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International Journal of Urology (2014) 21 (Suppl 1), 85–88
doi: 10.1111/iju.12379
Original Article
Gynecological disorders in bladder pain syndrome/interstitial cystitis patients Mauro Cervigni1,2 and Franca Natale2 1
Obstetrics and Gynecology Department, Catholic University, and 2Urogynecologic Department, S.Carlo-IDI, Rome, Italy
Abbreviations & Acronyms BPS/IC = bladder pain syndrome/interstitial cystitis CPP = chronic pelvic pain HPFD = hypertonic pelvic floor dysfunction IC = interstitial cystitis LPFD = low-tone pelvic floor dysfunction Correspondence: Mauro Cervigni M.D., Obstetrics and Gynecology Department, Catholic University, Largo Agostino Gemelli, 00100 Rome, Italy. Email: [email protected] Received 26 September 2013; accepted 19 November 2013.
Objectives: Bladder pain syndrome/interstitial cystitis, a chronic inflammatory condition of the bladder, is the source of pain in over 30% of female patients with chronic pelvic pain. The aim of the present study was to evaluate the most frequent associations between bladder pain syndrome/interstitial cystitis and gynecological disorders. Methods: A literature review of the previous 10 years was carried out to evaluate the incidence of gynecological diseases in patients with bladder pain syndrome/interstitial cystitis. Results: Hypertonic pelvic floor dysfunction with associated voiding dysfunction can be present in bladder pain syndrome/interstitial cystitis patients. It has been estimated that the prevalence ranges from 50% to 87%. Endometriosis affects 1–7% of the general population and up to 70% of women with endometriosis have some type of pain symptoms, a recent systematic review estimated the prevalence of bladder pain syndrome to be 61%, of endometriosis to be 70%, and coexisting bladder pain syndrome and endometriosis to be 48%. Vulvodynia is represented by pain, or an unpleasant altered sensation, in the vulva. Women with vestibulodynia are likely to have other additional pain conditions, such as fibromyalgia, irritable bowel syndrome or chronic fatigue syndrome. Recent data reported that vestibulodynia affects 25% of women with bladder pain syndrome/interstitial cystitis. Conclusions: Bladder pain syndrome/interstitial cystitis is a complex pathology often associated with vulvodynia, endometriosis and pelvic floor dysfunctions. Therefore, it is of utmost importance to obtain an accurate evaluation ruling out confusable disease, such as pudendal neuropathy. The optimal approach is a combined treatment oriented not only to treat the bladder, but also the other components responsible for the pain disorder.
Key words: bladder pain syndrome, endometriosis, hypertonic pelvic floor dysfunction, interstitial cystitis, vulvodynia.
Introduction BPS/IC is a chronic inflammatory condition of the bladder. This syndrome is the cause of pain in more than 30% of females with chronic pelvic pain. In 37% of patients, CPP is due to gastrointestinal involvement, in 20% to gynecological diseases and in 12% to musculoskeletal pathologies.1 Frequently, CPP is characterized by an overlapping of these different conditions. The aim of the present study was to evaluate the most frequent associations between BPS/IC and gynecological disorders.
Pelvic floor dysfunction Pelvic floor dysfunction affects the anterior, apical or posterior vaginal compartment. There are two types of dysfunction: hypotonic or LPFD and HPFD (see Table 1). Many patients with BPS/IC have concomitant HPFD, with muscle tenderness and spasms, and voiding dysfunction, both manifestations of pelvic floor hypertonicity.2 It has been estimated that the prevalence of HPFD in patients with BPS/IC ranges from 50% to 87%.3 Pelvic floor dysfunction exacerbates BPS/IC symptoms, and has been reported to appear in response to events such as bladder inflammation, gait disturbance, and trauma.4 Other pain disorders, such as irritable bowel syndrome, inflammatory bowel disease, fibromyalgia, and vulvodynia are all found to have a high prevalence in HPFD and myofascial pain.5 All these disorders are frequently associated with BPS/IC. © 2014 The Japanese Urological Association
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Table 1
Types of pelvic floor dysfunctions
Hypotonic disorders
Hypertonic disorders
Stress urinary incontinence Pelvic organ prolapse Fecal incontinence
Overactive bladder BPS/IC Vulvodynia CPP Overactive bowel Sexual dysfunction
Pathophysiology In a normal bladder, the peripheral way of transmission is mediated by A∂-fibers that transfer tension, pain and cold. Instead, the C-fibers transfer burning, heat, pain and itching; they are normally silent, only becoming active in response to bladder inflammation or irritation. An inflammatory disorder of the pelvic viscera, a trauma or exceptional behavior might elicit noxious stimuli to the sacral cord that sets up a pelvic floor muscle dysfunction with sacral nerve hypersensitivity and a sacral cord wind-up.6 The guarding reflex is a viscero-muscular reflex activated with the aim of increasing the tone of the pelvic floor during routine daytime activity. In BPS/IC patients, there is an afferent autonomic bombardment that can enhance and maintain a guarding reflex that manifests itself as a hypertone of the pelvic floor.
Physical examination Patients with HPFD are unable to produce more contractile strength and therefore cannot produce an effective squeeze. A single finger can be introduced in the vagina to assess pelvic floor awareness, and the ability to squeeze and relax the levator ani. Often patients with HPFD will have a “V” configuration of the introitus and, as a finger is advanced, it will drop off the shelf caused by the contracted levator muscles. Active “trigger points” are often identified by an exquisitely tender area palpable at the level of the pelvic side wall within a taut band that reproduces the patient’s pain, as well as the referral pattern of her pain.
Diagnostic studies Muscle activity can be measured using a perineometer or an electromyography probe.7 Urodynamics include fluctuating or interrupted flow, abnormal voiding studies, elevated urethral pressure and urethral instability. Schmidt and Vapnek observed pain episodes in such patients coinciding with behavioral increase in the sphincter tone, more than in the bladder.8 When symptoms involve obstructed defecation and rectal pain, defecography should be used to identify the presence of a non-relaxing pelvic floor or even parodoxic activity of the pelvic floor during defecation.
Treatment The pelvic floor therapy should be considered as a first-line treatment in the case of HPFD. The goal of these stretching exercises is to lengthen the contracted muscles by decreasing 86
tension, releasing trigger points in the levator muscles, re-educating the muscles to a normal range of motion and improving patient awareness. The therapy also includes: behavior modification, muscle relaxants, Thiele’s massage, sacral and tibial neuromodulation, trigger point injections, and botulinum toxin.
Endometriosis Endometriosis is the presence of endometrial glands or stroma outside of the endometrial cavity and affects 1–7% of the general population.9 Up to 70% of women with endometriosis have some type of pain symptoms, most commonly dysmenorrhea, cyclic pelvic pain or deep dyspareunia.10 In women who undergo a laparoscopy to evaluate CPP, the prevalence of endometriosis is 30–90%.11 Endometriosis pain is usually a visceral pain, and endometriotic lesions produce inflammatory mediators, particularly prostaglandins F2-α and E2.12 There is a high prevalence and association of IC and endometriosis. A study by Chung et al. of 178 women with CPP found that 65% of CPP patients suffered from both active endometriosis and IC.13 In a prospective study carried out of 162 patients with CPP, Paulson and Delgado found that 66% of the sample was diagnosed with both endometriosis and IC.14 A recent systematic review estimated the prevalence of BPS/ IC, and the coexistence of BPS/IC and endometriosis in women with CPP. Nine studies including 1016 patients with CPP showed the mean prevalence of BPS was 61%, of endometriosis 70%, and coexisting BPS and endometriosis 48% (range 16–78%, CI 44–51%). These data suggest the importance of considering the bladder as the source of pain even where endometriosis is confirmed, and in the case of unresolved endometriosis and persistent pelvic pain, patients must be evaluated to rule out the presence of BPS/IC.15
Vulvodynia and vestibulodinia Vulvodynia, also known as vulvar vestibulitis or vulvar dysesthesia syndrome, literally means pain, or an unpleasant altered sensation, in the vulva. Pain can be unprovoked, varying from constant to intermittent, or occurring only on provocation, such in sexual intercourse. The International Society for the Study of Vulvovaginal Disease defines vulvodynia as “vulvar discomfort, most often described as burning pain, occurring in the absence of relevant visible findings or a specific, clinically identifiable, neurologic disorder”. This “burning pain” is suggestive of a neuropathic pain response. Classification of the vulvar pain might include localized or generalized pain, or both, provoked or non-provoked and primary or secondary.16 Sometimes an area of redness might be visible, but more often the vagina and the vulva show no abnormalities on gynecological or dermatological evaluation. This pain can affect women’s sexual life, which makes sex painful and, in some cases, impossible. Women with vestibulodynia are likely to have at least two additional pain conditions, such as fibromyalgia, irritable bowel syndrome, BPS/IC or chronic fatigue syndrome.17 © 2014 The Japanese Urological Association
BPS/IC: Gynecological disorders
Peters et al. reported that vestibulodynia affects 25% of women with PBS/IC.18 The etiology of vulvodynia is presumed to involve many factors: infections and altered vaginal acidbase balance, and the upregulation of pro-inflammatory immune responses.19 Furthermore, a large community-based study found that vulvodynia was strongly associated with childhood physical or sexual abuse.20 Because both the vestibule of the vulva and the bladder are derived from the urogenital sinus, it could be hypothesized that the coexistence of vulvodynia and BPS/IC in some patients represents a generalized disorder of urogenital sinus-derived epithelium.21 From a histological point of view, various noxious stimula could cause changes in the vulvar epithelium: contraction in the pelvic floor, and mast cells activation with subsequent degranulation and release of histamine. This causes chronic pain and inflammation through the stimulation of peripheral neurons of the autonomic nervous system, an upregulation of the pain system, and a possible shift from nociceptive to neuropathic pain.22 The standard clinical test for vulvodynia is the cotton swab (Q-tip) test, measuring vulvar pain ratings on a visual analog scale.23
Therapy Several studies have shown that gabapentin has a role as tricyclic, yielding significant results in reducing pain perception.24 More than 80% of patients have reported improvements.25 More recently, botulinum toxin has been proposed for the treatment of vulvodynia. It was found that the visual analog scale score was reduced from 8.1 to 2.5 (P < 0.001), and eight (72.7%) out of 11 patients were satisfied.26
Pudendal neuropathy Pudendal neuropathy is a common feature of syndromes such as dysfunctional voiding, non-obstructive urinary retention, chronic pelvic pain syndromes, and urinary and fecal incontinence. It could be ruled out as a confusable disease in BPS/IC patients. Pudendal neuralgia is a functional entrapment of the pudendal nerve, and pain occurs during compression or stretch maneuvers, such as repetitive microtrauma, orthopedic fracture, straining with constipation and childbirth, falls onto the buttocks, and suture entrapment during pelvic surgery. The main symptom is pain aggravated by sitting/driving/exercise, reduced by recumbence or standing and relieved by sitting on a toilet. The quality of neuropathic pain varies and can be described as burning, stabbing, ache, or pressure, and can be induced by voiding, defecating, vaginal penetration or orgasm. It can occur anywhere in the pudendal territory, but primarily includes the perineum and urethra, and extends to suprapubic, inguinal regions and to the upper medial thighs. Urinary symptoms and rectal dysfunction might occur. Foreign body sensation in the rectum, vagina, urethra or perineum is frequent. Sexual dysfunction could be present. Females might suffer reduced clitoral sensation, pain at vaginal penetration, reduced lubrication and anorgasmia. © 2014 The Japanese Urological Association
Pinprick sensation is tested bilaterally at the level of the clitoris posterior labia and posterior perianal skin. Hyperalgesia is more common than hypoalgesia. Pressure is placed at the level of the Alcock canal attempting to reproduce pain, bladder or rectal symptoms (the Valleix phenomenon). The parasacral area is also examined for a back mouse (episacroiliac lipoma). Several tests can measure pudendal neuropathy including: biothesiometry, sacral latency test, sensory-evoked potentials, motor-evoked potentials and motor latency tests.
Management Pharmacotherapy Tricyclic antidepressants are the first medication category effective in placebo-controlled trials. Other drugs, such as gabapentin, pregabalin, oxcarbazepine, tramadol and duloxetine, significantly reduce pain and improve sleep, mood, and quality of life.
Mininvasive approach Transgluteal pudendal nerve blocks Two injections are given at the ischial spine at 1-month intervals. A third is given into the Alcock canal using computed tomography guidance.
Surgical therapy Transperineal and transgluteal approach The pudendal nerve decompression by the perineal route is a blind procedure carried out under local or regional anesthesia. To suppress the blind character of the procedure, a transgluteal approach has been proposed and the reported surgical success rates range from 60% to 70%. Pain-free status might take some years. Bladder, bowel and sexual dysfunctions show variable improvement.27 More recently, a transvaginal approach has also been proposed.28 Until now, the results on pain are the same as those obtained by the Shafik’s approach,29 but with the concurrent sections of one or two ligaments of the pelvis (sacro-spinal and/or sacro-tuberous ligaments). However, the long-term effects of these sections on the stability of the pelvic region are as yet unknown. Up to now, no data are available about a potential effect of the transgluteal or transvaginal procedures on urinary or anal incontinence.
Conclusions CPP in women can be related in more than 30% of patients to BPS/IC, which is frequently associated with pelvic floor dysfunction. For this group of patients, it is mandatory to observe not only the bladder but also all the other organs within the pelvis. Therefore, it is of utmost importance to evaluate concomitant pathologies, such as vulvodynia, endometriosis and pelvic floor dysfunctions, ruling out confusable disease, such as pudendal neuropathy. An optimal approach is a combined treatment oriented not only to treat the bladder, but also the other components responsible for the pain disorder. Patients with bladder tenderness 87
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alone responded better than patients with multiple tender trigger points, possibly because in these patients the bladder is the only target organ and the patients are less severely affected than patients with multiple trigger points. Multimodal therapy remains the gold standard in the management of BPS/IC patients.
Conflict of interest None declared.
References 1 Zondervan KT, Yudkin PL, Vessey MP et al. Prevalence and incidence of chronic pelvic pain in primary care: evidence from a national general practice database. Br. J. Obstet. Gynaecol. 1999; 106: 1149–55. 2 Butrick C. Interstitial cystitis and chronic pelvic pain: new insights in neuropathology, diagnosis, and treatment. Clin. Obstet. Gynecol. 2003; 46: 811–23. 3 De Paepe H, Renson C, Van Laecke E et al. Pelvic-floor therapy and toilet training in young children with dysfunctional voiding nad obstipation. BJU Int. 2000; 85: 889–93. 4 Travell JG, Simons DG. Myofascail Pain and Dysfunction: The Triggerpoint Manual. Williams and Wilkins, Baltimore, MD, 1992. 5 Alagiri M, Chottiner S, Ratner V, Slade D, Hanno PM. Interstitial cystitis: unexplained associations with other chronic diseare and pain syndromes. Urology 1997; 49 (5A Suppl): 52–7. 6 Butrick CW. Pelvic floor Hypertonic disorders: identification and management. Obstet. Gynecol. Clin. North Am. 2009; 36: 707–22. 7 Whitmore K, Kellogs-Spadt S, Fletcher E. Comprehensive assessment of the pelvic floor. Issues Incontinence 1998; Fall: 1–10. 8 Pezzone MA, Liang R, Frazer MO. A model of neural cross-talk and irritation in the pelvis: implications for the overlap of chronic pelvic pain disorders. Gastroenterology 2005; 128: 1953–64. 9 Barbieri RL. Etioloy and epidemiology of endometriosis. Am. J. Obstet. Gynecol. 1990; 162: 565–7. 10 Ballweg ML. Impact of endometriosis on momen’s health: comparative historical data show that the earlier the onset, the more severe the disease. Best Pract. Res. Clin. Obstet. Gynecol. 2004; 18: 201–18. 11 Howard FM. The role of laparoscopy in chronic pelvic pain: promise and pitfalls. Obstet. Gynecol. Surv. 1993; 48: 357–87. 12 Lessey BA, Metzger DA, Haney AF et al. Immunohistochemical analysis of oestrogen and progesterone receptors in endometriosis: comparison with normal endometrium during the menstrual cycle and the effect of medical therapy. Fertil. Steril. 1989; 51: 409–15.
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13 Chung MK, Chung RP, Gordon D. Interstitial cystitis and endometriosis in patients with chronic pelvic pain: the “Evil Twins” syndrome. JSLS 2005; 9: 25–9. 14 Paulson JD, Delgado M. The relationship between interstitial cystitis and endometriosis in patients with chronic pelvic pain. JSLS 2007; 11: 175–81. 15 Tirlapur SA, Kuhrt K, Chaliha C et al. The “Evil Twin Syndrome” in chronic pelvic Pain: a systematic review of prevalence studies of bladder pain syndrome and endometriosis. Int. J. Surg. 2013; 11: 233–7. 16 Leclair CM, Goetsch MF, Korcheva VB et al. Differences in primary compared with secondary vestibulodynia by immunohistochemistry. Obstet. Gynecol. 2011; 117: 1307–13. 17 Nguyen RHN, Veasley C, Smolenski D. Latent class analysis of comorbidity patterns among women with generalized and localized vulvodynia: preliminary findings. J. Pain Res. 2013; 6: 303–9. 18 Peters K, Girdler B, Carrico D, Ibrahim I, Diokno A. Painful bladder syndrome/interstitial cystitis and vulvodynia: a clinical correlation. Int. Urogynecol. J. Pelvic Floor Dysfunct. 2008; 19: 665–9. 19 Lowenstein L, Vardi Y, Deutsch M et al. Vulvar vestibulitis severity – assessment by sensory and pain testing modalities. Pain 2004; 107: 47–53. 20 Harlow BL, Stewart EG. Adult-onset vulvodynia in relation to childhood violence victimization. Am. J. Epidemiol. 2005; 161: 871–80. 21 Fitzpatrick CC, DeLancey JOL, Elkins TE, McGuire EJ. Vulvar vestibulitis and Interstitial Cystitis: a disorder of urogenital sinus derived epithelium. Obstet. Gynecol. 1993; 81: 860–2. 22 Glazer HI, Rodke G, Swencionis C, Herts R, Young AW. Treatment of vuvlvar vestibulitis syndrome with electromiographic biofeedback of pelvic floor musculature. J. Reprod. Med. 1995; 40: 283–90. 23 Pukall CF, Binik YM, Khalife S, Amsel R, Abbot FV. Vulvar tactile and pain thresolds in women with vulvar vestibulitis syndrome. Pain 2002; 96: 163–75. 24 van Lankveld JJ, Granot M, Weijmar Schultz WC et al. Women’s sexual pain disorders. J. Sex. Med. 2010; 7 (1 Pt 2): 615–31. 25 Ben-David B, Friedman M. Gabapentin therapy for vulvodynia. Anesth. Analg. 1999; 89: 1459–60. 26 Jeon Y, Kim Y, Shim B et al. A retrospective study of the management of vulvodynia. Korean J. Urol. 2013; 54: 48–52. 27 Robert R, Labat JJ, Bensignor M et al. Bases anatomiques de la chirurgie du nerf pudendal. Conséquences thérapeutiques dans certaines algies périnéales. Lyon Chir. 1993; 89: 183–7. 28 Bautrant E, de Bisschop E, Vaini-Elies V et al. La prise en charge moderne des névralgies pudendales. A partir d’une série de 212 patientes et 104 interventions de décompression. J. Gynecol. Obstet. Biol. Reprod. (Paris) 2003; 32: 705–12. 29 Shafik A. Pudendal canal syndrome. Description of a new syndrome and its treatment. Report of 7 cases. Coloproctology 1991; 13: 102–10.
© 2014 The Japanese Urological Association
International Journal of Urology (2014) 21 (Suppl 1), 85–88
doi: 10.1111/iju.12379
Original Article
Gynecological disorders in bladder pain syndrome/interstitial cystitis patients Mauro Cervigni1,2 and Franca Natale2 1
Obstetrics and Gynecology Department, Catholic University, and 2Urogynecologic Department, S.Carlo-IDI, Rome, Italy
Abbreviations & Acronyms BPS/IC = bladder pain syndrome/interstitial cystitis CPP = chronic pelvic pain HPFD = hypertonic pelvic floor dysfunction IC = interstitial cystitis LPFD = low-tone pelvic floor dysfunction Correspondence: Mauro Cervigni M.D., Obstetrics and Gynecology Department, Catholic University, Largo Agostino Gemelli, 00100 Rome, Italy. Email: [email protected] Received 26 September 2013; accepted 19 November 2013.
Objectives: Bladder pain syndrome/interstitial cystitis, a chronic inflammatory condition of the bladder, is the source of pain in over 30% of female patients with chronic pelvic pain. The aim of the present study was to evaluate the most frequent associations between bladder pain syndrome/interstitial cystitis and gynecological disorders. Methods: A literature review of the previous 10 years was carried out to evaluate the incidence of gynecological diseases in patients with bladder pain syndrome/interstitial cystitis. Results: Hypertonic pelvic floor dysfunction with associated voiding dysfunction can be present in bladder pain syndrome/interstitial cystitis patients. It has been estimated that the prevalence ranges from 50% to 87%. Endometriosis affects 1–7% of the general population and up to 70% of women with endometriosis have some type of pain symptoms, a recent systematic review estimated the prevalence of bladder pain syndrome to be 61%, of endometriosis to be 70%, and coexisting bladder pain syndrome and endometriosis to be 48%. Vulvodynia is represented by pain, or an unpleasant altered sensation, in the vulva. Women with vestibulodynia are likely to have other additional pain conditions, such as fibromyalgia, irritable bowel syndrome or chronic fatigue syndrome. Recent data reported that vestibulodynia affects 25% of women with bladder pain syndrome/interstitial cystitis. Conclusions: Bladder pain syndrome/interstitial cystitis is a complex pathology often associated with vulvodynia, endometriosis and pelvic floor dysfunctions. Therefore, it is of utmost importance to obtain an accurate evaluation ruling out confusable disease, such as pudendal neuropathy. The optimal approach is a combined treatment oriented not only to treat the bladder, but also the other components responsible for the pain disorder.
Key words: bladder pain syndrome, endometriosis, hypertonic pelvic floor dysfunction, interstitial cystitis, vulvodynia.
Introduction BPS/IC is a chronic inflammatory condition of the bladder. This syndrome is the cause of pain in more than 30% of females with chronic pelvic pain. In 37% of patients, CPP is due to gastrointestinal involvement, in 20% to gynecological diseases and in 12% to musculoskeletal pathologies.1 Frequently, CPP is characterized by an overlapping of these different conditions. The aim of the present study was to evaluate the most frequent associations between BPS/IC and gynecological disorders.
Pelvic floor dysfunction Pelvic floor dysfunction affects the anterior, apical or posterior vaginal compartment. There are two types of dysfunction: hypotonic or LPFD and HPFD (see Table 1). Many patients with BPS/IC have concomitant HPFD, with muscle tenderness and spasms, and voiding dysfunction, both manifestations of pelvic floor hypertonicity.2 It has been estimated that the prevalence of HPFD in patients with BPS/IC ranges from 50% to 87%.3 Pelvic floor dysfunction exacerbates BPS/IC symptoms, and has been reported to appear in response to events such as bladder inflammation, gait disturbance, and trauma.4 Other pain disorders, such as irritable bowel syndrome, inflammatory bowel disease, fibromyalgia, and vulvodynia are all found to have a high prevalence in HPFD and myofascial pain.5 All these disorders are frequently associated with BPS/IC. © 2014 The Japanese Urological Association
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Table 1
Types of pelvic floor dysfunctions
Hypotonic disorders
Hypertonic disorders
Stress urinary incontinence Pelvic organ prolapse Fecal incontinence
Overactive bladder BPS/IC Vulvodynia CPP Overactive bowel Sexual dysfunction
Pathophysiology In a normal bladder, the peripheral way of transmission is mediated by A∂-fibers that transfer tension, pain and cold. Instead, the C-fibers transfer burning, heat, pain and itching; they are normally silent, only becoming active in response to bladder inflammation or irritation. An inflammatory disorder of the pelvic viscera, a trauma or exceptional behavior might elicit noxious stimuli to the sacral cord that sets up a pelvic floor muscle dysfunction with sacral nerve hypersensitivity and a sacral cord wind-up.6 The guarding reflex is a viscero-muscular reflex activated with the aim of increasing the tone of the pelvic floor during routine daytime activity. In BPS/IC patients, there is an afferent autonomic bombardment that can enhance and maintain a guarding reflex that manifests itself as a hypertone of the pelvic floor.
Physical examination Patients with HPFD are unable to produce more contractile strength and therefore cannot produce an effective squeeze. A single finger can be introduced in the vagina to assess pelvic floor awareness, and the ability to squeeze and relax the levator ani. Often patients with HPFD will have a “V” configuration of the introitus and, as a finger is advanced, it will drop off the shelf caused by the contracted levator muscles. Active “trigger points” are often identified by an exquisitely tender area palpable at the level of the pelvic side wall within a taut band that reproduces the patient’s pain, as well as the referral pattern of her pain.
Diagnostic studies Muscle activity can be measured using a perineometer or an electromyography probe.7 Urodynamics include fluctuating or interrupted flow, abnormal voiding studies, elevated urethral pressure and urethral instability. Schmidt and Vapnek observed pain episodes in such patients coinciding with behavioral increase in the sphincter tone, more than in the bladder.8 When symptoms involve obstructed defecation and rectal pain, defecography should be used to identify the presence of a non-relaxing pelvic floor or even parodoxic activity of the pelvic floor during defecation.
Treatment The pelvic floor therapy should be considered as a first-line treatment in the case of HPFD. The goal of these stretching exercises is to lengthen the contracted muscles by decreasing 86
tension, releasing trigger points in the levator muscles, re-educating the muscles to a normal range of motion and improving patient awareness. The therapy also includes: behavior modification, muscle relaxants, Thiele’s massage, sacral and tibial neuromodulation, trigger point injections, and botulinum toxin.
Endometriosis Endometriosis is the presence of endometrial glands or stroma outside of the endometrial cavity and affects 1–7% of the general population.9 Up to 70% of women with endometriosis have some type of pain symptoms, most commonly dysmenorrhea, cyclic pelvic pain or deep dyspareunia.10 In women who undergo a laparoscopy to evaluate CPP, the prevalence of endometriosis is 30–90%.11 Endometriosis pain is usually a visceral pain, and endometriotic lesions produce inflammatory mediators, particularly prostaglandins F2-α and E2.12 There is a high prevalence and association of IC and endometriosis. A study by Chung et al. of 178 women with CPP found that 65% of CPP patients suffered from both active endometriosis and IC.13 In a prospective study carried out of 162 patients with CPP, Paulson and Delgado found that 66% of the sample was diagnosed with both endometriosis and IC.14 A recent systematic review estimated the prevalence of BPS/ IC, and the coexistence of BPS/IC and endometriosis in women with CPP. Nine studies including 1016 patients with CPP showed the mean prevalence of BPS was 61%, of endometriosis 70%, and coexisting BPS and endometriosis 48% (range 16–78%, CI 44–51%). These data suggest the importance of considering the bladder as the source of pain even where endometriosis is confirmed, and in the case of unresolved endometriosis and persistent pelvic pain, patients must be evaluated to rule out the presence of BPS/IC.15
Vulvodynia and vestibulodinia Vulvodynia, also known as vulvar vestibulitis or vulvar dysesthesia syndrome, literally means pain, or an unpleasant altered sensation, in the vulva. Pain can be unprovoked, varying from constant to intermittent, or occurring only on provocation, such in sexual intercourse. The International Society for the Study of Vulvovaginal Disease defines vulvodynia as “vulvar discomfort, most often described as burning pain, occurring in the absence of relevant visible findings or a specific, clinically identifiable, neurologic disorder”. This “burning pain” is suggestive of a neuropathic pain response. Classification of the vulvar pain might include localized or generalized pain, or both, provoked or non-provoked and primary or secondary.16 Sometimes an area of redness might be visible, but more often the vagina and the vulva show no abnormalities on gynecological or dermatological evaluation. This pain can affect women’s sexual life, which makes sex painful and, in some cases, impossible. Women with vestibulodynia are likely to have at least two additional pain conditions, such as fibromyalgia, irritable bowel syndrome, BPS/IC or chronic fatigue syndrome.17 © 2014 The Japanese Urological Association
BPS/IC: Gynecological disorders
Peters et al. reported that vestibulodynia affects 25% of women with PBS/IC.18 The etiology of vulvodynia is presumed to involve many factors: infections and altered vaginal acidbase balance, and the upregulation of pro-inflammatory immune responses.19 Furthermore, a large community-based study found that vulvodynia was strongly associated with childhood physical or sexual abuse.20 Because both the vestibule of the vulva and the bladder are derived from the urogenital sinus, it could be hypothesized that the coexistence of vulvodynia and BPS/IC in some patients represents a generalized disorder of urogenital sinus-derived epithelium.21 From a histological point of view, various noxious stimula could cause changes in the vulvar epithelium: contraction in the pelvic floor, and mast cells activation with subsequent degranulation and release of histamine. This causes chronic pain and inflammation through the stimulation of peripheral neurons of the autonomic nervous system, an upregulation of the pain system, and a possible shift from nociceptive to neuropathic pain.22 The standard clinical test for vulvodynia is the cotton swab (Q-tip) test, measuring vulvar pain ratings on a visual analog scale.23
Therapy Several studies have shown that gabapentin has a role as tricyclic, yielding significant results in reducing pain perception.24 More than 80% of patients have reported improvements.25 More recently, botulinum toxin has been proposed for the treatment of vulvodynia. It was found that the visual analog scale score was reduced from 8.1 to 2.5 (P < 0.001), and eight (72.7%) out of 11 patients were satisfied.26
Pudendal neuropathy Pudendal neuropathy is a common feature of syndromes such as dysfunctional voiding, non-obstructive urinary retention, chronic pelvic pain syndromes, and urinary and fecal incontinence. It could be ruled out as a confusable disease in BPS/IC patients. Pudendal neuralgia is a functional entrapment of the pudendal nerve, and pain occurs during compression or stretch maneuvers, such as repetitive microtrauma, orthopedic fracture, straining with constipation and childbirth, falls onto the buttocks, and suture entrapment during pelvic surgery. The main symptom is pain aggravated by sitting/driving/exercise, reduced by recumbence or standing and relieved by sitting on a toilet. The quality of neuropathic pain varies and can be described as burning, stabbing, ache, or pressure, and can be induced by voiding, defecating, vaginal penetration or orgasm. It can occur anywhere in the pudendal territory, but primarily includes the perineum and urethra, and extends to suprapubic, inguinal regions and to the upper medial thighs. Urinary symptoms and rectal dysfunction might occur. Foreign body sensation in the rectum, vagina, urethra or perineum is frequent. Sexual dysfunction could be present. Females might suffer reduced clitoral sensation, pain at vaginal penetration, reduced lubrication and anorgasmia. © 2014 The Japanese Urological Association
Pinprick sensation is tested bilaterally at the level of the clitoris posterior labia and posterior perianal skin. Hyperalgesia is more common than hypoalgesia. Pressure is placed at the level of the Alcock canal attempting to reproduce pain, bladder or rectal symptoms (the Valleix phenomenon). The parasacral area is also examined for a back mouse (episacroiliac lipoma). Several tests can measure pudendal neuropathy including: biothesiometry, sacral latency test, sensory-evoked potentials, motor-evoked potentials and motor latency tests.
Management Pharmacotherapy Tricyclic antidepressants are the first medication category effective in placebo-controlled trials. Other drugs, such as gabapentin, pregabalin, oxcarbazepine, tramadol and duloxetine, significantly reduce pain and improve sleep, mood, and quality of life.
Mininvasive approach Transgluteal pudendal nerve blocks Two injections are given at the ischial spine at 1-month intervals. A third is given into the Alcock canal using computed tomography guidance.
Surgical therapy Transperineal and transgluteal approach The pudendal nerve decompression by the perineal route is a blind procedure carried out under local or regional anesthesia. To suppress the blind character of the procedure, a transgluteal approach has been proposed and the reported surgical success rates range from 60% to 70%. Pain-free status might take some years. Bladder, bowel and sexual dysfunctions show variable improvement.27 More recently, a transvaginal approach has also been proposed.28 Until now, the results on pain are the same as those obtained by the Shafik’s approach,29 but with the concurrent sections of one or two ligaments of the pelvis (sacro-spinal and/or sacro-tuberous ligaments). However, the long-term effects of these sections on the stability of the pelvic region are as yet unknown. Up to now, no data are available about a potential effect of the transgluteal or transvaginal procedures on urinary or anal incontinence.
Conclusions CPP in women can be related in more than 30% of patients to BPS/IC, which is frequently associated with pelvic floor dysfunction. For this group of patients, it is mandatory to observe not only the bladder but also all the other organs within the pelvis. Therefore, it is of utmost importance to evaluate concomitant pathologies, such as vulvodynia, endometriosis and pelvic floor dysfunctions, ruling out confusable disease, such as pudendal neuropathy. An optimal approach is a combined treatment oriented not only to treat the bladder, but also the other components responsible for the pain disorder. Patients with bladder tenderness 87
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alone responded better than patients with multiple tender trigger points, possibly because in these patients the bladder is the only target organ and the patients are less severely affected than patients with multiple trigger points. Multimodal therapy remains the gold standard in the management of BPS/IC patients.
Conflict of interest None declared.
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