Women's Sexual Function and Dysfunction. Irwin Goldstein MD

Medical management: perspective of the sexual medicine physician

Irwin Goldstein

Introduction

Sexual dysfunctions in women are recognized to be common health problems1 (see Chapters 2.1—2.4 of this book). There has been limited clinical and basic science research investigation, and most of the investigatory focus has been on women who present with the most common of the sexual dysfunctions, that is, lack of interest, diminished orgasm, and/or decreased lubrication.2 The sexual dysfunction that has received the least research and clinical attention is genital sexual pain (see Chapters 6.5, 12.1-12.3, 12. 5 and 12.6).

Genital sexual pain is considered a multifactorial syndrome of pain, sexual dysfunction, and psychologic disability. There are numerous mind, body, and relationship pathophysiologies linked to genital sexual pain.3 Significant psychologic factors such as previous sexual trauma and abuse, sexual neuroses, sexual inhibitions or idiosyncracies, and/or interpersonal relationship issues may manifest as genital sexual pain disorders.Significant biologic factors may occur in a multitude of peripheral genital organs and tissues secondary to such pertinent biologic factors as genital tissue infection, inflammation, abnormal immunologic conditions, abnormal hormonal states, tumors, mechanical compartment syndromes, blunt or penetrating traumatic injury, tissue weakness with organ prolapse, and others.4

Recent research in the psychophysics of pain and touch thresholds, as well as modern functional magnetic resonance imaging studies, robustly shows that genital sexual pain is not limited to the genitals and that genital sexual pain is similar in many ways to nongenital sexual pain. Furthermore, afflicted women with genital sexual pain demonstrate the intellectual survival strategies, such as hypervigilance and catastrophiza- tion, noted in women with nongenital sexual pain conditions. Contemporary researchers are emphasizing that genital sexual pain is a pain syndrome and that genital sexual pain research should consider the biopsychosocial mechanisms underlying chronic and recurrent pain.5

Ideal clinical management in women with genital pain disorders is not controversial. Preferably, women with genital pain should be diagnosed and treated by a multidisciplinary team, including biologic-focused health-care professionals trained to identify and manage specific biologic pathophysiologies, psychologic-focused health-care professionals trained to identify and manage specific psychologic pathophysiologies, physical therapists trained in women’s sexual health disorders, and, if necessary, pain specialists trained in chronic and recurrent genital sexual pain problems and/or surgeons trained in genital sexual pain problems2 (see Chapters 6.5, 12.1-12. 3, 12. 5 and 12.6).

While recognizing the need for multiple trained specialists in the overall management of women afflicted with genital sexual pain syndrome, this chapter aims to selectively provide the biologic-focused health-care professional relevant clinical information to help identify and treat specific biologic-based pathophysiologies. For the biologic-focused clinician, the general logic is that if the pathology or pathologies causing the peripheral genital sexual pain can be identified, management outcome may be successfully directed to the source pathophysiology.

Genital sexual pain disorders are associated with multiple theories and anecdotes with little evidence-based medicine documentation. Unfortunately, randomized, controlled treatment-outcome studies in the management of women with genital sexual pain are limited. At the time of this writing, three treatments for genital sexual pain have been shown to be effective against placebo: cognitive-behavioral therapy, physical therapy/biofeedback, and surgery. No medications have yet been shown superior to placebo in randomized clinical trials for the management of women with genital sexual pain.4,5

The chapter will review the epidemiology, history, physical examination, laboratory testing, biologic pathophysiology, and medical treatment of many of the genital sexual pain disorders. The classification system for this chapter is an anatomic one that includes clitoral, urethral, vulvar, vestibular, vaginal, and pelvic floor disorders.

Anatomy/epidemiology

To understand genital sexual pain, it is important to appreciate relevant anatomy, especially the innervation of the various genital tissues such as the vagina, vestibule, and vulva (see Chapters 4.1-4.4).

Concerning vaginal innervation, many physicians concluded that, since biopsies of vaginal pathologic lesions can be performed without local anesthesia, the vagina is an organ devoid of sensory nerves. Contemporary research has shown that, based on immunohistochemical markers that stain axons and nerve terminals, all regions of the human vagina reveal profound innervation with multiple axons and nerve terminals.Regional differences in nerve density are noted, such as the distal areas of the vagina having more fibers than proximal parts. The anterior vaginal wall is more densely innervated than the posterior vaginal wall, and there are free nerve endings only in the introital region.6

Concerning vulvar and vestibular innervation, there are four sensory and motor nerves which innervate the region. These include the iliohypogastric nerve (T-12, L-1, and L-2), the ilioinguinal nerve (L-1 and L-3), the genitofemoral nerve (L-1 and L-2), and the pudendal nerve (S-2-4). The pudendal nerve also includes the perineal nerve, the dorsal nerve of the clitoris, and the inferior hemorrhoidal nerve. The pelvic autonomic nerves to the genital organs pass through the hypogastric plexus. Parasympathetic input passes via the pelvic nerve, while sympathetic input travels via the hypogastric nerve.

There is an anatomic distinction between the vestibule and the vulva that is based, in part, on genital embryology and the keratin content of the skin epithelium. The vestibule is a rhom- boidal area from the frenulum anteriorly to the mucocutaneous border of the perineum posteriorly. Laterally, the vestibule extends from the hymenal ring to Hart’s line. The skin epithelium of the vulva is fully keratinized, while the epithelium overlying the vestibule has limited keratin content.

There are few epidemiologic studies on genital sexual pain. Contemporary epidemiologic studies show that genital sexual pain of varying degrees exists in approximately 10-20% of women. A recently published, population-based study of 8000 women, aged 18-64 years from seven ethnically and socioeconomically varied Boston-area communities, has shown that 16% of women have experienced vulvar pain lasting 3 months or longer. It is estimated that there are more than 15 million American women with genital sexual pain. Risk factors for genital sexual pain include being victimized as a child, lacking childhood family support, use of oral contraceptives in women reporting no pain at the time of first tampon use, and women first having intercourse before age 18 with a frequency of once per week or more.3,7

History of the patient with genital sexual pain

There are as yet limited consensed management paradigms for the diagnosis and treatment of women with genital sexual pain complaints. The cornerstone of diagnosis of women with sexual dysfunction, including those with genital sexual pain, is a detailed history and physical examination. Specifically, in women with sexual dysfunction, history taking is crucial. History taking establishes the diagnostic impressions and forms the basis of search on physical examination2,4 (see Chapters 12.1 and 12.2).

For all clinical members of the multidisciplinary team, including biologic-focused health-care professionals, psychologic-focused health-care professionals, physical therapists, pain specialists, and surgeons, genital sexual pain problems in women are difficult, complex, and, unfortunately, not rare. Genital sexual pain disorders involve discomfort, throbbing, stinging, aching, soreness, burning, and/or tenderness. Genital sexual pain disorders usually interfere with satisfactory sexual function. Genital sexual pain disorders are often associated with a psychologic response, such as anxiety, depression, and/or an aversion response to sexual stimulation. Genital sexual pain disorders are often associated with a pelvic floor contraction and/or spasm response. There are multiple difficult interactive relationships that need to be addressed during history taking, in the overall assessment of women with genital sexual pain disorders.2,4

Genital sexual pain disorders are often so very distracting and disturbing that physiologic desire, arousal, and orgasm responses during sexual stimulation cannot manifest. In such cases, the patient with the genital sexual pain problem may erroneously consider the genital sexual pain a less relevant secondary sexual complaint. It is commonplace for women with significant, long-standing genital sexual pain history to present to the health-care provider mistakenly thinking that the primary sexual complaint is really a lack of interest, lubrication, or orgasm. A thorough and detailed sexual history will help elucidate the genuine primary sexual complaint of sexual pain, since defining the total sexual medical condition will be critical for successful clinical outcome.

For example, for women having sexual activity, inquiries should be made to assess whether the patient feels subjectively excited and feels her vagina become sufficiently moist during sexual stimulation. If the patient is engaging in intercourse, queries should be made concerning her thoughts and feelings prior, during, and after penetration. It is relevant to know whether the pain occurs with contact in the vulva or vestibule, with full entry, after thrusting, with her partner’s ejaculation, after withdrawal, after urinary voiding, or days after sexual intercourse. If the pain is related exclusively to ejaculation, a rare seminal plasma allergy may exist. It is appropriate to question whether other forms of penetration hurt (tampons, fingers, sex toys). Is her body tensing or does she experience muscle spasms during penetration? Does touching her outside the genital area (nipples) also cause nongenital pain? It is important to know whether there is pain during nonsexual events such as riding a bicycle or when wearing tight clothes. What are the consequences of the genital sexual pain on her relationship? What is the personal distress or bother?4

Another important factor in history taking is the patient’s age. Above age 50 years, it is likely that the cause is related to genital tissue atrophy and local estrogen deficiency. Below this age, the cause is probably related to vestibulodynia. The duration of the symptoms of the genital sexual pain is relevant. If the pain has always been a problem, even from the first sexual attempt, the cause is often related to vestibulodynia. The timing of the symptoms of the genital sexual pain is also of interest. A history of cyclical pain may indicate endometriosis. If the pain always occurs with certain partners, there may be an anatomic concern in the partner, such as Peyronie’s disease or excessive phallic size, or a conflicting relationship issue. The location of the genital sexual pain may be pertinent. Patient-friendly anatomic diagrams of the female sexual and reproductive tract should be used, and the differences explained simply among the vulva, vestibule, vagina, cervix, uterus, and ovaries. Patients should localize pain sites from the anatomic drawing. If the patient describes on history taking that the pain is superficial and not deep, the cause may be more dermatologic, or consistent with vaginitis or vulvodynia. If the patient says that the pain is really deep inside her body, the pain may be more consistent with abdominal wall focus, pelvic pathology, or impaction against the cervix.8-10

Basic questions should be asked concerning tests done by other health-care providers, treatments already received by the patient, and the outcome of these various treatments.

Concerning the sexual history, it is appropriate for the clinician to use validated questionnaires to characterize the genital sexual pain. To help assess treatment outcome, questions are assessed at baseline and repeated at follow-up visits. The Female Sexual Function Inventory11 utilizes three questions dedicated to the domain of genital sexual pain. The first question rates the patient’s experience with discomfort or pain during vaginal penetration. The second rates how often she experiences discomfort or pain after vaginal penetration. Answers for the first two questions vary from not attempting intercourse (0 points), to almost always or always (1 point), to more than half the time (2 points), to about half the time (3 points), to less than half the time (4 points), and to almost never or never (5 points). The third question rates the patient’s level (degree) of discomfort or pain during or after vaginal penetration. Answers vary from not attempting intercourse (0 points), to very high (1 point), to high (2 points), to moderate (3 points), to low (4 points), or to very low or none at all (5 points). The lower the score, the more the genital sexual pain complaints.

The McGill Pain Questionnaire12 is more specific and extremely valuable in assessing women with genital sexual pain. The first series of 10 items helps in pain characterization. Is the genital sexual pain throbbing, shooting, stabbing, sharp, cramping, gnawing, hot-burning, aching, heavy, tender, and/or splitting? The second series of five items helps in understanding the patient’s response to the pain. Is the genital sexual pain tiring- exhausting, sickening, fearful, and/or punishing-cruel? Answers are marked as none (0 points), mild (1 point), moderate (2 points), or severe (3 points). The worst score would be 45 points for the first two sets of queries. A Likert scale (10 cm) allows patients to rate the quality of the pain from no pain to worst pain (worst pain is 10 points). Finally, a series of descriptors is used to rate the overall intensity of the pain. Answers can range from no pain (0 points), to mild pain (1 point), discomforting pain (2 points), distressing pain (3 points), horrible pain (4 points), and excruciating pain (5 points). A patient with the worst genital sexual pain would score 60 points.

Physical examination of the patient with genital sexual pain

The peripheral genital examination is regularly a helpful and revealing clinical assessment, but its intimate character demands that a rational explanation exist for its inclusion in the diagnostic process. A focused peripheral genital examination is highly recommended in women with genital sexual pain disorder. It is particularly important that the patient with genital sexual pain have full communication with the health-care provider and final authority during the physical examination to terminate at any time, to ask questions, to have control over who is in attendance, and to understand the extent of the assessment. It is vital that the patient be aware of the purpose. Some of the goals of the physical examination are to confirm normal architecture; detect any existing lesions or abnormalities; and discover whether there are any tender areas of the vulva, vagina, or pelvis or whether there is any hypertonicity of the pelvic floor. Inclusion of the sexual partner, with permission of the patient, is advantageous and provides needed patient support. Allowing the patient (and the partner) to observe any pathology via mirrors or digital photography is often therapeutic, allowing, for the first time in many cases, an illustration and connection of a detected physical abnormality with the genital sexual pain problem. During the physical examination, the patient should point with her finger to the locations of the genital sexual pain.2,4,8-10

Whatever the gender of the examining health-care provider, it is strongly recommended that a female chaperone health-care provider be present in the examination room. The following equipment should be available: examination table, hospital gown, bedsheet, disposable absorbing chucks, patient covering sheet, surgical loupes or magnifying glass, examination light source, examination gloves, gauze, lubricant, Q-tip, speculum, pH paper, glass slide, saline, and microscope. The patient should wear a hospital gown and a sheet should cover her lower torso. The patient should be placed in the lithotomy position and the examining health-care provider, using magnified vision and a carefully focused light source, should be sitting comfortably.

The first part of the examination involves inspection. If appropriate, lubricant should be placed on the vulva. Gauzes can be grasped between thumb and index finger and used to retract the labia majora for a full inspection of the vestibular contents. Two gloved fingers are placed on either side of the clitoral shaft, and with an upward force in the cephalic direction, the prepuce is retracted to gain full exposure of the glans clitoris (usually more than 1 cm in diameter), corona, and right and left frenulum emanating at 5 o’clock and 7 o’clock off the posterior portion of the glans clitoris (Fig. 12.4.1). Using gauze to retract the labia minora, the labial-hymenal junction is identified. A Q-tip cotton-swab test is performed, gently applying pressure on Skene’s glands and minor vestibular glands and documenting the quality of the discomfort or pain (Fig. 12.4.2). The Q-tip cotton swab may also be placed at multiple locations in the vulva and vestibule. Palpation is next performed by a single-digit examination. This procedure occurs before speculum insertion or bimanual searches for vaginismus. Single-digit palpation is achieved by sliding a finger into the vaginal opening and depressing the bulbocavernosus muscle. The test is positive if there is hypertonicity and pain.

Figure 12.4.1. To examine the clitoris, place two gloved fingers on either side of the clitoral shaft, and, using an upward force in the cephalic direction, retract the prepuce to gain full exposure of the glans clitoris (usually more than 1cm in diameter), corona, and right and left frenulum emanating at 5 o'clock and 7 o'clock off the posterior portion of the glans clitoris.

Figure 12.4.2. A Q-tip test is performed by gently applying pressure on the Skene's glands and minor vestibular glands and documenting the quality of the discomfort or pain. The Q-tip may also be placed at multiple locations in the vulva and vestibule.

A bimanual examination and evaluation of pelvic floor may be subsequently performed if indicated. Two fingers are placed against the lateral walls, and the levators and underlying obturator are assessed for tenderness or taut bands. In addition, a bimanual examination can evaluate the integrity of the fornices, bladder and urethral bases, and pelvic organs. A rectovaginal examination and speculum examination can then be performed if indicated.

For the speculum examination, a warm, lubricated speculum is used. The vaginal wall is examined for estrogen milieu integrity, inflammation of the walls, and vaginal lesions.

Laboratory testing of the patient with genital sexual pain

Vaginal pH testing can be performed as indicated, especially if the physical examination identifies vaginal discharge or vaginal odor. The vaginal pH in a healthy vaginal milieu is approximately 3.5—4.5. An acid pH rules out bacterial vaginitis, but not candidiasis. For vaginal pH values more than 4.5, several conditions need to be considered, including vaginitis, vaginosis, and/or atrophic vaginitis8 (see Chapters 9.5 and 12.2).

During the speculum examination, an evaluation should be performed for discharge. Samples should be collected for wet mount and culture. Normal vaginal secretions are odorless and are not associated with the clinical complaint of itching or irritation. On wet mount, one should note a heterogeneous suspension of desquamated vaginal epithelial cells with lactobacilli as the predominant microorganism. The wet prep13,14 has four important elements: background flora, vaginal epithelial cells, pathogens, and white blood cells.

Concerning background flora, lactobacilli predominate in the healthy vagina. Lactobacilli may be best seen with the phase-contrast microscope. If lactobacilli are seen to dominate the background flora and the vaginal pH is acidic, it probably does not matter what vaginal culture grows. There is probably no bacterial infection, independent of any vaginal culture reports. If, however, there are absent lactobacilli in the background flora, there are several causes, including bacterial vaginosis, desquamative vaginitis, vaginal lichen planus, and the recent use of antibiotics. There is no evidence relating the presence of Candida albicans to lack of lactobacilli on a wet mount.13,14

Concerning vaginal epithelial cells, the frequency of noting parabasal cells on the wet mount can help the clinician assess the woman’s estrogen status and/or presence or absence of inflammation. In normally estrogenized vaginal epithelium, the wet mount should reveal very few parabasal epithelial cells. If there is an ongoing inflammatory process in the vagina, the inflamed epithelium will shed, thereby increasing the occurrence of parabasal epithelial cells. In addition, the presence of so-called “clue cells” may be a sign of the existence of the condition of bacterial vaginosis.13,14

Vaginal pathogens may include Trichomonas and Candida (either C. albicans or non-C. albicans). It should be noted that Trichomonas and Candida may be identified on wet mount in approximately 60% of cases, and that in the remaining 40% of cases of true infection with these pathogens, wet mount will not disclose them. Vaginal cultures that show Escherichia coli, enterococcus, and/or group B streptococcus are normal vaginal organisms that have a commensal relationship, whereby one organism derives food or other benefits from the association while the other organism remains unharmed and unaffected. It should be noted that Gardnerella may be seen in the vaginal cultures of 50-70% of normal women.13,14

Concerning white blood cells on the wet mount, the observation of an increased white blood cell count is considered to be more than one white blood cell per epithelial cell. The observation of an increased white blood cell count on a wet mount may occur under the following conditions: vaginitis secondary to Trichomonas, yeast, and/or atrophy; cervicitis secondary to gonorrhea, chlamydia, and/or herpes; and upper genital/reproduc- tive tract disease such as endometritis and/or pelvic inflammatory disease. An increased white blood cell count on a wet mount is not caused by bacterial vaginosis. One should consider a vaginal culture on Sabouraud’s medium with the finding of an increased white blood cell count on a wet mount, especially if the vaginal pH is not acidic.13,14

Other laboratory testing procedures for assessment of genital sexual pain disorders include biopsy of a genital lesion; serology for lesions suggesting a sexually transmitted disorder; pelvic ultrasound of the ovaries, fallopian tubes, uterus, and cervix; magnetic resonance imaging of pelvis or spine; and sex steroid hormone blood testing. A genital biothesiometric examination and duplex Doppler ultrasound study before and during sexual stimulation may also help to assess underlying genital tissue function.

Biologic pathophysiologies and treatment strategies

Biologic pathophysiologies resulting in genital sexual pain may occur in the clitoris, urethra, vulva and vestibule, vagina, and pelvic floor muscles. In this chapter, the treatment strategies will be discussed within each anatomic region.

Clitoris, prepuce, and fenulae

Phimosis/balanitis

Careful inspection of the glans clitoris should be performed, especially in cases where the history suggests focused clitoral pain. Any smegma material can be removed by gentle manipulation with a Q-tip and/or with gauze. Failure to visualize the whole glans clitoris with the corona is consistent with some degree of prepucial phimosis.15 Phimosis has been reported to occur in approximately one-quarter to one-third of all female patients with sexual dysfunction. There can be mild, moderate, or severe phimosis according to the elasticity of the prepuce and its ability to retract on examination (Fig. 12.4.3).

Phimosis per se does not imply clinically relevant disorder requiring intervention. Since phimosis may create a closed compartment, however, phimosis is often the underlying disorder in balanitis associated with the clinical symptoms of itching, burning, and recurrent fungal infections. Should clitoral glans balanitis be diagnosed, the physical examination usually reveals phimosis and the classic associated redness and tenderness of the visible glans clitoris. In cases of complete phimosis, the diagnosis cannot be made until surgical intervention. Initial treatment of clitoral glans balanitis should be conservative with topical estrogen and/or testosterone creams to see whether the prepuce can be made more elastic and retractile. Topical antifungal agents, such as nystatin, or oral antifungal agents, such as fluconazole, may be considered. Infections can also be related to herpes virus, and appropriate treatment, such as acyclovir, administered. If a chronic genital dermatitis condition, such as lichen sclerosus, involves the prepuce, phimosis and fusion of the prepuce are a likely sequela. An uncommon chronic genital dermatitis condition, lichen planus, may lead to hypertrophic, white scarring of the glans clitoris, prepuce, and frenulae. A useful treatment consideration for lichen sclerosus or lichen planus of the periglans clitoral tissue is clobetasol cream.

If appropriate conservative treatment is performed for any clitoral pain condition and the condition remains unchanged specifically due to the phimotic prepuce, surgical management by dorsal slit procedure should be considered so that medical management without the closed compartment can be reinitiated.

Prepucial infections

Severe swelling, pain, tenderness, fever, or purulent drainage may emanate from the prepuce. The most likely diagnosis is an infected sebaceous cyst with extension from surrounding vulvar or pubic skin into the prepuce (Fig. 12.4.4). Conservative treatments include antibiotics, soaking, and sitz baths. Often the situation is resolved only with incision and drainage.

Figure 12.4.4. Swelling, pain, tenderness, fever, or purulent drainage may emanate from the prepuce or from vulvar tissue near the prepuce. The most likely diagnosis is infected sebaceous cyst with extension from surrounding vulvar or pubic skin into the prepuce.

Traumatic neuropathy

In some cases, women with genital sexual pain claim that symptoms started after traumatic injuries to regional or local nerves associated with childbirth, especially forceps delivery, regional anesthetic procedures, or postoperative injuries to the ileo-hypogastric or ileo-inguinal nerves, especially with wide lower abdominal Pfanenstiel incisions. Some women provide a history of sudden onset pain in the clitoris following a sharp blow to the perineum. This may result from bicycle-riding injuries, especially after a fall onto the nose of the saddle or the bar of the bicycle frame, or from other blunt perineal or pelvic trauma, as in a fall onto a tree branch or horn of a horse saddle, or after pelvic fracture as a result of a motor vehicle accident. In many such cases, the genital sexual pain is described as sharp, shooting, and burning, consistent with neuropathy and severe hypersensitivity. There is often diminished sensation of the clitoris and perineum on physical examination, including biothesiometric evaluation. On physical examination, Q-tip testing shows regions with no pain but highly specific localized regions with sharp pain, typically to the side of the clitoris.

Treatment should aim at correcting the primary disorder, but usually that is not possible. Psychotropic agents, such as amitriptyline or gabapentine, have become the mainstay of conservative therapy. Local genital pudendal neuromata can be identified after blunt perineal trauma, and these may be excised surgically in certain circumstances (Fig. 12.4.5). Unfortunately, there is a high recurrence rate after surgical excision of neuromata. Injection of the region with steroids may prevent recurrence of the neuromata after surgical excision.

Tumors

In women with a history of focused clitoral pain and increased sensitivity to touch, especially that which worsens with sexual contact, physical inspection of the clitoris may reveal a clitoral tumor. Occasionally, such women can feel a mass associated with and related to the clitoris during self-examination or selfstimulation. The most likely tumor of the clitoris is fibroepithe- lioma, a skin tumor composed of fibrous tissue intersected by thin, anastomosing bands of basal cells of the epidermis. Differential diagnosis would include condyloma, sebaceous cyst, or compound nevi. Careful surgical excision of the mass should be performed under local/general anesthesia with care to preserve as much as possible of the clitoral structure. It should be noted that fibroepitheliomas have a propensity to recur locally despite surgical excision. Fibroepitheliomas may also give rise to basal cell carcinoma of the nodular type (Fig. 12.4.6).

Figure 12.4.5. Local genital pudendal neuromata can be identified after blunt perineal trauma (in this case, a fall onto a bicycle bar). These may be excised surgically in certain circumstances.

Urethral meatus, urethra/bladder neck, Skene’s glands

Gentle retraction of the labia minora should provide full view of the urethral meatus. This orifice is 1-2 cm posterior in the midline from the glans and frenula, and should be flush with the vestibule. The urethral meatus is 1-2 cm anterior to the 12 o’clock hymenal tissue at the vaginal introitus.

Urethral prolapse

Prolapse of the urethral mucosa out the urethral lumen is highly associated with estrogen-deficiency states such as after bilateral oophorectomy, natural menopause, or after chemotherapy for malignancy. Owing to breast cancer fears, many women with low estradiol values do not receive local or systemic estradiol treatment. It is not uncommon for such women with urethral prolapse to note urgency, frequency, and discomfort of urination and also observe spotting of blood on the toilet paper on wiping after voiding. The abnormal voiding history is often accompanied by a unique sexual history. Women with urethral prolapse often have the ability to have full sexual pleasure and satisfaction during self-stimulation of the clitoris; however, during sexual activity with the partner or with a mechanical device, they experience pain and/or urgency to urinate and/or inability to have orgasm secondary to distracting pain.

Physical examination of a urethral prolapse reveals a beefy, red, erythematous, protruding, inflamed, and edematous mucosa prolapsing from the meatus in different degrees. Conservative treatment options include topical or systemic estrogens. If necessary, surgical excision may be required (Fig. 12.4.7).

Skene’s glands adenitis/urethral-vesical hyperreflexia

Skene’s glands drain to the side of the meatus at various locations. It is at present unknown what the pathophysiology is of Skene’s glands adenitis, but there appears to be a relation to abnormal immunologic and/or sex-steroid-deficiency states. The diagnosis is made by a history of significant, localized, urethral meatal discomfort and/or dysuria during penetrative sexual activity. Women able to have self-stimulation sexual activity that avoids contact with the urethral meatus can have excellent sexual satisfaction. The classic physical finding of Skene’s glands adenitis is a swollen, edematous, protruding urethral meatus without mucosal prolapse. Conservative treatment is by topical and systemic sex steroid hormones, while in extreme cases surgical reconstruction of the meatus is required, with excision of the inflamed Skene’s glands.

Urethral-vesical hyperreflexia is an unusual condition involving severe urgency and frequency during urethral meatal contact that is not associated with inflammation of Skene’s glands. The pathophysiology is unknown and may be due to trauma to the perineum. The condition is diagnosed during physical examination. The urethral meatus appears otherwise normal except during Q-tip testing. Application of direct pressure by the Q-tip at the urethral meatus results in severe urgency. The diagnosis involves application of local lidocaine ointment at the meatus with immediate resolution of the irritative urethral symptoms. A long-term treatment plan may consist of local application of lidocaine prior to sexual activity. It is possible that recurrent kenalog steroid nerve blocks administered circumferentially around the meatus can help desensitize the hypersensitivity of the urethral meatus.

Urethra/bladder

Urethritis/recurrent urinary tract infections/interstitial cystitis

It is very common for women, especially during transition and in the menopause, to complain of irritative voiding symptoms, especially burning, frequency, urgency, and nocturia, which significantly interfere with sexual activity (see Chapter 17.4). In such women, the vulvovaginal and urinary irritative and burning symptoms are often poorly defined. The patient and physician confusion in this area occurs because the differential diagnosis of irritation and burning in the perineum, especially after coitus, involves multiple different urologic and gynecologic conditions. Urologic conditions that do not involve the urethral meatus include, for example, recurrent urethritis, recur rent urinary tract infections, urethral diverticulae, irritable bladder, cystocele, ureteral stones, and endometriosis of the ureter and/or bladder. Irritative symptoms during voiding, especially after coitus, may also be associated with inflammatory gynecologic conditions. In such situations, the contact of voided urine with the inflamed vestibular, vulvar, and/or vaginal tissues can result in significant perineal burning and stinging. Vaginal yeast infections, vulvar dermatitis conditions, and uterine prolapse with or without rectocele are common gynecologic pathophysiologies. In many cases, expert urologic and gynecologic consultations are required to confirm the various diagnoses involved in the individual patient so that varied specific focused treatments may be initiated.

A rare genital sexual pain condition that is confusing and ill-defined, interstitial cystitis, is associated with severe urogenital and pelvic pain, urgency, frequency, nocturia, and dyspareunia. Its pathophysiology is unknown, but it appears unrelated to any recognized bacterial pathophysiology. Interstitial cystitis symptoms, including dyspareunia, often increase with the menses, increase during bladder filling, and decrease with bladder emptying. There is no definitive diagnostic test for interstitial cystitis. History, physical examination, urinalysis, urine culture, and maintenance of a voiding log are mandatory. Specific patient questionnaires are often useful. Other specific testing procedures include potassium sensitivity testing and cystoscopy with or without hydrodistention. As it relates to the dyspareunia and genital sexual pain component associated with interstitial cystitis, treatment of the underlying condition is important. Treatment of interstitial cystitis involves specific agents, such as oral pentosan polysulfate, and bladder instillations of dimethyl sulfoxide or heparin. Nonspecific agents include various antibiotics, anticholinergics, antihistamines, analgesics, and antidepressants.16

Vulva

Genital sexual pain in the vulva may be related to various specific disorders, such as infection secondary to Candida, herpes, etc. Another example may be inflammation resulting from lichen sclerosus or lichen planus. A neurologic etiology may derive from blunt perineal trauma, such as traumatic neuromata formation, or hyperpathia from postherpetic neuralgia.

Obstruction of the duct draining Bartholin’s gland can lead to cyst formation, especially after sexual arousal. Another example is neoplasm from Paget’s disease or squamous cell carcinoma.

Bartholin’s cysts

Bartholin’s (major vestibular) glands are located at 5 o’clock and 7 o’clock at the vaginal introitus, one on each side. During sexual stimulation, the function of Bartholin’s glands is to secrete a lubricious fluid onto the mucosal surface of the labia. Bartholin’s cyst develops when the exiting Bartholin’s gland duct becomes obstructed. The fluid produced then accumulates, causing the gland to swell and form a cyst. Bartholin’s cyst, typically in one of the two glands, results in discomfort and swelling of the labia near the entrance to the vagina, especially during sexual arousal. The labial physical examination may be normal in the baseline, nonsexually stimulated state. Ultrasound studies before and during sexual stimulation may be needed to make the diagnosis. Significant pain, swelling, and tenderness of a cyst that interfere with walking and sitting suggest that an abscess has developed, usually with such organisms as chlamydia or E. coli. Home treatment of Bartholin’s cysts often involves sitz baths. Very large cysts or abscesses may require either incision and drainage or marsupialization, in which the lining of the cyst wall is sutured to the overlying skin in such a way as to create a permanent drain site. If needed, intraoperative ultrasound may help localize the cyst17,18 (Fig. 12.4.8).

Lichen sclerosus/lichen planus

Lichen sclerosus is a chronic genital dermatitis condition that is associated with varying intensity of symptoms, including vulvar itch or burning and various degrees of vulvar scarring leading to narrowing of the introitus and dyspareunia. There is a wide variation in presentation symptoms. In some women, especially those not sexually active, there can be minimal symptoms, and the patient may be unaware of the condition of lichen sclerosus for years. Alternatively, the burning and itching symptoms can be so intense as severely to interfere with sexual activity, day-to- day activities, and even sleep. If the scarring of lichen sclerosus involves the perianal area, the patient may also complain of perianal fissuring and painful defecation. The diagnosis of lichen sclerosus is suggested by physical examination showing white genital, vulvar, and vestibular tissue with paleness, loss of pigmentation, and characteristic “cigarette paper” wrinkling. Classically, the genital tissue changes do not involve the inside of the vagina, and if they involve the perianal area, there is a traditional figure of eight extension. The lichen sclerosus condition commonly involves the vestibule, with associated labia minora atrophy, and the vaginal introitus, with loss of elasticity and narrowing. The treatment of choice for lichen sclerosus is the ultrapotent fluorinated steroid, clobetasol. Safety and efficacy (especially for control of itching) studies for clobetasol treatment of vulvar lichen sclerosus have been reported for more than a decade19,20 (Fig. 12.4.9).

Lichen planus is another chronic genital dermatitis condition, probably pathophysiologically related to various altered immunologic disorders. The presenting symptoms vary widely in different patients, probably due to the varied pathophysiologies. Lichen planus may occur secondary to drugs, such as antihypertensives, diuretics, oral hypoglycemics, and nonsteroidal anti-inflammatory agents, that may rarely induce a lichen planus-like eruption. One type of lichen planus is primarily associated with itching and does not result in scarring. Another type of lichen planus is erosive and destructive. Overall, patient complaints may include severe vulvar itching, pain, burning, and irritation. Dyspareunia occurs in sexually active women secondary to vaginal introital scarring. Some types of lichen planus, unlike lichen sclerosus, may involve the vaginal mucosa. If there is vaginal involvement, a purulent malodorous discharge may be noted. Findings on physical examination of women with lichen planus vary widely. The pruritic type of lichen planus is associated with a purple color, and multiple papules and plaques on the vulva and vestibule. The erosive type is associated with vestibular ulcers, scarring, and clitoris and labia minora atrophy; occasionally, destruction of the vagina has been reported. A biopsy and dermatopathologic review may be needed to establish the diagnosis of lichen planus. Treatment of lichen planus involves use of ultrapotent topical steroids, such as clobetasol, topically and vaginally as indicated.21,22

Vestibule

Generalized vulvodynia

Generalized vulvodynia refers to a diffuse, constant, burning pain anywhere on the vulva, from mons to anus, which is hyper- pathic and greatly out of proportion to the stimulus. Afflicted patients have a constant or sporadic awareness of the vulva with widespread “everything hurts”, vulvar soreness, rawness, constant irritation, various paresthesias, aching, and/or stinging. Generalized vulvodynia may be considered as primary if it occurs with the first penetrative sexual encounter, or with a tampon or speculum examination. Generalized vulvodynia may be considered as secondary if it occurs after previous nonpainful vaginal penetrations. Although the pathophysiology of generalized vulvodynia is unknown, there are nociceptive and neuropathic theories.23,24

A nociceptive theory involves an initial “insult to genital tissues”, not to genital peripheral nerve tissue. There have been multiple conditions proposed to induce the genital tissue insult and injury, including exposure to C. albicans, oxalate metabolite, chemotherapy, early and frequent intercourse at a young age, and inadequate estrogen milieu as with an oral contraceptive agent or menopause. Continuing genital tissue insult and damage lead to continual stimulation of nerve endings. This continuous stimulation leads to local sensitization of fibers and central sensitization potentiating chronic genital sexual pain. The chronic pain may exist to a degree that a nonnoxious stimulus, such as simple touch, produces pain or there is continuous pain appreciation.

A neuropathic theory also is proposed to explain generalized vulvodynia where there is “injury to the sensory nervous system” and the genital tissue is intact. In the neuropathic theory, transmission of pain signals occurs secondary to nerve. There are multiple possible etiologies, including injury to the pudendal nerve from childbirth, from vaginal surgeries such as transvaginal sacrospinous colpopexy surgery, or from disorder within the obturator internus. Other neuropathic etiologies of generalized vulvodynia include abnormalities of the sacroiliac joint, piriformis, or pubococcygeus; ruptured disk; scarring around nerve roots after disk surgery; or spinal stenosis.

The diagnosis of generalized vulvodynia is made by ruling out, on physical examination and laboratory testing, such diagnoses as Candida vaginitis and chronic genital dermatitis conditions, while Q-tip testing shows all vulvar areas positive for pain and/or tenderness. The treatment of any genital sexual pain disorder involves the multidisciplinary team approach. This is especially true for the disabling condition of generalized vulvo- dynia. Patient management includes education and support, especially regarding avoidance of contacts and practice of healthy vulvar hygiene; pelvic floor physical therapy treatment; management of concomitant depression; and management of any associated neurologic, dermatologic, gynecologic, orthopedic, or urologic conditions. Medical management includes amitriptyline and/or gabapentin.23,24

Vulvar vestibulitis syndrome/vestibular adenitis

Friedrich’s criteria for vulvar vestibulitis syndrome are as follows. On history, there is severe pain on vestibular touch or attempted vaginal entry. On physical examination, there is erythema of various degrees within the vestibule. During Q-tip testing, there is tenderness to pressure “localized” within the vulvar vestibule. Often the tender localized region is along the labial hymen junction associated with the presence of minor vestibular glands.

Vulvar vestibulitis syndrome is one of the most likely causes of dyspareunia, especially in women less than 50 years old. Afflicted patients with vulvar vestibulitis syndrome complain of severe pain during sexual activity, often described as raw, red burning, and feeling like sandpaper or burnt tissue being rubbed. Most women experience the pain in the vulvar region with initial penetration. There is another group of women who do not experience pain during initial penetration, but experience severe pain upon deep penetration when the man’s perineum comes into contact with the woman’s perineum, resulting in the genital sexual pain. In the latter group of women, physicians are misguided to the cervix when the site of the pain trigger is really within the vulvar region. With vulvar vestibulitis syndrome, genital sexual pain may also be experienced with the use of ampons, during speculum examination, wearing tight pants, or straddling while cycling or horseback riding. Although there is no known pathophysiology, there are several possible pathophysiologic factors, including exposure to human papillomavirus, the irritant oxalate, abnormal immunologic conditions, psychopathology, and an abnormal sex steroid hormonal milieu.25

During physical examination, the diagnosis of vulvar vestibulitis syndrome is made by Q-tip testing. Q-tip point pressure mapping within the vestibule reveals localized areas without pain and localized areas with pain that can be graded on a 1-10 scale. Use of a spring-loaded Q-tip can result in a consistent pressure applied to the vestibule under all circumstances.26 In many cases, the regions of pain are consistent with redness and tenderness in the ostea of the minor vestibular glands in the labial-hymenal junction. The diagnosis of vulvar vestibulitis syndrome can be further established with a lidocaine diagnostic nerve block test. This is performed with a few milliliters of 1% lidocaine administered by insulin syringe and 31-gauge needle in the region of positive Q-tip testing. In vulvar vestibulitis syndrome, unlike generalized vulvodynia, lidocaine administration results in virtual complete loss of pain (Fig. 12.4.10).

The treatment of vulvar vestibulitis syndrome includes conservative measures, including education, support, counseling, physical therapy, and/or biofeedback. The pain trigger should be eliminated. Topical estrogen and topical xylocaine creams and/or ointments should be considered. Systemic medications include tricyclic antidepressants or gabapentin. In women with vulvar vestibulitis syndrome, unlike generalized vulvodynia, if medical management fails, surgery, such as vestibulectomy, can be considered.27-30

Vagina

Atrophic vaginitis

Unfortunately, contemporary clinical research data show that the use of exogenous estrogen by women in menopause for control of menopausal symptoms, such as hot flashes and night sweats, and for maintenance of urogenital health is perplexing and controversial. What is not controversial, however, is the role of estradiol in the genital tissues. Estradiol is a sex steroid that is critical for the structure and function of multiple genital tissues, especially the vagina. All three layers of the vagina, the endothelium, lamina propria, and muscularis, require estradiol for their integrity. Persistently low estradiol levels in the vaginal tissues result in atrophic vaginitis, which is associated with low estradiol states, including natural menopause, bilateral oophorectomy, gonadotropin-releasing hormone agonist use, hypothalamic amenorrhea, radiation, and chemotherapy.

The symptoms of atrophic vaginitis include vaginal dryness, dyspareunia, stinging, bleeding, and dysuria. On physical examination, women with atrophic vaginitis reveal vaginal mucosal changes. The classic, healthy-appearing vagina has a pink hue with vaginal folds and rugae; when touched with a Q-tip, it reveals a shiny, lubricating substance and, when rubbed with a Q-tip, it does not bleed. In atrophic vaginitis, the vagina changes to an unhealthy pale complexion, with a lack of vaginal folds and rugae, and a lack of lubricating substance on the surface, and the tissue bleeds with minimal contact (Fig. 12.4.11). On wet mount, the microscopic examination reveals parabasal cells, increased white blood cells, and absent background flora of lactobacilli. The vaginal pH is elevated to 6.0-7.0.

Figure 12.4.11. The classic, healthy-appearing vagina has a pink hue with vaginal folds and rugae; when touched with a Q-tip, it reveals a shiny, lubricating substance and, when rubbed with a Q-tip, does not bleed. In atrophic vaginitis, the vagina changes to an unhealthy pale complexion, with a lack of vaginal folds and rugae, and lubricating substance on the surface, and the tissue bleeds with minimal contact.

The conservative treatment involves the use of local topical vestibular and/or intravaginal estrogen. There are multiple products on the market, including intravaginal rings, and intravaginal pills and creams. In some rare patients, plasma estradiol levels may increase to values similar to those of systemic estrogen administration, so monitoring is required as indicated by the physician. Moreover, some patients are allergic to the various additives in the topical estradiol product. For example, some women react to the propylene glycol in several topical estrogen products. There are also multiple estrogen alternatives, such as soy, black cohosh, wild yam, chaste berry, angelica, damiana, nettle, red clover, and saw palmetto, although there are limited double-blind, placebo-controlled safety and efficacy trials with these products.31,32

Pelvic floor disorders (see Chapters 4.4 and 12.3)

In genital sexual pain disorders, spasms of the pelvic floor muscles may contribute to the overall pain and discomfort experienced by the patient. The spasms reflex to the spinal cord and back to the source of pain in a self-perpetuating circuit. Pain and spasm manifest as increased activity in the perineal muscles. Chronic spasm results in muscle anoxia responsible for deep, aching pain and additional genital sexual pain. Biofeedback utilizes an electromyographic transducer inserted into the vagina. The electromyographic output is connected to a computer for visual biofeedback. Physical therapists33 organize a training process of contraction and relaxation of pelvic floor muscles similar to Kegel exercises.

Conclusions

Genital sexual pain problems are common and often disabling. Genital sexual pain disorders are multifactorial syndromes of pain, sexual dysfunction, and psychologic disability. Ideal patient management involves a multimodal approach, including psychologic support, sexual therapy, physical therapy, and medical management of the pain. The aim of this chapter was to provide the biologic-concentrated health-care professional with germane clinical data to aid in the management of specific biologic-based pathophysiologies of genital sexual pain.

The major lessons from this chapter are that there are multiple physiologic causes for genital sexual pain and that, as in other medical issues, accurate diagnosis is essential to appropriate treatment. The basis for diagnosis is history and physical examination with appropriate laboratory testing. Additional research is needed in this very significant aspect of women’s sexual health.

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