Common diseases of the female reproductive system are discussed in the following chapter according to their anatomical sites. They comprise congenital alterations, inflammation, and infections (Table 8-1); benign and malignant tumors; and pregnancy-related disorders. TABLE 8-1 INFECTIOUS AND INFLAMMATORY DISEASES OF THE FEMALE REPRODUCTIVE SYSTEM The uterus is subdivided for diagnostic and therapeutic reasons into the uterine cervix, the endometrium, and the myometrium. Common diseases include functional disturbances, inflammation, and neoplasia. Cervicitis, which often results from sexually transmitted disease (STD), is common, whereas endometritis is rather rare. STDs include infections by papilloma virus, herpes simplex virus type II, syphilis, and gonorrhea (also see chapter 7). Chlamydia species cause infections of the female reproductive system with increasing frequency.
Diseases of the Female Reproductive System
Conditions
Causes
Dermatoses of the vulva
Folliculitis and furunculosis
Staphylococcus aureus, mixed organisms
Herpes genitalis (progenitalis)
Herpes simplex virus type 2
Intertrigo
Chafing plus dermatophytosis (fungal infection)
Tinea cruris
Ringworm of the groin, usually Epidermophyton floccosum
Molluscum contagiosum
Poxvirus
Psoriasis
Systemic noninfectious inflammatory disorder
Infections and other lesions of the vulva, vagina, and cervix
Diabetic vulvitis
Mycotic (fungal) infection
Gonorrhea
Neisseria gonorrhoeae
Syphilis
Treponema pallidum
Chancroid
Haemophilus ducreyi
Lymphogranuloma venereum
Chlamydia trachomatis types L1, L2, L3
Granuloma inguinale
Calymmatobacterium granulomatis (originally Donovania species)
Bartholin gland cyst and abscess
Neisseria gonorrhoeae, other pathogenic bacteria
Common vulvovaginitis, urethritis, and cervicovaginitis
Candida albicans (moniliasis), Chlamydia trachomatis (serotypes D-K), Trichomonas vaginalis, other organisms, including gram-positive and -negative bacteria (nonspecific vaginitis)
Genital (venereal) warts (condylomata acuminata)
Human papillomaviruses, especially types 6, 11, 42, and 44 (low risk for cervical cancer)
Tuberculosis
Mycobacterium tuberculosis
Chemical vaginitis
Douches (high-concentration chemicals)
Traumatic vaginitis
Foreign bodies, pessaries
Pelvic inflammatory disease
Vulvitis, cervicitis, endometritis, salpingitis, oophoritis
Neisseria gonorrhoeae, Chlamydia trachomatis, polymicrobial puerperal infections—staphylococci, streptococci, coliform bacteria, Clostridium perfringens
Puerperal infections
Endometritis, vaginitis, sepsis
Streptococcus species, Staphylococcus species, gram-negative bacteria
Diseases of the Uterus
Pathology of the Mammary Gland
The skin of the vulva can be involved by the same spectrum of dermatoses that affect the skin of the rest of the body. Some common dermatoses are shown here, and the causes are listed in Table 8-1. Folliculitis is a papular or pustular inflammation involving the apertures of the hair follicles, and furuncles are larger and more deeply seated lesions with a central core of purulent exudate. Herpes genitalis or progenitalis is a recurring, localized condition, beginning as groups of vesicles on an edematous, erythematous base and subsequently forming small ulcers that dry, crust, and heal. Intertrigo and tinea cruris are superficial dermatoses associated with fungal infection. Vulvar lesions of psoriasis, a systemic noninfectious inflammatory disorder, are typically pruritic, red, and covered with silvery-white scales. The presence of similar lesions on the scalp and extensor surfaces of the extremities and nail changes help to establish the diagnosis.
Presenting features of vulvitis or vulvovaginitis include pruritus vulvae, vaginal discharge, burning on urination, and dyspareunia. Diabetic vulvitis is characterized by an inflamed, dark-red or beefy appearance with a superimposed superficial fungal infection. The vulvovaginitis produced by Trichomonas vaginalis has a thick, odoriferous, bubbly discharge in the vestibule. Vulvovaginitis caused by Candida albicans and related yeast fungi (moniliasis) is characterized by white, cheesy, irregular plaques, partially adherent to the congested mucosa of the vagina and cervix (vaginal thrush). Acute gonorrhea often presents with vaginitis beginning 1 to several days after contact; occasionally, the disease may not manifest until after the following menses, when ascending infection has resulted in acute salpingitis. Examination of the external genitalia may reveal a congested vestibule with a purulent discharge and inflammation of the urethra, Skene ducts, and Bartholin ducts.
The painless ulcerated chancre, the primary lesion of syphilis, typically develops on the labia majora or vaginal mucosa approximately 3 to 4 weeks after infection and is easily overlooked. Inguinal lymphadenopathy develops slowly and is usually well demarcated 6 weeks after infection. Histologically, the chancre shows edema, congestion, and infiltration with lymphocytes, plasma cells, epithelioid macrophages, and multinuclear giant cells. The diagnosis is made by the demonstration of the spirochetes of Treponema pallidum by dark field examination of wet preparations of the lesions. Condylomata lata, the lesions of secondary syphilis, are slightly elevated, disk-shaped papules with depressed centers. Condyloma acuminata (genital or venereal warts) are caused by an infection with HPVs, often not the precancerous 16 and 18. The confluent, cauliflowerlike growths of squamous epithelium form multiple soft, pointed, watery excrescences about the labia and perineum.
Chancroid, a venereal infection caused by the Ducrey bacillus (Haemophilus ducreyi), is a painful ulcerated, inflamed lesion that develops 3 to 10 days after infection. It is associated with suppurative inguinal nodes or buboes. Lymphogranuloma venereum, which is caused by strains of Chlamydia trachomatis, begins as a papule, a pustule, or an erosion on the vulva or within the vagina. Lymphatic spread leads to the development of inguinal adenitis, a painful matted mass of glands with periadenitis and occasional suppuration and draining sinuses. Complications include rectal stricture. Granuloma inguinale is a chronic infectious disease that is widespread in the tropics and common in the southern United States. After a variable incubation period, the primary lesion develops as a vivid, circumscribed, granulomatous nodule involving the vulva, the vaginal mucosa, or the cervix. Healing occurs slowly, with the lesion persisting for many months or years.
Varicose veins of the vulva are associated with varicose veins of the lower extremities that develop as a result of retarded venous flow caused by increased intrapelvic pressure during pregnancy. Angioneurotic edema is a transient recurrent allergic reaction that manifests as painless swelling of the vulva and other areas of the body. Bartholin cysts result from obstruction of the excretory duct or one of its subdivisions due to specific or nonspecific infections and accidental or operative trauma. Sebaceous cysts result from occlusion of a sebaceous duct, which causes sebum and epithelial debris to be retained in the gland. Benign tumors of the vulva include the fibroma, fibromyoma, lipoma, papilloma, urethral caruncle, hydradenoma, angioma, myxoma, neuroma, and endometrial growths. Fibromas, which arise from vulvar connective tissue, become pedunculated as they increase in size and weight. Lipomas of the vulva are soft proliferations of benign adipose tissue.
Primary vulvar lesions account for approximately 5% of the malignant tumors of the female genital tract. Primary carcinoma of the vulva or clitoris almost always develops in elderly women. Most are SCCs, and approximately 50% are preceded by leukoplakia. The typical course is the development of a small, firm nodule that progressively enlarges and ulcerates. Lymphatic extension to the regional inguinal nodes occurs early, but distant metastases are rare. Basal cell carcinoma or adenocarcinoma of a Bartholin gland or other glandular tissue are less common. Secondary carcinoma of the vulva is uncommon, but it may occur particularly with renal cell carcinoma (hypernephroma), choriocarcinoma of the uterus, and carcinoma of the uterine body or cervix. Sarcoma of the vulva, which includes fibrosarcoma, spindle cell carcinoma, lymphosarcoma, myxosarcoma, and liposarcoma is also uncommon, but it is usually very malignant.
Most congenital anomalies of the uterus and vagina are caused by a failure of the müllerian ducts to fuse completely or to develop after fusion. Absence of the vagina (gynatresia) results from complete lack of union of the müllerian ducts. Each ovary, because it is derived from a different embryonic structure, is normal; the fallopian tubes may be rudimentary. A less extreme failure in müllerian development leads to a double vagina. The partial septate vagina is a milder degree of congenital malformation caused by a failure of the core of solid müllerian epithelium to slough completely at its lowermost portion. Another variation is a rudimentary second vagina. Failure of proper interaction and development of the lower müllerian ducts and the urogenital sinus can lead to an imperforate hymen (external gynatresia).
The decline in estrogen levels after the onset of menopause leads to vulvar and vaginal atrophy. The vagina is narrowed, especially near the apex, making visualization of the cervix difficult. The thin mucosa exhibits pallor and petechial hemorrhages, and some ulcerations may be present. Trichomonas or mixed bacterial infections may be present. As the condition advances, attempts at regeneration and repair lead to the formation of adhesions. Histologically, the epithelium is thin and focally interrupted, and the stroma is edematous and contains focal infiltrates of lymphocytes and polymorphonuclear leukocytes. Cytology of a cervical smear shows atrophic epithelial cells and neutrophils. Senile vaginitis is a common cause of postmenopausal bleeding.
A cystocele, a hernialike structure, occurs when the spreading and tearing of the principal muscular supports of the vagina and rupture of pelvic fascia during childbirth cause the bladder to push forward and downward through the anterior vaginal wall. Fistulae may develop between the vagina and urinary bladder and rectum, diverting the urinary or fecal streams and causing incontinence. The extent of the defect depends on the number and difficulty of previous deliveries and the quality of prepartum and postpartum care. A severe cystocele may produce urinary retention leading to recurrent attacks of cystitis with dysuria, frequency, nocturia, and stress incontinence and may necessitate surgical repair. The consequences of unrepaired posterior obstetric lacerations of the vagina depend on the direction and extent of the tear. Rectocele and varying degrees of prolapse of the pelvic floor may occur in subsequent months. Rectoceles are graded according to size; third degree is a hernia to or beyond the introitus.
Nonspecific or simple vaginitis occurs when the normal vaginal flora of microorganisms proliferate, stimulated by conditions such as age, debility, systemic disease, ovulation, menstruation, or pregnancy. Characteristic features of the infection caused by the protozoan parasite Trichomonas vaginalis are a vaginal and cervical epithelium with small petechial hemorrhages producing a “strawberry” appearance and a thin, greenish-yellow discharge containing many small bubbles, producing a foamy appearance. Vaginal infection due to the fungus C albicans (moniliasis) causes an aphthous ulcerative infection with patchy, white exudate that leaves a raw, bleeding surface when it is removed. Predisposing factors are diabetes and previous use of antibiotics. Vaginitis can be produced by chemical irritation from substances in douches and from foreign bodies in the vagina.
In the vagina, the syphilis chancre, with its raised indurated border surrounded by a shallow ulceration, is most likely to be near the vestibule, and inguinal lymphadenopathy may be present. In the primary stage, serologic test results are often negative, and dark field examination results of a smear from the lesion are positive for treponemes. In late syphilis, white mucosal patches that coalesce and ulcerate focally may be present in the vagina and the external genitalia. Gonorrhea involves the cervix, but spares the vagina during reproductive life because the vaginal epithelium is resistant to infection by Neisseria gonorrhoeae. Gonorrheal vaginitis is a recognized but rare clinical entity in the postmenopausal period and in childhood. Tuberculosis, which rarely affects the vagina, is secondary to tuberculosis of the fallopian tubes, the uterus, and the cervix. Typical ulcerated lesions usually involve the posterior vagina.
Most vaginal tumors are benign cysts. Gartner duct cysts are formed from embryonic epithelial remnants of Wolffian ducts, are located on the anterolateral vaginal walls, may be simple or multiple, and occasionally attain large enough size to produce pain and other symptoms. Congenital cysts of müllerian origin (inclusion cysts) may occur in the fornices or lower in the vagina. Malignant tumors include primary carcinoma of the vagina, which is usually an SCC that begins as a small growth of the posterior vaginal wall and progresses to infiltrate the vagina and eventually the adjacent pelvic viscera and regional lymphatics, and the rare vaginal sarcomas, fibrosarcoma, and variants in adults and sarcoma botryoides in children. Melanoma of the vagina is unusual, but can occur as an apparently primary lesion or, more commonly, as part of metastatic disease.
Approximately 60% of vaginal malignant tumors are secondary to other tumors, most often carcinomas of the cervix or endometrium. After hysterectomy for endometrial carcinoma, the vaginal vault is a common site of recurrence. Vulvar carcinomas may involve some or most of the vagina. The vagina is the most frequent site of metastases from uterine choriocarcinoma and may be the earliest clinical manifestation. A history of recent pregnancy may be elicited. The dark-purple hemorrhagic gross appearance and the histologic picture are characteristic. The lesion is made up of clusters of syncytiotrophoblasts and cytotrophoblasts, with the trophoblastic cells exhibiting large, hyperchromatic nuclei and frequent mitoses. Renal cell carcinoma, or hypernephroma, may metastasize to the vagina, forming a nodular, yellow tumor mass, usually composed of clear cells with hyperchromatic nuclei. Metastases or extensions may involve the vagina before or after treatment of carcinomas of the ovary, the bladder, or the rectum.
Endometriosis is characterized by the presence of hormonally responsive endometrial glands or stroma in abnormal locations outside the uterus. Endometrial tissue can result from retrograde menstruation through the fallopian tubes, metaplasia of coelomic epithelial implants, or vascular or lymphatic dissemination of tissue from the endometrium. Vaginal endometriosis is associated with similar lesions in the ovary and rectovaginal septum. The sagittal section shows a distribution of endometriosis on the surface of the ovary and other implants on the adjacent peritoneum of the posterior cul-de-sac and the lateral pelvic wall. Blue-domed endometrial cysts extend down the rectovaginal septum, which causes the anterior rectal wall to adhere to the posterior surface of the uterus. Occasionally, there may be involvement of the vulva or perineum and, rarely, a Bartholin gland.
A variety of congenital anomalies are related to the embryologic derivation of the female genital tract from the müllerian ducts. Complete failure of fusion of the müllerian ducts results in the formation of 2 separate genital tracts with completely independent uteri and a fallopian tube attached to the lateral angle of each uterus (uterus didelphys). Each uterus can function separately and sustain a normal pregnancy. More frequently, partial fusion of the müllerian ducts takes place, as is the case in the uterus duplex bicornis. If failure of fusion occurs only at a higher level, the result is 2 uterine bodies with a single cervix, the uterus bicornis unicollis. In some cases, the uterine cavities are completely or partially separated by a thin septum, giving rise to uterus septus or uterus subseptus, respectively. Uterus unicornis is a half uterus arising from only 1 formed müllerian duct. Uterine aplasia with blind fallopian tubes also is known to occur.
Displacement of the uterus occurs when it becomes fixed or rests chronically in an abnormal position. The anatomical configurations are retroversion, retroflexion, retrocession, and anteflexion. Prolapse refers to descent of the uterus down the vaginal canal so that it lies below its normal position. Some degree of retroversion is present. Usually, this occurs after parturition when the stretched uterine ligamentous supports cannot counteract the usual intraabdominal pressure and the involuting uterus lacks normal myometrial tone. In first-degree prolapse, the cervix does not protrude at the introitus. In second-degree prolapse (procidentia), the cervix protrudes. In complete procidentia, the entire uterus
protrudes. Cystocele and rectocele are frequent complications. Spontaneous rupture of the uterus is a rare complication of parturition or may occur during procedures such as dilatation and curettage, especially when there is preexisting displacement with anatomical malposition of the uterus.
Lacerations of the external cervical os are common after parturition, and, barring infection, most heal spontaneously. More complex lacerations penetrate deeply into the endocervical stroma or extend into the lateral fornix, which permits eversion of the lining of the endocervical canal, predisposing to infection. Stricture of the internal cervical os, which can result in hematometra (retained menstrual flow and endometrial debris), occurs after posttraumatic or postinfectious scarring, after radiation therapy, and in rare cases of partial or complete congenital atresia. The uterine mucosa can form polyps, which differ from endometrial polyps etiologically and clinically. Endocervical polyps, which are usually benign, typically contain all the elements of the endocervical mucosa (columnar epithelium, fibrous stroma, and glands). As the polyps grow to produce protruding soft, red, granular lesions, they can produce mild vaginal bleeding. The etiology of cervical polyps is unknown.
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