Vulvodynia: Causes, Diagnosis and Modern Therapy

Overview and Prevalence

Vulvodynia is defined as chronic vulvar discomfort with a duration of at least three months. This discomfort can be expressed as pain, burning, itching, dyspareunia (painful sexual intercourse), stinging, rawness, or "irritation" of a constant or intermittent nature. The prevalence rate is not well-established, but an article by Martha Goetsch, M.D., (American Journal of Obstetrics and Gynecology, 1991) reported the prevalence rate of Vulvar Vestibulitis Syndrome to be 15% in her general gynecologic private practice. Early results from a survey by the National Vulvodynia Association found the mean age of women with vulvodynia to be 43.  Age ranges have been reported from 11-75 years.

Classification

Vulvodynia can be classified as primary, meaning the pain has been present since first sexual experience or tampon use, or as secondary, beginning after first tampon use or first sexual experience. It can also be classified as pure, occurring only with touch (such as with sexual intercourse, riding a bicycle, during wiping of the perineum after voiding) or as mixed, occurring both with touch and at other times (e.g., spontaneously when one is sitting). It can be organic, i.e., have an identifiable cause, or idiopathic, meaning it does not have an identifiable cause. There are several classification schemes for dyspareunia (painful sexual intercourse). For example, it can be classified according to type and severity. The type can be superficial, i.e., located only at the opening of the vagina, or deep, i.e., located deep within the vaginal cavity, or both. Dyspareunia can also be classified according to its severity. In Type 1, sexual intercourse causes discomfort but does not prevent intercourse from occurring; in Type 2, the pain frequently prevents intercourse; and in Type 3, intercourse is completely prevented because of pain. 

Nervous Input to  the Vulva

To understand vulvodynia, it is important to review the anatomy of the nerve supply in the pelvis. The vulva receives nervous input from three major nerves: the pudendal, ilioinguinal, and genitofemoral. The pudendal nerve originates from the S2-S4 region of the spinal cord and has three branches: the inferior hemorrhoidal (rectal) nerve, the perineal nerve, and the dorsal nerve of the clitoris. It functions to transmit sensory signals to the genitalia and perineum and also supplies motor function to the pelvic floor and external sphincters of the urethra and rectum. Both the ilioinguinal and genitofemoral nerves originate in the L1-L2 area of the spinal cord; they have sensory functions, but not motor functions. The ilioinguinal nerve transmits sensory signals to the inguinal (groin) area, symphysis (mons pubis) and anterior vulva. The genitofemoral nerve transmits sensory signals to the anterior vulva and the labia majora. Types of Neuropathic Pain Many physicians have conceptualized vulvodynia as neuropathic (nerve) pain, which can be classified as one of the following three types. Allodynia is the term used to describe a painful response to a stimulus that does not ordinarily cause pain; for example, touching a healthy person’s arm with a feather does not normally cause a painful sensation, but if such touch was perceived as painful, the sensation would be classified as allopathic pain. Hyperalgesiadescribes an exaggerated pain reaction to a stimulus that is normally painful. Dysesthesia is the perception of an unpleasant and abnormal sensation produced by a non-noxious stimulus. Women with vulvodynia may experience all three types of neuropathic pain, but many pain specialists consider vulvodynia to be primarily an allodynia. 

Nociceptors are receptors that transmit pain caused by injury to body tissue. There are many types of nociceptive damage which can cause vulvar pain. Nociceptive damage can result from neurologic compression, either from a stretch injury, transection (cutting) of a nerve, or infection of a nerve. It also can occur from pelvic floor descent, i.e., prolapse of the pelvic floor with stretching of the pudendal nerve through "Alcock's canal." (Alcock's canal is an area where the pudendal nerve runs very near to the hipbone).

Vulvar pain also can result from damage to nerves from tumors, cysts or surgery; soft tissue injury from an episiotomy or vaginal delivery; laser ablation of the vulvar skin; a car accident; a straddle injury; vaginal atrophy caused by a lack of estrogen; chronic skin irritation from application of medications such as steroids or Aldara and infective agents such as viruses (e.g., HPV). Furthermore, vaginal surgery can increase the risk of pudendal nerve injury. 

DIAGNOSIS

A variety of factors need to be considered to make an accurate diagnosis of vulvodynia. Initially, it is important to take a careful medical history. In particular, one should look for an initiating factor such as surgery, childbirth, or change of sexual partner. Other factors to explore are a relationship between vulvodynia and the menstrual cycle; urethral symptoms; other medical conditions; any medications used ( past or present); any family history (other female family members who have experienced similar symptoms); the use of menstrual pads or panty liners; trauma to the perineum from excessive exercising or bicycle riding; and contraception use. 

Physical Examination

There are multiple aspects of the gynecologic examination. To minimize pain on the patient’s first visit, the use of an intravaginal speculum may not be necessary. Since pain is usually located on the vulva, a careful, thorough examination of this area may be all that is required initially to localize the pain. To start, it is important to evaluate the following: the size and shape of the clitoris, the clitoral hood and labia minora; possible inflammation of the Bartholin glands and minor vestibular glands (using the Q-tip test); and Fox Fordyce spots (normal anatomic spots on the labia minora). The labia should be examined for lesions, the perianal area should be checked for whiteness and/or fissuring, and the general color of the genital and anal skin should be noted. Next, a neurological examination should be carried out, evaluating reflexes in the vaginal and rectal areas, as well as allodynia.

During the internal examination of the vagina, the pelvic muscles should be palpated. Areas surrounding the vulva should be evaluated for pain including any coccygeal (tailbone) pain, mons pubis (area around the pubic bone) pain, upper inner thigh pain, pyriformis (pelvic floor muscle) pain, and sciatic pain. Any pelvic prolapse should also be noted. Colposcopic examination should be performed in some cases to rule out conditions such as condylomatous vaginitis, lichenoid vaginitis, lichen planus, and other dermatologic conditions. If there are any suspicious lesions, a biopsy should be performed to confirm the diagnosis.

Laboratory Testing

In some cases, an array of laboratory tests are also necessary. These include potassium hydroxide and normal saline wet mounts for microscopic examination of vaginal secretions. This evaluation also can be done with the vulvar skin and perianally, if needed. Laboratory tests should be performed to rule out metabolic diseases such as hypothyroidism, hyperthyroidism, glucose intolerance, and autoimmune diseases. In order to rule out menopause or premature ovarian failure, blood levels of certain hormones such as serum estradiol and follicle stimulating hormone should be measured.

Multidisciplinary Approach

Ideally, the evaluation and treatment of vulvodynia should involve a multi-disciplinary approach. A neurological examination, including an EMG of the pelvis, should be completed. A gastroenterologist may be needed to examine sphincter integrity and check for intestinal inflammatory disease. A dermatologist should evaluate if the pain is resulting from any dermatoses. To manage pain medications and evaluate trigger points, an anesthesiologist may be needed. If the patient exhibits depression, a referral to a psychiatrist may be appropriate. A physical therapist skilled in the use of biofeedback may be needed for a biofeedback evaluation of the pelvic floor musculature. In certain cases in which the patient has undergone prior vulvar surgery, a consultation with a plastic surgeon may be indicated if major skin grafting is required at subsequent surgery. Finally, a referral to a vulvar disease specialist may be appropriate.

TREATMENT

Once the initial work up and all necessary referral visits have been completed, it is important to treat any underlying conditions found during the course of the examinations. Some of these underlying conditions are infections: if yeast is found, antifungals are prescribed; if bacterial vaginosis is found, antibacterials are prescribed; for herpes simplex, antivirals are used; for human papillomavirus, ablative therapy (application of certain topical biochemicals) is performed; if there’s a Bartholin gland abscess, the gland should be excised.

All possible trauma to the area should be avoided including wearing tight clothing, excessive exercising, and motorcycle or bicycle riding. The physician should repair vaginal prolapses and revise scars created during previous surgeries, vaginal deliveries, or pelvic floor injury.

Any medications or vaginal products that might be causing an allergic reaction should be discontinued including anesthetics, topical antibiotics, antifungals, antiseptics, corticosteroids, and spermicides. All materials that come in contact with the vulvar region such as condoms, latex, rubber, and panty liners, as well as over-the-counter personal hygiene products which may contain additives that are irritating to the vulva should be avoided. Substances that sting upon application, e.g., soaps, alcohol, douches, and gentian violet, should also be avoided.

In some cases, vulvar symptoms may be the result of dermatological disorders such as lichen sclerosus, eczema, atrophic vulvitis, or contact dermatitis. Some other dermatological conditions to be ruled out are Candida vulvitis, lichen simplex, Herpes simplex and Crohn’s disease of the vulva. Vaginal or vulvar atrophy due to perimenopause, menopause, or prolonged breast feeding can be treated with hormone therapy. Similarly, one should treat any underlying metabolic diseases such as Crohn’s, Sjogren’s syndrome, and Systemic Lupus Erythematosus to see if vulvar symptoms are eliminated.

Patients with chronic vulvar pain which does not result from an underlying disease and does not have an identifiable cause are treated with an "antineuralgic" medication. The classic approach is to prescribe a tricyclic depressant such as Elavil, beginning with doses as low as 5 or 10 mg. daily to minimize side effects. Typically, doses are gradually increased to a maximum of 50-100mg. The difficulty with prescribing Elavil is that many patients discontinue the drug because of its sedating side effect. Tricyclic antidepressants with a better side effect profile include desipramine and nortriptyline. The serum serotonin reuptake inhibitors such as Zoloft and Paxil also may be prescribed. These medications have had most benefit in chronic pain syndromes such as fibromyalgia. They can be very useful because they have a less sedating effect than the tricyclics. Alternately, or in conjunction with an antidepressant, anticonvulsants such as Tegretol, Depakote, or Neurontinmay be prescribed for pain relief. Topical anesthetic agents that may provide short-term relief include Xylocaine (lidocaine), Hurricaine (benzocaine), and Zonalon (topical doxepin). In some cases, a referral to a pelvic floor dysfunction therapist for biofeedback training may be recommended. Finally, if the patient has Vulvar Vestibulitis Syndrome, surgery may be recommended. An important part of treatment is helping the patient to develop realistic outcome goals. In general, the shorter the duration of the syndrome, the better the outcome. The patient needs to develop positive coping mechanisms to control the pain such as exercise, distraction techniques, and active participation in managing the pain.

VULVAR VESTIBULITIS

Etiology

The etiology of Vulvar Vestibulitis Syndrome (VVS) is often idiopathic, i.e., without identifiable cause. Dyspareunia at the entrance to the vagina is the essential characteristic of this condition. Sometimes it occurs secondary to dermatological conditions such as lichen sclerosus or desquamative vaginitis. Frequently, the initiating factor in VVS appears to be a history of serial antibiotic use for urinary tract infections, acne, sinus infections, or vaginitis, or the use of highly progestational agents such as certain birth control medications. Symptoms always intensify from the use of panty liners or menstrual pads. VVS involves inflammation of the Bartholin glands and/or the minor vestibular glands at the base of the hymen.

Therapy

Treatment begins by instructing the patient to stop attempts at intravaginal penetration and inviting the husband/partner to the patient’s next examination. Topical estrogen such as Estrace cream should be applied to gland openings to relieve symptoms of burning and reduce inflammation. The use of topical and intravaginal steroids also may be beneficial.

Some of the commonly used treatments for VVS do not appear to have a high success rate. One of the popular alternative methods, the low-oxalate diet plus calcium citrate, does not work for most VVS patients. Likewise, oral corticosteroids are ineffective. Interferon injections, a fairly common medical treatment, also has not been successful in many cases. Another treatment sometimes used, the superficial lasering of the vestibular gland openings, has actually made symptoms worse in many patients.

For most patients with VVS, surgery appears to have the highest success rate, especially if the condition has been of short duration. Of 127 vestibulectomies performed at WomenNow Health care, 108 women were evaluated five years after surgery. “Success” was defined as the ability to engage in sexual intercourse without pain and with normal frequency. “Improved” was defined as the ability to engage in intercourse with occasional discomfort during or afterwards; the experienced discomfort, however, did not inhibit frequency of episodes.

Seventy-five percent of patients were determined to be a success post-surgically, eighteen percent were improved and three percent were failures. No patient’s symptoms worsened as a result of surgery. Complete removal of both the major and minor vestibular glands was critical to success of the procedure. Fifteen percent of patients subsequently became pregnant. Eight percent required a second procedure such as a skin graft at the perineal body posteriorly, removal of a suture granuloma, or, in one case, a second surgery to remove a gland that wasn’t removed during the first procedure.

SUMMARY

Chronic vulvar pain, whether intermittent or constant, can be a debilitating symptom that deserves appropriate work-up and disease specific therapy. As the millenium draws to a close, it is unwarranted and unjustified that a woman should have to live with the fear of suffering from such pain or being told that it’s “all in your head.”

There is hope associated with treating this disorder, and vulvodynia patients need to be directed toward health care providers who are interested, dedicated and competent in helping them. In particular, surgery for Vulvar Vestibulitis Syndrome should no longer be considered a last effort, because there is excellent data reported from various investigators nationwide showing high success rates with this form of therapy. Such surgery does not have to be cosmetically disfiguring and can help to restore normal sexual function between loving couples.

References:

Summer 1999 NVA News (Volume V, Issue III)