The vulval pain syndromes

The vulval pain syndromes

VULVAL PAIN SYNDROMES 19 Chavez M L, DeKorte C J. Valdecoxib: a review. Clin Ther 2003; 25: 817–51. 20 Carroll D, Moore R A, McQuay H J, Fairman F, T...

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VULVAL PAIN SYNDROMES

19 Chavez M L, DeKorte C J. Valdecoxib: a review. Clin Ther 2003; 25: 817–51. 20 Carroll D, Moore R A, McQuay H J, Fairman F, Tramer M, Leijon G. Transcutaneous electrical nerve stimulation (TENS) for chronic pain. Cochrane Database of Systematic Reviews, 2001. CD003222. 21 Proctor M L, Smith C A, Farquhar C M, Stones R W. Transcutaneous electrical nerve stimulation and acupuncture for primary dysmenorrhoea. Cochrane Database of Systematic Reviews, 2002. CD002123. 22 Habek D, Cerkez H J, Bobic-Vukovic M, Vujic B. Efficacy of acupuncture for the treatment of primary dysmenorrhea. Gynakol Rundsch 2003; 43: 250–3. 23 Hu J. Acupuncture treatment of dysmenorrhea. J Tradit Chin Med 1999; 19: 313–16. 24 Taylor D, Miaskowski C, Kohn J. A randomized clinical trial of the effectiveness of an acupressure device (relief brief) for managing symptoms of dysmenorrhea. J Altern Complement Med 2002; 8: 357–70. 25 Facchinetti F, Sgarbi L, Piccinini F, Volpe A. A comparison of glyceryl trinitrate with diclofenac for the treatment of primary dysmenorrhea: an open, randomized, cross-over trial. Gynecol Endo 2002; 16: 39–43. 26 Pittrof R, Lees C, Thompson C, Pickles A, Martin J F, Campbell S. Crossover study of glyceryl trinitrate patches for controlling pain in women with severe dysmenorrhoea. BMJ 1996; 312: 884. 27 Kwok A, Lam A, Ford R. Laparoscopic presacral neurectomy: a review. Obstet Gynecol Surv 2001; 56: 99–104. 28 Wilson M L, Farquhar C M, Sinclair O J, Johnson N P. Surgical interruption of pelvic nerve pathways for primary and secondary dysmenorrhoea. Cochrane Database of Systematic Reviews, 2000. CD001896. 29 Molnar B G, Baumann R, Magos A L. Does endometrial resection help dysmenorrhea? Acta Obstet Gynecol Scand 1997; 76: 261–5. 30 Vercellini P, Frontino G, De Giorgi O, Aimi G, Zaina B, Crosignani P G. Comparison of a levonorgestrel-releasing intrauterine device versus expectant management after conservative surgery for symptomatic endometriosis: a pilot study. Fertil Steril 2003; 80: 305–9. 31 Chan C L, Annapoorna V, Roy A C, Ng S C. Balloon endometrial thermoablation – an alternative management of adenomyosis with menorrhagia and dysmenorrhoea. Med J Malaysia 2001; 56: 370–3. 32 Zullo F, Palomba S, Zupi E et al. Effectiveness of presacral neurectomy in women with severe dysmenorrhea caused by endometriosis who were treated with laparoscopic conservative surgery: a 1-year prospective randomized double-blind controlled trial. Am J Obstet Gynecol 2003; 189: 5–10.

The vulval pain syndromes Anne Edwards

Vulval pain syndromes are now well recognized in the developed world. The International Society for the Study of Vulvovaginal Disease (ISSVD) has recently proposed changes to the classification of vulvodynia (vulval pain), Figure 1.

Epidemiology There are no good data on the prevalence of vulval pain syndromes, but these are not rare disorders. In a Genito-Urinary Medicine (GUM) Department with a total of 4000 new female attendances per annum and a weekly specialist vulval clinic, there are about 20–30 new cases of vulval pain each year. There will be more in specialist vulval clinics taking referrals from primary care and a high proportion of gynaecology patients presenting with superficial (introital) dyspareunia will have ‘localized vulval dysesthesia’. Vulval pain syndromes occur at any age throughout adult life. The localized vulval dysesthesia variant is reported more commonly in pre-menopausal women and ‘dysesthetic vulvodynia’ is seen more commonly in peri- and post-menopausal women. The possibility, and the potential challenges, of cases presenting in childhood has been raised although not yet formally reported in the literature. The diagnosis of a vulval pain syndrome is seldom made in developing countries, and is usually confined to white women.

Pathogenesis The aetiology remains unclear, but this group of disorders is best regarded as an example of the complex regional pain syndromes. The pathogenesis is similar to reflex sympathetic dystrophy, in which persistent pain occurs in the absence of tissue injury. This primary chronic pain syndrome subsequently impacts on the patient’s ability to function sexually.

Diagnosis

FURTHER READING Lippman S A, Warner M, Samuels S, Olive D, Vercellini P, Eskenazi B. Uterine fibroids and gynecologic pain symptoms in a populationbased study. Fertil Steril 2003; 80: 1488–94. Proctor M, Farquhar C. Dysmenorrhoea. Clin Evid 2002; 7: 1639–53. Wilson M L, Murphy P A. Herbal and dietary therapies for primary and secondary dysmenorrhoea. Nurs Times 2001; 97: 44.

A vulval pain syndrome is a diagnosis of exclusion based on a typical history, absence of vulval pathology on examination and, in localized vulval dysesthesia, the presence of touch tenderness. The symptoms are summarized in Figure 2. Other vulval disorders may coexist with a vulval pain syndrome.

Anne Edwards is Consultant/Honorary Senior Lecturer in Genito-Urinary Medicine at the Radcliffe Infirmary, Oxford, UK. She qualified from Oxford and trained at the Middlesex and St Thomas’ Hospitals, London. She is a Fellow of Brasenose College, Oxford, and Council Member of the British Society for the Study of Vulval Disease.

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VULVAL PAIN SYNDROMES

Glossary

When to consider a diagnosis of vulval pain

• Vulvodynia – vulval pain • Vulval dysesthesia – chronic vulval pain with no obvious cause • Generalized vulval dysesthesia – constant vulval pain, formerly known as dysesthetic vulvodynia • Localized vulval dysesthesia – vulval pain usually triggered by contact, formerly known as vestibulodynia

• Long history/repeated consultations with a range of doctors and others • Chronic/recurrent symptoms • No response/partial response to a range of therapies • Vulva appears normal/ mild erythema • Microbiological tests repeatedly negative – notably for clinically diagnosed candidiasis

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Course of the disease and prognosis

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The vulval pain syndromes typically follow a relapsing and remitting course. However, it is likely that most patients will improve and/or fully recover with time. In one study, 50% of patients recovered within 12 months of diagnosis.1 Data on the long-term outcome are not available, but most clinicians will have experience of a small minority of patients with intractable disease for whom no treatments are effective. It is also true that some patients will recover and then present again with a recurrence. In the author’s experience, if they have previously responded to treatment they are likely to do so again.

vestibulodynia, erythema was cited as one of the characteristics and part of the diagnostic criteria. It is now recognized that this is not an inflammatory process; the erythema reported in most cases reflects a subjective impression and is probably not real. • In generalized vulval dysesthesia, touching the affected area does not exacerbate symptoms. • In localized vulval dysesthesia, patients are symptom free until the vulva is touched with, for example, a cotton bud. Commonly this is the vestibule but in some women the whole labia minora is affected. The distribution may also be asymmetrical.

History and examination

Investigations

Typically the history is long, sometimes years. Patients may have tried treatments without success, and may see many doctors before a correct diagnosis is made (Figure 3). It is important to remember that in the most common form, pain is localized to the vestibule and commonly presents as dyspareunia. The vulva usually looks normal. In the original description of

There are no specific investigations to confirm a vulval pain syndrome. Biopsy and/or screening for infection is indicated when the vulva looks abnormal. It is, however, worth remembering that vulval pain and specific skin disease may coexist. Formal neurological assessment may be useful in women with symptoms or signs supporting a neurological diagnosis.

Differential diagnosis

Features and classification of the vulval pain syndromes

A full history and thorough examination of the mouth, genitalia and skin should exclude other diagnoses, most of which are uncommon. These include recurrent hymenal/posterior fourchette tears (examine patients within 48 hours of intercourse if this is suspected – a magnifying instrument such as a colposcope may be useful), symptomatic dermographism (where writing on the skin with a blunt instrument causes the development of a red weal) and neurological disease.

In all forms, the vulva appears normal. Some patients present with features of both generalized vulval dysesthesia and localized vulval dysesthesia. 1. Generalized vulval dysesthesia • Tends to occur in older pre- and post-menopausal women • Chronic vulval discomfort – burning, stinging, irritation or a feeling of rawness • May be worse at the end of the day • Intercourse does not usually aggravate symptoms 2. a. • • • • • b. c.

Management The approach to management is the same for the different vulval pain syndromes. Most specialists favour a combined medical and psychosexual/psychological approach. A definite diagnosis can be therapeutic in many patients with a long history of uncertainty, as can the knowledge that other women suffer the same problem. Reassurance about the lack of any sinister cause and the tendency to spontaneous improvement with time is essential. Written information, including details of support organizations, should be provided at the first consultation.

Localized vulval dysesthesia Vestibulodynia (commonest variant in this group) Young sexually active women, usually pre-menopausal Superficial dyspareunia Pain on insertion of tampon/riding bicycle Pain may persist after intercourse Pain free at other times Clitoridynia Other localized forms of vulval dysesthesia

Drug therapy Topical agents (e.g. moisturizing creams, steroids) may be soothing but rarely produce any long-term sustained

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VULVAL PAIN SYNDROMES

improvements. Some patients find temporary relief with topical local anaesthetic agents. Low-dose tricyclic agents used for their analgesic, and possibly anxiolytic, effects may accelerate recovery and are the mainstay of treatment. Treatment is usually started with low-dose amitryptiline or impramine (10 mg nocte) increasing after one month (to 25 mg nocte). Patients who are likely to respond, do so at doses of 40 to 75 mg, though higher doses of up to 125 mg may be tried with occasional benefit. Those who respond should remain on a maintenance regimen for three to six months or longer, depending on individual wishes. After a period of stability, dose reduction can be tried. When a trial of therapy with a tricyclic agent is undertaken compliance is improved if the patient is given a detailed explanation of what to expect (Figure 4). Interestingly, underlining the fact that the tricyclics are not acting as antidepressants in the vulval pain syndromes, the serotonin re-uptake inhibitors (SSRIs) appear to be ineffective in vulval pain. Those patients already on treatment for depression with SSRIs can take low-dose tricyclics in addition. Some patients may prefer to gradually increase to an antidepressant dose of the tricyclic and discontinue the SSRI. Others can be maintained on both a low-dose tricyclic and an SSRI. In complicated cases psychiatric advice should be sought.

Involvement of the partner in chronic cases can also be helpful. There may be an understandable tendency for the more considerate partner to avoid initiating sexual contact, often reinforcing the patient’s self-perceived feeling of not being attractive, thereby diminishing her libido. Psychological approaches – in some cases, especially those with generalized vulval dysesthesia, a psychological assessment may help both in providing an overview of the patient and in assisting her to develop strategies for coping with chronic pain. Non-responders The management of non-responders is difficult. • There are anecdotal reports suggesting that gabapentin (as an analgesic) may be beneficial. • Some authorities have advocated surgical resection of the vestibule in intractable localized vulval dysesthesia, but this is an invasive procedure, there is no good evidence to support its efficacy, and many patients subsequently relapse. • In the late 1980s superficial laser vulvectomy was tried for vulval dysesthesia, but has since been abandoned because it was ineffective and associated with complications. Further options are limited they include: • referral for advice from a chronic pain clinic • alternative therapies such as acupuncture • strong reassurance – the limited data available suggest most patients improve with time. Sadly, all specialists will have a small group of women for whom vulval pain remains an intractable problem and for whom there is no effective treatment.

Psychosexual and psychological management Most patients will benefit from a combined approach in addition to the medical management described above. Psychosexual approaches – whilst pain is the primary symptom, most patients, especially those with localized vulval dysesthesia, will experience impairment of sexual function. Commonly this results in loss of desire and/or avoidance of sex. Vaginismus can occur in localized vulval dysesthesia, which compounds the pain associated with sexual intercourse. Psychosexual approaches may help, including an explanation of how pain can be provoked during intercourse, as well as the effects of muscles contracting, which may worsen the pain. Some useful practical hints include: • the use of lubrication • Kegel’s exercises • positional advice showing the patient how penetration can be achieved with minimal discomfort. Exercises for relaxing pelvic floor musculature may speed recovery. In the USA and Europe this latter approach has been formalized with the development of biofeedback and surface electromyography. If available, a physiotherapist with an interest in biofeedback may provide additional help.

Follow-up Patients with a vulval pain syndrome probably benefit from follow-up in a specialist unit. Some patients elect to start treatment immediately, others prefer time to consider the diagnosis, read an information leaflet and access the web. An initial early follow-up at four to six weeks allows discussion about questions that may have arisen. For patients who have initiated therapy it is useful to review possible side-effects and to consider further dose increases. Where a particular tricyclic agent has not been tolerated it is possible, in most cases, to find an alternative that will suit. Once patients are stabilized on therapy, follow-up may be extended to every three to six months.

REFERENCE 1 Peckham B M, Maki D G, Patterson J J, Hafez G R. Focal vulvitis: a characteristic syndrome and cause of dyspareunia. Am J Obstet Gynecol 1986; 154: 855–64. FURTHER READING Edwards A, Wojnarowska F. The vulval pain syndromes. Int J STD AIDS 1998; 9: 74–7. Edward L, ed. Genital dermatology atlas. London: Lippincot, Williams and Wilkins, 2004. Jantos M. The role of biofeedback in the management of vulvodynia. Application of surface electromyography in chronic vulvar pain. Vulvar pain clinics. www.vulvodynia.com.au/articles/001.html The International Society for the Study of Vulvovaginal Disease (ISSVD). The terminology and classification of vulvodynia: past, present and future. ISSVD Newsletter, 2000. www.vulvodynia.com.au

Counselling for patients with vulval pain starting tricyclic therapy for analgesia • These drugs are not acting as antidepressants in the vulval pain syndromes • Side-effects tend to resolve after 2–3 weeks of use (or an alternative tricyclic can be tried) • A therapeutic benefit may not be noticed for 3–4 weeks or even longer (most commonly, not until doses of 40 mg are reached) • These are not drugs of dependency. 4

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