++
Detailed descriptions of hyperesthesia of the vulva can already
be found in U.S. and European textbooks of gynecology from the last
century.8,9 Surprisingly, despite these early reports
the medical literature did not mention vulvar pain again until the
early 1980s, when a new awareness of this chronic pain syndrome
developed. In 1984, the International Society for the Study of Vulvar
Disease Task Force defined vulvodynia as chronic vulvar discomfort,
characterized by the patient’s complaint of a burning and
sometimes stinging sensation in the vulvar area.10 Vulvodynia
includes several subgroups: vulvar dermatosis, cyclic vulvovaginitis,
vulvar vestibulitis, vulvar papillomatosis, and dysesthetic vulvodynia.11
++
The incidence or prevalence of vulvodynia is not known. A recent
survey of sexual dysfunction, analyzing data from the National Health
and Social Life Survey, reported that 16% of women between
the ages of 18 and 59 years living in households throughout the
United States experience pain during sex.12 When
these data were analyzed by age group, the highest number of women reporting
pain during sex was in the age group of 18 to 29 years. The location
and etiology of pain was not analyzed in this study. Goetsch13 reported
that 15% of all patients seen in her general gynecologic
private practice fulfilled the definition of vulvar vestibulitis,
a major subgroup of vulvodynia. It is important to point out that these
patients had not come for a gynecologic evaluation because of vulvar
pain, but for a routine gynecologic checkup. Fifty percent of these
patients had always experienced entry dyspareunia and pain with
inserting tampons, and most of these, since their teenage years.
++
It is important to be aware of the different subtypes of vulvodynia,
because treatment varies according to diagnosis. The clinical features
and treatment strategies for these different subtypes are discussed
in the remainder of this section. It is likely that the current
classification of different subtypes of vulvodynia will be modified
in the future, as physicians learn more about the etiologies of
the vulvar pain syndromes. Chronic infections of the vulvar area
should be treated before a diagnosis of vulvodynia is made. A number
of genital infections have been found to be a frequent cause of
chronic vulvar pain,14 and a thorough evaluation
for infections of the vulva and vagina should be carried out by
a gynecologist or dermatologist experienced in this area.
++
Vulvar dermatoses are a frequent cause of chronic vulvar pain.
A prospective study15 of patients presenting with
vulvar pain showed that the majority of patients had a corticosteroid-responsive dermatosis.
Unlike most other subsets of vulvodynia, vulvar dermatoses are associated
with physical signs: redness, blisters, and erosions that can be
recognized during a careful physical examination.16 For
the physician who specializes in pain management, these physical
signs on examination of the patient should be an indication to evaluate
more extensively for an etiology of the pain. The differential diagnosis
of these physical signs is complex and may include local as well
as systemic disease. Inflammatory dermatoses, chronic contact dermatitis,
lichen planus, lichen sclerosus, lichen simplex chronicus, seborrheic
dermatitis, psoriasis, herpetic infections, and systemic autoimmune
diseases such as Beháet’s disease and systemic
lupus erythematosus have to be considered.17 The
diagnosis usually needs to be confirmed by vulvar punch biopsy.
Vulvar and vaginal redness and erosions also may occur in diabetes
mellitus. These patients, however, typically present with vaginal
itching and not pain.
++
Cyclic vulvovaginitis is characterized by episodic “flares” of
vulvar pain, often after sexual intercourse (pain is typically worst
the next day) or during the luteal phase of the menstrual cycle.18 Cyclic
symptoms have been reported after hormonal changes, such as starting
or discontinuing oral contraceptives, or during pregnancy. Cyclic
vulvovaginitis might be multifactorial. Several contributing factors
have been suggested: hypersensitivity to the Candida antigen,19 immunoglobulin
A deficiency,20 and cyclic changes in the vaginal
environment.21 Prolonged maintenance therapy with
antimycotics, topically or systemically, is usually effective if
cultures show the presence of Candida.16,18
++
Vulvar vestibulitis is characterized by a history of entry dyspareunia,
pain at the introitus of the vagina when inserting a tampon, and
painful sensations when wearing pants, or with bicycle and horseback
riding. On examination of the vulva, there is often some vestibular
erythema. A typical characteristic of this type of vulvodynia during
examination is that touch of the vestibule is very painful.22 Often,
the patient herself can point to a specific spot at the vaginal
introitus that is painful to touch. The clinical observation of
vestibular erythema associated with dyspareunia was described in
1928 by Kelly23: “Exquisitely sensitive
deep-red spots in the mucosa of the hymenal ring are a fruitful
source of dyspareunia—tender enough at times to make a
vaginal examination impossible. Inflamed caruncles with or without
these spots often stand guard at the introitus labeled ‘noli
me tangere’.” Earlier studies implicated persistent
infection as the cause of vulvar vestibulitis, but histologic and
molecular studies have not supported this supposition.24–28 Interestingly,
two recent studies reported vestibular neural hyperplasia,29,30 which
might provide a morphologic and neurologic explanation for the pain
in vulvar vestibulitis syndrome.
++
Approximately half of the patients with clinical symptoms of
vulvar vestibulitis eventually experience spontaneous remission.31 Treatment
for vulvar vestibulitis is difficult. Most treatments have been
reported as uncontrolled case reports, and no long-term follow-up
data have been provided. As a first step in the treatment of vulvar
vestibulitis, local irritants and potential allergens should be
identified and eliminated. A change to a mild, hypoallergenic laundry
detergent should be considered.
++
The role of urinary oxalate excretion in vulvar vestibulitis
is controversial. One initial case report associated vulvar vestibulitis
with oxalate crystalluria32: the patient experienced
pain relief with calcium citrate and a low-oxalate diet. Withdrawal
of calcium citrate resulted in reoccurrence of vulvar pain, and
reinstitution of calcium citrate alleviated the pain again. A more
recent study (published as a commentary, only, without details of
the patient population studied) claimed successful pain relief in
75% of patients treated with low-oxalate diet and calcium
citrate.33 However, a controlled study in 130 patients
with vulvar pain and 23 volunteers without symptoms showed that
urinary oxalates may be nonspecific irritants that aggravate vulvodynia,
but that the role of oxalates as instigators is doubtful.34 Because
a low-oxalate diet has no side effects, a trial of this treatment
modality is certainly indicated in patients interested in pursuing
a dietary approach. Controlled, prospective studies with clearly
defined outcome parameters are necessary to further elucidate the
role of urinary oxalates in this chronic pain syndrome.
++
Biofeedback of the pelvic floor musculature has been reported
to result in pain improvement in 83% of patients suffering
from vulvar vestibulitis.35 Given that this treatment
modality is not associated with any side effects and given the high
response rate reported in the literature, a trial of biofeedback
is warranted, before considering any more invasive treatment strategies,
especially irreversible surgical approaches (see below). Various
topical creams or ointments applied to the vulvar vestibule have
resulted in pain relief in some patients. A mild hydrocortisone
cream or ointment or an estrogen cream applied to the vaginal introitus
have been helpful. In mild cases of vulvar vestibulitis, topical
lidocaine applied as a 4% solution is often sufficient
to make intercourse possible without serious discomfort.36 Intralesional α interferon
injections have been reported to provide substantial or partial
improvement in about 50% of patients.37 Isoprinosine, another
agent known to enhance immune function, was found to improve pain
in 60% of patients.38
++
Surgical approaches have been suggested with the aim of removing
the painful skin area. The most common procedure is perineoplasty.
The vulvar vestibule (the hyperalgesic area) is excised, and the
vaginal mucosa is then advanced to cover the defect.39 Risks
include general anesthesia, prolonged healing period, intraoperative
bleeding, and postoperative disfigurement.36 A
simplified surgical revision under local anesthesia has been suggested
in a pilot study,36 resulting in complete resolution
of pain in the majority of patients.
++
In vulvar papillomatosis, small papillae are seen around the
vulvar vestibule. These papillae can be seen in conjunction with
lichen simplex chronicus, or with subclinical infection with human papillomavirus.
In other cases, this is a normal variant. Colposcopy and biopsy
are important to rule out an infection with human papillomavirus.
Treatment of the papillomatosis is usually not necessary.16 If
pain persists, symptomatic treatment of the vulvar pain can be considered.
++
The term essential vulvodynia was originally used to describe
chronic vulvar pain for which no secondary cause could be determined.21 When
it was subsequently found that treatment of cutaneous neuralgia
was effective in some of these patients, the term essential was
replaced by the more appropriate term dysesthetic.40 Dysesthetic
vulvodynia is more common in perimenopausal or postmenopausal women.
In contrast to vulvar vestibulitis, in which pain is localized at
the vulvar vestibule and evoked by touch, patients with dysesthetic
vulvodynia report diffuse, constant hyperalgesia in the vulvar area,
often extending throughout the perineum. These women complain less
about dyspareunia. Some women present with features of both vulvar
vestibulitis and dysesthetic vulvodynia. One article has suggested
use of the term vestibulodynia for this symptom complex.41 It
has been hypothesized that the vulvar hyperalgesia in dysesthetic
vulvodynia is caused by a neuropathic pain syndrome, possibly of
the pudendal nerve.17 In support of this hypothesis,
Sonni and coworkers42 found that the pain threshold
for acid solutions is decreased in women with dysesthetic vulvodynia.
Many patients can be helped with medications recommended for other
neuropathic pain syndromes.43 Case reports have
demonstrated that low-dose amitriptyline may be effective in women
with dysesthetic vulvodynia, especially in elderly women.40,44 However,
no controlled studies have been reported so far, and further clinical
research is urgently needed.
++
Iatrogenic causes have to be considered when evaluating a patient
with vulvodynia. Especially as the use of potent topical corticosteroids
becomes more widespread, steroid rebound dermatitis in the vaginal
area is seen more frequently.18 Vulvodynia has
been recognized as a complication of CO2 laser therapy to the vulva.17 The
literature on the long-term effects of episiotomy on vaginal sensation
is sparse. In one older, retrospective study,45 16% to
47% of the women interviewed continued to report dyspareunia
1 to 5 years after episiotomy.
++
In summary, vulvodynia is a recognized disease entity, and an
emerging body of literature is reporting on several different etiologic
factors, attesting to the multifactorial aspects of this disease.
For the treating physician, it is important to realize that many
women with vulvodynia are in their reproductive ages and previously
had satisfying sexual relationships. In contrast to many other chronic
pain syndromes, vulvodynia may only interfere to a moderate extent with the daily activities of a woman, but the disease usually interferes
100% with her sexual life. To confirm the diagnosis of
vulvodynia, excluding secondary causes such as dermatitis or gynecologic
infections, and to design a treatment plan, a multidisciplinary
approach involving collaborations of gynecologists, dermatologists,
neurologists, pain specialists, psychologists, and psychiatrists
is necessary.
++
In contrast to the large body of literature that has emerged
over the past 15 years on vulvodynia, few reports exist on clitoral
pain. In clinical practice, clitoral pain occasionally is seen in
women presenting with dysesthetic vulvodynia, if the pain is extending
throughout the whole perineum, and the ongoing pain (often a burning,
stinging sensation) is usually exacerbated by mechanical stimuli
such as tight clothing and sexual contact. Chronic pain is reported
as one of the complications of female circumcision. This procedure
involves excision of the clitoris and the labia minora, and is still
performed on young females in many parts of the world before or
as they reach puberty, as an instrument to control female sexuality
and maintain cultural pride.46–48 As mobility
is increasing, some of these women have moved to western countries;
for example, it is estimated that 2,000 young women living in the
United Kingdom undergo this ritual per year.47 Few
of these women seem to seek medical attention, and the incidence
of chronic clitoral pain in this group is not known.
++
Many women present to the urologist, gynecologist, or family
physician with painful micturition but no evidence of organic disease,
and the urine culture is negative by standard techniques. Gallagher
and colleagues49 in 1965 coined the term urethral
syndrome to describe this problem. It has been estimated that urethral
syndrome accounts for as many as 5 million office visits a year
in the United States.50 This syndrome is defined
as a disease entity characterized by urinary urgency, frequency,
dysuria, and, at times, by suprapubic and back pain and urinary
hesitancy in the absence of objective urologic findings. Urethral
syndrome typically occurs in women during their reproductive years,
but it also has been reported in children and in men.51,52 In
contrast to other chronic perineal syndromes involving nonmalignant
pain, the rates of spontaneous remission are very high in this patient
population.53,54
++
Several different theories have been proposed to explain the
etiology of urethral syndrome, most, however, with little supporting
evidence. It has been suggested that symptoms are caused by urethral
obstruction and thus are surgically treatable.55,56 It
is important to note that there rarely is evidence to support an
anatomically obstructive etiology. Although surgical procedures
aimed at relieving a urethral obstruction claim excellent results,
it must be cautioned that long-term follow-up rarely is provided.
These procedures involve some risk of incontinence and are of uncertain and
usually temporary efficacy.57,58 Urinary hesitance,
which often is reported by patients with urethral syndrome, might
be the result of spasms of the external urethral sphincter, rather
than an anatomic obstruction. Several studies reported a staccato
or prolonged flow phase during uroflowmetry and increased external
sphincter tone detected on urethral pressure profilometry in patients with
urethral syndrome.51 However, these urodynamic
findings may also be produced voluntarily in a neurologically intact
person, and are, therefore, difficult to interpret.59 To
date, an inflammatory or infectious etiology of the urethral syndrome
has not been supported (reviewed in ref. 59), and controlled studies
using molecular techniques to assess for infection are necessary
to further clarify whether an occult infection is maintaining the
chronic pain syndrome.
++
A thorough diagnostic evaluation is very important, because the
symptoms of urethral syndrome are indistinguishable from those caused
by urinary infections, tumors, stones, interstitial cystitis, and
many other urologic diseases. Urethral syndrome is a diagnosis of
exclusion. The urologic evaluation includes urine analysis, culture,
and cytology. Radiographic studies, urodynamic studies, and cystoscopy
are indicated in selected patients.59 Systemic
diseases affecting the innervation of the urogenital area, including
multiple sclerosis, collagen diseases, and diabetes mellitus, have
to be included in the differential diagnosis. In female patients
a gynecologic examination is necessary to rule out symptoms that
may be secondary to a gynecologic cause. As in other chronic pain
syndromes, a psychological evaluation should be part of the multidisciplinary
evaluation to rule out a psychogenic etiology and to assess for
symptoms of depression associated with the chronic pain problem.
++
Various invasive and medical treatment options have been suggested
for patients with urethral syndrome59; most are
anecdotal clinical reports, and controlled clinical studies are
urgently needed to assess which approach might be most successful
for this painful disorder. Endoscopic and open surgical procedures
have been suggested to eliminate a presumed urethral stenosis. Fulguration,
scarification, resection, or cryosurgery has been considered to
obliterate cystoscopically apparent urethritis. Bladder instillations
with a variety of anti-inflammatory or cauterizing agents and systemic
therapy with anticholinergics, α-adrenergic blockers,
and muscle relaxants have been advocated. High rates of success
were found with skeletal muscle relaxants or electrostimulation
combined with biofeedback techniques.51,58 Realizing
the different surgical and nonsurgical treatment options discussed
in the literature, a conservative treatment approach has been recommended
as the first choice, because this usually is as effective as surgery,
less expensive, and, most importantly, less subject to risk.54,60
++
Similar to women who suffer from pain syndromes of the reproductive
organs, men with chronic testicular pain are usually embarrassed
to talk about it, and often wonder if they have a hidden sexual
aversion that presents itself as a pain syndrome in the genital
region. Many patients cannot recall any precipitating event that
led to the onset of the chronic pain syndrome. Secondary causes of
chronic testicular pain include infection, tumor, testicular torsion,
varicocele, hydrocele, spermatocele, trauma (bicycle accident),
and previous surgical interventions.61,62 The differential diagnosis
includes referred pain from the ureter or the hip or lumbar facet
joints and entrapment neuropathies of the ilioinguinal or genitofemoral
nerve. Chronic testicular pain has been reported as a complication
of vasectomy.63 This chronic genital pain syndrome
is usually not associated with erectile or ejaculatory dysfunction.61
++
A careful history, physical examination, and urologic evaluation
reveal most secondary causes of chronic testicular pain. In selected
patients, a gastroenterologic evaluation might be indicated to rule
out referred pain from the lower pelvic organs or herniography to
evaluate for an occult hernia. The neurologic evaluation is directed
toward the lumbosacral roots, the ilioinguinal, genitofemoral, and
pudendal nerves, and the autonomic nerve supply to the testis. In
many cases, however, the pain remains unexplained despite a very
thorough diagnostic workup. Treatment of chronic testicular pain
is directed toward the underlying etiology, if an underlying etiology
can be identified. A hydrocele, varicocele, or spermatocele rarely
is the cause of chronic testicular pain, but rather is a coincidental
finding.64
++
Traditionally, pain management for chronic testicular pain in
the urology clinics consisted of a trial of antibiotics and nonsteroidal
anti-inflammatory drugs (NSAIDs), with the aim of treating a possible
occult inflammatory process. Case reports suggest that medical management,
including medications used for other chronic pain syndromes such
as low-dose antidepressants, anticonvulsants, membrane-stabilizing
agents, and opiates, often are effective for treatment of chronic
testicular pain62,65,66; however, no placebo-controlled
studies have been published yet. Transcutaneous electrical nerve
stimulation (TENS) might be helpful.65 Repeated
lumbar sympathetic blocks with local anesthetic and oral sympatholytic
drugs have been reported to result in marked pain relief in selected
patients in whom a sympathetic component is suspected in the maintenance
of chronic testicular pain.67 In the past, drastic
surgical procedures have been recommended for the treatment of chronic
testicular pain, such as epididymectomy and orchiectomy. As an alternative
to surgical removal of these organs, microsurgical denervation has
been suggested.68
+++
Prostatitis
and Prostatodynia
++
“Prostatitis” is a diagnosis that is often
given to patients presenting with unexplained symptoms or condition
that might possibly originate from the prostate gland.69 In
the United States, approximately 25% of men presenting
with genitourinary tract problems are diagnosed with prostatitis.70,71 Drach
and colleagues72 defined four categories of prostatitis:
(1) acute bacterial prostatitis, (2) chronic bacterial prostatitis,
(3) nonbacterial prostatitis (including nonbacterial infections,
allergic and autoimmune prostatitis), and (4) prostatodynia. Prostatodynia
is defined as persistent complaints of urinary urgency, dysuria,
poor urinary flow, and perineal discomfort and pain, without evidence
of bacteria or purulence in the prostatic fluid.72 In
addition to the perineal pain, patients often report that the pain
is radiating to the lower back, suprapubic area, and groin. In contrast
to patients with chronic testicular pain, patients with prostatodynia
often complain about pain with ejaculation. Prostatodynia accounts
for approximately 30% of patients presenting with prostatitis,
the age range is from 20 to 60 years of age.73,74
++
Physical examination of the prostate is typically normal, without
any signs of tenderness. A thorough urologic evaluation is indicated,
including urinalysis, urine culture, urine cytology, and urethral
cultures.75 Referred pain from the colon or rectum
needs to be ruled out. Prostatodynia is a diagnosis of exclusion,
in which it is assumed that the chronic pain syndrome is related
to the prostate, but no inflammatory prostatic process can be identified.
++
The most frequently advocated treatment is antibiotics despite
the fact that usually no infectious etiology can be found. The urodynamic
abnormalities observed in some patients with prostatodynia suggest
that there is increased sympathetic tone. Oral α-adrenergic
blockers have been shown to improve the voiding abnormalities as
well as pain; however, their use is often limited by side effects,
most frequently hypotension.76 It has been suggested
that there is an increase in pelvic floor muscle tone in patients
presenting with prostatodynia, and pelvic floor relaxation techniques
and muscle-relaxing agents have been reported to result in marked
improvement of the symptomatology.77
++
Pain localized to the coccyx is a common perineal pain syndrome.
The term coccygodynia was first used by Simpson78 to
describe a chronic pain syndrome characterized by tenderness and pain
in the area of the coccyx, most severe with sitting. This chronic
pain syndrome occurs more frequently in women and among elderly
debilitated patients.79,80 Some patients can remember
a history of acute trauma to the coccyx, either a fall in the sitting
position or birth trauma. Chronic trauma to the coccyx might result
from poor sitting positions in which continued pressure occurs on
the coccyx. Although one series related 70% of all cases
of coccygodynia to a traumatic etiology,81 others
have suggested that trauma is an unlikely cause of coccygodynia,
and instead a rheumatic etiology should be considered.82
++
On physical examination the coccyx is usually tender on palpation.
Because pain can be referred to the coccyx from the lumbosacral
spine, sacrum, anus, rectum, pelvis, and genitourinary tract, a thorough
history is important, including questions regarding a precipitating
cause. It is important to evaluate for anal fissures, hemorrhoids,
anorectal or gynecologic infections, or rare causes such as space-occupying
lesions, including tumors. As in many other perineal pain syndromes,
despite a thorough evaluation, no cause can be found in many patients
with coccygodynia.
++
The first step in the treatment of coccygodynia is protection
of the painful coccyx from further irritation by sitting in such
a position that no pressure occurs on the coccyx.80 This
measure alone often results in significant pain improvement after
a few weeks. Physical therapy interventions, including hot sitz
bath, pelvic relaxation techniques, and pelvic massage, have been
reported to result in pain relief.77,83 Local infiltrations
with local anesthetic of the painful area, coccygeal nerve blocks,
and caudal injections with local anesthetic, alone or in combination
with corticosteroids, often are helpful.83,84 Wray
and coworkers84 reported that manipulations of
the coccyx under anesthesia resulted in marked pain relief. In these
manipulations, the coccyx is repeatedly flexed and extended with
the aim of stretching ligaments so that the ordinary ranges of motion were
no longer painful. In patients with intractable pain who experience
significant temporary pain relief with caudal local anesthetic injections,
cryoanalgesia of the posterior rami of the lower sacral nerve roots
and the coccygeal nerve should be considered.85 Surgical
removal of the coccyx, the treatment of choice during the nineteenth
and early twentieth centuries, is rarely necessary today because
conservative measures are usually sufficient.80,84 In
selected patients whose pain is clearly related to the coccyx and
in whom conservative measures have failed, coccygectomy is indicated
and has a high success rate.84,86 However, before
considering this irreversible surgical procedure, it is important
to make use of conservative measures first. Importantly, Wray and
coworkers84 reported a 20% relapse rate
in patients who had initial pain relief with conservative measures; however,
repeat therapy was usually effective in providing permanent pain
relief.
++
Pain in the rectal-anal area can occur as constant pain—proctodynia—or
as paroxysms of pain—proctalgia fugax.
++
Proctodynia is often caused by local disease of the anus or rectum
or it can be referred from the urogenital tract or the lumbosacral
spine. A comprehensive workup is indicated, because in most cases
of proctodynia—in contrast to many of the other perineal
pain syndromes—the underlying etiology can be found. Intractable
rectal pain has been associated with pudendal neuralgia in 24% of
the cases in one study, and has been treated successfully with neuropathic
pain medications.87 A pudendal nerve block with
local anesthetic might be helpful to assess the contribution of
the pudendal nerve to the chronic pain syndrome. Chronic idiopathic
anal pain has been associated with abnormal anorectal manometric
profiles, probably resulting from a dysfunction of the striated
external anal sphincter. Biofeedback training has been shown to
be effective in these cases.88
++
Proctalgia fugax is characterized by sudden attacks of intense
pain of short duration in the region of the internal anal sphincter
and the anorectal ring. The incidence of proctalgia fugax has been reported
to be as high as 14% in the general population, and as
high as 33% in patients with gastrointestinal disease.89,90 Familial
forms of proctalgia fugax have been described, and it is important
to take a family history. The immediate cause of proctalgia fugax
seems to be muscle spasms, but the etiology of this syndrome remains
unclear.91 A consistent phenomenon in all studies
seems to be gastrointestinal smooth muscle dysfunction. In the majority
of patients with proctalgia fugax, the physical examination is normal,
and common anorectal diseases such as hemorrhoids and anal fissures
seem to be unrelated to the paroxysmal pain problem, in contrast
to patients with constant rectal pain—proctodynia.
++
Simple and effective remedies have been suggested to end the
acute pain attack associated with proctalgia fugax: immediate taking
of food or drink, dilation of the anorectum (by digital dilation, attempting
a bowel movement or inserting a tap-water enema), hot sitz baths,
and firm pressure to the perineum.92–94 A
variety of drugs has been suggested in anecdotal reports: antispasmodics, nitroglycerin,
nifedipine, carbamazepine, diltiazem, and salbutamol (reviewed in
ref. 43). Eckhardt and colleagues95 showed in a
controlled crossover trial that salbutamol inhalation significantly
shortened the duration of the severe pain. Because this is an easy-to-use
medication during the acute attack, and given that this is the only
controlled study on medications for proctalgia fugax, salbutamol
should be the first choice, if a decision is made to use medications
to abort pain attacks. It is important to reassure the patient that
the symptoms, although quite troublesome (the pain often reaches
an intensity of 10/10 on a Visual Analog Scale) are not
signs of a life-threatening disease and may improve with time. Psychological
assessment is important to rule out a depressive symptomatology
contributing to the chronic pain syndrome.
+++
Generalized
Perineal Pain
++
Perineal pain can be localized to a specific area of the perineum,
as previously discussed. In some cases, the perineal pain syndrome
starts at a specific area and over time extends over the whole perineum.
In other cases, perineal pain starts as a diffuse discomfort involving,
from the beginning, most of the perineum and gradually increasing
in intensity—this is a chronic generalized perineal pain
syndrome.
++
The differential diagnosis is complex: gastrointestinal, proctologic,
urologic, gynecologic, and neurologic etiologies have to be excluded.
Systemic diseases associated with painful peripheral neuropathies,
such as diabetes mellitus and acquired immunodeficiency syndrome
(AIDS), have to be considered. French studies have reported a diagnosis
of pudendal nerve entrapment in 91% of patients with perineal
pain referred to a pain clinic.
++
Surgical neurolysis-transposition was recommended as the treatment
of choice, with best results obtained in patients in whom pudendal
nerve entrapment was diagnosed early.96,97 In patients presenting
with perineal pain and sacral meningeal cysts (Tarlov cysts), surgical
resection of the cysts has been reported to result in excellent
pain relief in the majority of the patients.98 Perineal pain
has been reported in the context of movement disorders. Chronic
perineal pain occurred as a complication of neuroleptic drug exposure.
Catecholamine depletors resulted in complete resolution of the painful
sensations.99 In addition, chronic perineal pain
has been reported in the context of Parkinson’s disease,
and excellent pain relief was achieved using medications regularly
used for Parkinson’s disease.100