Newborn and Prepubertal

In the newborn girl the physical appearance of the genitalia reflects stimulation by maternal sex hormones (Fig. 18-1). Separation of the labia minora reveals thick, redundant hymenal folds that often hide the small central vaginal opening and urethral meatus. The mucosa is moist, vaginal pH is acidic, and a milky discharge (physiologic leukorrhea) is often seen. Vaginal bleeding during the first week of life is common. It is caused by withdrawal of maternal estrogen after delivery, and parents can be reassured that this is normal. Breast development, with palpable breast tissue, engorgement, and less commonly a clear or cloudy discharge, is observed in full-term neonates of both genders (see Chapter 2). Without ongoing stimulation from maternal estrogen, these findings gradually subside over several months. During this period, infants are at increased risk for developing breast inflammation and infection (see Chapter 12). Local trauma, including squeezing, may increase the likelihood of infection.

Figure 18-1 Normal appearance of the genitalia in a newborn girl. The labia majora are full, and the thickened labia minora protrude between them. The mucosa is pink, and a milky white discharge is seen, reflecting stimulation by maternal hormones.

(Courtesy Ian Holzman, MD, Mt. Sinai Medical Center, New York, N.Y.)

Similarly, the effect of maternal hormones on the female genitalia gradually disappears; the labia majora lose their fullness, and the labia minora and hymen become thinner and flatter. Separation of the labia minora usually exposes the vaginal opening (Fig. 18-2, A and B). As the young infant matures, the labia cover less of the vaginal vestibule, particularly when the infant or child is sitting, and thus offer incomplete protection from external sources of irritation. The mucosa has a glistening, reddish pink hue that on first inspection sometimes is mistaken for inflammation by observers unaccustomed to examining prepubertal genitalia. Vaginal pH is now neutral or alkaline, and secretions are minimal.

Figure 18-2 A and B, Normal appearance of the genitalia of a 2-year-old girl. The labia majora are flattened, and the labia minora and hymen are thin and flat. The vaginal orifice is easily seen, and the mucosa is thin, relatively atrophic, and red in color. Patient was examined in the semisupine lithotomy position. Visualization was facilitated by use of the labial traction technique (A), which involves grasping the labia majora between the thumbs and index fingers and gently pulling them down, laterally and slightly toward the examiner, and by the labial separation technique (B), which is achieved by pressing down and laterally on the labia majora with the index and middle fingers on the lower portion of the labia majora.

Physiologic changes also cause variations in the appearance of the hymenal tissues during childhood. During early infancy the tissues are relatively thick and can be redundant into the second year (Fig. 18-3, A and B). Usually in the first months of life, the hymen becomes thin and translucent, with smooth edges (Fig. 18-2, A and B and Fig. 18-3, C-E). When the child enters puberty, the hymen again thickens under the influence of estrogen.

Figure 18-3 Normal variations in hymenal configuration. A, In this young infant, the redundant hymen totally obscures the vaginal orifice. B, In a 23-month-old the hymenal folds are redundant and the central orifice is visible. C, Annular orifice of a 2-year-old. Note the thin sharp edges of the hymenal membrane. D, Crescentic hymenal orifice. E, Crescentic orifice with septal remnants at 1 and 5 o’clock. F and G, Two variations of a septate hymen are seen, one in an infant and another in an older child. See also Fig. 18-7.

(A and B, Courtesy Janet Squires, MD, Children’s Hospital of Pittsburgh, Pittsburgh, Pa.; C, courtesy John McCann, MD, University of California at Davis, Davis, Calif.; D, E, and G, courtesy Pat Bruno, MD, Sunbury Community Hospital Center for Child Protection, Sunbury, Pa.; F, courtesy Carol Byers, CRNP, Children’s Hospital of Pittsburgh, Pittsburgh, Pa.)

The shape of the vaginal orifice also varies. Annular (Fig. 18-3, C), crescentic (Fig. 18-3, D and E), and fimbriated (with fingerlike projections around the hymenal rim) hymens are all normal variations. Other irregular shapes occur, such as a teardrop, for example, with the narrow portion formed by an anterior notch to one side of the clitoris. On rare occasions there is a completely imperforate hymen; more commonly there is a septated hymen (Fig 18-3, F and G), or a fenestrated hymen with small perforations is seen.

From about 6 to 8 weeks of age until puberty the perineum, perivaginal tissues, and pelvic supporting structures are relatively rigid and inelastic. This factor increases the likelihood of tearing as a result of trauma. In addition, before the onset of puberty, the ovaries are positioned above the pelvic brim (Table 18-1). This intraabdominal location accounts for the fact that ovarian disorders in childhood frequently present with abdominal, rather than pelvic, signs and symptoms.

Table 18-1 Developmental Gynecologic Anatomy and Physiology

Pubertal

With the onset of puberty the mons pubis begins to fill out and hair begins to grow, typically first in the mid-section. Fat deposition fills out the labia majora. The labia minora thicken, become softer and more rounded, and asymmetry and variations in size and shape become more noticed, particularly in girls who remove their pubic hair.

Girls whose labia minora protrude beyond the labia majora (Figure 18-4 often wonder if their genital appearance is normal. There is no consensus on the diagnostic criteria for labial hypertrophy, but normally the maximal width between the midline and lateral free edge of the labia minora should measure less than 3 to 5 cm. Patients with labial hypertrophy may present with pain, irritation, chronic infection, problems with personal hygiene (e.g., during menses), or problems during sports or sexual activity. Most symptoms can be alleviated by addressing personal hygiene and appropriate choice of clothing to minimize friction. Sometimes use of an antichafe product can help to prevent abrasions. Referral to a surgeon experienced in labioplasty may be considered for a mature, fully developed patient whose symptoms do not respond to conservative management. However, patients should be counseled that surgery can result in scarring, chronic vulvar pain, and dyspareunia. In most cases, reassurance and discussion of the variations in normal anatomy are all that is required.

Figure 18-4 Eleven-year-old patient with hypertrophy of the left labium minus.

(Reprinted with permission from Pardo J, Sola V, Ricci P, et al: Laser labioplasty of labia minora, Int J Gynaecol Obstet 93:38-43, 2006.)

Other normal changes during puberty include slight enlargement of the clitoris and increased prominence of the estrogen-responsive urethral mucosa. The hymen also thickens and its central orifice enlarges. The vaginal mucosa thickens and softens and becomes moist and pink as secretions increase and pH levels drop. Perineal and pelvic tissues become more elastic, and the ovaries gradually descend into the pelvis. In the months preceding menarche, physiologic leukorrhea increases and becomes noticeable. It consists of a white discharge containing mature epithelial cells and vaginal secretions stimulated by estrogen (Fig. 18-5). In some girls the unopposed estrogen secretion of puberty stimulates leukorrhea so profuse that it becomes concerning and irritating because the perineum is constantly moist.

Figure 18-5 Physiologic leukorrhea. A, A milky discharge is seen on the perineum of this normal adolescent. It consists of cervical and vaginal secretions produced in response to estrogen stimulation and can be seen in the newborn, peripubertal, and postpubertal periods. B, On microscopy the discharge is found to contain sheets of estrogenized vaginal epithelial cells. Leukocytes are not increased and lactobacilli are the predominant flora.

The developmental aspects of gynecologic anatomy and physiology are summarized in Table 18-1. There is variation in the range of normal reproductive organ size as seen on ultrasound. Some authors (Garel et al, 2001) have proposed the following as upper values for prepubertal girls: uterine length, 4.5 cm; uterine thickness, 1 cm; and ovarian volume, 4 to 5 mL. The Tanner stages of pubertal development are presented in Chapter 9.

Examination of the Prepubertal Patient

Technique

Whether the patient is being seen for a routine checkup or for a specific problem, the gynecologic portion of the assessment should occur only after establishing rapport to avoid frightening the child. Because parents and health care providers communicate their own comfort levels to children both verbally and nonverbally, a discussion of any parental anxieties and the clinician’s self-awareness of his or her own attitudes can facilitate successful examination. The use of pictures and terms or language familiar to the child may further enhance cooperation, but interspersing anatomically correct vocabulary also can be educational.

For routine checkups the task involves simple external inspection. In such instances, after abdominal and inguinal examination, the clinician generally can say to patients old enough to understand, “Now, I need to take a look at your bottom, and you can help me.” The desired position for examination can be explained or demonstrated and the patient shown how to maneuver into it. Drapes generally are unnecessary for toddlers and preschoolers because they are isolating and often perceived as threatening. However, similar to adolescents, school-age children may find drapes helpful in reducing embarrassment.

Young infants can be assessed easily on an examination table after being positioned by the examiner. An older infant, toddler, or preschool child tends to be more relaxed when examined on her mother’s lap, with the mother assisting by gently holding the child in either the frog-leg or lithotomy position (Fig. 18-6). School-age children usually can be examined on the table in the frog-leg, lithotomy, or knee–chest position (see Chapter 6). Knee–chest positioning can provide excellent visualization of the hymen and the distal vagina (Fig. 18-7, C), but proper positioning can be challenging; some patients may feel threatened by being examined from behind. An alternative means of achieving visualization of the distal vagina is with the patient supine and performing the Valsalva maneuver (i.e., asking the child to push down as if she were going to pass a stool). This often produces distention of the distal vagina and hymenal orifice, facilitating visualization and atraumatic collection of specimens.

Figure 18-6 Optimal positions for perineal inspection of the young prepubertal girl. This patient has skin desquamation subsequent to a streptococcal infection. A, Frog-leg position on the mother’s lap. B, Lithotomy position on the mother’s lap.

Figure 18-7 Perineal visualization in various positions and with various techniques of parting the labia (see also Figure 18-2, A and B). A-C, Views of the same child taken on the same day and clearly showing the variations in appearance when using different positions and different techniques to facilitate visualization of the introitus and lower third of the vagina. A, Supine frog-leg position. B, Supine frog-leg position using labial traction. C, knee–chest position.

(A-C, Courtesy Mary Carrasco, MD, Mercy Hospital, Pittsburgh, Pa.)

Use of good focused lighting is essential. In the office setting the otoscope provides excellent focused light and low magnification, which is sufficient for most examinations. Since some young children fear it because of prior painful experiences during otoscopy, the patient should be reassured ahead of time that no speculum will be attached and that she will not be forcibly restrained. Preliminary “examination” of the umbilicus may help allay anxiety. Colposcopes and hand-held lenses also provide excellent magnification when available.

Once the patient is properly positioned, visualization of the introitus, hymen, and lower portion of the vagina is facilitated by maneuvers that separate the labia. These maneuvers should be explained first and the child reassured that the examiner is just going to look. If the patient desires or is mildly anxious, she may place her hands beneath the examiner’s, or the mother may be enlisted to perform the maneuver. Some girls prefer to separate their labia themselves. The maneuvers include labial separation (see Fig. 18-2, B), and labial traction (see Figs. 18-2, A and 18-7, B). When using a hand-held light (otoscope) a second set of hands (parent or nurse) may be needed to maximize labial separation and focus the light. Care must be taken to ensure that excess traction is not applied during these maneuvers because it can result in painful tearing of labial adhesions, if present.

If the patient is unusually anxious about the procedure and cannot be reassured, the examination should be deferred to a later date. On occasion, use of an oral benzodiazepine, conscious sedation, or anesthesia may be needed for an adequate evaluation. At no time should a frightened, struggling child be physically restrained and forced to undergo examination; the yield is minimal and the experience physically and emotionally traumatic.

On inspection, the clinician can readily ascertain the presence or absence of pubic hair; note the appearance and configuration of the labia majora, labia minora, clitoris, urethra, hymen, and vaginal orifice; observe the color of the mucosa and the presence or absence of rash or discharge; and often visualize the distal vagina. Vaginoscopy is required only occasionally in the prepubertal child in order to perform a complete evaluation for complaints such as vaginal bleeding with or without evidence of trauma, discharge resistant to routine therapy, a suspected vaginal foreign body, and suspected vaginal tumors. Because of the high potential for inflicting pain, especially if the patient moves suddenly, vaginoscopy generally is best performed under anesthesia or sedation. Some older school-age children may tolerate internal examination by a highly skilled examiner without sedation if preparation is careful. Again, a traumatic experience should be avoided.

Patients with precocious puberty, suspected abdominal masses, suspected vaginal foreign body, and/or abdominal pain should undergo rectal bimanual examination (vaginal bimanual examination is rarely if ever necessary). Use of adequate lubricant, having the child perform a Valsalva maneuver as the finger is inserted into the rectum, and gentle technique reduce discomfort. In most cases this can be accomplished readily in the office. If the patient is unable to cooperate, the procedure should be deferred and an examination under anesthesia considered when warranted on the basis of clinical circumstances or the results of ancillary studies such as sonography or MRI. Computed tomography involves greater radiation exposure and provides poorer resolution of pelvic structures and thus should be used only to provide information not obtainable by other imaging studies.

Examination of the Pubertal Patient

Technique

A thorough and directed history precedes the examination. A comprehensive outline is suggested in Table 18-3. Adequate time should be devoted to interviewing the patient alone, which provides an opportunity to ask questions about voluntary and involuntary sexual activity and to explore other concerns that may be difficult to discuss in the presence of a parent. A similar opportunity should be given to the parents to express any particular concerns or worries that they have been reluctant to share in their daughter’s presence.

Table 18-3 Complete History of an Adolescent with Gynecologic Concerns

Category of Information	Specific Information Required
General
Home	Who lives there and quality of relationships; sources of conflict and support
Education/employment	School, grades, curriculum, repeated grades, goals, behavioral or learning difficulties; if working—type, occupational hazards, hours, literacy/numeracy
Activities	Exercise, nutritional content (specifically calcium, iron, fat, fiber, folate), body image, eating behaviors/patterns, peer activities, friends, hobbies
Drugs	Caffeine, tobacco, alcohol, marijuana, crack, cocaine, heroin, hallucinogens, pills, injectable drugs; rehabilitation or treatment history
Suicide	Depression, anxiety, psychiatric treatment, medications, major losses or disruptions, counseling history
Abuse/exploitation	Physical, sexual, or emotional; family, relationship, peer, school, and community violence or exploitation
Obstetric and Gynecologic History
Menstrual	Menarche (age); cycles (length, duration, quantity of flow, use of pads or tampons); first day of last menstrual period; dysmenorrhea and associated disability; premenstrual symptoms (PMS); abnormal bleeding; mid-cycle pain (mittelschmerz) and spotting; douching; feminine hygiene product use (including scented products and deodorants)
STI	Herpes, gonorrhea, Chlamydia, syphilis, PID, pubic lice (“crabs”), HPV (venereal warts), Trichomonas, HIV, hepatitis A, B, and C, undiagnosed pelvic pain
Pap	Abnormal results, colposcopy, biopsies, treatments, follow-up
Urologic	Urinary tract infection or kidney problems, enuresis, incontinence, dysuria, urgency, frequency
Vaginal discharge	Color, odor, quantity, duration, pruritus
Vaginal infections	Yeast, bacterial vaginosis, trichomoniasis
Obstetric	Previous pregnancies and outcomes, fertility plans and concerns
Sexual	Last and other recent intercourse and protection; specific HIV risk to self and partners; sexual experience and age at onset; sexual practices, condom use; gender of partners, sexual orientation; number of partners, lifetime and recent; satisfaction with sexual experience; sexual problems with self or partner
Contraceptive	Current and past methods, satisfaction, consistency of use, problems
Past Medical History	Prior sources of care (routine, episodic, and emergency); immunization status including hepatitis A and B and HPV; rubella and varicella status; hypertension; migraines with aura or neurologic signs; thromboembolic events
Medications/Treatment	History of self-treatment with over-the-counter or prescription medications, and any integrative therapies or medications
Family History	Thromboembolic events at an early age or associated with pregnancy or with hormonal contraceptives; disease or death caused by alcohol, drugs, tobacco; gynecologic or obstetric problems; age at childbearing; endocrine problems (especially thyroid); bleeding problems (especially OB/GYN-related bleeding, mucus membrane bleeding, or need for blood transfusion); congenital malformations; mental retardation; reproductive loss

HIV, human immunodeficiency virus; HPV, human papillomavirus; PID, pelvic inflammatory disease; PMS, premenstrual syndrome; STI, sexually transmitted infection.

The nature of the initial experience with pelvic examination may greatly affect a young woman’s comfort with her body and the ease with which she experiences routine gynecologic care and sexual relations throughout her adult life. Hence, the examiner’s approach should be sympathetic, unhurried, and sensitive to the modesty of the patient. When patients have had gynecologic examinations in the past, it is helpful to ask them about their prior experience to avoid repeating any previous emotional or physical trauma.

Young women should be given the choice of being examined with or without an accompanying adult in the room. Some patients, particularly early adolescents, may be conflicted between their extreme modesty and their desire for support from an accompanying friend, partner, or family member. Suggesting that the support person must stay at the head of the table and using drapes that allow visual (eye) contact between patient and examiner is often the most comfortable compromise for younger adolescents. A chaperone (such as a nurse) is desirable for all examinations. This should be offered to all patients and is considered standard when the examiner is male, is a trainee, or when there is a history of sexual abuse.

The pelvic examination is done after other components of the physical examination. The patient should empty her bladder beforehand, and a urine specimen can be collected at this time if needed for testing. Raising the head of the examining table 20 to 45 degrees helps relax abdominal muscles and facilitates maintenance of visual contact with the patient. She is then assisted into the lithotomy position at the end of the examination table. During the examination, the examiner should talk to the patient to explain what she or he is seeing and to provide reassurance and education. Maintaining a dialogue throughout the procedure also usually helps the patient relax. Conversation can confirm normal anatomic findings and provide the patient with examples of a correct and comfortable vocabulary describing her reproductive anatomy and function. A hand mirror held by the patient is often useful for similar reasons. Before beginning, the examiner should carefully explain the various parts of the examination: inspection of the external genitalia, speculum examination of the vagina and cervix (with specimen collection), and bimanual palpation. Use of anatomic drawings and/or models can be helpful and educational (Fig. 18-8). Gloves should be worn for both external and internal examinations.

Figure 18-8 Anatomic drawings are useful for education and to prepare adolescent patients for gynecologic examination.

Patient comfort with being touched may be increased by identifying and then touching distal areas first and moving proximally (e.g., knees, thighs, groin, labia, introitus). Next, the external genitalia are inspected. Pubic hair distribution should be noted as should the presence of any nits, lice, skin or vulvar lesions, or vaginal discharge on the perineum. The introital opening is examined and its edges palpated for any swellings in the regions of the Bartholin glands. Clitoral size is assessed. The urethral opening is then inspected for erythema or discharge. Any purulent material obtained should be cultured for gonorrhea, but swabs used to obtain Chlamydia cultures from the urethra and any other sites must have direct contact with the mucosal surface, rather than the discharge itself.

If a speculum examination is required, successful examination depends on adequate patient preparation and use of appropriate instruments. For virginal adolescents, the narrow-bladed Huffman speculum ( × inches) is recommended. Although long enough to expose the cervix, its narrow blades are usually inserted easily through the virginal introitus. Most sexually active adolescents can be examined with the straight-sided Pederson speculum ( × 4 inches); however, the Huffman speculum should be considered as an alternative for a first pelvic examination or for particularly anxious patients. The duck-billed Graves speculum ( × inches) is useful in parous patients (Fig. 18-9). Obese patients may require a Graves speculum or a longer Pederson (1 × inches) for adequate visualization of the cervix. Metal speculums are preferred because they are easier to manipulate and are available in a greater range of lengths and widths. If only a single size of disposable plastic speculum is routinely used at a facility, it is important to have a backup supply of smaller metal speculums.

Figure 18-9 From left to right: The Graves speculum is best reserved for large or gravid adolescents; the Pederson speculum is adequate for most sexually active adolescents; the narrow-bladed Huffman is ideal for virginal adolescents.

The patient should be shown the speculum and allowed to touch it if she so desires. Patients experiencing their first pelvic examination should be reassured that only the blades of the speculum will be inserted. Comparing the size of an open speculum to a finger or tampon often is reassuring. The patient should be told that she will feel “a sense of pressure,” not pain, during speculum insertion and should be reminded to breathe at a regular rate because tensing abdominal or pelvic muscles can produce discomfort and make the examination more difficult to perform.

The examiner may then gently insert the index finger into the vagina to assess the size of the introital opening and to locate the cervix. Vaginal muscle tone can be assessed by asking the patient to “tighten her muscles” around the examiner’s finger. Conscious relaxation can be practiced by asking the patient to relax those same muscles and to push her buttocks onto the examining table. Both plastic and metal speculums should be moistened with warm water to increase comfort and ease of insertion. With the index finger partially withdrawn but gently pressing on the vaginal floor, the speculum is inserted over the finger into the vagina, taking care to avoid catching pubic hairs or the labia in the mechanism of the speculum. This is done at an oblique angle along the posterior wall to accommodate the vertical introitus and avoid traumatizing the urethra, which lies above the anterior vaginal wall. Another technique that effectively assists insertion involves using the middle and index fingers to stretch the posterior labial folds down and out before inserting the speculum. With the speculum in place the vaginal walls are inspected for erythema, lesions, and quality and quantity of discharge, and specimens are collected (see Specimen Collection, below).

After specimens have been collected the speculum is removed, and the bimanual (vaginal–abdominal) examination is performed. Water-based lubricant is placed on the two gloved fingers to be used before inserting them carefully through the introitus into the vagina. The examiner should note the size, consistency, position, and mobility of the uterus and check for tenderness on cervical or fundal motion. The adnexa should be palpated for evidence of enlargement or tenderness. After changing the glove on the examining hand, a rectovaginal examination using the index and middle fingers may be performed to confirm an abnormal or uncertain finding on vaginal–abdominal examination, to palpate the cul-de-sac, and to examine a retroflexed uterus.

Once the examination is completed, the patient should be helped out of the lithotomy position, given tissues to wipe away any lubricant or discharge, and allowed privacy to get dressed. At the conclusion of the visit the examiner can present the results of any office-based testing (such as microscopic evaluation of wet mount and KOH preparation). The use of handouts, printed pictures, or line drawings can enhance the patient’s understanding of the results (see Fig. 18-8). This is also an opportunity to encourage communication between the young woman and her parent, as appropriate to the circumstance.

Specimen Collection

Adolescents should be advised not to douche or to use tampons or feminine hygiene products before a gynecologic examination. To obtain a vaginal sample for gonorrhea (GC) and Chlamydia (CT) NAAT, slide the swab 4 to 5 cm into the vagina and rotate it for 10 to 15 seconds, moistening it against the walls of the vagina. The vaginal pH level can be measured by moistening a cotton-tipped swab on the lateral vaginal wall and rolling it on pH paper (with an appropriate range of pH 3.6 to 6.1). Vaginal pH levels are elevated in bacterial vaginosis and tend to be increased with trichomonal and decreased with candidal infections, respectively. Vaginal secretions should also be obtained with a cotton or Dacron swab and placed in a tube with 1 mL of nonbacteriostatic normal saline for wet mount and potassium hydroxide (KOH) examination. The swabs for GC and CT NAAT and for wet mount may be obtained by the patient (“self-swab”) if the patient prefers, and additional examination is not required.

To review the wet mount, a drop of the saline solution containing vaginal secretions is examined under low (×10) and high (×40) power for distribution of epithelial cells, leukocytes, yeast forms, Trichomonas organisms, and clue cells. A drop of 10% KOH is added to a second drop of the saline solution. This preparation is immediately “whiffed” for the presence of the acrid odor associated with amines that is found in bacterial vaginosis and often in patients with trichomoniasis. After this, microscopic scanning of the KOH preparation facilitates identification of yeast forms that may be obscured by epithelial cells on the wet mount.

If a speculum examination is required (see Box 18-2), the vaginal pH level is measured by holding pH paper (described previously) against the lateral vaginal wall, away from pooled secretions. Visible vaginal secretions from the posterior vaginal pool should be obtained with a cotton or Dacron swab for wet mount. Before obtaining samples from the cervix, any surface mucus should be gently removed with cotton swabs. Purulent secretions (mucopus) typically turn the swab yellow and may be saved for microscopic examination. The normal nulliparous cervix usually has a small round os (Fig. 18-10). Any cervical lesions seen, such as cysts, warts, polyps, or vesicles, should be noted. An ectropion (or eversion) of the endocervical columnar epithelium onto the cervical surface is common and normal in adolescents (Fig. 18-11). Ectropion should be distinguished from cervicitis, the latter being suggested by erythema, friability, and/or mucopurulent cervical discharge (see Fig. 18-41, A).

Figure 18-10 Normal nulliparous cervix. The surface is covered with pink squamous epithelium that is uniform in consistency. The os is small and round. A small area of ectropion is visible inferior to the os.

(Courtesy C. Stevens.)

Figure 18-11 Ectropion. Columnar mucosal cells usually found in the endocervical canal have extended out onto the surface of the cervix, creating a circular raised erythematous appearance. Note the normal nonpurulent cervical mucus. This normal variant is not to be confused with cervicitis.

(Courtesy E. Jerome, MD.)

When a speculum examination is necessary, endocervical swabs can be sent to test for GC and CT as part of routine sexual health care or for the evaluation of pain, bleeding, or cervical discharge. Gonorrhea cultures are obtained by inserting a sterile swab into the endocervical canal and rotating it for at least 10 seconds. The swab is then placed immediately into a selective transport or culture medium. Either medium must be at room temperature before inoculation. Gonorrhea-specific media prevent bacterial overgrowth by other species and allow a longer transport time. Chlamydia cultures require mucosal surface cells because the pathogen is an obligate intracellular organism. Dacron swabs are placed in the endocervical canal and thoroughly rotated to obtain the necessary cellular material. Wooden swabs are not acceptable for Chlamydia tests. NAATs, because of their ability to detect minute quantities of pathogen DNA/RNA, do not require obtaining mucosal cells for either GC or CT.

Cervical cytology (Pap) screening using liquid-based cytology to check for cervical dysplasia (a precursor of cervical cancer) is done by rotating a plastic Ayre spatula circumferentially (360°) around the cervical os, including the entire squamocolumnar junction. A sample from the endocervical canal is collected with a cytobrush (or a cotton swab if the patient is pregnant). Each sample is swished in liquid ThinPrep solution according to laboratory protocol. Cervical cytology results can be uninterpretable in the presence of inflammation and bleeding; therefore it is preferable to defer collection until infections are treated and menstrual bleeding has finished. However, concerns about patient follow-up or urgent clinical needs can justify collection of specimens at less optimal times. It should be noted that a number of strains of human papillomavirus (HPV) have been identified as causative in cervical cancer, as well as genital warts. However, currently available vaccines against HPV do not cover all strains associated with cervical cancer. Therefore Pap smears remain indicated for all patients 21 years and older, unless the patient has immune suppression or HIV infection, in which case annual Pap should begin with the onset of sexual activity.

Labial Adhesions

The most common form of “vaginal obstruction” in prepubertal patients is really a pseudo-obstruction or partial obstruction produced by “fusion” of the labia minora as a result of labial adhesions. On inspection the clinician finds a smooth, flat membrane with a thin lucent central line overlying the introitus. It is postulated that inflammation and erosion of the superficial layers of the mucosa—whether caused by infection, dermatitis, or mechanical trauma—result in agglutination of the apposed labia minora by fibrous tissue on healing. The process typically begins posteriorly and extends forward. In most cases the fused portion is less than 1 cm in length, but on occasion it can extend to cover the vaginal vestibule and rarely the urethra (Fig. 18-12, A and B). Even when fusion is extensive, urine and vaginal secretions are able to exit through the opening anteriorly. However, some urine may become trapped behind the adhesions after toileting. This may cause further irritation, perpetuating the condition or fostering extension of the adhesions. Although most labial adhesions are asymptomatic, some patients have symptoms of lower urinary tract and vulval inflammation.

Figure 18-12 Labial adhesions. Agglutination and adhesion of the labia minora, as a result of healing after inflammation, produce the appearance of a smooth flat surface overlying the introitus, divided centrally by a thin lucent line. A, In this infant, the fused portion involves the posterior half of the introitus. B, In another, the fused area has extended much further anteriorly.

(A, Courtesy Carol Byers, CRNP, Children’s Hospital of Pittsburgh, Pittsburgh, Pa.; B, courtesy D. Lloyd, MD.)

If resolution of the fused labia is desired, the condition readily responds to application of estrogen cream along the line of fusion twice daily for 2 weeks followed by nightly application for an additional week. On occasion the course needs to be extended for an additional 2 weeks, or an increased volume of estrogen cream is advised. After the labia have separated, a zinc oxide–based cream should be applied nightly for several months to prevent recurrence. The patient’s parent should be informed that topical estrogen may cause transient hyperpigmentation of the labia and the areolae and an increase in breast tissue, but that these changes regress once therapy is completed. An estrogen withdrawal bleed (similar to that seen in the neonate) occasionally occurs. Removal of irritants, treatment of infections, and instructions on good perineal hygiene help prevent recurrence. Nonetheless, refusion can occur, although repeated treatment is not necessary if the child is asymptomatic.

Manual separation of fused labia is painful, traumatic, and frequently followed by a recurrence. Hence this practice should be abandoned. True fusion—adhesions present in the first months of life or adhesions that do not respond to the prescribed therapy—requires further evaluation for abnormalities in gender differentiation or androgen production.

Female Genital Cutting

Female genital cutting (FGC) is another cause of genital tract obstruction seen with increasing frequency by pediatricians, especially those who care for large numbers of patients from Africa, the Middle East, and Asia. This ritual cutting and alteration of female genitalia has no known medical benefits and carries potentially life-threatening short- and long-term health consequences. Figure 18-13, A-D illustrates the various types of FGC. Further information about this topic can be found at the World Health Organization website (see Websites, following the bibliography). The World Health Organization is working to eliminate this practice, considering it a human rights violation of girls and women. However, pediatricians who encounter girls who have undergone these procedures must be sensitive both to the complex religious and sociocultural norms that motivate families to practice FGC as well as to the consequences to the individual patient.

Figure 18-13 Female genital cutting (FGC) is classified into four types. A, Normal female genital anatomy. B, Type 1 FGC, “clitoredectomy,” involves partial or total removal of the clitoris, and/or the skin around it. C, Type 2 FGC, removal of the clitoris and labia minora. D, Type 3 FGC, removal of the clitoris and labia minora, and labia majora sewn together to cover urethral opening and most of the vaginal opening. Type 4 FGC involves all other alterations including pricking, piercing, incising, or cauterizing the genitalia.

Imperforate Hymen

The congenital anomaly referred to as imperforate hymen consists of a thick imperforate membrane located just inside the hymenal ring. This is the most common truly obstructive abnormality. It is frequently missed on the newborn examination because of the redundancy of hymenal folds. However, it may become evident by 8 to 12 weeks of age on careful perineal inspection, appearing as a thin, transparent hymenal membrane that bulges when the infant cries or strains. On occasion, young infants have copious vaginal secretions secondary to stimulation by maternal hormones, and as a result of this anomaly they develop hydrocolpos. In such cases the infant may have midline swelling of the lower abdomen (especially noticeable when the bladder is full) that feels cystic on palpation. Perineal inspection reveals a whitish, bulging membrane at the introitus. The cystic mass may also be palpable on rectal examination. In the presence of a neonatal withdrawal bleed or trauma, a hematocolpos may develop. This presents as a red or purplish bulge (Fig. 18-14). Treatment consists of incision of the membrane to allow drainage, followed by excision of redundant tissue.

Figure 18-14 Imperforate hymen with neonatal hematocolpos. A dark purplish bulge at the introitus was noted by the mother during a diaper change.

If her condition goes undetected, the patient with an imperforate hymen usually develops hematocolpos in late puberty. The major complaints are intermittent lower abdominal pain and low back pain, which rapidly progress in severity and duration. Over time difficulty in urination and defecation may develop, and a lower abdominal swelling may become noticeable. The patient has well-developed secondary sex characteristics but has had no menstrual periods. Perineal inspection reveals a thick, tense, bulging membrane, often bluish in color, at the introitus (Fig. 18-15, A). A low cystic swelling is palpable anteriorly on rectal examination. Operative incision allows drainage of the accumulated blood and vaginal secretions (Fig. 18-15, B) and is followed by excision of the membrane. Other partially obstructive hymenal abnormalities may allow menstrual blood to flow but later cause difficulty inserting tampons or initiating intercourse. Because hymens are not of müllerian origin, imperforate hymens are not associated with other genitourinary abnormalities.

Figure 18-15 Imperforate hymen with hematocolpos. This adolescent presented with a 2-month history of intermittent crampy lower abdominal pain, which had acutely worsened. She had well-developed secondary sex characteristics but was premenarchal by history. A, Examination revealed midline fullness and tenderness of the lower abdomen and a smooth bulging mass at the introitus. B, Incision of the imperforate membrane just inside the hymenal ring allowed the accumulated menstrual blood and vaginal secretions to drain.

(Courtesy D. Lloyd, MD.)

Other forms of genital tract obstruction (Box 18-4) are rare. In most cases early routine genital inspection reveals the absence of a vaginal orifice, enabling early delineation of the anomaly and thus facilitating treatment. Proximal obstructing anomalies may not be apparent on physical examination. If missed in infancy or childhood, partial or complete obstruction can present with a wide range of signs and symptoms, such as those listed in Box 18-5. As noted earlier, ultrasonography is a valuable screening tool in evaluating girls suspected of having genital tract obstruction, bearing in mind its limitations in visualization of internal structures after the neonatal period and before puberty, when they are very small given minimal amounts of estrogen and gonadotropins. When structures are not seen or when further anatomic detail is required, consultation with a radiologist regarding an MRI is recommended.