Reproductive System

Male reproductive system
	Reproductive organs
Female reproductive system
	Mammary glands
	External structures
	Internal structures
	The menstrual cycle
Pathophysiologic manifestations
	Sexual maturation alteration
	Hormonal alterations
	Menstrual alterations
	Sexual dysfunction
	Male structural alterations
Disorders
	Abnormal uterine bleeding
	Amenorrhea
	Benign prostatic hyperplasia
	Cryptorchidism
	Dysmenorrhea
	Endometriosis
	Erectile dysfunction
	Fibrocystic change of the breast
	Fibroid disease of the uterus
	Gynecomastia
	Hydrocele
	Ovarian cysts
	Precocious puberty
	Polycystic ovarian syndrome
	Prostatitis
	Testicular torsion
	Varicocele

T he reproductive system must function properly to ensure survival of the species. The male reproductive system produces sperm and delivers them to the female reproductive tract. The female reproductive system produces the ovum. If a sperm fertilizes an ovum, this system also nurtures and protects the embryo and developing fetus and delivers it at birth. The functioning of the reproductive system is determined not only by anatomic structure, but also by complex hormonal, neurologic, vascular, and psychogenic factors.

Anatomically, the main distinction between the male and female is the presence of conspicuous external genitalia in the male. In contrast, the major reproductive organs of the female lie within the pelvic cavity.

MALE REPRODUCTIVE SYSTEM

The male reproductive system consists of the organs that produce sperm, transfer mature sperm from the testes, and introduce them into the female reproductive tract, where fertilization occurs.

Besides supplying male sex cells (in a process called spermatogenesis), the male reproductive system plays a part in the secretion of male sex hormones. The penis also functions in urine elimination.

Reproductive organs

The male reproductive organs include the penis, scrotum, testes, duct system, and accessory reproductive glands.

Penis

The penis consists of three cylinders of erectile tissue: two corpora cavernosa, and the corpus spongiosum, which contains the urethra. The glans or tip of the penis contains the urethral meatus, through which urine and semen pass to the exterior, and many nerve endings for sexual sensation.

Scrotum

The scrotum, which contains the testes, epididymis, and lower spermatic cords, maintains the proper testicular temperature for spermatogenesis through relaxation and contraction. This is important because excessive heat reduces the sperm count.

Testes

The testes (also called gonads, which is a term for any reproductive organ, or testicles) produce sperm in the seminiferous tubules.

AGE ALERT Complete spermatogenesis develops in most males by age 15 or 16 years.

The testes form in the abdominal cavity of the fetus and descend into the scrotum during the seventh month of gestation.

Duct system

The vas deferens connects the epididymis, in which sperm mature and ripen for up to 6 weeks, and the ejaculatory ducts. The seminal vesicles ― two convoluted membranous pouches ― secrete a viscous liquid of fructose-rich semen and prostaglandins that probably facilitates fertilization.

Accessory reproductive glands

The prostate gland secretes the thin alkaline substance that comprises most of the seminal fluid; this fluid also protects sperm from acidity in the male urethra and in the vagina, thus increasing sperm motility.

The bulbourethral (Cowper's) glands secrete an alkaline ejaculatory fluid, probably similar in function to that produced by the prostate gland. The spermatic cords are cylindrical fibrous coverings in the inguinal canal containing the vas deferens, blood vessels, and nerves.

Testosterone

The testes produce and secrete hormones, especially testosterone, in their interstitial cells (Leydig's cells). Testosterone affects the development and maintenance of secondary sex characteristics and sex drive. It also regulates metabolism, stimulates protein anabolism (encouraging skeletal growth and muscular development), inhibits pituitary secretion of the gonadotropins (follicle-stimulating hormone and interstitial cell-stimulating hormone), promotes potassium excretion, and mildly influences renal sodium reabsorption.

In males, the reproductive and urinary systems are structurally integrated; most disorders, therefore, affect both systems. Congenital abnormalities or prostate enlargement may impair both sexual and urinary function. Abnormal findings in the pelvic area may result from pathologic changes in other organ systems, such as the upper urinary and GI tracts, endocrine glands, and neuromusculoskeletal system.

FEMALE REPRODUCTIVE SYSTEM

Female reproductive structures include the mammary glands, external genitalia, and internal genitalia. Hormonal influences determine the development and function of these structures and affect fertility, childbearing, and the ability to experience sexual pleasure.

In no other part of the body do so many interrelated physiologic functions occur in such proximity as in the area of the female reproductive tract. Besides the internal genitalia, the female pelvis contains the organs of the urinary and GI systems (bladder, ureters, urethra, sigmoid colon, and rectum). The reproductive tract and its surrounding area are thus the site of urination, defecation, menstruation, ovulation, copulation, impregnation, and parturition.

Mammary glands

Located in the breasts, the mammary glands are specialized accessory glands that secrete milk. Although present in both sexes, they normally function only in females.

External structures

Female genitalia include the following external structures, collectively known as the vulva: mons pubis (or mons veneris), labia majora, labia minora, clitoris, and the vestibule. The perineum is the external region between the vulva and the anus. The size, shape, and color of these structures ― as well as pubic hair distribution and skin texture and pigmentation ― vary greatly among individuals. Furthermore, these external structures undergo distinct changes during the life cycle.

Mons pubis

The mons pubis is the pad of fat over the symphysis pubis (pubic bone), which is usually covered by the base of the inverted triangular patch of pubic hair that grows over the vulva after puberty.

Labia majora

The labia majora are the two thick, longitudinal folds of fatty tissue that extend from the mons pubis to the posterior aspect of the perineum. The labia majora protect the perineum and contain large sebaceous glands that help maintain lubrication. Virtually absent in the young child, their development is a characteristic sign of onset of puberty. The skin of the more prominent parts of the labia majora is pigmented and darkens after puberty.

Labia minora

The labia minora are the two thin, longitudinal folds of skin that border the vestibule. Firmer than the labia majora, they extend from the clitoris to the posterior fourchette.

Clitoris

The clitoris is the small, protuberant organ located just beneath the arch of the mons pubis. The clitoris contains erectile tissue, venous cavernous spaces, and specialized sensory corpuscles that are stimulated during coitus. It's homologous to the male penis.

Vestibule

The vestibule is the oval space bordered by the clitoris, labia minora, and fourchette. The urethral meatus is located in the anterior portion of the vestibule, and the vaginal meatus is in the posterior portion. The hymen is the elastic membrane that partially obstructs the vaginal meatus in virgins. Its absence doesn't necessarily imply a history of coitus, nor does its presence obstruct menstrual blood flow.

Several glands lubricate the vestibule. Skene's glands (also known as the paraurethral glands) open on both sides of the urethral meatus; Bartholin's glands, on both sides of the vaginal meatus.

The fourchette is the posterior junction of the labia majora and labia minora. The perineum, which includes the underlying muscles and fascia, is the external surface of the floor of the pelvis, extending from the fourchette to the anus.

Internal structures

The internal structures of the female genitalia include the vagina, cervix, uterus, Fallopian tubes (or oviducts), and ovaries.

Vagina

The vagina occupies the space between the bladder and the rectum. A muscular, membranous tube approximately 2 to 3 inches (5 to 7.5 cm) long, the vagina connects the uterus with the vestibule of the external genitalia. It serves as a passageway for sperm to the Fallopian tubes, a conduit for the discharge of menstrual fluid, and the birth canal during parturition.

Cervix

The cervix is the most inferior part of the vagina, protruding into the vaginal canal. The cervix provides a passageway between the vagina and the uterine cavity.

Uterus

The uterus is the hollow, pear-shaped organ in which the conceptus grows during pregnancy. The thick uterine wall consists of mucosal, muscular, and serous layers. The inner mucosal lining (the endometrium) undergoes cyclic changes to facilitate and maintain pregnancy.

The smooth muscular middle layer (the myometrium) interlaces the uterine and ovarian arteries and veins that circulate blood through the uterus. During pregnancy, this vascular system expands dramatically. After abortion or childbirth, the myometrium contracts to constrict the vasculature and control loss of blood.

The outer serous layer (the parietal peritoneum) covers all of the fundus, part of the corpus, but none of the cervix. This incompleteness allows surgical entry into the uterus without incision of the peritoneum, thus reducing the risk for peritonitis in the days before effective antibiotic therapy.

Fallopian tubes

The Fallopian tubes extend from the sides of the fundus and terminate near the ovaries. Each tube has a fimbriated (fringelike) end adjacent to the ovary that serves to capture an oocyte after ovulation. Through ciliary and muscular action, these small tubes carry ova from the ovaries to the uterus and facilitate the movement of sperm from the uterus toward the ovaries. The same ciliary and muscular action helps move a zygote (fertilized ovum) down to the uterus, where it may implant in the blood-rich inner uterine lining, the endometrium.

Ovaries

The ovaries are two almond-shaped organs, one on either side of the pelvis, situated behind and below the Fallopian tubes. The ovaries produce ova and two primary hormones ― estrogen and progesterone ― in addition to small amounts of androgen. These hormones in turn produce and maintain secondary sex characteristics, prepare the uterus for pregnancy, and stimulate mammary gland development. The ovaries are connected to the uterus by the utero-ovarian ligament.

In the 30th week of gestation, the fetus has about 7 million follicles, which degenerate, leaving about 2 million present at birth. By puberty, only 400,000 remain, and these ova precursors become Graafian follicles in response to the effects of pituitary gonadotropic hormones (follicle-stimulating hormone [FSH] and luteinizing hormone [LH]). Fewer than 500 of each woman's ova mature and become potentially fertile.

The menstrual cycle

Maturation of the hypothalamus and the resultant increase in hormone levels initiate puberty. In the young girl, the appearance of pubic and axillary hair (pubarche) and the characteristic adolescent growth spurt follow breast development (thelarche) ― the first sign of puberty. The reproductive system begins to undergo a series of hormone-induced changes that result in menarche, or the onset of menstruation (or menses).

The menstrual cycle consists of three different phases: menstrual, proliferative (estrogen dominated), and secretory (progesterone dominated). (See Understanding the menstrual cycle .)

At the end of the secretory phase, the uterine lining is ready to receive and nourish a zygote. If fertilization doesn't occur, increasing estrogen and progesterone levels decrease LH and FSH production. Because LH is needed to maintain the corpus luteum, a decrease in LH production causes the corpus luteum to atrophy and halt the secretion of estrogen and progesterone. The thickened uterine lining then begins to slough off, and menstruation begins again.

In the nonpregnant female, LH controls the secretions of the corpus luteum, thereby increasing progesterone levels in the bloodstream. In the pregnant woman, human chorionic gonadotropin (HCG), produced by the nascent placenta, controls these secretions.

If fertilization and pregnancy occur, the endometrium grows even thicker and vascular ingrowth occurs. After implantation of the zygote (about 5 or 6 days after fertilization), the endometrium becomes the decidua. Trophoblastic cells produce HCG soon after implantation, stimulating the corpus luteum to continue secreting estrogen and progesterone, which prevents further ovulation and menstruation.

HCG continues to stimulate the corpus luteum until the placenta (the vascular organ that develops to transport materials to and from the fetus) forms and starts producing its own estrogen and progesterone. After the placenta takes over hormonal production, secretions of the corpus luteum are no longer needed to maintain the pregnancy, and the corpus luteum gradually decreases its function and begins to degenerate. This is termed the luteoplacental shift and commonly occurs by the end of the first trimester.

PATHOPHYSIOLOGIC MANIFESTATIONS

Alterations may occur in the structure, process, or function of both the male and female reproductive systems.

UNDERSTANDING THE MENSTRUAL CYCLE The menstrual cycle is divided into three distinct phases. During the menstrual phase, which starts on the first day of menstruation, the top layer of the endometrium breaks down and flows out of the body. This flow, the menses, consists of blood, mucous, and unneeded tissue. During the proliferatve (follicular) phase, the endometrium begins to thicken, and the level of estrogen in the blood increases. During the secretory (luteal) phase, the endometrium begins to thicken to nourish an embryo should fertilization occur. Without fertilization, the top layer of the endometrium breaks down and the menstrual phase of the cycle begins again. <center></center>

Sexual maturation alteration

Sexual maturation, or puberty, can be affected by various congenital and endocrine disorders. The timing of puberty may be too early (precocious puberty) or too late (delayed puberty). Precocious puberty is the onset of sexual maturation before age 9 years in boys and before age 8 years in girls. It occurs more often in girls than boys. In girls, the cause is most commonly idiopathic, whereas in boys it is more likely to be organic.

In delayed puberty, there's no evidence of the development of secondary sex characteristics in a boy age 14 years or a girl age 13 years. There's usually no evidence of hormonal abnormalities. The hypothalamic-pituitary-ovarian axis, a system that stimulates and regulates the production of hormones necessary for normal sexual development and function, is intact, but maturation is slow. The cause is unknown.

Hormonal alterations

Complex hormonal interactions determine the normal function of the female reproductive tract and require an intact hypothalamic-pituitary-ovarian axis. A defect or malfunction of this system can cause infertility due to insufficient gonadotropin secretions (both LH and FSH). The ovary controls, and is controlled by, the hypothalamus through a system of negative and positive feedback mediated by estrogen production. Insufficient gonadotropin levels may result from infections, tumors, or neurologic disease of the hypothalamus or pituitary gland. A mild hormonal imbalance in gonadotropin production and regulation, possibly caused by polycystic disease of the ovary or abnormalities in the adrenal or thyroid gland that adversely affect hypothalamic-pituitary functioning, may sporadically inhibit ovulation. Because gonadotropins are released in a pulsatile fashion, a significant disturbance in this pulsatility will adversely affect ovulatory function.

Male hypogonadism, or an abnormal decrease in gonad size and function, results from decreased androgen production in males, which may impair spermatogenesis (causing infertility) and inhibit the development of normal secondary sex characteristics. The clinical effects of androgen deficiency depend on age at onset. Primary hypogonadism results directly from interstitial (Leydig's cell) cellular or seminiferous tubular damage due to faulty development or mechanical damage. Androgen deficiency causes increased secretion of gonadotropins by the pituitary in an attempt to increase the testicular functional state and is therefore termed hypergonadotropic hypogonadism. This form of hypogonadism includes Klinefelter's syndrome (47 XXY), Reifenstein's syndrome, male Turner's syndrome, Sertoli-cell-only syndrome, anorchism, orchitis, and sequelae of irradiation.

Secondary hypogonadism is due to faulty interaction within the hypothalamic-pituitary axis, resulting in failure to secrete normal levels of gonadotropins, and is therefore termed hypogonadotropic hypogonadism. This form of hypogonadism includes hypopituitarism, isolated FSH deficiency, isolated LH deficiency, Kallmann's syndrome, and Prader-Willi syndrome. Depending on the patient's age at onset, hypogonadism may cause eunuchism (complete gonadal failure) or eunuchoidism (partial failure).

Symptoms vary depending on the specific cause of hypogonadism. Some characteristic findings may include delayed bone maturation, delayed puberty; infantile penis and small, soft testes; less than average muscle development and strength; fine, sparse facial hair; scant or absent axillary, pubic, and body hair; and a high-pitched, effeminate voice. In an adult, hypogonadism diminishes sex drive and potency and causes regression of secondary sex characteristics.

Menstrual alterations

Alterations in menstruation include the absence of menses, abnormal bleeding patterns, or painful menstruation. Menopause is the cessation of menstruation. It results from a complex continuum of physiologic changes ― the climacteric ― caused by declining ovarian function. The climacteric produces various changes in the body, the most dramatic being the cessation of menses.

AGE ALERT The climacteric, a normal gradual reduction in ovarian function due to aging, begins in most women between ages 40 and 50 years and results in infrequent ovulation, decreased menstrual function and, eventually, cessation of menstruation (usually between ages 45 and 55 years).

Premature menopause, the gradual or abrupt cessation of menstruation before age 35 years, occurs without apparent cause in about 5% of women in the United States. Certain diseases, especially autoimmune diseases such as premature ovarian failure, may cause pathologic menopause. Other factors that may precipitate premature menopause include malnutrition, debilitation, extreme emotional stress, pelvic irradiation, and surgical procedures that impair ovarian blood supply. Artificial menopause may follow radiation therapy or surgical procedures such as removal of both ovaries (bilateral oophorectomy). Hysterectomy decreases the interval before menopause even when the ovaries are not removed. It's speculated that hysterectomy may decrease ovarian blood flow in some fashion.

Ovarian failure, in which no ova are produced, may result from a functional ovarian disorder from premature menopause. Amenorrhea is a natural consequence of ovarian failure.

ABNORMAL PREMENOPAUSAL BLEEDING Causes of abnormal premenopausal bleeding vary with the type of bleeding: Oligomenorrhea (infrequent menses) and polymenorrhea (menses occurring too frequently) usually result from anovulation due to an endocrine or systemic disorder. Hypomenorrhea (decreased amount of menstrual fluid) results from local, endocrine, or systemic disorders or blockage caused by partial obstruction by the hymen or cervical obstruction. Hypermenorrhea (excessive bleeding occurring at the regular intervals) usually results from local lesions, such as uterine leiomyomas, endometrial polyps, and endometrial hyperplasia. It may also result from endometritis, salpingitis, and anovulation. Cryptomenorrhea (no external bleeding, although menstrual symptoms are experienced) may result from an imperforate hymen or cervical stenosis. Metrorrhagia (bleeding occurring at irregular intervals) usually results from slight physiologic bleeding from the endometrium during ovulation but may also result from local disorders, such as uterine malignancy, cervical erosions, polyps (which tend to bleed after intercourse), or inappropriate estrogen therapy. Complications of pregnancy can also cause premenopausal bleeding, which may be as mild as spotting or as severe as hypermenorrhea.

Pain is often associated with the menstrual cycle; in many common diseases of the female reproductive tract, such pain may follow a cyclic pattern. A patient with endometriosis, for example, may report increasing premenstrual pain that decreases at the end of menstruation. For a description of the types of abnormal menstrual bleeding, see Abnormal premenopausal bleeding .

Sexual dysfunction

Sexual dysfunction includes arousal problems, orgasmic problems, and sexual pain (dyspareunia, vaginismus). Dysfunction may be caused by a general medical condition, psychological condition, substance use or abuse, or a combination of these factors.

Arousal disorder is an inability to experience sexual pleasure. According to the Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV), the essential feature is a persistent or recurrent inability to attain or to maintain an adequate lubrication-swelling response of sexual excitement until completion of the sexual act. Orgasmic disorder, according to DSM-IV, is a persistent or recurrent delay in or absence of orgasm after a normal sexual excitement phase.

Both arousal and orgasmic disorders are considered primary if they exist in a female who has never experienced sexual arousal or orgasm; they are secondary when a physical, mental, or situational condition has inhibited or obliterated a previously normal sexual function. The prognosis is good for temporary or mild disorders resulting from misinformation or situational stress but is guarded for disorders that result from intense anxiety, chronically discordant relationships, psychological disturbances, or drug or alcohol abuse in either partner.

The following factors, alone or in combination, may cause arousal or orgasmic disorder:

• certain drugs, including central nervous system depressants, alcohol, street drugs, and, rarely, oral contraceptives
• general systemic illnesses, diseases of the endocrine or nervous system, or diseases that impair muscle tone or contractility
• gynecologic factors, such as chronic vaginal or pelvic infection or pain, congenital anomalies, and genital cancers
• stress and fatigue
• inadequate or ineffective stimulation
• psychological factors, such as performance anxiety, guilt, depression, or unconscious conflicts about sexuality
• relationship problems, such as poor communication, hostility, or ambivalence toward the partner, fear of abandonment or independence, or boredom with sex.

All these factors may contribute to involuntary inhibition of the orgasmic reflex. Another crucial factor is the fear of losing control of feelings or behavior. Whether these factors produce sexual dysfunction and the type of dysfunction depend on how well the woman copes with the resulting pressures. Physical factors may also cause arousal or orgasmic disorder.

Female sexual function and responses decline, along with estrogen levels, in the perimenopausal period. The decrease in estradiol levels during menopause affects nerve transmission and response in the peripheral vascular system. As a result, the timing and degree of vasoconstriction during the sexual response is affected, vasocongestion decreases, muscle tension decreases, and contractions are fewer and less intense during orgasm.

A female with arousal disorder has limited or absent sexual desire and experiences little or no pleasure from sexual stimulation. Physical signs of this disorder include lack of vaginal lubrication or absence of signs of genital vasocongestion.

Dyspareunia is genital pain associated with intercourse. Insufficient lubrication is the most common cause. Other physical causes of dyspareunia include:

• endometriosis
• genital, rectal, or pelvic scar tissue
• acute or chronic infections of the genitourinary tract
• disorders of the surrounding viscera (including residual effects of pelvic inflammatory disease or disease of the adnexal and broad ligaments).

Among the many other possible physical causes are:

• deformities or lesions of the introitus or vagina
• benign and malignant growths and tumors
• intact hymen
• radiation to the pelvis
• allergic reactions to diaphragms, condoms, or other contraceptives.

Psychological causes include:

• fear of pain or injury during intercourse
• previous painful experience, including sexual abuse
• guilt feelings about sex
• fear of pregnancy or injury to the fetus during pregnancy
• anxiety caused by a new sexual partner or technique
• mental or physical fatigue.

Vaginismus is an involuntary spastic constriction of the lower vaginal muscles, usually from fear of vaginal penetration. This disorder may coexist with dyspareunia and, if severe, may prevent intercourse (a common cause of unconsummated marriages). Vaginismus may be physical or psychological in origin. It may occur spontaneously as a protective reflex to pain or result from organic causes, such as hymenal abnormalities, genital herpes, obstetric trauma, and atrophic vaginitis.

Psychological causes may include:

• childhood and adolescent exposure to rigid, punitive, and guilt-ridden attitudes toward sex
• fear resulting from painful or traumatic sexual experiences, such as incest or rape
• early traumatic experience with pelvic examinations
• fear of pregnancy, sexually transmitted disease, or cancer.

In males, the normal sexual response involves erection, emission, and ejaculation. Sexual dysfunction is the impairment of one or all of these processes.

Erectile disorder, or impotence, refers to inability to attain or maintain penile erection sufficient to complete intercourse. Transient periods of impotence aren't considered dysfunction and probably occur in half the adult males. Erectile disorder affects all age groups but increases in frequency with age.

Psychogenic factors are responsible for approximately 50% to 60% of the cases of erectile dysfunction; organic factors, for the rest. In some patients, psychogenic and organic factors coexist, making isolation of the primary cause difficult.

Most problems with emission and ejaculation usually have structural causes.

Male structural alterations

Structural defects of the male reproductive system may be congenital or acquired. Testicular disorders such as cryptorchidism or torsion may result in infertility.

In cryptorchidism, a congenital disorder, one or both testes fail to descend into the scrotum, remaining in the abdomen or inguinal canal or at the external ring. If bilateral cryptorchidism persists untreated into adolescence, it may result in sterility, make the testes more vulnerable to trauma, and significantly increase the risk for testicular cancer, particularly germ cell tumors. In about 80% of affected infants, the testes descend spontaneously during the first year; in the rest, the testes may descend later.

AGE ALERT The testes of an older male may be slightly smaller than those of a younger male, but they should be equal in size, smooth, freely moveable, and soft, without nodules. The left testis is often lower than the right. Benign prostatic hyperplasia is a disorder of prostate enlargement due to androgen-induced growth of prostate cells. It's more prevalent with aging and may result in urinary obstructive symptoms.

Hypospadias is the most common penile structural abnormality. The midline fusion of the urethral folds is incomplete, so the urethral meatus opens on the ventral (anterior, or “belly”) side of the penis. In epispadias, the urethral meatus is located on the dorsal (posterior, or “back”) side of the penis.

Priapism is prolonged, painful erection in the absence of sexual stimulation. It results from arteriovenous shunting within the corpus cavernosum that leads to obstructed venous outflow from the penis. In adults, it's usually idiopathic due to trauma. In children, it may be associated with sickle cell disease. Without prompt treatment, it can lead to ischemic fibrosis and infertility.

A urethral stricture is a narrowing of the urethra caused by scarring. It may result from trauma or infection. Common complications include prostatitis and secondary infection.

During the first 3 years of life, congenital adhesions between the foreskin and the glans penis separate naturally with penile erections. Phimosis is a condition in which the foreskin can't be retracted over the glans penis; poor hygiene and chronic infection can cause it. Paraphimosis is a condition in which the foreskin is retracted and can't be reduced to cover the glans; the penis becomes constricted, causing edema of the glans. Severe paraphimosis is a surgical emergency.

To prevent threatened spontaneous abortion, millions of women took diethylstilbestrol (DES) between 1946 and 1971. Men whose mothers took DES during their eighth to sixteenth weeks of pregnancy have experienced structural abnormalities, such as urethral meatal stenosis, hypospadias, epididymal cysts, varicoceles, cryptorchidism, and decreased fertility.

DISORDERS

Disorders of the reproductive system may affect sexual, reproductive, or urinary function.

Abnormal uterine bleeding

Abnormal uterine bleeding refers to abnormal endometrial bleeding without recognizable organic lesions. Abnormal uterine bleeding is the indication for almost 25% of gynecologic surgical procedures. The prognosis varies with the cause. Correction of hormonal imbalance or structural abnormality yields a good prognosis.

Causes

Abnormal uterine bleeding usually results from an imbalance in the hormonal-endometrial relationship in which persistent and unopposed stimulation of the endometrium by estrogen occurs. Disorders that cause sustained high estrogen levels are:

• polycystic ovary syndrome
• obesity (because enzymes present in peripheral adipose tissue convert the androgen androstenedione to estrogen precursors)
• immaturity of the hypothalamic-pituitary-ovarian mechanism (postpubertal teenagers)
• anovulation (women in their late thirties or early forties).

Other causes include:

• trauma (foreign object insertion or direct trauma)
• endometriosis
• coagulopathy such as thrombocytopenia or leukemia (rare).

Pathophysiology

Irregular bleeding is associated with hormonal imbalance and anovulation (failure of ovulation to occur). When progesterone secretion is absent but estrogen secretion continues, the endometrium proliferates and become hypervascular. When ovulation does not occur, the endometrium is randomly broken down, and exposed vascular channels cause prolonged and excessive bleeding. In most cases of abnormal uterine bleeding, the endometrium shows no pathologic changes. However, in chronic unopposed estrogen stimulation (as from a hormone-producing ovarian tumor), the endometrium may show hyperplastic or malignant changes.

Signs and symptoms

Abnormal uterine bleeding usually occurs as:

• metrorrhagia (episodes of vaginal bleeding between menses)
• hypermenorrhea (heavy or prolonged menses, longer than 8 days, also incorrectly termed menorrhagia)
• chronic polymenorrhea (menstrual cycle less than 18 days) or oligomenorrhea (infrequent menses)
• fatigue due to anemia
• oligomenorrhea and infertility due to anovulation.

Complications

Possible complications are:

• iron-deficiency anemia (blood loss of more than 1.6 L over a short time) and hemorrhagic shock or right-sided cardiac failure (rare)
• endometrial adenocarcinoma due to chronic estrogen stimulation.

Diagnosis

Abnormal uterine bleeding may be caused by anovulation. Diagnosis of anovulation is based on:

• history of abnormal bleeding, bleeding in response to a brief course of progesterone, absence of ovulatory cycle body temperature changes, and low serum progesterone levels
• diagnostic studies ruling out other causes of excessive vaginal bleeding, such as organic, systemic, psychogenic, and endocrine causes, including certain cancers, polyps, pregnancy, and infection
• dilatation and curettage (D&C) or office endometrial biopsy to rule out endometrial hyperplasia and cancer
• hemoglobin levels and hematocrit to determine the need for blood or iron replacement.

Treatment

Possible treatment of abnormal uterine bleeding includes:

• high-dose estrogen-progestogen combination therapy (oral contraceptives) to control endometrial growth and reestablish a normal cyclic pattern of menstruation (usually given four times daily for 5 to 7 days even though bleeding usually stops in 12 to 24 hours; drug choice and dosage determined by patient's age and cause of bleeding); maintenance therapy with lower dose combination oral contraceptives
• endometrial biopsy to rule out endometrial adenocarcinoma (patients over age 35 years)
• progestogen therapy (alternative in many women, such as those susceptible to such adverse effects of estrogen as thrombophlebitis)
• I.V. estrogen followed by progesterone or combination oral contraceptives if the patient is young (more likely to be anovulatory) and severely anemic (if oral drug therapy is ineffective)
• D&C (short-lived treatment and not clinically useful, but an important diagnostic tool)
• iron replacement or transfusions of packed cells or whole blood, as indicated, due to anemia caused by recurrent bleeding
• explaining the importance of following the prescribed hormonal therapy; explaining D&C or endometrial biopsy procedure and purpose (if ordered)
• stressing the need for regular checkups to assess the effectiveness of treatment.

Amenorrhea

Amenorrhea is the abnormal absence or suppression of menstruation. Absence of menstruation is normal before puberty, after menopause, or during pregnancy and lactation; it's abnormal, and therefore pathologic, at any other time. Primary amenorrhea is the absence of menarche in an adolescent (after age 16 years). Secondary amenorrhea is the failure of menstruation for at least 3 months after the normal onset of menarche. Primary amenorrhea occurs in 0.3% of women; secondary amenorrhea is seen in 1% to 3% of women. Prognosis is variable, depending on the specific cause. Surgical correction of outflow tract obstruction is usually curative.

Causes

Amenorrhea usually results from:

• anovulation due to hormonal abnormalities, such as decreased secretion of estrogen, gonadotropins, luteinizing hormone, and follicle-stimulating hormone (FSH)
• lack of ovarian response to gonadotropins
• constant presence of progesterone or other endocrine abnormalities.

Amenorrhea may also result from:

• absence of a uterus
• endometrial damage
• ovarian, adrenal, or pituitary tumors
• emotional disorders (common in patients with severe disorders such as depression and anorexia nervosa); mild emotional disturbances tending to distort the ovulatory cycle; severe psychic trauma abruptly changing the bleeding pattern or completely suppressing one or more full ovulatory cycles
• malnutrition and intense exercise, causing an inadequate hypothalamic response.

Pathophysiology

The mechanism varies depending on the cause and whether the defect is structural, hormonal, or both. Women who have adequate estrogen levels but a progesterone deficiency don't ovulate and are thus infertile. In primary amenorrhea, the hypothalamic-pituitary-ovarian axis is dysfunctional. Because of anatomic defects of the central nervous system, the ovary doesn't receive the hormonal signals that normally initiate the development of secondary sex characteristics and the beginning of menstruation.

Secondary amenorrhea can result from several central factors (hypogonadotropic hypoestrogenic anovulation), uterine factors (as with Asherman syndrome, in which the endometrium is sufficiently scarred that no functional endometrium exists), cervical stenosis, premature ovarian failure, and others.

Signs and symptoms

Amenorrhea is a symptom of many disorders; signs and symptoms depend on the specific cause, and include:

• absence of menstruation
• vasomotor flushes, vaginal atrophy, hirsutism (abnormal hairiness), and acne (secondary amenorrhea).

Complications

Complications of amenorrhea include:

• infertility
• endometrial adenocarcinoma (amenorrhea associated with anovulation that gives rise to unopposed estrogen stimulation of the endometrium).

Diagnosis

Diagnosis of amenorrhea is based on:

• history of failure to menstruate in a female over age 16 years, if consistent with bone age (confirms primary amenorrhea)
• absence of menstruation for 3 months in a previously established menstrual pattern (secondary amenorrhea)
• physical and pelvic examination and sensitive pregnancy test ruling out pregnancy, as well as anatomic abnormalities (such as cervical stenosis) that may cause false amenorrhea (cryptomenorrhea), in which menstruation occurs without external bleeding
• onset of menstruation (spotting) within 1 week after giving pure progestational agents such as medroxyprogesterone (Provera), indicating enough estrogen to stimulate the lining of the uterus (if menstruation doesn't occur, special diagnostic studies such as gonadotropin levels are indicated)
• blood and urine studies showing hormonal imbalances, such as lack of ovarian response to gonadotropins (elevated pituitary gonadotropin levels), failure of gonadotropin secretion (low pituitary gonadotropin levels), and abnormal thyroid levels (without suspicion of premature ovarian failure or central hypogonadotropism, gonadotropin levels aren't clinically meaningful because they're released in a pulsatile fashion; at a given time of day, levels may be elevated, low, or average)
• complete medical workup, including appropriate X-rays, laparoscopy, and a biopsy, to identify ovarian, adrenal, and pituitary tumors.

Tests to identify dominant or missing hormones include:

• “ferning” of cervical mucus on microscopic examination (an estrogen effect)
• vaginal cytologic examination
• endometrial biopsy
• serum progesterone level
• serum androgen levels
• elevated urinary 17-ketosteroid levels with excessive androgen secretions
• plasma FSH level more than 50 IU/L, depending on the laboratory (suggests primary ovarian failure); or normal or low FSH level (possible hypothalamic or pituitary abnormality, depending on the clinical situation).

Treatment

Treatment of amenorrhea may include:

• appropriate hormone replacement to reestablish menstruation
• treatment of the cause of amenorrhea not related to hormone deficiency (for example, surgery for amenorrhea due to a tumor)
• inducing ovulation; with intact pituitary gland, clomiphene citrate (Clomid) may induce ovulation in women with secondary amenorrhea due to gonadotropin deficiency, polycystic ovarian disease, or excessive weight loss or gain if it's reversed
• FSH and human menopausal gonadotropins (Pergonal) for women with pituitary disease
• providing reassurance and emotional support (psychiatric counseling if amenorrhea results from emotional disturbances)
• teaching the patient how to keep an accurate record of her menstrual cycles to aid in early detection of recurrent amenorrhea (after treatment).

Benign prostatic hyperplasia

Although most men over age 50 years have some prostatic enlargement, in benign prostatic hyperplasia (BPH, also known as benign prostatic hypertrophy), the prostate gland enlarges enough to compress the urethra and cause overt urinary obstruction. Depending on the size of the enlarged prostate, the age and health of the patient, and the extent of obstruction, BPH is treated symptomatically or surgically.

AGE ALERT BPH is common, affecting up to 50% of men over age 50 years and 75% of men over age 80 years.

Causes

The main cause of BPH may be age-associated changes in hormone activity. Androgenic hormone production decreases with age, causing imbalance in androgen and estrogen levels and high levels of dihydrotestosterone, the main prostatic intracellular androgen.

Other causes include:

• arteriosclerosis
• inflammation
• metabolic or nutritional disturbances.

Pathophysiology

Regardless of the cause, BPH begins with nonmalignant changes in periurethral glandular tissue. The growth of the fibroadenomatous nodules (masses of fibrous glandular tissue) progresses to compress the remaining normal gland (nodular hyperplasia). The hyperplastic tissue is mostly glandular, with some fibrous stroma and smooth muscle. As the prostate enlarges, it may extend into the bladder and obstruct urinary outflow by compressing or distorting the prostatic urethra. There are periodic increases in sympathetic stimulation of the smooth muscle of the prostatic urethra and bladder neck. Progressive bladder distention may also cause a pouch to form in the bladder that retains urine when the rest of the bladder empties. This retained urine may lead to calculus formation or cystitis.

Signs and symptoms

Clinical features of BPH depend on the extent of prostatic enlargement and the lobes affected. Characteristically, the condition starts with a group of symptoms known as prostatism, which include:

• reduced urinary stream caliber and force
• urinary hesitancy
• difficulty starting micturition (resulting in straining, feeling of incomplete voiding, and an interrupted stream).

As the obstruction increases, it causes:

• frequent urination with nocturia
• sense of urgency
• dribbling
• urine retention
• incontinence
• possible hematuria.

Complications

As BPH worsens, a common complication is complete urinary obstruction after infection or while using decongestants, tranquilizers, alcohol, antidepressants, or anticholinergics.

Other complications include:

• infection
• renal insufficiency and, if untreated, renal failure
• urinary calculi
• hemorrhage
• shock.

Diagnosis

Diagnosis includes physical examination showing:

• visible midline mass above the symphysis pubis (sign of an incompletely emptied bladder)
• enlarged prostate with rectal palpation.

Clinical features and a rectal examination are usually sufficient for diagnosis. Other findings that help confirm BPH may include:

• excretory urography to rule out urinary tract obstruction, hydronephrosis (distention of the renal pelvis and calices due to obstruction of the ureter and consequent retention of urine), calculi or tumors, and filling and emptying defects in the bladder
• alternatively, if patient is not cooperative, cystoscopy to rule out other causes of urinary tract obstruction (neoplasm, stones)
• elevated blood urea nitrogen and serum creatinine levels (suggest renal dysfunction)
• elevated prostate-specific antigen (PSA) (prostatic carcinoma must be ruled out)
• urinalysis and urine cultures showing hematuria, pyuria, and, with bacterial count more than 100,000/μl, urinary tract infection (UTI)
• cystourethroscopy for severe symptoms (definitive diagnosis) showing prostate enlargement, bladder wall changes, and a raised bladder (only done immediately before surgery to help determine the best procedure).

Treatment

Conservative therapy includes:

• prostate massages
• sitz baths
• fluid restriction for bladder distention
• antimicrobials for infection
• regular ejaculation to help relieve prostatic congestion
• alpha-adrenergic blockers, such as terazosin (Hytrin) and prazosin (Minipress), to improve urine flow rates to relieve bladder outlet obstruction by preventing contractions of the prostatic capsule and bladder neck
• finasteride (Proscar) to possibly reduce the size of the prostate in some patients
• continuous drainage with an indwelling urinary catheter to alleviate urine retention (high-risk patients).

Surgery is the only effective therapy to relieve acute urine retention, hydronephrosis, severe hematuria, recurrent UTIs, and other intolerable symptoms. The following procedures involve open surgical removal:

• transurethral resection (if prostate weighs less than 2 oz [56.7 g]); tissue removed with a wire loop and electric current using a resectoscope
• suprapubic (transvesical) resection (most common and useful for prostatic enlargement remaining within the bladder)
• retropubic (extravesical) resection allowing direct visualization (potency and continence usually maintained)
• monitoring and recording the patient's vital signs, intake and output, and daily weight; watching closely for signs of postobstructive diuresis (such as increased urine output and hypotension) that may lead to serious dehydration, reduced blood volume, shock, electrolyte loss, and anuria
• indwelling urinary catheter for urine retention (usually difficult in a patient with BPH)
• suprapubic cystostomy under local anesthetic if indwelling urinary catheter can't be passed transurethrally (watching for rapid bladder decompression)
• balloon dilation of the urethra and prostatic stents to maintain urethral patency (occasionally)
• laser excision to relieve prostatic enlargement
• nerve-sparing surgical techniques to reduce common complications such as erectile dysfunction.

After prostatic surgery, interventions may include:

• maintaining patient comfort; watching for and preventing postoperative complications; observing for immediate dangers of prostatic bleeding (shock, hemorrhage); checking the catheter often (every 15 minutes for the first 2 to 3 hours) for patency and urine color; checking dressings for bleeding
• three-way catheter with continuous bladder irrigation (inserted postoperatively by many urologists); involves keeping the catheter open at a rate sufficient to maintain clear, light-pink returns; watching for fluid overload from absorption of the irrigating fluid into systemic circulation; observing an indwelling regular catheter closely (if used); irrigating a catheter with stopped drainage due to clots with 80 to 100 ml of normal saline solution, as ordered, maintaining strict aseptic technique
• watching for septic shock (most serious complication of prostatic surgery); immediately reporting severe chills, sudden fever, tachycardia, hypotension, or other signs of shock; starting rapid infusion of I.V. antibiotics, as ordered; watching for pulmonary embolus, heart failure, and renal shutdown; monitoring vital signs, central venous pressure, and arterial pressure continuously (supportive care in the intensive care unit may be needed)
• belladonna and opium suppositories or other anticholinergics, as ordered, to relieve painful bladder spasms that often occur after transurethral resection
• after an open procedure, patient comfort measures, such as providing suppositories (except after perineal prostatectomy), analgesic medication to control incisional pain, and frequent dressing changes
• I.V. fluids until the patient can drink sufficient fluids (2 to 3 L/day) to maintain adequate hydration
• stool softeners and laxatives, as ordered, to prevent straining (don't check for fecal impaction because a rectal examination may precipitate bleeding)
• reassuring patient that temporary frequency, dribbling, and occasional hematuria will likely occur after the catheter is removed
• reinforcing prescribed limits on activity, such as lifting, strenuous exercise, and long automobile rides that increase bleeding tendency; cautioning patient to restrict sexual activity for several weeks after discharge
• instructing the patient about the prescribed oral antibiotic drug regimen and indications for using gentle laxatives; urging him to seek medical care immediately if he can't void, passes bloody urine, or develops a fever
• encouraging annual digital rectal exams and screening for PSA to identify a possible malignancy.

Cryptorchidism

Cryptorchidism is a congenital disorder in which one or both testes fail to descend into the scrotum, remaining in the abdomen or inguinal canal or at the external ring. Although this condition may be bilateral, it more commonly affects the right testis. True undescended testes remain along the path of normal descent, while ectopic testis deviate from that path.

Cryptorchidism occurs in 30% of premature male newborns but in only 3% of those born at term. In about 80% of affected infants, the testes descend spontaneously during the first year; in the rest, the testes may descend later. If indicated, surgical therapy is successful in up to 95% of the cases if the infant is treated early enough.

Causes

The mechanism by which the testes descend into the scrotum is still unexplained. Possible causes of cryptorchidism include:

• hormonal factors, most likely androgenic hormones from the placenta, maternal or fetal adrenals, or the immature fetal testis and possibly maternal progesterone or gonadotropic hormones from the maternal pituitary
• testosterone deficiency resulting in a defect in the hypothalamic-pituitary-gonadal axis, causing failure of gonadal differentiation and gonadal descent
• structural factors impeding gonadal descent, such as ectopic location of the testis or short spermatic cord
• genetic predisposition in a small number of cases (greater incidence of cryptorchidism in infants with neural tube defects)
• premature newborns most commonly affected due to normal descent of testes into the scrotum during the seventh month of gestation.

Pathophysiology

A prevalent but still unsubstantiated theory links undescended testes to the development of the gubernaculum, a fibromuscular band that connects the testes to the scrotal floor. Normally in the male fetus, testosterone stimulates the formation of the gubernaculum. This band probably helps pull the testes into the scrotum by shortening as the fetus grows. Thus, cryptorchidism may result from inadequate testosterone levels or a defect in the testes or the gubernaculum. Because the testis is maintained at a higher temperature, spermatogenesis is impaired, leading to reduced fertility.

Signs and symptoms

Possible signs and symptoms of cryptorchidism include:

• testis on the affected side not palpable in the scrotum; underdeveloped scrotum (unilateral cryptorchidism)
• scrotum enlarged on the unaffected side due to compensatory hypertrophy (occasionally)
• infertility after puberty due to prevention of spermatogenesis (uncorrected bilateral cryptorchidism) despite normal testosterone levels.

Complications

Bilateral cryptorchidism that is untreated into adolescence may result in:

• sterility because of testicular temperature higher than optimal for spermatogenesis
• significantly increased risk for testicular cancer because the higher tempertures can cause abnormal division of germ cells.
• increased vulnerability of the testes to trauma.

Diagnosis

Physical examination confirms after sex is determined by the following laboratory tests:

• buccal smear (cells from oral mucosa) to determine genetic sex ( a male sex chromatin pattern)
• serum gonadotropin to confirm the presence of testes by showing presence of circulating hormone.

Treatment

If the testes don't descend spontaneously by age 1 year, surgical correction is generally indicated. Surgery should be performed by age 2 years, because by this time about 40% of undescended testes can no longer produce viable sperm. Treatment includes:

• orchiopexy to secure the testes in the scrotum and to prevent sterility, excessive trauma from abnormal positioning, and harmful psychological effects (usually before age 4 years; optimum age, 1 to 2 years)
• human chorionic gonadotropin I.M. to stimulate descent (rarely); ineffective for testes located in the abdomen
• providing information on causes, available treatments, and effect on reproduction; emphasizing that testes may descend spontaneously (especially in premature infants).

After orchiopexy, treatment includes:

• monitoring vital signs, intake, and output; checking dressings; encouraging coughing and deep breathing; watching for urine retention
• keeping the operative site clean, telling the child to wipe from front to back after defecating
• maintaining tension on rubber band applied to keep the testis in place but checking that it isn't too tight
• encouraging parents to participate in postoperative care, such as bathing or feeding the child; urging the child to do as much for himself as possible.

Dysmenorrhea

Dysmenorrhea is painful menstruation associated with ovulation that isn't related to pelvic disease. It's the most common gynecologic complaint and a leading cause of absenteeism from school (affecting 10% of high school girls each month) and work (estimated 140 million work hours lost annually).

AGE ALERT The incidence peaks in women in their early twenties and then slowly decreases.

Dysmenorrhea can occur as a primary disorder or secondary to an underlying disease. Because primary dysmenorrhea is self-limiting, the prognosis is generally good. The prognosis for secondary dysmenorrhea depends on the underlying disorder.

Causes

Although primary dysmenorrhea is unrelated to an identifiable cause, possible contributing factors include:

• hormonal imbalance
• psychogenic factors.

Dysmenorrhea may also be secondary to such gynecologic disorders as:

• endometriosis
• cervical stenosis
• uterine leiomyomas (benign fibroid tumors)
• pelvic inflammatory disease
• pelvic tumors.

Pathophysiology

The pain of dysmenorrhea probably results from increased prostaglandin secretion in menstrual blood, which intensifies normal uterine contractions. Prostaglandins intensify myometrial smooth muscle contraction and uterine blood vessel constriction, thereby worsening the uterine hypoxia normally associated with menstruation. This combination of intense muscle contractions and hypoxia causes the intense pain of dysmenorrhea. Prostaglandins and their metabolites can also cause GI disturbances, headache, and syncope.

Because dysmenorrhea almost always follows an ovulatory cycle, both the primary and secondary forms are rare during the anovulatory cycle of menses. After age 20 years, dysmenorrhea is generally secondary.

Signs and symptoms

Possible signs and symptoms of dysmenorrhea include sharp, intermittent, cramping, lower abdominal pain, usually radiating to the back, thighs, groin, and vulva, and typically starting with or immediately before menstrual flow and peaking within 24 hours.

Dysmenorrhea may also be associated with signs and symptoms suggestive of premenstrual syndrome, including:

• urinary frequency
• nausea
• vomiting
• diarrhea
• headache
• backache
• chills
• abdominal bloating
• painful breasts
• depression
• irritability.

Complications

A possible but rare complication is dehydration due to nausea, vomiting, and diarrhea.

Diagnosis

Diagnosis of dysmenorrhea may include:

• pelvic examination and a detailed patient history to help suggest the cause
• ruling out secondary causes for menses painful since menarche (primary dysmenorrhea)
• tests such as laparoscopy, D&C, hysteroscopy, and pelvic ultrasound to diagnose underlying disorders in secondary dysmenorrhea.

Treatment

Initial treatment aims to relieve pain and may include:

• analgesics such as nonsteroidal anti-inflammatory drugs for mild to moderate pain (most effective when taken 24 to 48 hours before onset of menses), especially effective due to inhibition of prostaglandin synthesis through inhibition of the enzyme cyclooxygenase
• narcotics for severe pain (infrequently used)
• heat applied locally to the lower abdomen (may relieve discomfort in mature women), used cautiously in young adolescents because appendicitis may mimic dysmenorrhea.

For primary dysmenorrhea:

• sex steroids (effective alternative to treatment with antiprostaglandins or analgesics), such as oral contraceptives to relieve pain by suppressing ovulation and inhibiting endometrial prostaglandin synthesis (patients attempting pregnancy should rely on antiprostaglandin therapy)
• psychological evaluation and appropriate counseling due to possible psychogenic cause of persistently severe dysmenorrhea.

Treatment of secondary dysmenorrhea is designed to identify and correct the underlying cause and may include surgical treatment of underlying disorders, such as endometriosis or uterine leiomyomas (after conservative therapy fails).

Effective management of the patient with dysmenorrhea focuses on relief of symptoms, emotional support, and appropriate patient teaching, especially for the adolescent, and includes:

• complete history focusing on the patient's gynecologic complaints, including detailed information on symptoms of pelvic disease, such as excessive bleeding, changes in bleeding pattern, vaginal discharge, and dyspareunia (painful intercourse)
• patient teaching, including explanation of normal female anatomy and physiology as well as the nature of dysmenorrhea (depending on circumstances, providing the adolescent patient with information on pregnancy and contraception)
• encouraging the patient to keep a detailed record of her menstrual symptoms and to seek medical care if symptoms persist.

Endometriosis

Endometriosis is the presence of endometrial tissue outside the lining of the uterine cavity. Ectopic tissue is generally confined to the pelvic area, usually around the ovaries, uterovesical peritoneum, uterosacral ligaments, and cul de sac, but it can appear anywhere in the body.

Active endometriosis may occur at any age, including adolescence. As many as 50% of infertile women may have endometriosis, although the true incidence in both fertile and infertile women remains unknown.

Severe symptoms of endometriosis may have an abrupt onset or may develop over many years. Thirty percent to 40% of women with endometriosis become infertile. Endometriosis usually manifests during the menstrual years; after menopause, it tends to subside. Hormonal treatment of endometriosis (continuous use of oral contraceptives, danazol [Danocrine], and gonadotropin-releasing hormone [GnRH] antagonists) is potentially effective in relieving discomfort, although treatment for advanced stages of endometriosis is usually not as successful because of impaired follicular development. However, nonsurgical treatment of endometriosis generally remains inadequate. Surgery appears to be the more effective way to enhance fertility, although definitive class I evidence doesn't currently exist. Pharmacologic and surgical treatment of endometriosis may be beneficial for managing chronic pelvic pain.

Causes

The cause of endometriosis remains unknown. The main theories to explain this disorder (one or more are perhaps true for certain populations of women) are:

• retrograde menstruation with implantation at ectopic sites (retrograde menstruation alone may not be sufficient for endometriosis to occur because it occurs in women with no clinical evidence of endometriosis)
• genetic predisposition and depressed immune system (may predispose to endometriosis)
• coelomic metaplasia (repeated inflammation inducing metaplasia of mesothelial cells to the endometrial epithelium)
• lymphatic or hematogenous spread (extraperitoneal disease).

Pathophysiology

The ectopic endometrial tissue responds to normal stimulation in the same way as the endometrium, but more unpredictably. The endometrial cells respond to estrogen and progesterone with proliferation and secretion. During menstruation, the ectopic tissue bleeds, which causes inflammation of the surrounding tissues. This inflammation causes fibrosis, leading to adhesions that produce pain and infertility.

Signs and symptoms

Signs and symptoms of endometriosis include:

• dysmenorrhea, abnormal uterine bleeding, and infertility (classic symptoms)
• pain that begins 5 to 7 days before menses peaks and lasts for 2 to 3 days (varies between patients); severity of pain not indicative of extent of disease.

Other signs and symptoms depend on the location of the ectopic tissue and may include:

• infertility and profuse menses (ovaries and oviducts)
• deep-thrust dyspareunia (ovaries or cul de sac)
• suprapubic pain, dysuria, and hematuria (bladder)
• abdominal cramps, pain on defecation, constipation; bloody stools due to bleeding of ectopic endometrium in the rectosigmoid musculature (large bowel and appendix)
• bleeding from endometrial deposits in these areas during menses; pain on intercourse (cervix, vagina, and perineum).

Complications

Complications of endometriosis include:

• infertility due to fibrosis, scarring, and adhesions (major complication)
• chronic pelvic pain
• ovarian carcinoma (rare).

Diagnosis

The only definitive way to diagnose endometriosis is through laparoscopy or laparotomy. Pelvic examination may suggest endometriosis or be unremarkable. Findings suggestive of endometriosis include:

• multiple tender nodules on uterosacral ligaments or in the rectovaginal septum (in one-third of the patients)
• ovarian enlargement in the presence of endometrial cysts on the ovaries.

Although laparoscopy is recommended to diagnose and determine the extent of disease, some clinicians recommend:

• empiric trial of GnRH agonist therapy to confirm or refute the impression of endometriosis before resorting to laparoscopy (controversial, but may be cost-effective)
• biopsy at the time of laparoscopy (helpful to confirm the diagnosis), although in some instances, diagnosis is confirmed by visual inspection.

Treatment

Treatment of endometriosis varies according to the stage of the disease and the patient's age and desire to have children. Conservative therapy for young women who want to have children includes:

• androgens such as danazol (Danocrine)
• progestins and continuous combined oral contraceptives (pseudopregnancy regimen) to relieve symptoms by causing a regression of endometrial tissue
• GnRH agonists to induce pseudomenopause (medical oophorectomy), causing remission of the disease (commonly used).

No pharmacologic treatment has been shown to cure the disease or be effective in all women. Some disadvantages of nonsurgical therapy include:

• adverse reaction to drug-induced menopause (including osteoporosis if used for more than 6 months), high expense of use for an extended duration, and possible recurrence of endometriosis after discontinuation of GnRH agonists
• high expense and weight gain when using danazol (Danocrine)
• lowest fertility rates of any medical treatment for endometriosis when using continuous oral contraceptive pills
• weight gain and depressive symptoms when using progestin (but as effective as GnRH antagonists).

When ovarian masses are present, surgery must rule out cancer. Conservative surgery includes:

• laparoscopic removal of endometrial implants with conventional or laser techniques (no benefit shown for laser laparoscopy over electrocautery or suture methods)
• presacral neurectomy or laparoscopic uterosacral nerve ablation (LUNA) for central pelvic pain; effective in about 50% or less of appropriate candidates (clinical studies of both presacral neurectomy and LUNA use different surgical techniques, degrees of resection, and definitions of success)
• advising a patient who wants children not to postpone childbearing, as she may become infertile (pregnancy may temporarily improve endometriosis�associated chronic pelvic pain)
• total abdominal hysterectomy with or without bilateral salpingo-oophorectomy; success rates vary; unclear whether ovarian conservation is appropriate (treatment of last resort for women who don't want to bear children or for extensive disease)
• annual pelvic examination and Papanicolaou test (all patients).

Erectile dysfunction

Erectile dysfunction, or impotence, refers to a male's inability to attain or maintain penile erection sufficient to complete intercourse. The patient with primary impotence has never achieved a sufficient erection. Secondary impotence is more common but no less disturbing than the primary form, and implies that the patient has succeeded in completing intercourse in the past.

Transient periods of impotence aren't considered dysfunction and probably occur in half of adult males.

AGE ALERT Erectile disorder affects all age groups but increases in frequency with age.

The prognosis for erectile dysfunction patients depends on the severity and duration of their impotence and the underlying causes.

Causes

Causes of erectile dysfunction include psychogenic factors (50% to 60% of cases), organic causes, or both psychogenic and organic factors in some patients. This complexity makes the isolation of the primary cause difficult.

Psychogenic causes of erectile dysfunction include:

• intrapersonal psychogenic causes reflecting personal sexual anxieties and generally involving guilt, fear, depression, or feelings of inadequacy resulting from previous traumatic sexual experience, rejection by parents or peers, exaggerated religious orthodoxy, abnormal mother-son intimacy, or homosexual experiences
• psychogenic factors reflecting a disturbed sexual relationship, possibly stemming from differences in sexual preferences between partners, lack of communication, insufficient knowledge of sexual function, or nonsexual personal conflicts
• situational impotence, a temporary condition in response to stress.

Organic causes include:

• chronic diseases that cause neurologic and vascular impairment, such as cardiopulmonary disease, diabetes, multiple sclerosis, or renal failure
• liver cirrhosis causing increased circulating estrogen due to reduced hepatic inactivation
• spinal cord trauma
• complications of surgery, particularly radical prostatectomy
• drug- or alcohol-induced dysfunction
• genital anomalies or central nervous system defects (rare).

Pathophysiology

Neurologic dysfunction results in lack of the autonomic signal and, in combination with vascular disease, interferes with arteriolar dilation. The blood is shunted around the sacs of the corpus cavernosum into medium-sized veins, which prevents the sacs from filling completely. Also, perfusion of the corpus cavernosum is initially compromised because of partial obstruction of small arteries, leading to loss of erection before ejaculation.

Psychogenic causes may exacerbate emotional problems in a circular pattern; anxiety causes fear of erectile dysfunction, which causes further emotional problems.

Signs and symptoms

Secondary erectile disorder is classified as:

• partial; inability to achieve a full erection
• intermittent; sometimes potent with the same partner
• selective; potent only with certain partners.

Some men lose erectile function suddenly, and others lose it gradually. If the cause isn't organic, erection may still be achieved through masturbation.

Immediately before a sexual encounter, patients with psychogenic impotence may:

• feel anxious
• perspire
• have palpitations
• lose interest in sexual activity.

Complications

A complication of erectile dysfunction is severe depression (patients with psychogenic or organic drug-induced erectile dysfunction), causing the impotence or resulting from it.

Diagnosis

A detailed sexual history helps differentiate between organic and psychogenic factors and primary and secondary impotence. Questions should include:

• Does the patient have intermittent, selective nocturnal or early morning erections?
• Can he achieve erections through other sexual activity?
• When did his dysfunction begin, and what was his life situation at that time?
• Did erectile problems occur suddenly or gradually?
• What prescription or nonprescription drugs is he taking?

Diagnosis also includes:

• ruling out such chronic diseases as diabetes and other vascular, neurologic, or urogenital problems
• fulfilling the diagnostic criteria for a DSM-IV diagnosis (when the disorder causes marked distress or interpersonal difficulty).

Treatment

Treatment for psychogenic impotence includes:

• sex therapy including both partners (course and content of therapy depend on the specific cause of dysfunction and nature of the partner relationship)
• teaching or helping the patient to improve verbal communication skills, eliminate unreasonable guilt, or reevaluate attitudes toward sex and sexual roles.

Treatment for organic impotence includes:

• reversing the cause, if possible
• psychological counseling to help the couple deal realistically with their situation and explore alternatives for sexual expression if reversing the cause is not possible
• sildenafil citrate (Viagra) to cause vasodilatation within the penis (may effectively manage erectile dysfunction in appropriate patients)
• adrenergic antagonist, yohimbine, to enhance parasympathetic neurotransmission
• testosterone supplementation for hypogonadal men (not given to men with prostate cancer)
• prostaglandin E injected directly into the corpus cavernosum (may induce an erection for 30 to 60 minutes in some men)
• surgically inserted inflatable or noninflatable penile implants (some patients with organic impotence); patient should be instructed to avoid intercourse until the incision heals (usually in 6 weeks) after penile implant surgery.

Measures to help prevent impotence include providing information on resuming sexual activity as part of discharge instructions for a patient with a condition that requires modification of daily activities, including those with cardiac disease, diabetes, hypertension, and chronic obstructive pulmonary disease, and all postoperative patients.

Fibrocystic change of the breast

Also incorrectly known as fibrocystic disease of the breast, this is a disorder of benign breast tissue alterations. The condition is usually bilateral.

AGE ALERT Fibrocystic change is the most common benign breast disorder, affecting an estimated 10% of women ages 21 years and younger, 25% of women ages 22 years and older, and 50% of postmenopausal women.

Although most lesions are benign, some may proliferate and show atypical cellular growth. Fibrocystic change by itself is not a precursor to breast cancer, but if atypical cells are present, the risk for breast carcinoma increases.

Causes

The precise cause is unknown. Some theories include:

• imbalance between estrogen (excess) and progesterone (deficiency) during the luteal phase of the menstrual cycle
• altered prolactin levels
• enzymatic alteration of breast tissue caused by methylxanthines (found in caffeinated food and drinks), tyramine (found in cheese, wine, and nuts), and tobacco, which inhibit cyclic guanosine monophosphate enzymes.

Pathophysiology

Changes in the breast tissue appear to respond to hormonal stimulation, although the exact mechanism is unknown.

The first type of breast tissue alteration is cyst formation. Cysts may form within lobular or subareolar areas. Cysts are classified as microcysts (smaller than 1 mm) and grow to macrocysts (3 mm or larger).

Ductal epithelial proliferation (hyperplasia) is a condition in which dilations of the ductal system occur below the areola and the nipple. The ductal epithelium may undergo metaplastic changes. Fibrotic areas may form as a result of inflammation caused by either the cysts or ductal hyperplasia.

Signs and symptoms

Signs and symptoms of fibrocystic change of the breast include:

• breast pain due to inflammation and nerve root stimulation (most common symptom), beginning 4 to 7 days into the luteal phase of the menstrual cycle and continuing until the onset of menstruation
• pain in the upper outer quadrant of both breasts (common site)
• palpable lumps that increase in size premenstrually and are freely moveable (about 50% of all menstruating women)
• granular feeling of breasts on palpation
• occasional greenish-brown to black nipple discharge that contains fat, proteins, ductal cells, and erythrocytes (ductal hyperplasia).

Complications

A possible complication is the greater risk for developing breast cancer in women with proliferative lesions that have atypical cells and who also have a family history of malignancy.

Diagnosis

Diagnosis of fibrocystic change of the breast includes:

• ultrasound to distinguish cystic (fluid-filled) from solid masses
• tissue biopsy to distinguish benign from malignant changes.

Treatment

Treatment is often symptomatic and may include:

• diet low in caffeine and fat and high in fruits and vegetables to help alleviate pain
• support bra to reduce pain
• draining painful cysts under local anesthesia (if the aspirated fluid is bloody, it should be sent for cytologic analysis to rule out possible malignancy)
• synthetic androgens (danazol [Danocrine]) for severe pain (occasionally).

Fibroid disease of the uterus

Uterine leiomyomas, the most common benign tumors in women, are also known as myomas, fibromyomas, or fibroids. They're tumors composed of smooth muscle and fibrous connective tissue that usually occur in the uterine corpus, although they may appear on the cervix or on the round or broad ligament.

CULTURAL DIVERSITY Uterine leiomyomas occur in 20% to 25% of women of reproductive age and may affect three times as many blacks as whites, although the true incidence in either population is unknown.

The tumors become malignant (leiomyosarcoma) in less than 0.1% of patients, which should serve to comfort women concerned with the possibility of a uterine malignancy in association with a fibroid.

Causes

The cause of uterine leiomyomas is unknown, but some factors implicated as regulators of leiomyoma growth include:

• several growth factors, including epidermal growth factor
• steroid hormones, including estrogen and progesterone (leiomyomas typically arise after menarche and regress after menopause, implicating estrogen as a promoter of leiomyoma growth).

Pathophysiology

Leiomyomas are classified according to location. They may be located within the uterine wall (intramural) or protrude into the endometrial cavity (submucous) or from the serosal surface of the uterus (subserous). Their size varies greatly. They're usually firm and surrounded by a pseudocapsule composed of compressed but otherwise normal uterine myometrium. The uterine cavity may become larger, increasing the endometrial surface area. This can cause increased uterine bleeding.

Signs and symptoms

Most leiomyomas are asymptomatic. Signs and symptoms of leiomyoma include:

• abnormal bleeding, typically menorrhagia with disrupted submucosal vessels (most common symptom)
• pain only associated with torsion of a pedunculated (stemmed) subserous tumor or leiomyomas undergoing degeneration (fibroid outgrows its blood supply and shrinks down in size; can be artificially induced through myolysis, a laparoscopic procedure to shrink fibroids, or uterine artery embolization)
• pelvic pressure and impingement on adjacent viscera (indications for treatment, depending on severity) resulting in mild hydronephrosis (not believed to be an indication for treatment because renal failure rarely, if ever, results).

Complications

Various disorders have been attributed to uterine leiomyomas, including:

• recurrent spontaneous abortion
• preterm labor
• malposition of the fetus
• anemia secondary to excessive bleeding
• bladder compression
• infection (if tumor protrudes out of the vaginal opening)
• secondary infertility (rare).

Diagnosis

Diagnosis of leiomyoma may be based on:

• clinical findings (enlarged uterus) and patient history suggesting uterine leiomyomas
• blood studies showing anemia from abnormal bleeding (may support the diagnosis)
• bimanual examination showing enlarged, firm, nontender, and irregularly contoured uterus (also seen with adenomyosis and other pelvic abnormalities)
• ultrasound for accurate assessment of the dimension, number, and location of tumors
• magnetic resonance imaging (especially sensitive with regard to fibroid imaging).

Other diagnostic procedures include:

• hysterosalpingography
• hysteroscopy
• endometrial biopsy (to rule out endometrial cancer in patients over age 35 years with abnormal uterine bleeding)
• laparoscopy.

Treatment

Treatment depends on the severity of symptoms, size and location of the tumors, and the patient's age, parity, pregnancy status, desire to have children, and general health.

Treatment options include nonsurgical as well as surgical procedures. Pharmacologic treatment generally isn't effective in the long term for fibroids. Although often prescribed by gynecologists, progestational agents are ineffective as primary treatment for fibroids.

Besides observation, nonsurgical methods include:

• gonadotropin-releasing hormone (GnRH) agonists to rapidly suppress pituitary gonadotropin release, which leads to profound hypoestrogenemia, a 50% reduction in uterine volume (peak effects occurring in the 12th week of therapy), and consequent benefit of reductions in tumor size before surgery and blood loss during surgery, and an increase in preoperative hematocrit (This treatment is not a cure, as tumors increase in size after cessation of therapy. [Increases in tumor size during therapy can indicate uterine sarcoma.] The treatment is best used preoperatively or for up to 6 months in a perimenopausal woman who might soon experience a natural menopause and thus avoid surgery.)
• nonsteroidal anti-inflammatory drugs for dysmenorrhea or pelvic discomfort.

Surgical procedures include:

• abdominal, laparoscopic, or hysteroscopic myomectomy (removal of tumors in the uterine muscle) for patients of any age who want to preserve their uterus
• myolysis (a laparoscopic procedure to treat fibroids without hysterectomy or major surgery, performed on an outpatient basis) to coagulate the fibroids and preserve the uterus and childbearing potential
• uterine artery embolization (radiologic procedure) to block uterine arteries using small pieces of polyvinyl chloride (This is a promising alternative to surgery in many women, but no existing long-term studies confirm if this procedure is appropriate in women desiring future childbearing or establish long-term success or side effects. Recent anecdotal data suggest decreased time to menopause after embolization.)
• hysterectomy (Though this is the definitive treatment for symptomatic women who have completed childbearing, it is critical to inform women of all their choices because hysterectomy usually isn't the only available option.)
• blood transfusions (with severe anemia due to excessive bleeding).

Prior to undergoing surgery, patients should be helped to understand the effects of hysterectomy or oophorectomy, if indicated, on menstruation, menopause, and sexual activity. Patients should also understand that pregnancy is still possible if multiple myomectomy is necessary, though cesarean delivery may be necessary. Extensive scar tissue may rupture during the contractions of vaginal delivery. (Violation of the endometrial cavity is the classic indication for cesarean section in such patients, but it's unclear why a cell layer 1 to 2 cells thick should be protective against uterine dehiscence in subsequent pregnancy.)

Gynecomastia

Gynecomastia is the enlargement of breast tissue in males.

AGE ALERT Gynecomastia is usually bilateral, but in men over age 50 years, it's usually unilateral.

Usually the cause is physiologic. Gynecomastia often resolves spontaneously in 6 to 12 months.

Causes

Excessive estrogen production from conditions including:

• testicular tumors
• obesity
• pituitary tumors
• some hypogonadism syndromes

Systemic disorders associated with gynecomastia that may alter the estrogen-testosterone ratio include:

• liver disease causing inability to break down normal male estrogen secretions
• chronic renal failure
• chronic obstructive lung disease.

Pharmacologic agents that may cause gynecomastia include marijuana and exogenous estrogen, as given for prostatic malignancy.

Pathophysiology

A disturbance in the normal ratio of active androgen to estrogen results in proliferation of the fibroblastic stroma and the duct system of the breast.

Signs and symptoms

Signs and symptoms of gynecomastia include:

• enlarged breast tissue (at least ?" [2 cm] in diameter), either unilateral or bilateral, beneath the areola
• bilateral enlargement (hormone-induced gynecomastia).

Complications

A possible complication of gynecomastia is malignant changes in the breast tissue.

Diagnosis

Diagnosis depends on:

• biopsy to rule out malignancy
• excessively high estrogen levels and normal testosterone levels (in drug- and tumor-induced hyperestrogenism)
• very low testosterone levels and normal estrogen levels (hypergonadism).

Treatment

Gynecomastia usually resolves spontaneously without treatment. If indicated, treatments include:

• treatment of the cause to reduce excess breast tissue
• resection of extra breast tissue for cosmetic reasons.

Hydrocele

A hydrocele is a collection of fluid between the visceral and parietal layers of the tunica vaginalis of the testicle or along the spermatic cord. It's the most common cause of scrotal swelling.

Causes

Possible causes of hydrocele include:

• congenital malformation (infants)
• trauma to the testes or epididymis
• infection of the testes or epididymis
• testicular tumor.

Pathophysiology

Congenital hydrocele occurs because of a patency between the scrotal sac and the peritoneal cavity, allowing peritoneal fluids to collect in the scrotum. The exact mechanism of congenital hydrocele is unknown.

In adults, the fluid accumulation may be caused by infection, trauma, tumor, an imbalance between the secreting and absorptive capacities of scrotal tissue, or an obstruction of lymphatic or venous drainage in the spermatic cord. This leads to a displacement of fluid in the scrotum, outside the testes. Subsequent swelling results, leading to reduced blood flow to the testes.

Signs and symptoms

Possible signs and symptoms of hydrocele include:

• scrotal swelling and feeling of heaviness
• inguinal hernia (often present in congenital hydrocele)
• size from slightly larger than the testes to the size of a grapefruit or larger
• fluid collection with either flaccid or tense mass
• pain with acute epididymal infection or testicular torsion
• scrotal tenderness due to severe swelling.

Complications

Complications may include:

• epididymitis
• testicular atrophy.

Diagnosis

Diagnosis of hydrocele may include:

• transillumination to distinguish fluid-filled from solid mass (a tumor doesn't transilluminate)
• ultrasound to visualize the testes and determine the presence of a tumor
• fluid biopsy to determine the cause and differentiate between normal cells and malignancy.

Treatment

Usually, no treatment of congenital hydrocele is indicated, as this condition frequently resolves spontaneously by age 1 year. Otherwise, possible treatments for hydrocele include:

• surgical repair to avoid strangulation of the bowel (inguinal hernia with bowel present in the sac)
• aspiration of fluid and injection of sclerosing drug into the scrotal sac for a tense hydrocele that impedes blood circulation or causes pain
• excision of the tunica vaginalis for recurrent hydroceles
• suprainguinal excision for testicular tumor detected by ultrasound.

Ovarian cysts

Ovarian cysts are usually nonneoplastic sacs on an ovary that contain fluid or semisolid material. Although these cysts are usually small and produce no symptoms, they may require thorough investigation as possible sites of malignant change. Cysts may be single or multiple (polycystic ovarian disease). Common physiologic ovarian cysts include follicular cysts, theca-lutein cysts, and corpus luteum cysts. Ovarian cysts can develop any time between puberty and menopause, including during pregnancy. The prognosis for nonneoplastic ovarian cysts is excellent. The risk for ovarian malignancy isn't increased with a functional (physiologic) ovarian cyst.

Causes

Possible causes of ovarian cyst are:

• granulosa-lutein cysts, which occur within the corpus luteum, are functional (arising during some variation of the ovulatory process), non-neoplastic enlargements of the ovaries caused by excessive accumulation of blood during the hemorrhagic phase of the menstrual cycle
• Theca-lutein cysts are commonly bilateral and filled with clear, straw-colored liquid; they are often associated with hydatidiform mole, choriocarcinoma, or hormone therapy (with human chorionic gonadotropin [HCG] or clomiphene citrate).

Pathophysiology

Follicular cysts are generally very small and arise from follicles that overdistend, either because they haven't ruptured or have ruptured and resealed before their fluid is reabsorbed. (See Follicular cyst .) Luteal cysts develop if a mature corpus luteum persists abnormally and continues to secrete progesterone. They consist of blood or fluid that accumulates in the cavity of the corpus luteum and are typically more symptomatic than follicular cysts. When such cysts persist into menopause, they secrete excessive amounts of estrogen in response to the hypersecretion of follicle-stimulating hormone and luteinizing hormone that normally occurs during menopause.

Signs and symptoms

Possible signs and symptoms of ovarian cysts are:

• no symptoms (small ovarian cysts such as follicular cysts)
• mild pelvic discomfort, low back pain, dyspareunia, or abnormal uterine bleeding, secondary to a disturbed ovulatory pattern (large or multiple cysts)
• acute abdominal pain similar to that of appendicitis (ovarian cysts with torsion)
• unilateral pelvic discomfort (from granulosa-lutein cysts appearing early in pregnancy and growing as large as 2 to 2?" [5 to 6 cm] in diameter), delayed menses, followed by prolonged or irregular bleeding (granulosa-lutein cysts in nonpregnant women).

Complications

Complications of ovarian cysts may include torsion or rupture causing signs of an acute abdomen (abdominal tenderness, distention, and rigidity) due to massive intraperitoneal hemorrhage or peritonitis.

Diagnosis

Generally, characteristic clinical features suggest ovarian cysts. They're confirmed by visualization of the ovary through ultrasound, laparoscopy, or surgery (often for another condition).

Treatment

Treatment depends on the type of cyst and symptoms and may include:

• no treatment due to tendency of cyst to disappear spontaneously within one to two menstrual cycles; persisting cyst indicates excision to rule out malignancy (follicular cysts)
• hormonal treatment (common, but no proven benefit in ovarian cyst management)
• analgesics to relieve symptoms (functional cysts that occur during pregnancy); cysts usually diminish during the third trimester (rarely needing surgery)
• elimination of the hydatidiform mole, destruction of choriocarcinoma, or discontinuation of HCG or clomiphene citrate (Clomid) therapy (theca-lutein cysts)
• laparoscopy or exploratory laparotomy with possible ovarian cystectomy or oophorectomy for persistent or suspicious ovarian cyst (These may be performed during pregnancy, if necessary. Optimal timing for surgery is the second trimester; laparoscopic management during pregnancy is promising.)
• surgery for ongoing hemorrhage from ruptured corpus luteum cyst. (Otherwise, ruptured ovarian cysts may be treated by draining intraperitoneal fluid through culdocentesis in the emergency room or office setting.)

FOLLICULAR CYST A common type of ovarian cyst, a follicular cyst is usually semitransparent and overdistended, with watery fluid visible through its thin walls. <center></center>

Counseling and support required by surgical cyst patients may include:

• teaching to explain the nature of the cyst; type of discomfort, if any; and how long condition may last
• preoperatively, watching for signs of cyst rupture, such as increasing abdominal pain, distention, and rigidity; monitoring vital signs for fever, tachypnea, or hypotension (possibly indicating peritonitis or intraperitoneal hemorrhage)
• postoperatively, encouraging frequent movement in bed and early ambulation as ordered to prevent pulmonary embolism
• providing emotional support; offering appropriate reassurance if patient fears cancer or infertility
• advising the patient to gradually increase her activities at home over 4 to 6 weeks after surgery.

Precocious puberty

Precocious puberty may occur in males or females. Males begin to mature sexually before age 9 years. This disorder occurs most commonly as true precocious puberty ― early maturation of the hypothalamic-pituitary-gonadal axis, development of secondary sex characteristics, gonadal development, and spermatogenesis ― or as pseudoprecocious puberty, marked by development of secondary sex characteristics without gonadal development. Males with true precocious puberty are reported to have fathered children as early as age 7 years.

In most males with precocious puberty, sexual characteristics develop in essentially normal sequence; these children function normally when they reach adulthood.

In females, precocious puberty is the early onset of pubertal changes, such as breast development, pubic and axillary hair development, and menarche, before the age of 8 years. The occurrence is 5 times more common in females than males. Normally, the mean age for menarche is 13 years. In true precocious puberty, the ovaries mature and pubertal changes progress in an orderly manner.

In pseudoprecocious puberty, pubertal changes occur without corresponding ovarian maturation. (See Precocious puberty .) In many cases, precocious puberty can be reversed.

Polycystic ovarian syndrome

Polycystic ovarian syndrome is a metabolic disorder characterized by multiple ovarian cysts. About 22% of the women in the United States have the disorder, and obesity is present in 50% to 80% of these women. Among those who seek treatment for infertility, more than 75% have some degree of polycystic ovarian syndrome, usually manifested by anovulation alone. Prognosis is very good for ovulation and fertility with appropriate treatment.

Causes

The precise cause of polycystic ovarian syndrome is unknown. Theories include:

• abnormal enzyme activity triggering excess androgen secretion from the ovaries and adrenal glands
• endocrine abnormalities causing the full spectrum of polycystic ovarian disease; amenorrhea, polycystic ovaries on ultrasound, hyperandrogenism (part of the Stein-Leventhal syndrome).

Pathophysiology

A general feature of all anovulation syndromes is a lack of pulsatile release of gonadotropin-releasing hormone. Initial ovarian follicle development is normal. Many small follicles begin to accumulate because there's no selection of a dominant follicle. These follicles may respond abnormally to the hormonal stimulation, causing an abnormal pattern of estrogen secretion during the menstrual cycle. Endocrine abnormalities may be the cause of polycystic ovarian syndrome or cystic abnormalities; muscle and adipose tissue are resistant to the effects of insulin, and lipid metabolism is abnormal.

Signs and symptoms

Signs and symptoms of classic polycystic ovarian syndrome (Stein-Leventhal syndrome) include:

• mild pelvic discomfort
• low back pain
• dyspareunia
• abnormal uterine bleeding secondary to disturbed ovulatory pattern
• hirsutism
• acne
• male-pattern hair loss.

Complications

Possible complications of polycystic ovarian syndrome include:

• malignancy due to sustained estrogenic stimulation of the endometrium
• increased risk for cardiovascular disease and type 2 diabetes mellitus due to insulin resistance.

Polycystic ovarian disease may produce:

• secondary amenorrhea
• oligomenorrhea
• infertility.

Diagnosis

Diagnosis of polycystic ovarian syndrome includes:

• history and physical examination showing bilaterally enlarged polycystic ovaries and menstrual disturbance, usually dating back to menarche
• visualization of the ovary through ultrasound, laparoscopy, or surgery, often for another condition (may confirm ovarian cysts)
• slightly elevated urinary 17-ketosteroid levels and anovulation (shown by basal body temperature graphs and endometrial biopsy)
• elevated ratio of luteinizing hormone to follicle-stimulating hormone (usually 3:1 or greater), and elevated levels of testosterone, and androstenedione
• unopposed estrogen action during the menstrual cycle due to anovulation
• direct visualization by laparoscopy to rule out paraovarian cysts of the broad ligament, salpingitis, endometriosis, and neoplastic cysts.

Treatment

Treatment of polycystic ovarian syndrome includes monitoring patient's weight to maintain a normal body mass index in order to reduce risks associated with insulin resistance, which may cause spontaneous ovulation in some women.

Treatment of polycystic ovarian disease may include the administration of drugs such as:

• clomiphene citrate (Clomid) to induce ovulation
• medroxyprogesterone acetate (Provera) for 10 days each month for a patient wanting to become pregnant
• low-dose oral contraceptives to treat abnormal bleeding for the patient needing reliable contraception.

Other treatments inlcude:

• preoperatively, watching for signs of cyst rupture, such as increasing abdominal pain, distention, and rigidity; monitoring vital signs for fever, tachypnea, or hypotension (possibly indicating peritonitis or intraperitoneal hemorrhage)
• postoperatively, encouraging frequent movement in bed and early ambulation as ordered to prevent pulmonary embolism
• providing emotional support, offering appropriate reassurance if the patient fears cancer or infertility.

PRECOCIOUS PUBERTY Although precocious puberty occurs in both male and female children, the origins of the disorder are different, so treatment varies.   FEMALE MALE Causes About 85% of the cases of true precocious puberty in females are idiopathic. Other causes of true precocious puberty are pathologic, including central nervous system (CNS) disorders resulting from tumors, trauma, infection, or other lesions, such as: hypothalamic tumors intracranial tumors (pinealoma, granuloma, hamartoma) hydrocephaly degenerative encephalopathy tuberous sclerosis neurofibromatosis encephalitis skull injuries meningitis peptic arachnoiditis. Other conditions associated with precocity include: Albright's syndrome Silver's syndrome juvenile hypothyroidism. Pseudoprecocious puberty may result from: increased levels of sex hormones due to ovarian and adrenocortical tumors adrenal cortical virilizing hyperplasia ingestion of estrogens or androgens increased end-organ sensitivity to low levels of circulating sex hormones, with estrogens promoting premature breast development and androgens promoting premature pubic and axillary hair growth. True precocious puberty may be: idiopathic and genetically transmitted as a dominant trait cerebral (neurogenic). Pseudoprecocious puberty may result from: testicular tumors (hyperplasia, adenoma, or carcinoma) that produce excessive testosterone levels congenital adrenogenital syndrome, producing high levels of adrenocortical steroids. Pathophysiology Idiopathic precocious puberty results from early development and activation of the endocrine glands without corresponding abnormality. Idiopathic precocious puberty results from pituitary or hypothalamic intracranial lesions that cause excessive secretion of gonadotropin. Signs and symptoms Changes that may occur independently or simultaneously before the age of 8 years include: rapid growth spurt thelarche (breast development) pubarche (pubic hair development) menarche. All boys with precocious puberty experience: early bone development, causing an initial growth spurt early muscle development premature closure of the epiphyses, resulting in stunted adult stature adult hair pattern penile growth bilateral enlarged testes. Symptoms of precocity due to cerebral lesions include: nausea vomiting headache visual disturbances internal hydrocephalus. Symptoms of pseudoprecocity caused by testicular tumors include: adult hair patterns acne discrepancy in testis size; with the enlarged testis feeling hard or containing a palpable, isolated nodule. Adrenogenital syndrome produces: adult skin tone excessive hair (including beard) deepened voice stocky and muscular appearance penile, scrotal sac, and prostate enlargement (but not the testes).   FEMALE MALE Complications Development of ovarian or adrenal malignancy. Development of testicular tumors In precocious puberty caused by a brain tumor, the outlook is less encouraging, and may be fatal. Diagnosis Diagnosis requires: complete patient history thorough physical examination special tests to differentiate between true and pseudoprecocious puberty and to indicate the necessary treatment X-rays of the hands, wrists, knees, and hips to determine bone age and possible premature epiphyseal closure ultrasound, laparoscopy, or exploratory laparotomy to verify a suspected abdominal lesion EEG, ventriculography, pneumoencephalography, computed axial tomography scan, or angiography to detect CNS disorders. Other tests detect abnormally high hormonal levels for the patient's age and may include: vaginal smear for estrogen secretion urinary tests for gonadotropic activity and excretion of 17-ketosteroids radioimmunoassay for both luteinizing and follicle-stimulating hormones. Assessing the cause of precocious puberty requires: complete physical examination detailed patient history to evaluate the patient's recent growth pattern, behavior changes, family history of precocious puberty, or ingestion of hormones. In true precocity, laboratory results include: elevated serum levels of luteinizing and follicle-stimulating hormones and corticotropin elevated plasma testosterone levels (equal to those of an adult male) evaluation of ejaculate showing the presence of live spermatozoa brain scan, skull X-rays, and EEG to detect possible CNS tumors skull and hand X-rays showing advanced bone age. In pseudoprecocity, diagnosis includes: chromosomal karyotype analysis showing abnormal pattern of autosomes and sex chromosomes elevated levels of 24-hour urinary 17-ketosteroids and other steroids.   FEMALE MALE Treatments Treatment of constitutional true precocious puberty may include medroxyprogesterone (Provera) to reduce gonadotropin secretion and prevent menstruation. Other therapy depends on the cause of precocious puberty and its stage of development and includes: cortical or adrenocortical steroid replacement for adrenogenital syndrome surgery to remove ovarian and adrenal tumors, resulting in regression of secondary sex characteristics, especially in young children surgery and chemotherapy for choriocarcinomas thyroid extract or levothyroxine to decrease gonadotropic secretions in hypothyroidism discontinuation of medication for drug ingestion no treatment in precocious thelarche and pubarche. The dramatic physical changes produced by precocious puberty can be upsetting and alarming for the child and her family. Interventions include: providing a calm, supportive atmosphere encouraging the patient and family to express their feelings about these changes explaining all diagnostic procedures and telling the patient and family that surgery may be necessary explaining the condition to the child in terms she can understand to prevent feelings of shame and loss of self-esteem providing appropriate sex education, including information on menstruation and related hygiene telling parents that although their daughter seems physically mature, she's not psychologically mature, and the discrepancy between physical appearance and psychological and psychosexual maturation may create problems; warning them against expecting more of her than expected of other children her age suggesting that parents continue to dress their daughter in clothes appropriate for her age that don't call attention to her physical development reassuring parents that precocious puberty doesn't usually precipitate precocious sexual behavior. Boys with idiopathic precocious puberty generally require no medical treatment and have no physical complications in adulthood. Supportive psychological counseling is the most important therapy. Interventions for specific conditions include: reassessing regularly for possible tumors in a child with an initial diagnosis of idiopathic precocious puberty neurosurgery for brain tumors (they commonly resist treatment) removing the affected testis (orchiectomy) for testicular tumors; chemotherapy and lymphatic radiation therapy for malignant tumors (poor prognosis) lifelong therapy with maintenance doses of glucocorticoids (cortisol) to inhibit corticotropin production in adrenogenital syndrome causing precocious puberty. Interventions to help the child undergoing these changes and his family include: emphasizing to parents that the child's social and emotional development should remain consistent with his chronological age, not with his physical development; advising parents not to place unrealistic demands on the child reassuring the child that although his body is changing more rapidly than those of other boys, they'll eventually experience the same changes helping him feel less self-conscious about his changing body; suggesting clothing that de-emphasizes sexual development providing sex education for the child with true precocity explaining adverse effects of medication (cushingoid symptoms) to family if a child must take glucocorticoids for the rest of his life.

Prostatitis

Prostatitis, or inflammation of the prostate gland, may be acute or chronic. It's usually nonbacterial and idiopathic in origin (95%). The nonbacterial form of the disorder is also known as prostatodynia. Acute prostatitis most often results from gram-negative bacteria and is easy to recognize and treat. However, chronic prostatitis, the most common cause of recurrent urinary tract infections (UTIs) in men, is less easy to recognize.

AGE ALERT As many as 35% of men aged over 50 years have chronic prostatitis.

Causes

The cause of nonbacterial prostatitis is unknown. Bacterial prostatitis is caused by:

• Escherichia coli (80% of cases)
• Klebsiella, Enterobacter, Proteus, Pseudomonas, Streptococcus , or Staphylococcus organisms (20% of cases).

These organisms probably spread to the prostate by:

• an ascending urethral infection or through the blood stream
• invasion of rectal bacteria through lymphatics
• reflux of infected bladder urine into prostate ducts
• infrequent or excessive sexual intercourse
• procedures such as cystoscopy or catheterization (less commonly)

AGE ALERT Acute prostatitis is associated with benign prostatic hypertrophy in older men.

• bacterial invasion from the urethra (chronic prostatitis).

Pathophysiology

Spasms in the genitourinary tract or tension in the pelvic floor muscles may cause inflammation and nonbacterial prostatitis.

Bacterial prostatic infections can be the result of a previous or concurrent infection. The bacteria ascend from the infected urethra, bladder, lymphatics, or blood through the prostatic ducts and into the prostate. Infection stimulates an inflammatory response in which the prostate becomes larger, tender, and firm. Inflammation is usually limited to a few of the gland's excretory ducts.

Signs and symptoms

Acute prostatitis begins with:

• chills
• low back pain, especially when standing, due to compression of the prostate gland
• perineal fullness
• suprapubic tenderness
• frequent and urgent urination
• dysuria, nocturia, and urinary obstruction due to blocked urethra by enlarged prostate
• cloudy urine.

Signs of systemic infection include:

• fever
• myalgia
• fatigue
• arthralgia.

Signs and symptoms of chronic bacterial prostatitis may include:

• the same urinary symptoms as the acute form but to a lesser degree
• recurrent symptomatic cystitis.

Other possible signs include:

• evidence of UTI, such as urinary frequency, burning, cloudy urine
• painful ejaculation
• bloody semen
• persistent urethral discharge
• sexual dysfunction.

Complications

Possible complications of prostatitis include:

• UTI (common)
• infected and abscessed testis (removed surgically).

Diagnosis

Diagnosis of prostatitis may include:

• rectal examination finding evidence of acute prostatitis, such as very tender, warm, and enlarged prostate (characteristic)
• rectal examination finding firm, irregularly shaped, and slightly enlarged prostate due to fibrosis (chronic bacterial prostatitis)
• palpation showing normal prostate gland by exclusion (nonbacterial prostatitis)
• pelvic X-ray showing prostatic calculi
• urine culture identifying the causative infectious organism
• urine culture identifying no UTI or causative organism (nonbacterial prostatitis).

Firm diagnosis depends on a comparison of urine cultures of specimens obtained by the Meares and Stamey technique. A significant increase in colony count in the prostatic specimens confirms prostatitis. This test requires four specimens:

• first specimen when the patient starts voiding (voided bladder one [VB1])
• second specimen midstream (VB2)
• third specimen after the patient stops voiding and the doctor massages the prostate to produce secretions (expressed prostate secretions [EPS])
• final voided specimen (VB3).

Treatment

Systemic antibiotic therapy is the treatment of choice for acute prostatitis, and may include:

• co-trimoxazole (Bactrim) orally for 30 days (for culture showing sensitivity)
• I.V. co-trimoxazole (Bactrim) or I.V. gentamicin (Garamycin) plus ampicillin (Unasyn) until sensitivity test results are known (sepsis)
• parenteral therapy for 48 hours to 1 week; then oral agent for 30 more days (with favorable test results and clinical response)
• co-trimoxazole (Bactrim) for at least 6 weeks (chronic prostatitis due to E. coli ).

Supportive therapy includes:

• bed rest
• adequate hydration
• analgesics
• antipyretics
• sitz baths
• stool softeners as necessary.

In symptomatic chronic prostatitis, treatment may include:

• instructing patient to drink at least 8 glasses of water daily
• regular careful massage of the prostate to relieve discomfort (vigorous massage may cause secondary epididymitis or septicemia)
• regular ejaculation to help promote drainage of prostatic secretions
• anticholingerics and analgesics to help relieve nonbacterial prostatitis symptoms
• alpha-adrenergic blockers and muscle relaxants to relieve pain
• continuous low-dose anabolic steroid therapy (effective in some men).

If drug therapy is unsuccessful, surgical treatment may include:

• transurethral resection of the prostate removing all infected tissue (not usually performed on young adults; may cause retrograde ejaculation and sterility)
• total prostatectomy (curative but may cause impotence and incontinence).

Testicular torsion

Testicular torsion is an abnormal twisting of the spermatic cord due to rotation of a testis or the mesorchium (a fold in the area between the testis and epididymis), which causes strangulation and, if left untreated, eventual infarction of the testis. Onset may be spontaneous or may follow physical exertion or trauma. This condition is almost always (90%) unilateral. The greatest risk occurs during the neonatal period and again between ages 12 and 18 years (puberty), but it may occur at any age. Infants with torsion of one testis have a greater incidence of torsion of the other testis later in life than do males in the general population. The prognosis is good with early detection and prompt treatment.

Causes

In intravaginal torsion (the most common type of testicular torsion in adolescents), testicular twisting may result from:

• abnormality of the coverings of the testis and abnormally positioned testis
• incomplete attachment of the testis and spermatic fascia to the scrotal wall, leaving the testis free to rotate around its vascular pedicle.

In extravaginal torsion (most common in neonates):

• loose attachment of the tunica vaginalis to the scrotal lining causing spermatic cord rotation above the testis
• sudden forceful contraction of the cremaster muscle (may precipitate this condition).

Pathophysiology

Normally, the tunica vaginalis envelops the testis and attaches to the epididymis and spermatic cord. Normal contraction of the cremaster muscle causes the left testis to rotate counterclockwise and the right testis to rotate clockwise. In testicular torsion, the testis rotates on its vascular pedicle and twists the arteries and vein in the spermatic cord, causing an interruption of circulation to the testis. Vascular engorgement and ischemia develop, causing scrotal swelling unrelieved by rest or elevation of the scrotum. If manual reduction is unsuccessful, it must be surgically corrected within 6 hours after the onset of symptoms to preserve testicular function (70% salvage rate). After 12 hours, the testis becomes dysfunctional and necrotic. (See Extravaginal torsion .)

Signs and symptoms

Signs and symptoms of testicular torsion include:

• excruciating pain in the affected testis or iliac fossa of the pelvis.
• edematous, elevated, and ecchymotic scrotum with loss of the cremasteric reflex (stimulation of the skin on the inner thigh retracts the testis on the same side) on the affected side.

Associated symptoms include:

• abdominal pain
• nausea and vomiting.

EXTRAVAGINAL TORSION In extravaginal torsion, rotation of the spermatic cord above the testis causes strangulation and, eventually, infarction of the testis. <center></center>

Complications

Possible complications of testicular torsion are:

• testicular infarction and necrosis
• infertility.

Diagnosis

Diagnosis of testicular torsion is based on:

• physical examination showing tense, tender swelling in the scrotum or inguinal canal; persistent reddening of the overlying skin; possibly palpable twisting of the spermatic cord (when examined before severe edema develops)
• Doppler ultrasonography to help distinguish testicular torsion from strangulated hernia, undescended testes, or epididymitis (absent blood flow and avascular testis in torsion).

Treatment

Treatment consists of immediate surgical repair by:

• orchiopexy (fixation of a viable testis to the scrotum and prophylactic fixation of the contralateral testis)
• orchiectomy (excision of a nonviable testis) to decrease the risk for autoimmune response to necrotic testis and its contents, damage to unaffected testis, and subsequent infertility
• manual manipulation of the testis counterclockwise in order to improve blood flow before surgery (not always possible).

Varicocele

A mass of dilated and tortuous varicose veins in the spermatic cord is called a varicocele. It is classically described as a “bag of worms.” Thirty percent of all men diagnosed with infertility have a varicocele. Varicocele occurs in the left spermatic cord 95% of the time.

AGE ALERT It occurs in 10% to 15% of all males, usually between age 13 and 18 years.

Causes

Causes of varicocele include:

• incompetent or congenitally absent valves in the spermatic veins
• tumor or thrombus obstructing the inferior vena cava (unilateral left-sided varicocele).

Pathophysiology

Because of a valvular disorder in the spermatic vein, blood pools in the pampiniform plexus of veins that drain each testis rather than flowing into the venous system. One function of the pampiniform plexus is to keep the testes slightly cooler than the body temperature, which is the optimum temperature for sperm production. Incomplete blood flow through the testis thus interferes with spermatogenesis. Testicular atrophy also may occur because of the reduced blood flow.

Signs and symptoms

Usually no symptoms are associated with the presence of a varicocele. Occasionally, symptoms may include:

• feeling of heaviness on the affected side
• testicular pain and tenderness on palpation.

Complications

Possible complications of a variocele include:

• infertility due to the elevated temperature caused by increased blood flow to the testes
• metastasis from a renal tumor leading to sudden development of a varicocele in an older man (late sign).

Diagnosis

Physical examination shows:

• palpation of “bag of worms” when the patient is upright
• drained varicocele can't be felt when the patient is recumbent.

Treatment

Possible treatment includes:

• conservative treatment with a scrotal support to relieve discomfort (mild varicocele and fertility not an issue)
• surgical repair or removal involving ligation of the spermatic cord at the internal inguinal ring (if infertility is an issue).