Vulvodynia: a Common and Underrecognized Pain Disorder in Women and Female Adolescents Integrating Current
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21/4/2017 www.medscape.org/viewarticle/877370_print www.medscape.org This article is a CME / CE certified activity. To earn credit for this activity visit: http://www.medscape.org/viewarticle/877370 Vulvodynia: A Common and UnderRecognized Pain Disorder in Women and Female Adolescents Integrating Current Knowledge Into Clinical Practice CME / CE Jacob Bornstein, MD; Andrew Goldstein, MD; Ruby Nguyen, PhD; Colleen Stockdale, MD; Pamela Morrison Wiles, DPT Posted: 4/18/2017 This activity was developed through a comprehensive review of the literature and best practices by vulvodynia experts to provide continuing education for healthcare providers. Introduction Slide 1. http://www.medscape.org/viewarticle/877370_print 1/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 2. Historical Perspective Slide 3. http://www.medscape.org/viewarticle/877370_print 2/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 4. What we now refer to as "vulvodynia" was first documented in medical texts in 1880, although some believe that the condition may have been described as far back as the 1st century (McElhiney 2006). Vulvodynia was described as "supersensitiveness of the vulva" and "a fruitful source of dyspareunia" before mention of the condition disappeared from medical texts for 5 decades. Slide 5. http://www.medscape.org/viewarticle/877370_print 3/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 6. Slide 7. http://www.medscape.org/viewarticle/877370_print 4/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 8. Slide 9. Magnitude of the Problem http://www.medscape.org/viewarticle/877370_print 5/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 10. Slide 11. http://www.medscape.org/viewarticle/877370_print 6/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 12. Slide 13. Studies of women found that the incidence of symptom onset is highest between the ages of 18 and 25. Once thought to affect primarily white women, recent studies indicate that Hispanic women are significantly more likely to develop vulvodynia and may present with different vulvar pain subtypes. http://www.medscape.org/viewarticle/877370_print 7/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 14. Because vulvodynia is rarely covered in medical school curricula and residency programs, symptoms mimic those of common vulvovaginal infections, and, in many cases, no abnormalities of the vulvar tissue can be seen upon examination, women are often misdiagnosed. In 2003, the first federally funded populationbased epidemiologic study in Boston found that almost 60% of patients reported visiting ≥3 healthcare providers to receive a diagnosis, 40% of whom remained undiagnosed after 3 consultations. Using prevalence estimates of 3% to 7%, Xie and colleagues demonstrated the significant economic impact of vulvodynia in the United States: $31 to $72 billion in direct and indirect costs. http://www.medscape.org/viewarticle/877370_print 8/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 15. Slide 16. Anatomy and Neurobiology of the Urogenital Tract Slide 17. http://www.medscape.org/viewarticle/877370_print 9/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 18. The vulva is the external female genitalia. It includes the mons pubis, labia majora, labia minora, prepuce or clitoral hood, clitoris, and vulvar vestibule. The vulva is the center of a woman's sexual response. The anterior and posterior boundaries of the vulva extend from the mons pubis to the anus, respectively. Its lateral boundaries lie at the genitocrural folds. The mons pubis is comprised of a fat pad at the anterior of the vulva and is covered in pubic hair. The labia majora are derived embryologically from labioscrotal swellings. They fuse posteriorly and attach anteriorly to the mons pubis. The labia minora, hairless folds of skin embryologically derived from urethral folds, lie within the labia majora. The labia minora fuse anteriorly, forming the prepuce (hood) of the clitoris, and extend posteriorly to either side of the vaginal opening. They fuse posteriorly at the vulvar vestibule, creating a fold of skin called the posterior fourchette. The mons pubis, perineum, and labia are derived from the embryonic ectoderm. Vulvar skin is a keratinized, stratified, squamous epithelial structure that contains sebaceous glands and sweat glands. The keratin thickness of vulvar skin decreases progressively from the labia majora, over the clitoris to the labia minora. The vulvar vestibule extends from the frenulum of the clitoris anteriorly to the fourchette posteriorly. Hart's line marks the juncture of nonkeratinized epithelium of the vestibule and the keratinized epithelium of the inner surface of the labia minora. The vulvar vestibule, derived from the urogenital sinus endoderm, contains the major vestibular glands (Bartholin's and Skene's) and the minor vestibular glands. The vestibular glands secrete mucous during sexual arousal and orgasm. The clitoris is located under the prepuce and is embryologically derived from the genital tubercle. It is formed of erectile corpora cavernosa tissue, which becomes engorged with blood during sexual stimulation (Farage 2006; Krantz 1977; Woodruff 1985). http://www.medscape.org/viewarticle/877370_print 10/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 19. The vulva is innervated by the anterior labial branches of the (A) ilioinguinal nerve (L1); (B) genitofemoral nerve (L12); and (C, D) branches of the pudendal nerve (S24). Near the medial aspect of the ischial tuberosity, the pudendal nerve divides into 3 branches: (C) the dorsal nerve of the clitoris (shown deeper as dashed lines in muscles of the urogenital diaphragm), (D) the perineal nerve, which innervates the labia majora and perineum, and (E) the inferior rectal nerve, which innervates the perianal area. The pudendal nerve also innervates the external anal sphincter and deep muscles of the urogenital triangle (Peng 2009). For a thorough review of the neurobiology of the urogenital tract, see Wesselmann (1997). Slide 20. http://www.medscape.org/viewarticle/877370_print 11/69 21/4/2017 www.medscape.org/viewarticle/877370_print The pelvic floor muscles are divided into 3 categories. The superficial pelvic floor muscles (bulbocavernosus or bulbospongiosus, ischiocavernosus, superficial transverse perineal/perineus muscle) are collectively known as the urogenital diaphragm. The function of the urogenital diaphragm muscles includes a role in sexual function (eg, clitoral engorgement, vaginal closure, reflexive response to enhance sexual pleasure, and facilitating closure of the urethra and anus for continence). The middle layer is comprised of the deep transverse perineal muscle and sphincter urethra. The deep pelvic floor muscles, sometimes called the anal triangle, include the levator ani (pubococcygeus, iliococcygeus, and puborectalis), and coccygeus. Other associated pelvic and hip muscles include the piriformis, obturator internus muscles, and gluteus maximus. The perineal body is the central tendon and attachment site for the superficial, middle, and deep pelvic floor muscles. The internal pudendal artery, vein, and nerve, which pass through Alcock's canal, provide neurovascular function to the pelvic floor musculature. Alcock's canal is comprised of fascia or connective tissue from the obturator internus. Although the pudendal nerve is known to innervate the levator ani muscles, there have been more recent studies describing innervation by the levator ani nerve and direct nerve roots S3 and/or S4 (Barber 2002, Grigorescu 2008). Specifically, the pubococcygeus muscle is innervated by the levator ani nerve S35 and the perineal branch of the pudendal nerve S34. The iliococcygeus is innervated by the levator ani nerve S34, and the puborectalis is innervated by the inferior rectal branches of the pudendal nerve S2S4. The coccygeus is innervated by direct nerve roots S34. The function of the deep pelvic floor muscles includes supporting the abdominal viscera or organs, providing pelvic and spinal stability, assisting in respiration, and providing sphincteric closure for bowel and bladder function. They also play a role in sexual function. For a thorough review of the anatomy and physiology of the pelvic floor, see Herschorn 2004. Terminology and Classification Slide 21. http://www.medscape.org/viewarticle/877370_print 12/69 21/4/2017 www.medscape.org/viewarticle/877370_print Slide 22. The current classification differentiates: vulvar pain due to a known cause vs idiopathic chronic vulvar pain (ie, vulvodynia). Slide 23. Pain is first classified by location, ie, generalized (several areas of the vulva), localized (a specific area of the vulva), or mixed; secondly, it is classified by provocation (provoked, spontaneous, or mixed). It is also described by onset (either primary or secondary) and temporal pattern (intermittent, persistent, constant, immediate, or delayed). The 2 most common forms of vulvodynia are: generalized vulvodynia and provoked vestibulodynia (formerly Vulvar Vestibulitis http://www.medscape.org/viewarticle/877370_print 13/69 21/4/2017 www.medscape.org/viewarticle/877370_print Syndrome). It should be noted that women may have both a specific disorder (eg, lichen sclerosus) and vulvodynia. Please see Slide 26 for potential associated factors. Slide 24. Since 1975, many terms have been used to describe the 2 most common vulvodynia subtypes, causing confusion among the medical, scientific, and patient