonadotropic hypogonadism/ovarian fail ure.


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First_Aid_Step2. progestin challenge (no bleed): Indicates uterine abnormality or estrogen defciency. Check FSH levels. ■↑ FSH: Indicates hypergonadotropic hypogonadism/ovarian fail ure. ■↓ FSH: Obtain a cyclic estrogen/progesterone test. A withdrawal bleed points to hypogonadotropic hypogonadism; a withdrawal bleed suggests an endometrial or anatomic problem. Signs of hyperglycemia (polydipsia, polyuria) or hypotension: Conduct a 1-mg overnight dexamethasone suppression test to distinguish CAH, Cushing’s syndrome, and Addison’s syndrome. If clinical virilization is present: Measure testosterone, DHEAS, and 17–hydroxyprogesterone. Mild pattern: PCOS, CAH, or Cushing’s syndrome. Moderate to severe pattern: Look for an ovarian or adrenal tumor. ■ Hypothalamic: Reverse the underlying cause and induce ovulation with gonadotropins. Tumors: Excision; medical therapy for prolactinomas (e.g., bromocriptine, cabergoline).


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First_Aid_Step2. ■ Hypothalamic: Reverse the underlying cause and induce ovulation with gonadotropins. Tumors: Excision; medical therapy for prolactinomas (e.g., bromocriptine, cabergoline). Premature ovarian failure (age < 40 years): If the uterus is present, treat with estrogen plus progestin replacement therapy. Menstrual pain associated with ovulatory cycles in the absence of pathologic f ndings. Caused by uterine vasoconstriction, anoxia, and sustained contractions mediated by an excess of prostaglandin (PGF2α). Presents with low, midline, spasmodic pelvic pain that often radiates to the back or inner thighs. Cramps occur in the frst 1–3 days of menstruation and may be associated with nausea, diarrhea, headache, and f ushing. There are no pathologic fndings on pelvic exam. A diagnosis of exclusion. Rule out 2° dysmenorrhea (see workup below). NSAIDs; topical heat therapy; combined OCPs, Mirena IUD.


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First_Aid_Step2. There are no pathologic fndings on pelvic exam. A diagnosis of exclusion. Rule out 2° dysmenorrhea (see workup below). NSAIDs; topical heat therapy; combined OCPs, Mirena IUD. Menstrual pain for which an organic cause exists. Common causes include endometriosis and adenomyosis, tumors, fbroids, adhesions, polyps, and PID. Look for pathology. Patients may have a palpable uterine mass, cervical motion tenderness, adnexal tenderness, a vaginal or cervical discharge, or visible vaginal pathology (mucosal tears, masses, prolapse). However, normal abdominal and pelvic exams do not rule out pathology. See Table 2.12-4 for distinguishing features of endometriosis vs. adenomyosis. First step: Obtain a β-hCG to exclude ectopic pregnancy. Second step: Order the following: A CBC with differential to rule out infection or neoplasm. UA to rule out UTI. Gonococcal/chlamydial swabs to rule out STDs/PID. Stool guaiac to rule out GI pathology.


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First_Aid_Step2. A CBC with differential to rule out infection or neoplasm. UA to rule out UTI. Gonococcal/chlamydial swabs to rule out STDs/PID. Stool guaiac to rule out GI pathology. Third step: Look for pelvic pathology causing pain (see Table 2.12-4). Treatment is etiology specif c. T AB LE 2.1 2-4. Endometriosis vs. Adenomyosis


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First_Aid_Step2. Def nition Functional endometrial glands and stroma outside the uterus. Endometrial tissue in the myometrium of the uterus. History/PE Presents with cyclical pelvic and/or rectal pain and dyspareunia (painful intercourse). Presents with the classic triad of noncyclical pain, menorrhagia, and an enlarged uterus. Diagnosis Requires direct visualization by laparoscopy or laparotomy. Classic lesions have a blue-black (“raspberry”) or dark brown (“powder-burned”) appearance. Ovaries may have endometriomas (the characteristic “chocolate cysts”). Ultrasound is useful but cannot distinguish between leiomyoma and adenomyosis. MRI can aid in diagnosis but is costly. Treatment Pharmacologic: Inhibit ovulation. Combination OCPs are frst line; other options include GnRH analogs (leuprolide) and danazol. Conservative surgical treatment: Excision, cauterization, or ablation of the lesions and lysis of adhesions. Twenty percent of patients can become pregnant subsequent to treatment. Defnitive


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First_Aid_Step2. danazol. Conservative surgical treatment: Excision, cauterization, or ablation of the lesions and lysis of adhesions. Twenty percent of patients can become pregnant subsequent to treatment. Defnitive surgical treatment: TAH/BSO +/– lysis of adhesions. Pharmacologic: Largely symptomatic relief. NSAIDs (frst line) plus OCPs or progestins. Conservative surgical treatment: Endometrial ablation or resection using hysteroscopy. Complete eradication of deep adenomyosis is diffcult and results in high treatment failure. Defnitive surgical treatment: Hysterectomy is the only def nitive treatment. Complications Infertility (the most common cause among menstruating women > 30 years of age). Rarely, can progress to endometrial carcinoma.


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First_Aid_Step2. Pregnancy is the most common cause of abnormal uterine bleeding and amenorrhea. Always check a pregnancy test! Normal menstrual bleeding ranges from two to seven days. Vaginal bleeding that occurs six or more months following the cessation of menstrual function is cancer related until proven otherwise. Assess the extent of bleeding: Menorrhagia: ↑ amount of fow (> 80 mL of blood loss per cycle) or prolonged bleeding (fow lasting > 8 days); may lead to anemia. Oligomenorrhea: An ↑ length of time between menses (35–90 days between cycles). Polymenorrhea: Frequent menstruation (< 21-day cycle); anovular. Metrorrhagia: Bleeding between periods. Menometrorrhagia: Excessive and irregular bleeding. Pelvic exam: Look for an enlarged uterus, a cervical mass, or polyps to assess for myomas, pregnancy, or cervical cancer. First step: Obtain a β-hCG to rule out ectopic pregnancy. Second step: Order a CBC to rule out anemia. Third step:


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First_Aid_Step2. First step: Obtain a β-hCG to rule out ectopic pregnancy. Second step: Order a CBC to rule out anemia. Third step: Pap smear to rule out cervical cancer (which can present with bleeding). TFTs to rule out hyper-/hypothyroidism and hyperprolactinemia. Obtain platelet count, bleeding time, and PT/PTT to rule out von Willebrand’s disease and factor XI def ciency. Order an ultrasound to evaluate the ovaries, uterus, and endometrium. Look for uterine masses, polycystic ovaries, and thickness of the endometrium. If the endometrium is ≥ 4 mm in a postmenopausal woman, obtain an endometrial biopsy. An endometrial biopsy should also be obtained if the patient is > 35 years of age, obese (BMI > 35), and diabetic. Heavy bleeding: Since heavy or prolonged uterine bleeding has likely denuded the endometrial cavity, estrogen is needed to rapidly promote endometrial growth.


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First_Aid_Step2. Heavy bleeding: Since heavy or prolonged uterine bleeding has likely denuded the endometrial cavity, estrogen is needed to rapidly promote endometrial growth. First step: For hemorrhage, high-dose estrogen IV stabilizes the endometrial lining and stops bleeding within one hour. Next step: If bleeding is not controlled within 12–24 hours, a D&C is indicated. Ovulatory bleeding: The goal is to ↓ blood loss. First step: NSAIDs to ↓ blood loss. Next step: If the patient is hemodynamically stable, treat with OCPs or a Mirena IUD to thicken the endometrium and control the bleeding. If this is not effective within 24 hours, look for an alternative diagnosis. Anovulatory bleeding: The goal is to convert proliferative endometrium to secretory endometrium. Give progestins × 10 days to stimulate withdrawal bleeding.


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First_Aid_Step2. Anovulatory bleeding: The goal is to convert proliferative endometrium to secretory endometrium. Give progestins × 10 days to stimulate withdrawal bleeding. For young patients with anovulatory bleeding who may also have a bleeding disorder, give desmopressin followed by a rapid ↑ in von Willebrand’s factor and factor VIII (lasts roughly six hours). If medical management fails, options include the following: D&C: An appropriate diagnostic/therapeutic option. Hysteroscopy: Can help identify endometrial polyps as well as aid in the performance of directed uterine biopsies. Hysterectomy or endometrial ablation: Appropriate in women who fail or do not want hormonal treatment, have symptomatic anemia, and/or experience a disruption in their quality of life from persistent, unscheduled bleeding. First-line treatment of abnormal uterine bleeding consists of NSAIDs to ↓ blood loss! OCPs and the Mirena IUD are highly effective treatment options for menorrhagia.


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First_Aid_Step2. First-line treatment of abnormal uterine bleeding consists of NSAIDs to ↓ blood loss! OCPs and the Mirena IUD are highly effective treatment options for menorrhagia. Complications of abnormal uterine bleeding are anemia and endometrial hyperplasia +/− carcinoma. A 21-hydroxylase defciency that can present in its most severe, classic form as a newborn female infant with ambiguous genitalia and life-threatening salt wasting (see the Endocrinology chapter for a discussion of early-onset CAH). Milder forms present later in life. 11β-hydroxylase defciency is a less common cause of adrenal hyperplasia. Presents with excessive hirsutism, acne, amenorrhea and/or abnormal uterine bleeding, infertility, and, rarely, a palpable pelvic mass. Hirsutism = male hair pattern. Virilization = frontal balding, muscularity, clitoromegaly, and deepening of the voice. Defeminization = ↓ breast size; loss of feminine adipose tissue.


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First_Aid_Step2. Hirsutism = male hair pattern. Virilization = frontal balding, muscularity, clitoromegaly, and deepening of the voice. Defeminization = ↓ breast size; loss of feminine adipose tissue. The most severe form of PCOS is HAIR-AN syndrome: HyperAndrogenism, Insulin Resistance, and Acanthosis Nigricans. ■↑ androgens (testosterone > 2 ng; DHEAS > 7 μg/mL): Rule out adrenal or ovarian neoplasm. ■↑ serum testosterone: Suspect an ovarian tumor. ■↑ DHEAS: Suspect an adrenal source (adrenal tumor, Cushing’s syn drome, CAH). ■↑ 17-OH progesterone levels (either basally or in response to ACTH stimulation). ■Table 2.12-5 outlines the differential diagnosis of hyperandrogenism. Glucocorticoids (e.g., prednisone). Medical therapy for adrenal and ovarian disorders prevents new terminal hair growth but does not resolve hirsutism. Laser ablation, electrolysis, or conventional hair removal techniques must be used to remove unwanted hair.


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First_Aid_Step2. One of the most common endocrine disorders in reproductive women, with a prevalence of 6–10% among U.S. women of reproductive age. Also known as Stein-Leventhal syndrome. Diagnosis requires two of the following three criteria: 1. 2. 3. Clinical or biochemical evidence of hyperandrogenism T AB LE 2.1 2-5. Differential Diagnosis of Hyperandrogenism


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First_Aid_Step2. PCOS Irregular menses, slow-onset hirsutism, obesity, infertility, hypertension, a family history of PCOS or DM. Fasting glucose, insulin/lipid prof le, BP, ultrasound for ovarian cysts. 21-hydroxylase def ciency—nonclassic (late-onset) CAH Severe hirsutism or virilization, a strong family history of CAH, short stature, signs of defeminization. More common among Ashkenazi Jews. 17-hydroxyprogesterone (17-HP) levels before and after ACTH stimulation test > 10 ng/dL; CYP21 genotype. 21-hydroxylase def ciency—classic (congenital) CAH Same as above but with congenital virilization. 17-HP levels > 30 ng/dL. Hypothyroidism Fatigue, weight gain, amenorrhea. TSH. Hyperprolactinemia Amenorrhea, galactorrhea, infertility. Prolactin. Androgen-secreting neoplasm Pelvic mass, rapid-onset hirsutism or virilization, > 30 with onset of symptoms. Pelvic ultrasound or abdominal/pelvic CT. Cushing’s syndrome Hypertension, buffalo hump, purple striae, truncal obesity. Elevated BP plus dexamethasone


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First_Aid_Step2. or virilization, > 30 with onset of symptoms. Pelvic ultrasound or abdominal/pelvic CT. Cushing’s syndrome Hypertension, buffalo hump, purple striae, truncal obesity. Elevated BP plus dexamethasone suppression test.


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First_Aid_Step2. High BP. BMI > 30 (obesity). Stigmata of hyperandrogenism or insulin resistance (menstrual cycle disturbances, hirsutism, obesity, acne, androgenic alopecia, acanthosis nigricans). Women with PCOS are also at ↑ risk for the following: Metabolic syndrome—insulin resistance, obesity, atherogenic dyslipidemia, and hypertension Biochemical hyperandrogenemia: ↑ testosterone (total +/− free); DHEAS, DHEA. Exclude other causes of hyperandrogenism: TSH, prolactin. 17-OH progesterone to rule out nonclassical CAH. Consider screening in the setting of clinical signs of Cushing’s syndrome (e.g., moon facies, buffalo hump, abdominal striae) or acromegaly (e.g., ↑ head size). Evaluate for metabolic abnormalities: Two-hour oral glucose tolerance test. Fasting lipid and lipoprotein levels (total cholesterol, HDL, LDL, triglycerides). Optional tests:


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First_Aid_Step2. Evaluate for metabolic abnormalities: Two-hour oral glucose tolerance test. Fasting lipid and lipoprotein levels (total cholesterol, HDL, LDL, triglycerides). Optional tests: Ultrasound: Look for > 8 small, subcapsular follicles forming a “pearl necklace” sign (see Figure 2.12-4). Seen in roughly two-thirds of women with PCOS. Gonadotropins: ↑ LH/FSH ratio (> 2:1). Fasting insulin levels. 24-hour urine for free cortisol: Adult-onset CAH or Cushing’s syndrome. F IGU R E 2.1 2-4. Polycystic ovary with prominent multiple cysts. (Reproduced, with permission, from DeCherney AH. Current Obstetric & Gynecologic Diagnosis & Treatment, 8th ed. Stamford, CT: Appleton & Lange, 1994: 747.) ↓ the risk of endometrial hyperplasia/carcinoma among women with PCOS. Women who are not attempting to conceive: Treat with a combination of OCPs, progestin, and metformin (or other insulin-sensitizing agents).


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First_Aid_Step2. Women who are not attempting to conceive: Treat with a combination of OCPs, progestin, and metformin (or other insulin-sensitizing agents). Women who are attempting to conceive: Clomiphene +/− metformin is frst-line treatment for ovulatory stimulation. Symptom-specif c treatment: Hirsutism: Combination OCPs are frst line; antiandrogens (spironolactone, f nasteride) and metformin may also be used. Cardiovascular risk factors and lipid levels: Diet, weight loss, and exercise plus potentially lipid-controlling medication (e.g., statins). ↑ risk of early-onset type 2 DM; ↑ risk of miscarriages; ↑ long-term risk of breast and endometrial cancer due to unopposed estrogen secretion. Defned as inability to conceive after 12 months of normal, regular, unprotected sexual activity. 1° infertility is characterized by no prior pregnancies; 2° infertility occurs in the setting of at least one prior pregnancy. Etiologies are shown in Figure 2.12-5 and Table 2.11-6.


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First_Aid_Step2. Cyst and Abscess of Bartholin’s Duct Obstruction of the gland leads to pain, swelling, and abscess formation. Infertility14% of reproductive aged women 5 million couples in the U.S. Female causes 58% Male causes 25% Unexplained 17% Amenorrhea/ ovulatory dysfunction 46% Hypothalamic/ pituitary causes 51% Polycystic ovary syndrome 30% Premature ovarian failure 12% Tubal defect 38% Primary hypogonadism ( FSH) 30–40% Secondary hypogonadism ( FSH, LH) 2% Disordered sperm transport 10–20% Unknown 40–50% Endometriosis 9% Other 7% Uterine or outflow tract disorders 7% FIGURE 2.12-5. Causes of infertility. (Reproduced, with permission, from Fauci AS et al. Harrison’s Principles of Internal Medicine, 17th ed. New York: McGraw-Hill, 2008: Fig. 341-9.) TABLE 2.12-6. Infertility Workup


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First_Aid_Step2. Male causes Testicular injury or infection Medications (corticosteroids, cimetidine, spironolactone) Thyroid or liver disease Signs of hypogonadism Varicocele TSH Prolactin Karyotype (to rule out Klinefelter’s syndrome) Semen analysis Treatment of hormonal def ciency Intrauterine insemination (IUI) Donor insemination In vitro fertilization (IVF) Intracytoplasmic sperm injection Ovulatory factors Age (incidence ↑ with age) Symptoms of hyper-/ hypothyroidism Galactorrhea Menstrual cycle abnormalities Basal body temperature Ovulation predictor Midluteal progesterone Early follicular FSH +/– estradiol level (ovarian reserve) TSH, prolactin, androgens Ovarian sonography (antral follicle count) Endometrial biopsy (luteal phase defect) Treatment depends on the etiology (e.g., levothyroxine, dopamine) Induction of ovulation with clomiphene, gonadotropins, and pulsatile GnRH IUI IVF Tubal/ pelvic factors History of PID, appendicitis, endometriosis, pelvic adhesions, tubal surgery


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First_Aid_Step2. dopamine) Induction of ovulation with clomiphene, gonadotropins, and pulsatile GnRH IUI IVF Tubal/ pelvic factors History of PID, appendicitis, endometriosis, pelvic adhesions, tubal surgery Hysterosalpingogram, endometrial biopsy Laparoscopic resection or ablation of endometriomas or f broids IVF Cervical factors Abnormal Pap smears, postcoital bleeding, cryotherapy, conization, or DES exposure in utero Pap smear Physical exam Antisperm antibodies IUI with washed sperm IVF


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First_Aid_Step2. Presents with periodic painful swelling on either side of the introitus and dyspareunia. A fuctuant swelling 1–4 cm in diameter is seen in the inferior portion of either labium minus. Tenderness is evidence of active infection. Asymptomatic cysts do not require therapy. Frequent warm soaks may be helpful. If an abscess develops, treat with aspiration or incision and drainage. Culture for Chlamydia and other pathogens. Antibiotics are unnecessary unless cellulitis is present. A spectrum of conditions that cause vulvovaginal symptoms such as itching, burning, irritation, and abnormal discharge. The most common causes are bacterial vaginosis, vulvovaginal candidiasis, and trichomoniasis (see Table 2.12-7). Presents with a change in discharge, malodor, pruritus, irritation, burning, swelling, dyspareunia, and dysuria. Normal secretions are as follows: Midcycle estrogen surge: Clear, elastic, mucoid secretions.


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First_Aid_Step2. Normal secretions are as follows: Midcycle estrogen surge: Clear, elastic, mucoid secretions. Luteal phase/pregnancy: Thick and white secretions; adhere to the vaginal wall.


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First_Aid_Step2. TABLE 2.12-7. Causes of Vaginitis 15–50% (most common). 5–50%. 15–30%. Refects a shift in vaginal f ora. Protozoal f agellates (an STD). Usually Candida albicans. Risk factors Pregnancy, > 1 sexual partner, female sexual partner, frequent douching. An STD. Unprotected sex with multiple partners. DM, broad-spectrum antibiotic use, pregnancy, corticosteroids, HIV, OCP use, IUD use, young age at f rst intercourse, ↑ frequency of intercourse. History Odor, ↑ discharge. ↑ discharge, odor, pruritus, dysuria. Pruritus, dysuria, burning, ↑ discharge. Exam Mild vulvar irritation. “Strawberry petechiae” in the upper vagina/cervix (rare). Erythematous, excoriated vulva/ vagina. Discharge Homogenous, grayish-white, f shy/stale odor. Profuse, malodorous, yellow-green, frothy. Thick, white, curdy texture without odor. Wet mounta “Clue cells” (epithelial cells coated with bacteria; see Figure 2.12-6); shift in vaginal f ora (↑ cocci, ↓ lactobacilli). Motile trichomonads (fagellated organisms that


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First_Aid_Step2. without odor. Wet mounta “Clue cells” (epithelial cells coated with bacteria; see Figure 2.12-6); shift in vaginal f ora (↑ cocci, ↓ lactobacilli). Motile trichomonads (fagellated organisms that are slightly larger than WBCs). — KOH prep whiff test (fshy smell). — Hyphae (see Figure 2.12–6). Treatment PO or vaginal metronidazole or clindamycin. Single-dose PO metronidazole or tinidazole. Treat partners; test for other STDs. Topical azole or PO f uconazole. Complications Chorioamnionitis/endometritis, infection, preterm delivery, miscarriage, PID. Same as for bacterial vaginosis. Oral azoles should be avoided in pregnancy. a If there are many WBCs and no organism on saline smear, suspect Chlamydia. Incidence Etiology


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First_Aid_Step2. AB FIGURE 2.12-6. Causes of vaginitis. (A) Candidal vaginitis. Candida albicans organisms are evident on KOH wet mount. (B) Gardnerella vaginalis. Note the granular epithelial cells (“clue cells”) and indistinct cell margins. (Image A reproduced, with permission, from Wolff K et al. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology, 5th ed. New York: McGraw-Hill, 2005: 717. Image B reproduced, with permission, from Kasper DL et al. Harrison’s Principles of Internal Medicine, 16th ed. New York: McGraw-Hill, 2005: 767.) Conduct a thorough examination of the vulva, vaginal walls, and cervix. If there are many WBCs and no organism on saline smear, suspect Chlamydia. Samples from the speculum exam should be obtained for vaginal pH, amine (“whiff”) test, wet mount (with saline), and 10% hydroxide (KOH) microscopy.


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First_Aid_Step2. Samples from the speculum exam should be obtained for vaginal pH, amine (“whiff”) test, wet mount (with saline), and 10% hydroxide (KOH) microscopy. To rule out cervicitis, DNA tests or cultures for Neisseria gonorrhoeae or Chlamydia trachomatis should be obtained in patients with a purulent discharge, numerous leukocytes on wet prep, cervical friability, and any symptoms of PID. Treatment is etiology specifc (see Table 2.12-7). Infammation of the uterine cervix. Because the female genital tract is contiguous from the vulva to the fallopian tubes, there is some overlap between vulvovaginitis and cervicitis. Etiologies are as follows: Infectious (most common): Chlamydia, gonococcus, Trichomonas, HSV, HPV. Noninfectious: Trauma, radiation exposure, malignancy. Hx/PE: Yellow-green mucopurulent discharge; cervical motion tenderness; absence of other signs of PID. Dx/Tx: See the discussion of STDs in the Infectious Disease chapter.


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First_Aid_Step2. Hx/PE: Yellow-green mucopurulent discharge; cervical motion tenderness; absence of other signs of PID. Dx/Tx: See the discussion of STDs in the Infectious Disease chapter. Criteria for the clinical diagnosis of bacterial vaginosis (three of four are required): Vaginal pH > 4.5 Clue cells comprise > 20% of epithelial cells on wet mount A polymicrobial infection of the upper genital tract that is associated with Neisseria gonorrhoeae (one-third of cases), Chlamydia trachomatis (one-third of cases), and endogenous aerobes/anaerobes. The lifetime risk is 1–3%. Risk IUDs do not ↑ PID risk. The chandelier sign is def ned as severe cervical motion tenderness that makes the patient “jump for the chandelier” on exam. Mild and subclinical PID is a major cause of tubal factor infertility, ectopic pregnancy, and chronic pelvic pain due to pelvic scarring. factors include non-Caucasian ethnicity, douching, smoking, multiple sex partners, and prior STDs and/or PID.


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First_Aid_Step2. factors include non-Caucasian ethnicity, douching, smoking, multiple sex partners, and prior STDs and/or PID. Presents with lower abdominal pain, fever and chills, menstrual disturbances, and a purulent cervical discharge. are also seen. Diagnosed by the presence of acute lower abdominal or pelvic pain plus one of the following: A WBC count > 10,000 has poor positive and negative predictive value for PID. Order a β-hCG and ultrasound to rule out pregnancy and to evaluate for the possibility of tubo-ovarian abscess. Ultrasound is a noninvasive means of diagnosing PID. Look for: Thickening or dilation of the fallopian tubes Fluid in the cul-de-sac Antibiotic treatment should not be delayed while awaiting culture results. All sexual partners should be examined and treated appropriately. Outpatient regimens: Regimen A: Of oxacin or levof oxacin × 14 days +/– metronidazole × 14 days.


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First_Aid_Step2. Outpatient regimens: Regimen A: Of oxacin or levof oxacin × 14 days +/– metronidazole × 14 days. Regimen B: Ceftriaxone IM × 1 dose or cefoxitin plus probenecid plus doxycycline × 14 days +/– metronidazole × 14 days. Inpatient antibiotic regimens: Cefoxitin or cefotetan plus doxycycline × 14 days. Clindamycin plus gentamicin × 14 days. Surgery: Drainage of a tubo-ovarian/pelvic abscess is appropriate if the mass persists after antibiotic treatment; the abscess is > 4–6 cm; or the mass is in the cul-de-sac in the midline and drainable through the vagina. If the abscess is dissecting the rectovaginal septum and is fxed to the vaginal membrane, colpotomy drainage is appropriate. If the patient’s condition deteriorates, perform exploratory laparotomy. Surgery may range from TAH/BSO with lysis of adhesions in severe cases to conservative surgery for women who desire to maintain fertility.


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First_Aid_Step2. Surgery may range from TAH/BSO with lysis of adhesions in severe cases to conservative surgery for women who desire to maintain fertility. Some 25% of women with acute disease develop repeated episodes of infection, chronic pelvic pain, dyspareunia, ectopic pregnancy, or infertility. RUQ pain (Fitz-Hugh–Curtis syndrome) may indicate an associated perihepatitis (abnormal liver function, shoulder pain). The risk of infertility ↑ with repeated episodes of salpingitis and is esti mated to approach 10% after the frst episode, 25% after the second episode, and 50% after a third episode. Caused by preformed S. aureus toxin (TSST-1); often occurs within f ve days of the onset of a menstrual period in women who have used tampons. The incidence in menstruating women is now 6–7:100,000 annually. Nonmenstrual cases are nearly as common as menstrual cases. Presents with abrupt onset of fever, vomiting, and watery diarrhea, with fever 38.9°C (102°F) or higher.


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First_Aid_Step2. Presents with abrupt onset of fever, vomiting, and watery diarrhea, with fever 38.9°C (102°F) or higher. A diffuse macular erythematous rash is also seen. Nonpurulent conjunctivitis is common. Desquamation, especially of the palms and soles, generally occurs during recovery within 1–2 weeks of illness. Blood cultures are because symptoms result from preformed toxin and are not due to the invasive properties of the organism. Rapid rehydration. Antistaphylococcal drugs (nafcillin, oxacillin); vancomycin for women with penicillin allergy. Corticosteroids can reduce the severity of illness and ↓ fever. Manage renal or cardiac failure. The mortality rate associated with TSS is 3–6%. Three major causes of death are ARDS, intractable hypotension, and hemorrhage 2° to DIC.


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First_Aid_Step2. Manage renal or cardiac failure. The mortality rate associated with TSS is 3–6%. Three major causes of death are ARDS, intractable hypotension, and hemorrhage 2° to DIC. TSS is a rare but potentially fatal reaction to S. aureus toxin. Diagnosis is clinical because reaction is to the toxin produced by the bacteria, not to the bacterium itself. The f rst steps in treatment are rapid rehydration and antibiotic treatment. Gynecologic cancers include uterine, endometrial, ovarian, cervical, and vulvar neoplasms. Ovarian cancer carries the highest mortality. The most common benign neoplasm of the female genital tract. The tumor is discrete, round, frm, and often multiple and is composed of smooth muscle and connective tissue. Tumors are estrogen and progesterone sensitive, so they often ↑ in size during pregnancy and ↓ after menopause. Malignant transformation to leiomyosarcoma is rare (0.1–0.5%). Prevalence is 25% among Caucasian women and 50% among African-American women.


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First_Aid_Step2. HISTORY/PE Uterine myomas are benign The majority of cases are asymptomatic. Symptomatic patients may present with the following: menorrhagia. ■Bleeding: Longer, heavier periods; anemia. If a uterine mass continues to grow after menopause, malignancy must be ruled out with a biopsy. Eighty percent of women with endometrial carcinoma have vaginal bleeding, but only 5–10% of women with abnormal vaginal bleeding have endometrial cancer. Pressure: Pelvic pressure and bloating; constipation and rectal pressure; urinary frequency or retention. Pain: 2° dysmenorrhea, dyspareunia. Pelvic symptoms: A frm, nontender, irregular enlarged (“lumpybumpy”), or cobblestone uterus may be seen. CBC: To look for anemia. Ultrasound: To look for uterine myomas; can also exclude ovarian masses. MRI: Can delineate intramural and submucous myomas. Pharmacologic: NSAIDs. Combined hormonal contraception. Medroxyprogesterone acetate or danazol to slow or stop bleeding.


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First_Aid_Step2. MRI: Can delineate intramural and submucous myomas. Pharmacologic: NSAIDs. Combined hormonal contraception. Medroxyprogesterone acetate or danazol to slow or stop bleeding. GnRH analogs (leuprolide or nafarelin) to ↓ the size of myomas, suppress further growth, and ↓ surrounding vascularity. Also used prior to surgery. Surgery: Emergent surgery is indicated for torsion of a pedunculated myoma. Women of childbearing years: Myomectomy or hysteroscopy with leiomyoma resection. Women who have completed childbearing: Total or subtotal abdominal or vaginal hysterectomy. Uterine artery embolization (~ 25% will need further invasive treatment). Infertility may be due to a myoma that distorts the uterine cavity and plays a role similar to that of an IUD.


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First_Aid_Step2. Uterine artery embolization (~ 25% will need further invasive treatment). Infertility may be due to a myoma that distorts the uterine cavity and plays a role similar to that of an IUD. Type I endometrioid adenocarcinomas derive from atypical endometrial hyperplasia and are the most common female reproductive cancer in the United States (~35,000 cases/year). Type II cancers derive from serous or clear cell histology (see Table 2.12-8). Type I: Vaginal bleeding (early fnding); pain (late fnding); metabolic syndrome. Type II: No vaginal bleeding. Endometrial/endocervical biopsy. Vaginal ultrasound shows a thickened endometrium leading to hypertrophy and neoplastic change. TYPE I: ENDOMETRIOID TYPE II: SEROUS TABLE 2.12-8. Types of Endometrial Cancer


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First_Aid_Step2. Vaginal ultrasound shows a thickened endometrium leading to hypertrophy and neoplastic change. TYPE I: ENDOMETRIOID TYPE II: SEROUS TABLE 2.12-8. Types of Endometrial Cancer Epidemiology 75% of endometrial cancers. 25% of endometrial cancers. Etiology Unopposed estrogen stimulation (e.g., tamoxifen use, exogenous estrogen-only therapy). Unrelated to estrogen; the p53 mutation is present in 90% of cases. Precursor lesion Hyperplasia and atypical hyperplasia. None. Mean age at diagnosis 55 years. 67 years. Prognosis Favorable. Poor. Type I: High-dose progestins for women of childbearing age; TAH/BSO +/– radiation for postmenopausal women. Type II: TAH/BSO with adjuvant chemotherapy for advanced-stage cancer.


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First_Aid_Step2. Type I: High-dose progestins for women of childbearing age; TAH/BSO +/– radiation for postmenopausal women. Type II: TAH/BSO with adjuvant chemotherapy for advanced-stage cancer. The upper third of the cervix is made up of columnar cells (similar to the lower uterine segment). The lower two-thirds of the cervix is made up of squamous cells (similar to the vagina). The exposure of columnar cells to an acidic vaginal pH results in metaplasia to squamous cells. The normal squamocolumnar junction (transformation zone) is located in the ectocervix and can be exposed to carcinogens, resulting in cervical intraepithelial neoplasia (CIN), an abnormal proliferation or overgrowth of the basal cell layer. HPV DNA is found in 99.7% of all cervical carcinomas. HPV 16 is the most prevalent type in squamous cell carcinoma; HPV 18 is most prevalent in adenocarcinoma.


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First_Aid_Step2. HPV DNA is found in 99.7% of all cervical carcinomas. HPV 16 is the most prevalent type in squamous cell carcinoma; HPV 18 is most prevalent in adenocarcinoma. Additional risk factors for cervical cancer include immunosuppression, infection with HIV or a history of STDs, tobacco use, high parity, and OCPs. The Gardasil vaccine may protect against HPV types 6, 11, 16, and 18 and may also prevent the development of cervical cancer. Metrorrhagia, postcoital spotting, and cervical ulceration are the most common signs. A bloody or purulent, malodorous, nonpruritic discharge may appear after invasion. ■Starting at age 21 or no more than three years after becoming sexually active, women should have a Pap smear with conventional cervical cytology on a yearly basis or liquid-based cervical cytology once every two years. Table 2.12-9 outlines the classifcation of Pap smear results. Hormonal contraceptives are protective against endometrial cancer.


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First_Aid_Step2. Hormonal contraceptives are protective against endometrial cancer. Screening of asymptomatic women for endometrial cancer is not recommended. Fifty percent of women with cervical cancer had not had a Pap smear in the three years preceding their diagnosis, and another 10% had not been screened in f ve years. T AB LE 2.1 2-9. Classifcation Systems for Pap Smears 1 Benign Benign Normal 2 Benign with inf ammation Benign with inf ammation Normal, atypical squamous cells (ASC) 3 Mild dysplasia CIN I LSIL 3 Moderate dysplasia CIN II HSIL 3 Severe dysplasia CIN II HSIL 4 Carcinoma in situ CIN II HSIL 5 Invasive cancer Invasive cancer Invasive cancer Women ≥ 30 years of age who have had three consecutive normal tests may ↑ their screening interval to once every three years. For women ≥ 30 years of age, HPV DNA testing for high-risk strands may be used for screening as well.


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First_Aid_Step2. For women ≥ 30 years of age, HPV DNA testing for high-risk strands may be used for screening as well. Screening should be discontinued for women ≥ 70 years of age who have had three or more normal Pap smears. Women with DES exposure and/or immunocompromised status (including HIV positivity) should continue as long as they do not have a life-limiting condition. Women who have had the HPV vaccine should continue cervical cancer screening according to established guidelines. Recent guidelines for the diagnosis and follow-up of cervical cancer distinguish women ≤ 21 from those > 21 years of age for all subtypes of cervical lesions except atypical glandular cells. The diagnosis and follow-up of specifc subtypes of cervical lesions should thus proceed as follows: Atypical glandular cells (AGC): < 35 years of age with no endometrial cancer risk factors: Proceed to colposcopy, endocervical curettage (ECC), and HPV DNA testing.


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First_Aid_Step2. Atypical glandular cells (AGC): < 35 years of age with no endometrial cancer risk factors: Proceed to colposcopy, endocervical curettage (ECC), and HPV DNA testing. ≥ 35 years of age, endometrial cancer factors, or abnormal bleeding: Add an endometrial biopsy. Atypical squamous cells of undetermined signif cance (ASC-US): ≤ 21 years of age: Repeat Pap smear at 12 months. If Pap smear is or reveals ASC-US or LSIL, repeat at 12 months. > 21 years of age: Immediate colposcopy, HPV DNA testing, and repeat Pap smear at 6 months. Low-grade squamous intraepithelial lesions (LSIL): ≤ 21 years of age: Same as ASC-US. > 21 years of age: Immediate colposcopy. High-grade squamous intraepithelial lesions (HSIL) or atypical squamous cells suspicious of high-grade dysplasia (ASC-H): Immediate colposcopy is indicated for all age groups. ■Treatment is based on fndings of colposcopy: If colposcopy is satisfactory, proceed to treatment based on f ndings.


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First_Aid_Step2. ■Treatment is based on fndings of colposcopy: If colposcopy is satisfactory, proceed to treatment based on f ndings. If colposcopy is unsatisfactory, perform ECC and cervical biopsy and proceed to treatment based on f ndings. Noninvasive disease: Treatment based on biopsy results for noninvasive lesions (stage 0 disease) is as follows (see Figure 2.12-7): CIN I: ■Untreated CIN I will regress in 60% of patients, progress in 10%, and persist in 30%. Thus, the mainstay of treatment for CIN I is close observation. For women > 21 years of age, Pap smear screening at 6 and 12 months and/or HPV DNA testing at 12 months is indicated. For women ≤ 21 years of age, HPV testing is not recommended. After two Pap smears or a DNA test, patients can be managed with routine annual follow-up. Persistent CIN I can be treated with ablative (cryotherapy or laser ablation) or excisional therapy (loop electrosurgical excision procedure [LEEP]; laser and cold-knife conization).


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First_Aid_Step2. Persistent CIN I can be treated with ablative (cryotherapy or laser ablation) or excisional therapy (loop electrosurgical excision procedure [LEEP]; laser and cold-knife conization). CD F IGU R E 2.1 2-7. Cervical intraepithelial neoplasia. (A) Colpophotograph illustrating a low-grade cervical intraepithelial neoplasia (CIN) in the transformation zone. (B)–(D).Histopathology of CIN I, II, and III. (Reproduced, with permission, from Kantarjian HM et al. MD Anderson Manual of Medical Oncology, 1st ed. New York: McGraw-Hill: Fig. 24-4.) CIN II and III: Untreated CIN II will regress in 43% of patients, progress in 22%, and persist in 35%. Untreated CIN III will regress in 32% of patients, progress in 14%, and persist in 56%. CIN II and III should be treated with ablative (cryotherapy or laser ablation) or excisional therapy (LEEP; laser and cold-knife conization). Hysterectomy is a treatment option for recurrent CIN II or III.


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First_Aid_Step2. Hysterectomy is a treatment option for recurrent CIN II or III. Postablative or excisional therapy follow-up is as follows: CIN I, II, or III with margins: Pap smear at 12 months and/or HPV testing. CIN II or III with margins: Pap smear at 6 months; consider repeat ECC. If margins are unknown, obtain a Pap smear at 6 months and HPV DNA testing at 12 months. ■ Invasive disease: Treatment based on biopsy results for invasive carcinoma is as follows (for staging, see Figure 2.12-8): Microinvasive carcinoma (stage IA1): Treat with cone biopsy and close follow-up or simple hysterectomy. Stages IA2, IB1, and IIA: May be treated either with radical hysterectomy with concomitant radiation and chemotherapy or with radiation plus chemotherapy alone. Stages IB2, IIB, III, and IV: Treat with radiation therapy plus concurrent cisplatin-based chemotherapy. ■The overall fve-year relative survival rate for carcinoma of the cervix is 68% in Caucasian women and 55% in African-American women.


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First_Aid_Step2. ■The overall fve-year relative survival rate for carcinoma of the cervix is 68% in Caucasian women and 55% in African-American women. FIGURE 2.12-8. Staging of cervical cancer. Anatomic display of the stages of cervix cancer, defned by location, extent of tumor, frequency of presentation, and f ve-year survival. (Reproduced, with permission, from Fauci AS et al. Harrison’s Principles of Internal Medicine, 17th ed. New York: McGraw-Hill, 2008: Fig. 93-1.) Survival rates are inversely proportionate to the stage of cancer: ■Stage 0: 99–100%. Stage IA: > 95%. Stage IB-IIA: 80–90%. Stage IIB: 65%. Stage III: 40%. Stage IV: < 20%. Almost two-thirds of patients with untreated carcinoma of the cervix die of uremia when ureteral obstruction is bilateral.


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First_Aid_Step2. Stage IB-IIA: 80–90%. Stage IIB: 65%. Stage III: 40%. Stage IV: < 20%. Almost two-thirds of patients with untreated carcinoma of the cervix die of uremia when ureteral obstruction is bilateral. Responsible for 1–4% of gynecologic malignancies. Some 90–95% are squamous lesions occurring in women > 50 years of age, followed by melanoma, basal cell carcinoma, adenocarcinoma, sarcoma, Bartholin’s gland tumors, and metastatic disease. Risk factors include HPV (types 16, 18, and 31), lichen sclerosus, infrequent medical exams, diabetes, obesity, hypertension, cardiovascular disease, and immunosuppression. Presents with pruritus, pain, or ulceration of the mass. Early: Lesions may appear white, pigmented, raised, thickened, nodular, or ulcerative. Late: Presents with a large, caulifower-like or hard ulcerated area in the vulva. The frst step is vulvar punch biopsy for any suspicious lesions.


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First_Aid_Step2. Late: Presents with a large, caulifower-like or hard ulcerated area in the vulva. The frst step is vulvar punch biopsy for any suspicious lesions. Vulvar intraepithelial neoplasia (VIN) is considered precancerous and more commonly occurs in premenopausal women. VIN I and II: Associated with mild and moderate dysplasia. VIN III: Carcinoma in situ. Precancerous lesions: Reduce irritative or other predisposing causes. Topical corticosteroids (e.g., betamethasone, clobetasol) and crotamiton are particularly effective for pruritus. High-grade VIN: Topical chemotherapy, laser ablation, wide local excision, skinning vulvectomy, and simple vulvectomy. Invasive: Treated with (1) radical vulvectomy and regional lymphadenectomy or (2) wide local excision of the 1° tumor with inguinal lymph node dissection +/– preoperative radiation, chemotherapy, or both.


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First_Aid_Step2. Accounts for 1–2% of all gynecologic malignancies. Squamous cell carcinoma usually occurs in postmenopausal woman, whereas other histologic types (rhabdosarcoma, endodermal sinus tumor, adenocarcinoma, and clear cell adenocarcinoma from DES) usually affect younger women. Risk factors include immunosuppression, chronic irritation (e.g., long-term pessary use or prolapse of female organs), low socioeconomic status, radiation for cervical cancer, hysterectomy for dysplasia, multiple sexual partners, and DES exposure. Frequency of female genital tract cancers: endometrial > ovarian > cervical. Number of deaths: ovarian > endometrial > cervical. Any palpable ovarian or adnexal mass in a premenarchal or postmenopausal patient is suggestive of an ovarian neoplasm. Characterized by abnormal vaginal bleeding, an abnormal discharge, or postcoital bleeding. Presents in the upper third of the vagina in 75% of patients. Cytology, colposcopy, and biopsy.


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First_Aid_Step2. Characterized by abnormal vaginal bleeding, an abnormal discharge, or postcoital bleeding. Presents in the upper third of the vagina in 75% of patients. Cytology, colposcopy, and biopsy. Local excision of involved areas when they are few and small. Extensive involvement of the vaginal mucosa may require partial or complete vaginectomy. Invasive disease requires radiation therapy or radical surgery. Most ovarian tumors are benign, but malignant tumors are the leading cause of death from reproductive tract cancer. Risk factors include the following: Age, low parity, ↓ fertility, or delayed childbearing. A family history. Patients with one affected frst-degree relative have a 5% lifetime risk. With two or more affected frst-degree relatives, the risk is 7%. The BRCA1 mutation carries a 45% lifetime risk of ovarian cancer. The BRCA2 mutation is associated with a 25% lifetime risk.


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First_Aid_Step2. The BRCA1 mutation carries a 45% lifetime risk of ovarian cancer. The BRCA2 mutation is associated with a 25% lifetime risk. Lynch II syndrome, or hereditary nonpolyposis colorectal cancer (HNPCC), is associated with an ↑ risk of colon, ovarian, endometrial, and breast cancer. OCPs taken for fve years or more ↓ risk by 29%. Both benign and malignant ovarian neoplasms are generally asymptomatic. Mild, nonspecifc GI symptoms or pelvic pressure/pain may be seen. Early disease is typically not detected on routine pelvic exam. Some 75% of woman present with advanced malignant disease, as evidenced by abdominal pain and bloating, a palpable abdominal mass, and ascites. Table 2.12-10 differentiates the benign and malignant characteristics of pelvic masses. Tumor markers (see Table 2.12-11): ↑ CA-125 is associated with epithelial cell cancer (90% of ovarian cancers) but is used only as a marker for progression and recurrence.


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First_Aid_Step2. Tumor markers (see Table 2.12-11): ↑ CA-125 is associated with epithelial cell cancer (90% of ovarian cancers) but is used only as a marker for progression and recurrence. Premenopausal women: ↑ CA-125 may point to benign disease such as endometriosis. Postmenopausal women: ↑ CA-125 (> 35 units) indicates an ↑ likelihood that the ovarian tumor is malignant. Transvaginal ultrasound: Used to screen high-risk women. T AB LE 2.1 2-1 0. Benign vs. Malignant Pelvic Masses Treatment of ovarian masses is as follows: Premenarchal women: Masses > 2 cm require exploratory laparotomy. Premenopausal women: Observation for 4–6 weeks is suffcient for asymptomatic, mobile, unilateral, simple cystic masses < 8–10 cm. Most resolve spontaneously. Surgical evaluation is warranted for masses > 8–10 cm as well as for those that are unchanged on repeat pelvic exam and ultrasound. Postmenopausal women:


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First_Aid_Step2. Surgical evaluation is warranted for masses > 8–10 cm as well as for those that are unchanged on repeat pelvic exam and ultrasound. Postmenopausal women: Asymptomatic, unilateral simple cysts < 5 cm in diameter with a normal CA-125 should be closely followed with ultrasound. Palpable masses warrant surgical evaluation by exploratory laparotomy. Treatment of ovarian cancer is as follows: Surgery: Surgical staging followed by TAH/BSO with omentectomy and pelvic and para-aortic lymphadenectomy. TABLE 2.12-11. Ovarian Tumor Markers Epithelial CA-125 Endodermal sinus AFP Embryonal carcinoma AFP, hCG Choriocarcinoma hCG Dysgerminoma LDH Granulosa cell Inhibin ■Benign neoplasms warrant tumor removal or unilateral oophorectomy. Postoperative chemotherapy is routine except for women with early-stage or low-grade ovarian cancer. Radiation therapy is effective for dysgerminomas.


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First_Aid_Step2. Postoperative chemotherapy is routine except for women with early-stage or low-grade ovarian cancer. Radiation therapy is effective for dysgerminomas. Women with the BRCA1 gene mutation should be screened annually with ultrasound and CA-125 testing. Prophylactic oophorectomy is recommended by age 35 or whenever childbearing is completed. OCP use ↓ risk. Risk factors for pelvic organ prolapse include vaginal birth (particularly with use of forceps), genetic predisposition, advancing age, prior pelvic surgery, connective tissue disorders, and ↑ intra-abdominal pressure associated with obesity or straining with chronic constipation. Presents with the sensation of a bulge or protrusion in the vagina. Urinary or fecal incontinence, a sense of incomplete bladder emptying, and dyspareunia are also seen. The degree of prolapse can be evaluated by having the woman perform the Valsalva maneuver while in the lithotomy position.


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First_Aid_Step2. The degree of prolapse can be evaluated by having the woman perform the Valsalva maneuver while in the lithotomy position. Supportive measures include a high-fber diet and weight reduction in obese patients and limitation of straining and lifting. Pessaries may temporarily reduce prolapse and are helpful in women who do not wish to undergo surgery or who are chronically ill. The most common surgical procedure is vaginal or abdominal hysterectomy with vaginal vault suspension. Defned as the involuntary loss of urine due to either bladder or sphincteric dysfunction. Table 2.12-12 outlines the types of incontinence along with their distinguishing features and treatment (see also the mnemonic DIAPPERS). Exclude fstula in cases of total incontinence. Look for neurologic abnormalities in cases of urge incontinence (spasticity, faccidity, rectal sphincter tone) or distended bladder in overf ow incontinence. First step: Obtain a UA and urine culture to exclude UTI.


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First_Aid_Step2. First step: Obtain a UA and urine culture to exclude UTI. Next step: ■Voiding diary; possible urodynamic testing. Causes of urinary incontinence without specif c urogenital pathology— Excessive urinary output (hyperglycemia, hypercalcemia, CHF) TABLE 2.12-12. Types of Incontinence


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First_Aid_Step2. Total Uncontrolled loss at all times and in all positions. Loss of sphincteric eff ciency (previous surgery, nerve damage, cancer inf ltration). Abnormal connection between the urinary tract and the skin (f stula). Surgery. Stress After ↑ intra-abdominal pressure (coughing, sneezing, lifting). Urethral sphincteric insuff ciency due to laxity of pelvic f oor musculature; common in multiparous women or after pelvic surgery. Kegel exercises and pessary. Vaginal vault suspension surgery. Strong, unexpected urge to void that is unrelated to position or activity. Detrusor hyperrefexia or sphincter dysfunction due to inf ammatory conditions or neurogenic disorders of the bladder. Anticholinergic medications or TCAs; behavioral training (biofeedback). Chronic urinary retention. Chronically distended bladder with ↑ intravesical pressure that just exceeds the outlet resistance, allowing a small amount of urine to dribble out. Placement of urethral catheter in acute settings. Treat underlying


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First_Aid_Step2. bladder with ↑ intravesical pressure that just exceeds the outlet resistance, allowing a small amount of urine to dribble out. Placement of urethral catheter in acute settings. Treat underlying diseases. Timed voiding. a Etiologies include inhibited contractions, local irritation (cystitis, stone, tumor), and CNS causes. b Etiologies include physical agents (tumor, stricture), neurologic factors (lesions), and medications.


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First_Aid_Step2. Serum creatinine to exclude renal dysfunction. Cystogram to demonstrate fstula sites and descensus of the bladder neck. Table 2.12-12 outlines treatment options according to subtype. The most common of all benign breast conditions. Involves exaggerated stromal tissue response to hormones and growth factors. Findings include cysts (gross and microscopic), papillomatosis, adenosis, fbrosis, and ductal epithelial hyperplasia. Primarily affects women 30–50 years of age; rarely found in postmenopausal woman. Associated with trauma and caffeine use. Presents with cyclic bilateral mastalgia and swelling, with symptoms most prominent just before menstruation. Rapid f uctuation in the size of the masses is common. Other symptoms include an irregular, bumpy consistency to the breast tissue (“oatmeal with raisins”). The differential diagnosis of a breast mass includes f brocystic disease, f broadenoma, mastitis/ abscess, fat necrosis, and breast cancer.


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First_Aid_Step2. The differential diagnosis of a breast mass includes f brocystic disease, f broadenoma, mastitis/ abscess, fat necrosis, and breast cancer. Intraductal papilloma is a common cause of bloody nipple discharge. See Figure 2.12-9 for an algorithm of a breast mass workup. Mammography is of limited use. Ultrasound can help differentiate a cystic from a solid mass. Fine-needle aspiration (FNA) of a discrete mass that is suggestive of a cyst is indicated to alleviate pain as well as to confrm the cystic nature of the mass. Perform an excisional biopsy if no fuid is obtained or if the fuid is bloody on aspiration. There is an ↑ risk of breast cancer if ductal epithelial hyperplasia or cellular atypia is present. Dietary modifcations (e.g., caffeine restriction). Danazol may be given for severe pain but is rarely used in view of its side effects (acne, hirsutism, edema). Consider use of OCPs, which ↓ hormonal f uctuations.


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First_Aid_Step2. Danazol may be given for severe pain but is rarely used in view of its side effects (acne, hirsutism, edema). Consider use of OCPs, which ↓ hormonal f uctuations. A benign, slow-growing breast tumor with epithelial and stromal components. The most common breast lesion in women < 30 years of age. Cystosarcoma phyllodes is a large f broadenoma. Suspicious mass: -Age > 35 -Family history -Firm, rigid -Axillary adenopathy -Skin changes FNA Excisional biopsy Excisional biopsy Follow-up monthly × 3 Clear fluid, mass disappears Bloody fluid Residual mass or thickening DCIS/cancer: Treat as indicated Mammography Core or excisional biopsy Negative: Reassure, routine follow-up Nonsuspicious mass: -Age < 35 -No family history -Movable, fluctuant -Size change w/cycle CystSolid Cytology Malignant Treatment Repeat FNA or open surgical biopsy Benign or inconclusive FIGURE 2.12-9. Workup of a breast mass.


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First_Aid_Step2. FIGURE 2.12-9. Workup of a breast mass. Presents as a round or ovoid, rubbery, discrete, relatively mobile, non-tender mass 1–3 cm in diameter. Usually solitary, although up to 20% of patients develop multiple f broadenomas. Tumors do not change during the menstrual cycle. Does not occur after menopause unless the patient is on HRT. Breast ultrasound can differentiate cystic from solid masses. Needle biopsy or FNA. Excision with pathologic exam if the diagnosis remains uncertain. Excision is curative, but recurrence is common. The most common cancer (affects one in eight women) and the second most common cause of cancer death in women (after lung cancer). Sixty percent occur in the upper outer quadrant. Risk factors include the following (most women have no risk factors): Female gender, older age. A personal history of breast cancer. Breast cancer in a f rst-degree relative. BRCA1 and BRCA2 mutations (associated with early onset). A high-fat and low-f ber diet.


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First_Aid_Step2. Female gender, older age. A personal history of breast cancer. Breast cancer in a f rst-degree relative. BRCA1 and BRCA2 mutations (associated with early onset). A high-fat and low-f ber diet. A history of fbrocystic change with cellular atypia. ■↑ exposure to estrogen (nulliparity, early menarche, late menopause, f rst full-term pregnancy after age 35). Ninety percent of breast cancers are found by the patient. Clinical manifestations include the following: Early f ndings: May present as a single, nontender, frm-to-hard mass with ill-defned margins or as mammographic abnormalities with no palpable mass. Later f ndings: Skin or nipple retraction, axillary lymphadenopathy, breast enlargement, redness, edema, pain, fxation of the mass to the skin or chest wall. Late f ndings: Ulceration; supraclavicular lymphadenopathy; edema of the arm; metastases to the bone, lung, and liver.


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First_Aid_Step2. Late f ndings: Ulceration; supraclavicular lymphadenopathy; edema of the arm; metastases to the bone, lung, and liver. Prolonged unilateral scaling erosion of the nipple with or without discharge (Paget’s disease of the nipple). Metastatic disease: Back or bone pain, jaundice, weight loss. A frm or hard axillary node > 1 cm. Axillary nodes that are matted or fxed to the skin (stage III); ipsilateral supraclavicular or infraclavicular nodes (stage IV). ↑ exposure to estrogen (early menarche, late menopause, nulliparity) ↑ the risk of breast cancer. In a postmenopausal woman with a new breast lesion, maintain a high degree of clinical suspicion for breast cancer. The f rst step in the workup of a suspicious mass in postmenopausal women and in those > 30 years of age is a mammogram. For premenopausal women < 30 years of age, get an ultrasound. Breast cancer stages: Stage I: Tumor size < 2 cm. Stage II: Tumor size 2–5 cm. Stage III: Axillary node involvement.


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First_Aid_Step2. Breast cancer stages: Stage I: Tumor size < 2 cm. Stage II: Tumor size 2–5 cm. Stage III: Axillary node involvement. Stage IV: Distant metastasis. Diagnostic measures differ for postmenopausal and premenopausal women. Postmenopausal women: The frst step is mammography. Look for ↑ density with microcalcifcations and irregular borders. Mammography can detect lesions roughly two years before they become clinically palpable (see Figure 2.12-10A). Premenopausal women: The frst step for women < 30 years of age is ultrasound, which can distinguish a solid mass from a benign cyst (see Figure 2.12-10B). Additional measures include the following: Tumor markers for recurrent breast cancer: Include CEA and CA 15-3 or CA 27-29. Receptor status of tumor: Determine estrogen receptor (ER), progesterone receptor (PR), and HER2/neu status. Metastatic disease: Labs: ↑ ESR, ↑ alkaline phosphatase (liver and bone metastases), ↑ calcium.


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First_Aid_Step2. Metastatic disease: Labs: ↑ ESR, ↑ alkaline phosphatase (liver and bone metastases), ↑ calcium. Imaging: CXR; CT of the chest, abdomen, pelvis, and brain; bone scan. Pharmacologic: All hormone receptor– patients should receive tamoxifen. ER-patients should receive chemotherapy. Trastuzumab, a monoclonal antibody that binds to HER2/neu receptors on the cancer cell, is highly effective in HER2/neu-expressive cancers. Surgical options include the following: Partial mastectomy (lumpectomy) plus axillary dissection followed by radiation therapy. Modifed radical mastectomy (total mastectomy plus axillary dissection). Contraindications to breast-conserving therapy include large tumor size, subareolar location, multifocal tumors, fxation to the chest wall, or involvement of the nipple or overlying skin. Stage IV disease should be treated with radiotherapy and hormonal therapy; mastectomy may be required for local symptom control.


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First_Aid_Step2. Stage IV disease should be treated with radiotherapy and hormonal therapy; mastectomy may be required for local symptom control. TNM staging (I–IV) is the most reliable indicator of prognosis. ERand PRstatus is associated with a favorable course. Cancer localized to the breast has a 75–90% cure rate. With spread to the axilla, the fve-year survival is 40–50%. Aneuploidy is associated with a poor prognosis. Pleural effusion occurs in 50% of patients with metastatic breast cancer; edema of the arm is common. B F IGU R E 2.1 2-1 0. Palpable breast mass on mammography and ultrasound. (A) Craniocaudal mammography of a palpable mass (arrows). (B) Ultrasound image demonstrating a solid mass with irregular borders (arrows) consistent with cancer. (Reproduced, with permission, from Brunicardi FC et al. Schwartz’s Principles of Surgery, 8th ed. New York: McGraw-Hill, 2005: Fig. 16-26.)


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First_Aid_Step2. Also known as nonaccidental trauma; includes neglect as well as physical, sexual, and psychological maltreatment of children. Suspect abuse if the history is discordant with physical findings or if there is a delay in obtaining appropriate medical care. Certain injuries in children such as retinal hemorrhages and specific fracture types are pathognomonic for abuse. Abuse or neglect in infants may present as apnea, seizures, feeding intolerance, excessive irritability or somnolence, or failure to thrive (FTT). Neglect in older children may present as poor hygiene or behavioral abnormalities. Exam findings may include the following: Injuries in atypical places (e.g., the face or thighs) or patterns (stockingglove burns, cigarette burns, belt marks). Spiral fractures of the humerus and femur (strongly suggest abuse in children < 3 years of age) or epiphyseal/metaphyseal “bucket fractures,” which suggest shaking or jerking of the child’s limbs. Posterior rib fractures.


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First_Aid_Step2. Posterior rib fractures. Genital trauma, bleeding, or discharge. Rule out conditions that mimic abuse—e.g., bleeding disorders or Mongolian spots (bruises), osteogenesis imperfecta (fractures), bullous impetigo (cigarette burns), and “coining” (an alternative treatment in certain cultures). A skeletal survey and bone scan can show fractures in various stages of healing. Test for gonorrhea, syphilis, chlamydia, and HIV if sexual abuse is suspected. Rule out shaken baby syndrome (SBS) by performing an ophthalmologic exam for retinal hemorrhages and a noncontrast CT for subdural hematomas. Infants with SBS often do not exhibit external signs of abuse. Consider an MRI to visualize white matter changes (diffuse axonal injury associated with violent shaking) and the extent of intraand extracranial bleeds. MRI often requires the young patient to be sedated and/or intubated, while CT usually does not.


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First_Aid_Step2. Document injuries, including location, size, shape, color, and the nature of all lesions, bruises, or burns. Notify child protective services (CPS) for evaluation and possible removal of the child from the home. Hospitalize if necessary to stabilize injuries or to protect the child. Consider abuse if the caretaker’s story and the child’s injuries don’t match. Reporting any suspicion of child abuse is mandatory; you cannot be sued for doing so. Intrauterine risk factors for congenital heart disease include maternal drug use (alcohol, lithium, thalidomide, phenytoin), maternal infections (rubella), and maternal illness (DM, PKU). Disease is classified by the presence or absence of cyanosis: Transposition of the great vessels = two arteries switched Tetralogy of Fallot (four) VSD is the most common congenital heart defect. Acyanotic conditions (“pink babies”): Have left-to-right shunts in which oxygenated blood from the lungs is shunted back into the pulmonary circulation.


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First_Aid_Step2. Acyanotic conditions (“pink babies”): Have left-to-right shunts in which oxygenated blood from the lungs is shunted back into the pulmonary circulation. Cyanotic conditions (“blue babies”): Have right-to-left shunts in which deoxygenated blood is shunted into the systemic circulation. A condition in which an opening in the ventricular septum allows blood to ﬂow between ventricles. VSD is the most common congenital heart defect. It is more common among patients with Apert’s syndrome (cranial deformities, fusion of the fingers and toes), Down syndrome, fetal alcohol syndrome, TORCH syndrome (toxoplasmosis, other agents, rubella, CMV, HSV), cri du chat syndrome, and trisomies 13 and 18. Small defects are usually asymptomatic at birth, but exam reveals a harsh holosystolic murmur heard best at the lower left sternal border.


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First_Aid_Step2. Small defects are usually asymptomatic at birth, but exam reveals a harsh holosystolic murmur heard best at the lower left sternal border. Large defects can present with frequent respiratory infections, dyspnea, FTT, and CHF. If present, the holosystolic murmur is softer and more blowing but can be accompanied by a systolic thrill, crackles, a narrow S2 with an ↑ P1, and a mid-diastolic apical rumble reﬂ ecting ↑ ﬂow across the mitral valve. Echocardiogram is diagnostic. ECG and CXR can demonstrate LVH with small defects and show both LVH and RVH with larger VSDs. CXR may show ↑ pulmonary vascular markings. Most small VSDs close spontaneously; patients should be monitored via echocardiography. Surgical repair is indicated in symptomatic patients who fail medical management, children < 1 year of age with signs of pulmonary hypertension, and older children with large VSDs that have not ↓ in size over time.


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First_Aid_Step2. Treat existing CHF with diuretics, inotropes, and ACEIs; treat respiratory infections as needed. A condition in which an opening in the atrial septum allows blood to ﬂ ow between the atria, leading to left-to-right shunting. Associated with Holt-Oram syndrome (absent radii, ASD, first-degree heart block), fetal alcohol syndrome, and Down syndrome. Ostium primum defects present in early childhood with findings of a murmur or fatigue with exertion. Ostium secundum defects (most common) tend to present in late childhood or early adulthood. Symptom onset and severity depend on the size of the defect. Symptoms of easy fatigability, frequent respiratory infections, and FTT can be observed, but patients are frequently asymptomatic. ASD has a f xed, widely split S2.


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First_Aid_Step2. Symptoms of easy fatigability, frequent respiratory infections, and FTT can be observed, but patients are frequently asymptomatic. ASD has a f xed, widely split S2. ■Exam reveals a right ventricular heave; a wide and fxed, split S2; and a systolic ejection murmur at the upper left sternal border (from ↑ ﬂ ow across the pulmonary valve). There may also be a mid-diastolic rumble at the left lower sternal border. Echocardiogram with color ﬂow Doppler reveals blood ﬂow between the atria (diagnostic), paradoxical ventricular wall motion, and a dilated right ventricle. ECG most commonly shows right axis deviation and RVH, although other patterns are possible depending on the type of defect. PR prolongation is common. CXR reveals cardiomegaly and ↑ pulmonary vascular markings. Most lesions are small defects that may close spontaneously and do not require treatment.


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First_Aid_Step2. CXR reveals cardiomegaly and ↑ pulmonary vascular markings. Most lesions are small defects that may close spontaneously and do not require treatment. Surgical closure is indicated in infants with CHF and in patients with more than a 2:1 ratio of pulmonary to systemic blood ﬂow. Early correction prevents complications such as arrhythmias, right ventricular dysfunction, and Eisenmenger’s syndrome. Failure of the ductus arteriosus to close in the first few days of life, leading to a left-to-right shunt from the aorta to the pulmonary artery. Risk factors include maternal first-trimester rubella infection, prematurity, and female gender. Typically asymptomatic; patients with large defects may present with FTT, recurrent lower respiratory tract infections, lower extremity clubbing, and CHF. Exam reveals a wide pulse pressure; a continuous “machinery murmur” at the second left intercostal space at the sternal border; a loud S2; and bounding peripheral pulses.


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First_Aid_Step2. Exam reveals a wide pulse pressure; a continuous “machinery murmur” at the second left intercostal space at the sternal border; a loud S2; and bounding peripheral pulses. A color ﬂow Doppler demonstrating blood ﬂow from the aorta into the pulmonary artery is diagnostic. With larger PDAs, echocardiography shows left atrial and left ventricular enlargement. ECG may show LVH, and CXR may show cardiomegaly if large lesions. Give indomethacin unless the PDA is needed for survival (e.g., transposition of the great vessels, tetralogy of Fallot, hypoplastic left heart) or if indomethacin is contraindicated (e.g., intraventricular hemorrhage). If indomethacin fails or if the child is > 6–8 months of age, surgical closure is required. In Eisenmenger’s syndrome, left-to-right shunt leads to pulmonary hypertension and shunt reversal. In infants presenting in a shocklike state within the f rst few weeks of life, look for: 1. 2. Inborn error of metabolism 3.


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First_Aid_Step2. In infants presenting in a shocklike state within the f rst few weeks of life, look for: 1. 2. Inborn error of metabolism 3. Ductal-dependent congenital heart disease, usually left-sided lesions (as the ductus is closing) 4. Come IN and CLOSE the door: give INdomethacin to CLOSE a PDA. What maternally ingested drug is associated with Ebstein’s anomaly (tricuspid valve displacement into the right ventricle)? Lithium. Coarctation is a cause of 2° hypertension in children. Coarctation of the Aorta Constriction of a portion of the aorta, leading to ↑ ﬂow proximal to and ↓ ﬂow distal to the coarctation. Occurs just below the left subclavian artery in 98% of patients. The condition is associated with Turner’s syndrome, berry aneurysms, and male gender. More than two-thirds of patients have a bicuspid aortic valve. Often presents in childhood with asymptomatic hypertension. Lower extremity claudication, syncope, epistaxis, and headache may be present.


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First_Aid_Step2. Often presents in childhood with asymptomatic hypertension. Lower extremity claudication, syncope, epistaxis, and headache may be present. The classic physical exam finding is a systolic BP that is higher in the upper extremities; the difference in BP between the left and right arm can indicate the point of coarctation. Additional findings include weak femoral pulses, radiofemoral delay, a short systolic murmur in the left axilla, and a forceful apical impulse. In infancy, critical coarctation requires a patent PDA for survival. Such infants may present in the first few weeks of life in a shocklike state when the PDA closes. Differential cyanosis may be seen with lower oxygen saturation in the left arm and lower extremities (postductal areas) as compared to the right arm (preductal area). Echocardiography and color ﬂow Doppler are diagnostic. CXR in young children may demonstrate cardiomegaly and pulmonary congestion.


First_Aid_Step2

First_Aid_Step2. Echocardiography and color ﬂow Doppler are diagnostic. CXR in young children may demonstrate cardiomegaly and pulmonary congestion. In older children, the following compensatory changes may be seen: LVH on ECG; the “3” sign on CXR due to preand postdilatation of the coarctation segment with aortic wall indentation; and “rib notching” due to collateral circulation through the intercostal arteries. If severe coarctation presents in infancy, the ductus arteriosus should be kept open with prostaglandin E1 (PGE1). Surgical correction or balloon angioplasty (controversial). Monitor for restenosis, aneurysm development, and aortic dissection. Transposition of the Great Vessels


First_Aid_Step2

First_Aid_Step2. Surgical correction or balloon angioplasty (controversial). Monitor for restenosis, aneurysm development, and aortic dissection. Transposition of the Great Vessels The most common cyanotic congenital heart lesion in the newborn. In this condition, the aorta is connected to the right ventricle and the pulmonary artery to the left ventricle, creating parallel pulmonary and systemic circulations. Without a septal defect or a PDA, it is incompatible with life. Risk factors include diabetic mothers and, rarely, DiGeorge syndrome (see the mnemonic CATCH 22). ■Critical illness and cyanosis typically occur immediately after birth. Reverse differential cyanosis may be seen if left ventricular outﬂow tract obstruction (e.g., coarctation, aortic stenosis) is also present. ■Exam reveals tachypnea, progressive hypoxemia, and extreme cyanosis. Some patients have signs of CHF, and a single loud S2 is often present. There may not be a murmur if no VSD is present. Echocardiography.


First_Aid_Step2

First_Aid_Step2. Echocardiography. CXR may show a narrow heart base, absence of the main pulmonary artery segment (“egg-shaped silhouette”), and ↑ pulmonary vascular markings. Start IV PGE1 to maintain or open the PDA. If surgery is not feasible within the first few days of life or if the PDA cannot be maintained with prostaglandin, perform balloon atrial septostomy to create or enlarge an ASD. Surgical correction (arterial or atrial switch). Tetralogy of Fallot Consists of pulmonary stenosis, overriding aorta, RVH, and VSD. The most common cyanotic congenital heart disease in children. Early cyanosis results from right-to-left shunting across the VSD. As right-sided pressures ↓ in the weeks after birth, the shunt direction reverses and cyanosis may ↓. If the degree of pulmonary stenosis is severe, the right-sided pressures may remain high and cyanosis may worsen over time. Risk factors include maternal PKU and DiGeorge syndrome.