Ann J. Davis
Pediatric and adolescent gynecology is frequently viewed as a single focused aspect of gynecology. In fact, these two areas are reasonably distinct, with a logical division being at the onset of puberty and the activation of the hypothalamic–pituitary–ovarian (HPO) axis. Prepubertal girls differ from postpubertal girls in anatomy, etiologies of similar symptoms, and the spectrum of likely and common syndromes. Both groups however require specific communication skills. Psychosocial and developmental milestones and characteristics help guide the obstetrician and gynecologist in how to communicate with each age group in an effective manner. Involvement of the family or adult caretaker is also critical in achieving the goals of providing excellence in gynecologic care. This chapter will discuss topics in both of these age groups, with an emphasis on the differences in children and teens as compared to mature reproductive women.
EXAMINATION
Examination of the Prepubertal Child
The genital examination of the prepubertal child should be approached quite differently from the gynecologic examination of an adolescent or adult. However, the complete exam may include all of the same elements as the examination of the more mature reproductive female: examination of the external genitalia, examination of the vagina and palpation of the uterus and adnexal structures.
Examination of External Genitalia
It is often helpful to examine a toddler on her mother's lap while the mother elevates or abducts the child's hips for the so-called “frog leg position.” It is important to place the child on a towel or chuck pad in case urination occurs. An older child can sit straddling her mother's lap fully clothed while mother places her legs in the stirrups. Children between the ages of 4 and 6, and sometimes as young as 3, can position themselves in classic lithotomy with use of the stirrups. Clinicians can use their hands to provide lateral and downward traction on the area of the labia majora (Fig. 29.1). This will allow full visualization of the hymen and vaginal orifice.
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FIG. 29.1. Diagram of the genital anatomy of a prepubertal girl. This drawing shows a crescentic hymen. (From Pokorny SF. Pediatric and adolescent gynecology. New York: Chapman and Hall, 1996.) |
Care should be taken to inform the child of the necessary steps of the exam. Continuously conversing with the child during the exam will allow her to relax.
Visualization of the Vagina
Various positions have been described to visualize the vagina. In the very young infant or toddler a Valsalva maneuver can be helpful in the exam; the child can be asked to pretend she is blowing up a balloon or blowing out her birthday candles. This will often allow visualization of the distal 1 to 2 cm of the vagina. The knee–chest position is very helpful in the older child and can often be used in children 3 years of age and older. The child places her buttocks in the air with knees placed apart and allows her abdomen to sag. The examining physician and one assistant provide lateral and upward traction on the labia and buttocks. An otoscope can also be used as a magnification instrument and light source to shine into the vagina allowing visualization to the level of the cervix even without inserting the instrument. A vaginal speculum is neither appropriate nor indicated in the examination of the prepubertal child in the office (Fig. 29.2).
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FIG. 29.2. The knee–chest position can be used to examine the vagina of a prepubertal child: the otoscope is used for a light source and magnification and not inserted into the vagina. |
Examination of the Uterus and Adnexa
Examination of the uterus and adnexa requires a rectal examination and should be reserved for the pediatric gynecology patient in which information regarding the uterus or adnexa is necessary in the evaluation. Rectal bimanual examination should not be done routinely in every child requiring gynecologic examination.
In prepubertal children the adnexa should not be palpable. In children, the ovaries lie at the level of the pelvic brim and drop into the pelvis with the onset of puberty. If an adnexa is palpable there is, by definition, an adnexal enlargement that will require swift and careful evaluation of a possible ovarian neoplasm.
In the normal prepubertal child the uterus should be easily palpable on rectal examination. Prior to puberty two-thirds of the uterine volume is cervical in contrast to the one-third proportion in adults. The cervix, therefore, is a relatively easy structure to palpate on rectal examination in prepubertal children.
Examination of the Newborn
The obstetrician/gynecologist should be encouraged to observe the normal genitalia of the female infants that he or she delivers. Under the influence of maternal estrogens, the labia are generous in size, and the hymen is prominent and fimbriated or redundant in appearance. The female infant will sometimes experience an estrogen-withdrawal spotting episode within several days after birth. Mothers should be informed of this normal phenomenon in an effort to preclude maternal anxiety and even unnecessary visits to the pediatric emergency department (ED). In a series of pediatric patients seen in the ED of Cleveland's Children's Hospital for vaginal bleeding, the vast majority of those under the age of 2 were seen for this reason. These ED visits are completely avoidable through parental education.
Observation of the genitalia of female infants at birth allows the detection of various developmental and congenital abnormalities, some of which may be life threatening. If ambiguous genitalia are observed the obstetrician must use excellent communication skills in the delivery room to help set the stage for the evaluation of the infant and help decrease parental anxiety. The parents should be informed that the baby's genitals are not fully developed and a simple examination of the external genitalia cannot determine the actual sex. The parents should be told that they definitely have either a girl or a boy; but because development is not complete, data will have to be collected before they are told what sex the baby is and what treatment is required. Guesses must be avoided. It is critical to wait until all the information allows that the initial sex assignment given to the parents is the final and correct assignment.
The problem of ambiguous genitalia represents a social and potential medical emergency that is best handled by a team of specialists, which may include urologists, neonatologists, endocrinologists, and pediatric gynecologists. The differential diagnosis of ambiguous genitalia includes chromosomal abnormalities, enzyme deficiencies (such as 21-hydroxylase deficiency which is a form of congenital adrenal hyperplasia), and prenatal masculinization of a female fetus resulting from maternal androgen-secreting ovarian tumors or, rarely, drug exposures. The etiology of these problems, as well as intersex disorders that may be discovered in an older child can be complex.
The possibility of congenital adrenal hyperplasia (CAH) is especially critical to exclude. With the salt-wasting form of this disease, death can occur in the neonatal period so electrolytes should be immediately obtained. The presence of gonads in the labial scrotal folds in the infant with ambiguous genitalia eliminates the diagnosis of CAH.
One additional benefit of an observation of the genitalia of female infants at the time of birth is that some genital anomalies such as an imperforate hymen, vaginal agenesis, or other hymeneal anomalies (i.e., bands) may be diagnosed. Hymeneal abnormalities occur in less than 1% of newborn females and include imperforate hymens, cribriform hymens, and septate hymens. Normal hymeneal variations include hymeneal bumps, ridges, or bands. If there is any doubt about hymeneal patency, a rectal thermometer or small plastic catheter may be used to gently test for the vaginal space. Obstructive lesions include imperforate hymen, vaginal agenesis, or vaginal septa. The timing of repair of an imperforate hymen remains controversial. Some experts recommend repair at puberty after full estrogenization. Others repair imperforate hymens after the neonatal interval when convenient for the family. This approach may avoid anxiety during the critical preadolescent years of psychosexual identity if the girl perceives there is something wrong with her genitalia which is awaiting repair. In some cases a mucocolpos develops behind the imperforate hymen. This may be seen in the newborn in which the mucus production is under the direction of maternal estrogens, or at the time of breast budding in which endogenous estrogens orchestrate stimulation of mucus production. Rarely the mucocolpos may cause urinary obstruction that would necessitate urgent hymenectomy. Care must also be taken to delineate the exact anatomic nature of the obstruction and the clinician performing a hymenectomy should be comfortable that the obstruction is at the level of the hymen. Opening a thin imperforate hymen is a relatively easy surgical procedure, whereas the correction of other types of obstructive lesions such as vaginal septae requires careful planning, experience, and a high degree of skill.
Vulvovaginitis
Vulvovaginitis is the most common cause of vulvar symptoms in the prepubertal age group and the most common gynecologic complaint in prepubertal children. In children the primary site of infection is often the vulva; in contrast to mature reproductive women in which the vagina is usually the primary site of infection. Vulvovaginits in prepubertal children may be caused by specific pathogens including Streptococcus pneumoniae, Neisseria gonorrhoeae, Chlamydia trachomatis, Shigella, Haemophilus influenzae, and pinworms. More commonly however, the vulvovaginitis is nonspecific with no pathogenic organism responsible.
Cultures of the vagina in children with nonspecific vulvovaginitis will often reveal normal rectal flora such as Escherichia coli. Overgrowth of enteric bacteria can cause a primary vulvitis and a secondary vaginitis. In prepubertal children the normal flora may invade and irritate the vulvar area. This invasion in prepubertal children is due to several circumstances. First, the labia minora are thin and unestrogenized. Second, there is no anatomic barrier between the vaginal orifice and the anus since the labia majora are undeveloped and, prior to somatic growth, the anal and vaginal orifices are almost abutting one another. The unestrogenized vulva and vestibule normally appear mildly erythematous and may appear to be infected even when they are not if examined by clinicians unaccustomed to routinely examining prepubertal children. Smegma around and beneath the prepuce resembles patches of candidal vulvitis to the inexperienced examiner. The prepubertal vagina is alkaline in contrast to the acidity of the mature reproductive woman's vagina. At puberty, bacilli in the completely estrogenized vagina begin to produce larger amounts of lactic acid.
Presentation
It is often difficult for a young child to describe vulvar sensations, but she may describe pruritis, pain, or a burning sensation. Parents sometimes note that the child cries during urination, scratches herself, touches herself frequently, or squirms when sitting in an effort to rub the sore vulva. Often, the child's pediatrician will have evaluated the child for a urinary tract infection (UTI) and pinworms. Vulvovaginal complaints of any sort in a young child should prompt the consideration of possible sexual abuse.
Diagnosis
Most cases of vulvovaginitis are nonspecific. Specific pathogenic organisms should also be considered. When the initial presentation is compatible with nonspecific vulvovaginitis, some clinicians recommend proceeding with therapy without performing diagnostic tests. In cases where treatment was unsuccessful it is important to perform diagnostic testing for specific pathogenic organisms. This may include cultures of the vagina to rule out S. pneumoniae, N. gonorrhoeae, C. trachomatis, Shigella, and H. influenzae. Both N. gonorrhoeae and C. trachomatis cause a vaginitis rather than cervicitis in children so a vaginal culture is appropriate to exclude these pathogens. Use of DNA technology to diagnosis sexually transmitted diseases (STDs) in children is currently not recommended as a first-line diagnostic strategy by the Centers for Disease Control and Prevention (CDC). Use of indirect tests for STDs (such as enzyme-linked immunosorbent assay-based technology) is inappropriate in prepubertal children given the high possibility of false-positive results.
Several methods of obtaining vaginal cultures from the vagina of children are applicable. One is insertion of a Dacron-tipped swab into the vagina moistened with nonbacteriostatic saline. Avoiding touching the hymen helps avoid any unnecessary discomfort to the child. Another method for obtaining cultures consists of placement of a catheter through which nonbacteriostatic saline can be injected, aspirated, and sent for culture.
Therapy
The first step in treatment of prepubertal vulvovaginitis involves attention to vulvar hygiene and toileting. Proper wiping will decrease rectal flora in the vulvovaginal areas. In addition avoidance of vulvar irritants such as shampoo and deodorant soaps decreases the vulvar abrasion and irritation making it more difficult for the rectal flora to invade the vulvar epithelium. Sitz baths are very helpful in relieving symptomatology. Scrubbing the vulvar area should be avoided since this will only abrade the epithelium. Soaking the vulva clean is the preferable hygienic approach. A short course of broad-spectrum antibiotics can eliminate the overgrowth of enteric bacteria; however, unless the child changes her hygienic practices the nonspecific vulvovaginitis is likely to recur when colonization recurs.
Fungal Infections and Vulvovaginitis
Fungal infections as a cause of vulvovaginitis are uncommon in prepubertal children. The prepubertal vagina is very alkaline and will not support fungal growth, which requires a more acidic environment. Exceptions to this rule may occur in immunosuppressed children such as organ recipients, children with human immunodeficiency virus (HIV), or children receiving high-dose steroids. Diaper rash, which may present with erythema, excoriations, and satellite lesions primarily outside the vulvovaginal area, is usually fungal related.
Labial Agglutination
Labial agglutination is another common presenting complaint in prepubertal children between 3 months and 6 years of age. These are the years of a nadir in circulating estrogens. The abraded unestrogenized labia minora agglutinate and form a telltale line at the point of the agglutination that is visible on genital examination. These girls sometimes complain of genital discomfort and dripping of urine. Urine can be trapped in the “pouch” behind the agglutination. Despite the fact that the urethra may not be visible on genital examination urinary obstruction is typically not a feature of this pediatric gynecologic problem.
Labial adhesions are extremely common, and usually asymptomatic. Some small degree of adhesions is seen in many 3- to 4-year old girls. Most experts agree that treatment should be reserved for symptomatic adhesions. The treatment consists of a short course of externally applied estrogen cream for several weeks. The area of agglutination will become thin and may spontaneously separate or can easily be separated in the office with the use of topical lidocaine jelly or anesthetic creams. It is critical that the estrogen be applied to the telltale line of adhesion and not lateral to the adhesion line. If pigmentation is seen lateral to the line of adhesion after estrogen application this indicates improper application of the estrogen cream. Manual separation of thick adhesions should not be done in the office, as it is very painful.
Attention must be given to the prevention of subsequent adhesions, as they clearly have a risk of recurring. One option is to recommend the use of a topical emollient, such as vitamins A and D ointment to prevent reagglutination in a tapering-like manner after initial separation has occurred. Resolution of labial agglutination occurs at the onset of signs of endogenous estrogen production (breast budding).
Lichen Sclerosus et Atrophicus and Other Chronic Skin Conditions
Chronic skin conditions such as lichen sclerosus, seborrhea dermatitis, and atopic dermatitis may occur in young children. Lichen sclerosus et atrophicus (LSA) is a skin dystrophy usually seen in prepubertal girls or postmenopausal women. The appearance of LSA is consistent in both these age groups: a cigarette paper type of appearance in a figure-of-eight distribution around the vulva and anus, ending at the labia majora. Breaks in the integument with small blood blisters and abrasion are common, with inexperienced clinicians misinterpreting the condition as trauma possibly secondary to sexual abuse. LSA is particularly likely to occur in irritated vulvar areas in susceptible children. Appropriate first-line treatment in children is to prevent genital irritation and trauma. This may include avoidance of straddle activities, use of gel bike seats, and so forth. The extremely potent steroid (clobetasol) has been used in adults with success and is being used by experts in children despite a paucity of studies in this age group and the fact that clobetasol is not labeled for pediatric use. When this condition begins in childhood, it may regress with puberty, although this is not invariable.
Vaginal Bleeding
Vaginal bleeding in a prepubertal child warrants a careful investigation. The differential diagnosis of vaginal bleeding hinges on the absence or presence of other signs of pubertal development. In young children with breast development an evaluation for precocious puberty is warranted. Most children with genital bleeding will not have concomitant signs of pubertal development and local causes of the bleeding are probably present. The differential diagnosis in a child with genital bleeding without pubertal development is extensive and includes vulvar irritation/vulvovaginitis, Shigella vaginitis, breakdown of labial adhesions, urethral prolapse, malignant tumors of the vagina, foreign objects in the vagina, LSA, and sexual abuse.
Evaluation should include genital cultures, careful genital examination, and vaginal visualization. In cases that the vagina cannot be visualized in the office, or bleeding continues despite a negative exam, an examination under anesthesia is indicated. The exam should rule out foreign objects and rare malignant vaginal tumors (sarcoma botryroides and endodermal vaginal sinus tumors) primarily seen in girls less than 6 years of age.
Sexual Abuse
The possibility of sexual abuse should be considered in children presenting with a variety of presenting complaints including, but not limited to, vulvar vaginal symptoms, vaginal discharge, and genital bleeding. Sensitive but direct questioning of the parent or caretaker and the child by herself should be a part of any evaluation. The parent should be asked about any significant changes in behavior (such as the recent onset of nightmares, difficulties in school, changes in personality) that may accompany sexual abuse. Questioning the child who is verbal can be a useful “teachable moment” in which the physician explains the concept of the genital area as a “private zone,” and the idea that touching in this area should be reported to a parent. One concrete way to explain the “private zone” concept to a young child is to describe this as the areas that are covered by two-piece bathing suits. If the history is suspect or the injuries or physical findings are inconsistent with the reported history, a report must be made to the appropriate social service agency.
The possibility of sexual abuse must also be assessed in children presenting with genital bleeding. However it should be noted that the vast majority of children who have been sexually abused will have normal exams and not present with bleeding symptomatology. Furthermore, hymeneal size is not an accurate way to determine if a child has been abused. The genital examination in abused children usually does not differ from the exam in nonabused children. The child's history is of primary importance in the prosecution of sexual abuse. If forensic evidence is found, the source in the majority of cases is clothing and linens, which should always be collected in cases of recent assault.
Acute trauma and bleeding may result from a straddle injury or from sexual assault. Unintended trauma most commonly results in injury to the anterior vulva or laterally to the labia. Straddle injuries may result in the formation of a large vulvar hematoma, which may require evaluation. Penetrating injuries with transection of the hymen are most commonly the result of sexual assault. Any bleeding laceration of the vulva requires a careful examination to assure that there are no vaginal lacerations and to completely repair the injury. This may require an examination under anesthesia or the use of conscious sedation in the ED.
Precocious Puberty
Precocious puberty should be considered in children presenting with vaginal bleeding with or without other signs of pubertal development or in children presenting with early breast development or adrenarche. The definition of what is early development is changing. Data from a large study involving pediatric office practices indicate that African-American girls have an earlier onset of pubertal development than do Caucasian girls. Precocious puberty has traditionally been defined as pubertal development occurring before age 8. Data from pediatric office practices reveal that 27% of African-American girls and 7% of Caucasian girls had signs of breast budding or pubic hair development at age 7. Thus, the definition for precocious puberty should be reassessed in light of these data. Some experts recommend that precocious puberty be defined as breast budding prior to age 6 in African-American girls and prior to age 7 in Caucasian girls. Others argue that when other neurologic/behavior changes in African-American girls after age 6 or Caucasian girls after age 7 are associated with menarche, evaluation is warranted since these may be hallmarks of serious central nervous system (CNS) lesions. Most gynecologists will refer girls with possible precocious development to specialists for a thorough evaluation because of the rarity of the condition. The causes of precocious puberty include ovarian neoplasm, CNS lesions and tumors, McCune-Albright syndrome, and idiopathic precocious puberty.
ADOLESCENT GYNECOLOGY
Normal and Abnormal Puberty
The average age of menarche in the United States is 12.8 years (Fig. 29.3). Menstrual cycles in the first 2 years after menarche are frequently anovulatory, although the cycle length is typically regular within a range of about 22 to 45 days. Mean duration of bleeding is generally less than 7 days. The patient's estimate of quantity of menstrual flow is typically unreliable, and adolescents may have less basis for comparison than older women. Measurement of hemoglobin/hematocrit provides objective evidence of heavy bleeding.
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FIG. 29.3. Classic description of puberty. Numbers under each event represent the normal range of ages within which the event may occur. Newer data indicates that puberty may occur earlier in some ethnic groups. (From Tanner JM. Growth at adolescence, second ed. Oxford: Blackwell Scientific, 1962.) |
First Gynecologic Visit
The American College of Obstetricians and Gynecologists (ACOG) Guidelines for Women's Healthcare indicates that the adolescent's first visit to an obstetrician/gynecologist should take place sometime between the ages of 13 and 15. This is in recognition of the role that obstetrician/gynecologists can potentially play in providing preventive guidance, screening, and preventive services to adolescents. Obstetrician/gynecologists are uniquely suited to provide these services in that the consequences of adolescent risk-taking behaviors include unintended pregnancies, STDs, ectopic pregnancies, pelvic inflammatory disease (PID), and infertility—all conditions with which the gynecologist is familiar. This initial visit is an ideal opportunity to discuss normal adolescent development and concerns related to adolescents with a girl and her parents. In at least two surveys, adolescents have indicated their desire to discuss health issues such as STDs, contraception, and sexual abuse. These surveys have also indicated that these issues were infrequently addressed by clinicians.
At this visit, issues of confidentiality should be discussed with the adolescent and her parents. Numerous studies have concluded that without assurances of confidentiality, many teens will not divulge their health concerns, particularly those that relate to sex, substance use, and other risk-taking behaviors. However confidentiality does not mean secrecy. Involvement of the parent or guardian should be strongly encouraged and developed. Facilitating a discussion regarding risk behaviors between the parent/guardian and the adolescent (with the adolescent's approval) is an ideal approach.
The initial visit does not necessarily need to include a pelvic examination. The ACOG guidelines state that the provision of additional services beyond guidance and screening should be based on information obtained at this visit. If the adolescent has had intercourse, a pelvic exam, Pap test, and screening for STDs are appropriate.
When a pelvic exam is required careful attention to education and gentle technique at the first exam is particularly critical. Adolescents are less apprehensive if the clinicians describes sensations (i.e., “this will cause a pressure sensation that is not painful but may be a bit uncomfortable”) rather than only describing the purpose of the exam (i.e., “I am now putting the speculum in your vagina”). It is more important that this exam not be traumatic than that it confirm uterine or ovarian dimensions. The exam should be tailored to the needed information. If an adolescent has not been sexually active but is experiencing severe dysmenorrhea, the bimanual and rectovaginal components of the exam are more important than a speculum examination of the cervix. If a speculum exam is deemed appropriate and necessary, as with an adolescent who has had intercourse and thus requires a Pap smear and cervical cultures, an appropriately sized speculum is indicated. The Huffman or “virginal” speculum is quite narrow and can be used with a narrow or rigid hymen. However, visualization of the cervix with the Huffman speculum often requires manipulation that may be more uncomfortable than that required with the more frequently used Pederson speculum. The Pederson speculum should be the most frequently used speculum for adolescents and can be used comfortably for almost all teens. The Graves speculum, which is commonly used for adults, may be necessary for some obese or parous adolescents or when cervical procedures (colposcopy and biopsy) are indicated. Adolescents should be informed about the need for an examination; the amount of information that is provided before the exam should be tailored to the adolescent's wishes. The adolescent should be offered the opportunity to have a parent or friend accompany her during the exam. The exam itself should be performed slowly and gently with forewarning of each successive step of the exam. Pap smears are indicated for adolescents who have been sexually active, and routine screening for STDs is also recommended by the CDC.
The Guidelines for Adolescent Preventive Services (GAPS) is a set of recommendations arrived at by a panel of experts from a number of different disciplines. They are based on the rationale that:
1. The primary health threats to adolescents are behavioral rather than biomedical.
2. An increasing number of adolescents are involved in behaviors with the potential for serious consequences.
3. Adolescents are engaging in these behaviors at earlier ages.
The comorbidities that adolescents experience are related to the risk-taking behaviors of unsafe sexual practices, substance use/abuse, and violence. The GAPS report concludes that many adolescents are engaged in multiple health risks simultaneously, and most adolescents engage in some type of behavior that is a threat to their health and well-being. The multiplicity of risk factors points out the futility of trying to deal with only one specific issue. For example alcohol and drug abuse is related to irresponsible sexuality decisions and motor vehicle accidents. The ACOG guidelines were developed in an effort to screen for and detect the risk-taking behaviors that result in significant morbidities for adolescents and to provide early or preventive interventions and services.
Abnormal Bleeding
Menstrual irregularity is one of the most common presenting problems in the adolescent years. After menarche most adolescents will have an interval of anovulatory cycles. The duration of anovulation varies with the age of menarche. The earlier menarche occurs the sooner ovulatory cycles will occur. If a teen is less than 12 years of age at menarche approximately half of her cycles will be ovulatory within one year, in contrast to the teen who is 12 to 13 at menarche in whom it will be 3 years before half of the cycles are ovulatory.
During this anovulatory interval, menstrual cycles will generally be somewhere between 22 to 45 days apart with great cycle-to-cycle variation. This pattern is due to an intact HPO feedback system creating estrogen withdrawal bleeds. As estrogen climbs, follicle-stimulating hormone (FSH) levels decline, lessening follicular stimulation with a corresponding drop in estrogen. At the estrogen nadir, an estrogen withdrawal bleed is initiated. As puberty proceeds, HPO maturity progresses and levels of estrogen production become high enough to induce a luteinizing hormone (LH) surge with resulting ovulation. Cycles consistently outside the 22- to 45-day range, even in the year after menarche, are often signs of true pathology.
Differential Diagnosis of Menstrual Disorders in Adolescents
The differential diagnosis of menstrual disorders in the adolescent is very extensive and specific diseases are covered in other chapters of this text. However a basic approach is the division of the disorders into two distinct groups: those due to aberrations involving the HPO axis and those unrelated to the HPO axis (Table 29.1).
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TABLE 29.1. Menstrual irregularity in the adolescent |
Examples of disorders unrelated to aberrations in the HPO axis include cervical bleeding (from cervicitis), endometritis, polyps, uterine fibroids, bleeding disorders, and abnormal bleeding related to congenital anomalies of the reproductive tract. It is most important to exclude pregnancy.
Every adolescent with a menstrual disorder should be assumed to be pregnant until proven otherwise by pregnancy testing. The potential medical consequences of missing a pregnancy-related complication dictate that a pregnancy test be performed in all cases of abnormal bleeding in an adolescent. The adolescent should be questioned privately without her parents about a history of sexual intercourse, whether voluntary or involuntary. In one study in which pregnancies were diagnosed in a pediatric ED, 10% had denied sexual activity. The clinician's skills will certainly be tested in the situation of an unexpected diagnosis of pregnancy. The adolescent should be strongly encouraged to tell her parent(s) or a responsible adult. Counseling about pregnancy options should take place immediately. The clinician must be aware of any relevant state laws that mandate parental notification or consent when an adolescent chooses abortion.
Bleeding from cervicitis is another important consideration. Cervicitis due to C. trachomatis can cause abnormal bleeding patterns. Bleeding disorders also deserve careful consideration including idiopathic thrombocytopenic purpura (ITP) and von Willebrand disease. These clotting disorders should be considered in all teens with heavy bleeding but are particularly likely to present with menorrhagia at menarche. In one series approximately half of all girls presenting with menorrhagia at menarche had a bleeding disorder.
Most menstrual irregularity in adolescents is related to aberrations in the HPO axis. These aberrations can be divided into two general groups: hypothalamic menstrual abnormalities and the endocrinopathies (see Table 29.1).
A variety of factors such as stress, body weight, exercise, and diet are related to hypothalamic menstrual disorders. The factors affect neurotransmitter patterns which in turn impact a pulsatile release of gonadotropin-releasing hormone (GnRH).
The endocrinopathies include thyroid disease, hyperprolactinemia, adult-onset CAH, and hyperandrogenic anovulation sometimes referred to as polycystic ovarian syndrome. Other rarer diagnoses include Cushing syndrome and steroid-producing ovarian tumors. All of these disorders are covered in detail in other chapters.
In teens, the hypothalamic disorders are the most common cause of menstrual cycling abnormalities. The diagnosis of a hypothalamic menstrual disorder is a diagnosis of exclusion. Historical questioning will often point toward hypothalamic causes. All teens with menstrual disorders should be specifically questioned about stress, dietary practices, their current, past, and desired body weight, and exercise practices. The physical exam should always include height, weight, temperature, and vital signs. The exam should also include consideration of physical signs of eating disorders. For example, the presence of bradycardia or carotene pigmentation may be critical findings leading to the diagnosis of a serious eating disorder.
The psychiatric definition of anorexia nervosa includes amenorrhea as one criterion. Amenorrhea may even precede severe weight loss. Anorexia nervosa should be managed by a skilled clinician familiar with the medical effects of the disorder, and psychological counseling is always indicated. When the adolescent is in counseling, the approach to the amenorrhea may become a wait-and-watch approach. There is evidence that the bone loss associated with anorexia nervosa may not be rapidly or completely reversible with the use of estrogen supplementation. In one study, approximately 50% of women with bulimia had menstrual abnormalities. Thus, eating disorders should always be considered as a possible cause when adolescents present with menstrual irregularities.
An individual with menorrhagia and signs of hirsutism should be evaluated with hormonal testing for disorders of androgen excess. For individuals without ovarian or adrenal androgen-producing tumors, oral contraceptives usually provide menstrual management and a decrease in acne and hirsutism.
Acute adolescent menorrhagia should initially be managed similarly to acute menorrhagia occurring in older women with hormonal therapy. Curettage is very rarely necessary. Various hormonal protocols have been published for severe menorrhagia including tapering regimens of combination oral contraceptives and intravenous estrogens. Well-controlled studies have not adequately compared these approaches but there is some evidence that there is no additional benefit to intravenous over oral therapy. Some clinicians are adamant that in patients with unopposed estrogens, such as those with polycystic ovary syndrome, the most critical aspect of the hormonal therapy is the progestin. The long-term approach to the patient with menorrhagia is dictated by the diagnosis; a patient with hyperandrogenic anovulation will require different ongoing therapy than the patient with a bleeding diathesis.
Primary Amenorrhea
Primary amenorrhea is defined as the absence of menses by age 15 or 16. The young woman who shows no signs of breast development by age 12 should be evaluated for delayed puberty, as should the young woman with breast development but no menses by age 15 or 16. In one large series from a tertiary referral center, ovarian failure was the most common cause of delayed sexual development. Congenital absence of the uterus and vagina and a physiologic delay of puberty were also frequently diagnosed etiologies. Other etiologies were diverse and numerically less frequent. Only 14% of all patients presenting with abnormalities of pubertal development had subsequent normal reproductive potential. All of these patients were in the physiologic delay category. Thus, the authors concluded that pubertal aberrancy should not be considered a benign entity because it is associated with significant morbidity, mortality, and compromise of reproductive potential.
Pregnancy must always be considered as a possible etiology of amenorrhea, whether it is secondary or primary amenorrhea. Just as with excessive or abnormal bleeding, the consequences of missing the diagnosis of pregnancy are serious, and a pregnancy test should always be performed to confirm the history.
Müllerian agenesis, obstructing vaginal septa and an imperfect hymen are associated with primary amenorrhea and thus are most frequently diagnosed during adolescence. Treatment options may include both surgical and nonsurgical management but should also focus attention on the psychological ramifications of this diagnosis.
Dysmenorrhea
Primary dysmenorrhea, beginning with the onset of ovulatory menstrual cycles, is common, occurring in up to 90% of adolescents. The use of nonsteroidal antiinflammatory drugs (NSAIDs) is usually helpful in relieving the prostaglandin-mediated symptoms, and many adolescents with dysmenorrhea have already tried over-the-counter (OTC) medications, although not always in appropriately therapeutic doses. Adolescents may be unaware that these drugs are more effective in relieving dysmenorrhea than other OTC analgesics. Severe dysmenorrhea and premenstrual molimina can affect the performance of adolescent activities (particularly school attendance, but also athletic endeavors) and these girls can benefit from the use of oral contraceptives. Parents may need to be informed of the potential noncontraceptive benefits of these medications, the rare risk of serious complications, and the fact that oral contraceptive use does not accelerate the initiation of sexual activity in this age group.
Adolescents who have persistent dysmenorrhea in spite of the use of NSAIDs and oral contraceptives should be evaluated for other causes of pelvic pain, such as irritable bowel syndrome and endometriosis. At one time, it was thought that endometriosis did not occur in adolescents. However, when teenagers with severe dysmenorrhea undergo laparoscopy, endometriosis can be found in a significant percentage. The percentage of adolescents with chronic pain who have endometriosis is not well established. In reported series of adolescents undergoing laparoscopy for chronic pain (generally defined as pain unresponsive to oral contraceptives and NSAIDs) up to 75% with this complaint have endometriosis. However, the percentage of teenagers with endometriosis found at laparoscopy depends on the indications for the surgical procedure and the criteria for diagnosis. Traditionally, visual confirmation was deemed sufficient; however, when strict criteria are used for diagnosis, endometriosis is not always confirmed. Endometriosis in adolescents is most frequently minimal or mild and may be atypical, with clear, white, or red lesions rather than the classic “powder-burn” lesion seen most frequently in older women. It has been suggested that there is an age-related change in the appearance and color of endometriotic lesions. There is good evidence supporting a familial occurrence of endometriosis; the evidence is most consistent with a polygenic/multifactorial etiology. An asymptomatic individual with a first-degree relative with endometriosis has a 7% risk of developing the disease.
Pelvic Masses
Pelvic masses in adolescents may be detected as a result of symptoms (pain, pressure, urinary symptoms) or signs (the presence of a pelvic or abdominal mass on examination). Pelvic masses in adolescents are most likely to be ovarian rather than uterine, although pregnancy should always be considered a possibility and ruled out.
Fewer than 5% of ovarian malignancies occur in children and adolescents. Ovarian tumors account for only 1% of all tumors in these age groups. Germ cell tumors make up one half to two thirds of ovarian neoplasms in individuals younger than 20. A review of studies conducted from 1940 until 1975 concluded that 35% of the neoplasms occurring during childhood and adolescence were malignant. In girls younger than 9 years of age, approximately 80% of ovarian neoplasms were found to be malignant. Germ cell tumors make up approximately 60% of ovarian neoplasms in girls, compared with only 20% in adults. Because neoplastic tumors are rare, these studies come from tertiary care centers and may not be representative of the true prevalence of these lesions. Some reports include only neoplastic masses, whereas others include nonneoplastic masses. One community survey of ovarian masses revealed that the frequency of malignancy was much lower; only 10% of masses were neoplastic, and only 6% of all masses were malignant. Another series reported that nonneoplastic masses in individuals younger than 20 constituted two thirds of the total; even in girls younger than 10, 60% of the masses were nonneoplastic, and two thirds of the neoplastic masses were benign.
The mature cystic teratoma (commonly referred to as a “dermoid”) is the most frequent neoplastic tumor of children and adolescents, accounting for more than one-half of ovarian neoplasms in women younger than 20. Ovarian cystectomy with careful palpation of the contralateral ovary is the best approach in order to maximize future reproductive potential in young women. Bivalving the contralateral ovary is not necessary as most contralateral tumors will be palpable.
Functional follicular cysts can occur at any age and have been reported in female fetuses, newborns, prepubertal children, adolescents, and mature reproductive women. Unilocular cysts will usually resolve spontaneously, and surgical therapy should be reserved for symptomatic masses, masses that do not resolve, suspected torsion, or masses that include a solid or multiloculated appearance on ultrasound. Attention to the long-term effects of ovarian function and future fertility dictate a conservative approach to ovarian masses in young girls; preservation of ovarian tissues with oophorocystectomy is a priority for benign tumors. Functional cysts in prepubertal girls may rarely be associated with sexual precocity, particularly when recurrent.
Unintended Pregnancy
The teen pregnancy rate in the United States is significantly higher than those seen in all other industrialized countries. However, a real decline in the U.S. teen pregnancy rate was seen in the 1990s. In 1990 the teen pregnancy rate was 117 pregnancies per 1,000 teen girls; this decreased to 97 pregnancies per 1,000 teen girls in 1996. Approximately 80% of this decline can be attributed to greater use of contraception and 20% to delay of coitus/interval abstinence.
The 1995 National Survey of Family Growth reported that more than 50% of adolescents have had intercourse. Although most of the youngest teens have nothad intercourse, the percentage of young teens who have had intercourse has been increasing. Some of the adolescents in this survey reported the experience of involuntary intercourse, and the younger the age at initiation of intercourse the more likely the experience was involuntary. Seventy-four percent of those who reported first intercourse at age 13 or younger reported that they had experienced involuntary intercourse.
At least 75% of adolescent pregnancies are unintended. Most (approximately 80%) adolescents use a method of contraception but do not always use the method consistently, correctly, and continuously. Developmental factors contribute to an adolescent's risk for unintended pregnancy. During early and mid-adolescence, concrete thinking is developmentally normal, and the “personal fable” and magical thinking mitigate against the reality that pregnancy could happen to the individual. Middle adolescents enjoy showing off their new “adult” bodies, frequently seek peer group approval, and feel invulnerable. However, they also have increased mobility and independence coupled with less adult supervision and protection. These factors frequently lead to risk-taking behaviors and experimentation with driving, substance use, and sexual activity. These teens may be unable to anticipate or prevent the consequences of these activities because of inexperience in abstract thinking. While they are developing the ability to perceive causal relationships and future consequences, this ability is variably applied, particularly in stressful situations such as an intimate or sexual relationship. There may be discordance among an individual adolescent's physical, social, sexual, and cognitive development. Thus, adolescents may not be developmentally equipped to use contraceptives effectively. Postponing sexual intercourse therefore is the preferred form of sexual behavior for most adolescents until they are developmentally capable of responsible sexual behavior.
Various approaches to promotion of abstinence have been studied. Curriculum-based approaches have been divided into two groups: abstinence-only and abstinence plus or sometimes labeled “comprehensive sex education.” Abstinence-only education is common given the 1996 federal law that gave states $85 million dollars in funding solely for this approach. Interestingly, in an analysis of published or known U.S. or Canadian studies with an experimental or quasi-experimental design, no programs that were abstinence-only based demonstrated a delay in sexual activity or increased contraceptive use in sexuality active teens. In contrast there have been some abstinence-plus curricula with positive results in delay of sexuality and increasing contraceptive use. It should be noted however that there are only a handful of studies on abstinence-only programs. Fortunately, a well-designed evaluation of the Title V abstinence-only programs will be available in the near future and help resolve the issue of whether abstinence- only education is effective. Given the lack of available evidence, ACOG issued a committee opinion noting the limitation of the abstinence-only approach in 1998.
Contraceptive Patterns in Adolescents
Approximately one third of adolescents wait a year after initiating intercourse before seeking medical contraceptive services, and another one third have not sought medical care. In spite of these figures, however, an increasing percentage of adolescents are using contraception at first intercourse. In 1982, 52% used no method of contraception at the time of first intercourse; in 1988, 35% reported using no method; and in 1995, 23% did not use any method of contraception at first intercourse. Birth control pills and condoms are the most popular methods of contraception among adolescents.
Adolescents generally have higher failure rates of various methods of contraception during typical use, primarily because of problems with compliance—defined for contraception as the use of a method in both a consistent and ongoing manner. Failure rates among adolescents using oral contraceptive pills can be as high as 15% to 18%; as many as 50% or more of adolescents have discontinued the method by the end of 1 year. Missed pills are frequently a problem for women of all ages but are particularly frequent among the youngest adolescents. In one study, only one-fourth of adolescents 14 or younger took their oral contraceptive pill every day. For many adolescents, the longer-term methods of contraception—depot medroxyprogesterone acetate, contraceptive patches, or contraceptive rings—may more appropriate methods, given the problems of compliance.
Sexually Transmitted Diseases
Biologic factors that impact an adolescent's risk of STD acquisition or complications include the active cervical metaplasia and ectopy, which may increase the risk of Chlamydia or human papilloma virus (HPV) acquisition. Aspects of adolescent development also affect the risks for STDs. The feeling of invulnerability may result in decreased use of condoms or denial of symptoms. In addition, the clinical presentation of STDs may be affected by both an excessive attention to hygiene (e.g., douching) or excessive neglect of perineal hygiene. When infection is suspected, the adolescent typically reacts with embarrassment and fear, which results in delays in seeking treatment. Once an STD is diagnosed, adolescents may fail to complete therapy, especially if symptoms decrease; they also frequently fail to keep follow-up appointments and have difficulty informing their partners of the STD acquisition.
Behavioral factors placing adolescents at increased risk include the fact that they may be more likely to have multiple sexual partners rather than single, long-term relationships; adolescents may have either concurrent partners or engage in serial monogamy. Almost half of all sexually active women between ages 15 and 19 have had two or more partners during the previous year. Teens may be more likely to engage in unprotected intercourse and may select partners at higher risk for STDs.
Adolescents have the highest rates of gonorrhea and HPV of any age group. Routine screening for Chlamydia is recommended by the CDC for all sexually active adolescents, regardless of other risk factors. Routine screening for N. gonorrhoeae should at least be done in all high-risk teens (previous STDs, multiplicity of partners), and teens living in areas where prevalence levels warrant routine screening, such as the southern United States. In many areas of the U.S. it is cost-effective to screen for both these STDS in the sexually active teen population. On careful examination the definition of high-risk teens often is the majority of sexually active teens.
Chlamydia screening has been shown in a randomized clinical trial to be associated with a lower risk of PID among those screened when compared to those individuals who were not screened. When control rates of sexual activity are applied (approximately 50% for adolescents between the ages of 15 and 19), adolescents have the highest rates of PID.
Clinicians should be aware that in the United States, all adolescents can legally consent to be screened and treated for STDs and have the right to these services without parental consent or knowledge. Barriers to STD prevention in adolescents and young adults include financial constraints, lack of transportation, discomfort with facilities and services designed for adults, and concerns about confidentiality.
Hepatitis B is the only completely preventable STD. The Advisory Committee on Immunization Practices now recommends the hepatitis B vaccination series at age 11 to 12. However, a cohort of adolescents currently exists who did not receive the vaccine at this age. Based on this finding, the American Academy of Pediatrics and ACOG both recommend that all adolescents receive the vaccine at the time of their next visit to their health care provider.
Treatment of STDs among adolescents is identical to treatment in adults. The difference in STD presentation in adolescents compared with adults relates to the developmental and risk-profile differences noted previously, which place adolescents at increased risk. In addition, problems of compliance with medication are common; single-dose therapies may thus be more appropriate for teens with uncomplicated gonorrhea or Chlamydia cervicitis.
SUMMARY POINTS
· This chapter has outlined and summarized the basic aspects of pediatric and adolescent gynecology, highlighting the differences from the manner in which conditions are evaluated and managed in adults. Psychosocial and behavioral factors greatly influence the health of adolescents. Preventive guidance and screening may be able to prevent or minimize these health problems.
· The gynecologic care of children and adolescents requires attention to a set of pathologic entities and treatments as well as psychosocial issues that are different from those of adults.
· A non-specific vaginitis is the most common cause of vulvovaginal symptoms in prepubertal children; sexual abuse must always be considered as a possible etiology.
· Most prepubertal children who have been sexually molested will have normal genital examinations.
· Most health threats in adolescents are related to risk-taking behaviors such as early sexual activity, alcohol, and other substance abuse.
· Menstrual irregularity in adolescents can be divided into two groups: Those due to an aberration of the hypothalamic–pituitary–ovarian (HPO) axis and those with a normal HPO axis. The aberrations are usually due to either the hypothalamic menstrual disorders or endocrinopathies. The most common cause of menstrual irregularity in teens with a normal HPO axis is pregnancy.
· Contraceptive compliance is difficult for adolescents and is the major factor that leads to higher failure rates during typical use for this age group than for older individuals.
· Adolescents with dysmenorrhea unresponsive to nonsteroidal antiinflammatory drugs and oral contraceptives may have pelvic endometriosis.
SUGGESTED READINGS
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Puberty and Menstrual Disorders
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