Text of above pdf. sans pics and appendix-
PELVIC PAIN SYNDROMES
In the United States, nearly one-third of the population experiences severe chronic pain at some
time in their lives. It is currently the most common cause of long-term disability affecting up to 50
million people. 12% of hysterectomies performed are due to chronic pelvic pain, yet 23% of
these patients will still have pelvic pain postoperatively [1]. In addition, 40% of all laparoscopies
are done for chronic pelvic pain, but when minimal endometriosis is found, only one-third will
have resolution of symptoms after that surgery [2].
Common pelvic pain syndromes include functional bowel disease, myofascial pain syndromes,
vulvodynia and/or dyspareunia syndromes, and interstitial cystitis. It is very common that these
puzzling problems coexist in many of our patients with chronic pelvic pain syndromes. For
example, 30% of patients with interstitial cystitis also have severe vulvodynia, 40% will have
functional bowel disease and 85-90% will have pelvic floor tension myalgia. 30% of patients
with interstitial cystitis will go on to be diagnosed to have fibromyalgia later in life. The
pathophysiology of complex pelvic pain syndromes and why these so frequently coexist in our
patients is easily explained once we understand the neuropathophysiology involved. The Key to
this neuropathic response is the up regulation of the sacral cord. Important components of that
up regulation include the activation of silent C-fibers arising from the viscera of the pelvis, and a
resultant increase in noxious input to the dorsal horn causing biochemical changes that become
permanent [3]. These changes result in the findings of allodynia with obvious sensory
processing abnormalities, expansion of receptive fields, and neuropathic output states including
neurogenic inflammation, hypertonic pelvic floor muscle dysfunction and the development of
trigger points not only within the pelvic floor but also within the abdominal wall. Once we
understand the importance of this neuropathology, suddenly we gain insight into why our
INTRODUCTION:
patients so often have coexisting pain syndromes and thus we can more easily explain this
findings to our patients and provide a more effective therapeutic approach.
Functional bowel disease is a classic example of visceral hyperalgesia. It is characterized by a
chronic relapsing pattern of bloating and cramping pain with alterations in stool frequency and/or
consistency. Often, the pain is relieved by defecation. It is present in 50-80% of patients with
any form of chronic pelvic pain. It is three times more likely to be found in women than in men,
and these women are three to four times more likely to have already undergone a hysterectomy
frequently for the treatment of pain [4]. We also know that if a hysterectomy in a patient who
has coexisting functional bowel disease was done for causes of pelvic pain, this patient is much
more likely to have a poor outcome with significant pain or persistence of pain postoperatively
(P value less than .05). Also, 50% of patients who have negative laparoscopies at the time of
evaluation of chronic pelvic pain are often found to have functional bowel disease once they are
referred to a clinic specializing in the identification and treatment of functional bowel disease [5].
Patients with functional bowel disease often give a history of certain foods worsening or
inducing their symptoms. These sensitivities might include a high fat diet, lactose, sorbitol,
caffeine or alcohol excess or simply chewing gum frequently. These sensitivities do not
represent true food allergies as much as an exaggerated gastric colonic reflex that is brought on
by the particular food products. Patients with functional bowel disease often have other GI
symptoms including esophageal reflux and peptic ulcer disease. Research has demonstrated
that the visceral hypersensitivity can be easily demonstrated in all parts of the GI tract even
though the patient might present with symptoms primarily arising from just one part of the GI
FUNCTIONAL BOWEL DISEASE:
tract (colon) [6]. 50-80% of patients with significant functional bowel disease often have
associated problems of pelvic floor muscle dysfunction resulting in problems of hemorrhoids,
anismus, obstructed defecation and generalized pelvic pain.
Treatment of functional bowel disease must be directed towards the particular symptomatology
of the patient. In patients with primarily severe cramping pain related to spasms in the colon,
multiple medications including Dicyclomine, Donnatal, Librax and Hyoscyamine are beneficial.
In patients who primarily complain of gaseous distention and cramping, products such as
Simethicone, peppermint oil (Colpermin) and Beano can be quite beneficial. In patients who
primarily have constipation dominated symptoms, fiber supplements are key. Psyllium is quite
beneficial and new osmotic laxatives, such as Miralax , can be quite successful. On the other
hand, if patients have predominantly diarrhea type symptomatology, then again fiber
supplements, Psyllium, Loperamide and tricyclics are very beneficial. 10-20% of patients who
have undergone a cholecystectomy will have bile salt related colitis and their diarrhea responds
very nicely to Cholestyramine.
Myofascial pain syndromes represent a large group of
muscle disorders that are characterized by
hypersensitive or tender spots that are referred to as
“trigger points.” (Figure 1) Trigger points are often
found within taught bands that are quite tender to
palpation, and patients usually demonstrate very
Figure 1
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MYOFASCIAL PAIN SYNDROMES:
nicely the localization of those trigger points if they happen to be within the abdominal wall.
Myofascial pain syndrome is the most common cause of chronic low back pain, shoulder or
neck pain. 30-50% of individuals will have latent trigger points. There are multiple risk factors
that can turn a latent trigger point into an active or symptomatic trigger point. The key finding on
examination is that an active trigger point will produce not only a localized site of tenderness but
typically a referral pattern of pain radiating into the area that the patient describes her general
pain to be in. Myofascial pain syndromes are the most frequent cause of chronic disabling pain,
particularly so in patients who have failed more traditional therapies for their pelvic pain
disorders. Primary fibromyalgia can be differentiated from a more localized myofascial pain
syndrome in that fibromyalgia has a widespread distribution.
Sinaki in 1977 described pelvic floor tension myalgia. This represents a myofascial pain
syndrome that involves the pelvic floor muscles. The literature is filled with multiple separate
syndromes that have been associated with many different pelvic pain disorders. These specific
syndromes include levator syndrome, coccygodynia and proctalgia fugax [7]. These are all
myofascial pain syndromes. Pelvic floor tension myalgia is a term that tends to consolidate all
of these previously used terms under a single umbrella. Pelvic floor myofascial pain syndromes
can be brought on either as a visceral muscular reflex as was described earlier, or as a primary
injury to pelvic floor muscles. Examples of a reflexic pelvic muscle disorder would include any
chronic painful inflammatory process within the pelvis since they can all potentially induce pelvic
floor tension. These include endometriosis, functional bowel disease, chronic vulvar vestibulitis
[8] or interstitial cystitis. Examples of primary injuries to the pelvic floor include pelvic floor
reconstructive surgeries (particularly sacrospinous vault suspensions), extensive dissection
within the pelvic floor, postoperative complications such as a large pelvic hematoma, a straddle
injury, a broken tailbone or a difficult delivery (particularly ones associated with forceps).
DeLancey has demonstrated that 29% of multiparous women have levator ani abnormalities on
MRI while nulliparous women have none [9]. It is important to note that many patients associate
the onset of chronic pain with problems that developed post delivery. Finally, pelvic myofascial
pain disorders can develop as a pelvic floor dysbehavior, which is often associated with pelvic
muscles simply not working in a coordinated
manner. A classic example of this would be a
patient who has had bed-wetting or recurrent
UTIs since her childhood and while those
urologic symptoms may resolve with time, the
underlying pelvic floor dysbehavior often
persists. It is important to note that patients with
a history of bed-wetting are ten times more
likely to develop interstitial cystitis. (Figure 2)
Typical symptoms of pelvic floor tension myalgia include an achy pelvic discomfort often
described as pressure. Patients are frequently referred for evaluation and/or have undergone
surgery for pelvic relaxation when in fact there are no true anatomic deficiencies found. The
patient often reports problems with urinary hesitancy, voiding dysfunction and anismus, and
gives a very typical history of the discomfort being made worse with prolonged sitting,
particularly on hard surfaces. Finally, one of the key symptoms includes dyspareunia, which
goes on for several hours after the completion of intercourse (muscular pain is often delayed
pain). On examination, these patients are found to have very little pelvic floor muscle awareness
and have difficulty demonstrating a simple squeeze or relaxation. The keys to the digital exam
are not only this poor muscle awareness and poor relaxation but also the muscles are quite
tender to palpation. In these cases it is sometimes easy to observe taught bands and trigger
points within the iliococcygeus or more commonly the pubococcygeus muscles near their
insertion along the “white line” or behind the pubic symphysis. (Figure 3) Objective
Figure 2
Chronic Pelvic Pain
Vestibulitis
Urgency / Frequency
Syndrome
Dyspareunia
Chronic
inflammatory
painful
conditions of
the pelvis
And
Pelvic Floor
Dysbehaviors
Primary Insult
To PFM
Trigger
Points
Myofascial
Pain
measurement of this pelvic floor muscle dysfunction is easily seen with urodynamic evaluation
of urethral pressures (the urethral pressures tend to be exaggerated and extremely unstable).
Objective evidence using surface EMG’s has also been demonstrated, particularly by authors
such as Howard Glazer [8].
Therapy for pelvic floor tension myalgia
includes the traditional therapies for any
muscle disorders with the keys here
being localized heat, muscle relaxants
and neurolytic therapy. Particularly
beneficial in this regard is the use of
Amitriptyline or other tricyclics. The
studies that show the best response in this myofascial disorder of the pelvic floor revolve around
a specially trained physical therapist who is knowledgeable in soft tissue work including
myofascial release, muscle mobilization and the use of diathermy or ultrasound heat therapy to
help relax these muscles. Biofeedback is commonly added with emphasis not on performing
Kegels but instead learning to relax the muscles or performing “reverse Kegels.”
Trigger point therapy, whether it involves the abdominal wall or the pelvic floor, is very important
in the management of myofascial pain syndromes. If conservative therapy is not able to
eradicate these highly sensitive localized areas of pain, then blocking the pain cycle with an
injection of ½% Lidocaine, using a series of three to five injections, is extremely beneficial.
Trigger point injections therefore not only become therapeutic but they are also diagnostic,
which will demonstrate to both the patient and you the importance of eradicating these areas of
extreme tenderness.
Figure 3
VULVODYNIA:
The international study of vulvar disease in 1983 defined vulvodynia as the chronic vulvar
discomfort that patients suffer with that is characterized by complaints of burning, stinging,
irritation and/or rawness and this generally needs to be differentiated from problems of itching or
pruritus vulvae. There are six different subtypes or subsets of vulvodynia [10]. The three most
common, which will be covered in this text, include vulvar vestibulitis, cyclic vulvovaginitis, and
dysesthetic vulvodynia. History is extremely important in these patients with the first and most
important question being whether or not the discomfort is a continuous discomfort or one that
occurs only with intercourse. When discomfort is present only with intercourse, then the primary
diagnoses to be included in the differential includes vulvar vestibulitis, cyclic vulvovaginitis and
primary pelvic floor muscle dysfunction.
Cycle vulvovaginitis typically includes a history of intercourse related flare of burning and
irritation. Typically, these patients will have redness. They will describe fissures and a minimal
discharge. These patients will often describe menstrual cycling of their symptomatic
dyspareunia. Important in these patients is that yeast cultures must be obtained despite the
minimal findings on wet prep and gross examination. These are also found to have colonization
with fungal organisms and thus long-term antifungal therapy is certainly indicated with the
classic treatment being Diflucan 150 mg each week for four to six months.
Vulvar vestibulitis, on the other hand, is found in patients who typically have dyspareunia with
localized areas of pinpoint tenderness at the site of vestibular gland openings with the pain and
discomfort always being reproduced by palpation to the area of the vestibule. We have now
come to realize that these patients may have only the pinpoint tenderness and no punctate
areas of redness, and therefore the lack of redness should not limit this diagnosis being made.
An important associated finding in patients with vulvar vestibulitis is significant problems of
pelvic floor tension myalgia and pelvic floor muscle dysfunction. Howard Glazer has
demonstrated that in patients with severe vulvar vestibulitis who have failed all other therapy,
pelvic floor rehabilitation and relaxation therapy has actually been found to be quite beneficial.
Traditional therapy for vulvar vestibulitis typically includes topical steroids, Amitriptyline 25-50
mg q HS. Surgical excision (Woodruff procedure) (Figure 4) is very beneficial in patients who
have first undergone treatment of their
pelvic floor tension myalgia and whose
symptoms are truly related to
intercourse and not those patients who
have shifted into a continuous form of
vulvodynia (Woodruff procedures
demonstrate a very poor outcome in
those patients who have continuous
pain and vulvar burning).
Dysesthetic vulvodynia represents patients who report continuous vulvar burning and
discomfort, yet often have no skin changes whatsoever. Their burning and discomfort are often
associated with the light touch of clothing that significantly aggravates the burning that is
already present. These patients quite frequently have severe pelvic floor muscle tension
disorders that are often associated with other forms of chronic pain syndromes such as
urgency/frequency syndrome, interstitial cystitis, functional bowel disease and fibromyalgia. It is
thought that dysesthetic vulvodynia has a neuropathic etiology and probably represents a
classic example of visceral somatic hyperalgesia. Therefore, neurolytic agents such as
Amitriptyline represent the mainstay of therapy. It is extremely important in patients with
Figure 4
Vestibulectomy
dysesthetic vulvodynia to look for other sources of visceral pain with the vulvodynia representing
only the referral hyperalgesia. It has been my experience and the experience of many others
that if one performs a potassium chloride test in these patients (Parson’s test) it is often positive
and therefore the patients actually have a form of interstitial cystitis [15]. Once the interstitial
cystitis is treated, the vulvodynia resolves. Glazer has shown impressive results with pelvic
floor rehabilitation in patients with dysesthetic vulvodynia [11]. Bergeron and Glazer have
recently published an excellent study comparing behavioral therapy, biofeedback and
vestibulectomy in the treatment of vulvar vestibulitis. They showed that vestibulectomy resulted
in the best outcome in patients with
vulvar vestibulitis [12]. The key is
vulvar pain only with sex and therefore
not dysesthetic vulvodynia. (Figure 5)
Pudendal neuralgia is simply
dysesthetic vulvodynia in which the
patient has a known history of
neurologic trauma such as
lumbosacral disease, post delivery
avulsion of the pelvic floor with resultant localized pudendal defects, etc. Neurolytic agents are
again used for this subtype of neuropathic vulvodynia.
Interstitial cystitis represents a clinical syndrome with symptoms that include urgency,
frequency, suprapubic pain and irritable voiding symptoms that are often made worse with
Figure 5
INTERSTITIAL CYSTITIS:
Paiin IIndex
0
1
2
3
4
5
6
7
Pre Tx Post Tx 6 months
Vestibulectomy
Surface EMG
Group CB Tx
intercourse. Throughout the literature, inconsistent use of other nomenclatures including
urethritis, urethral syndrome, trigonitis and chronic cystitis have been used to the point that
NIDDK developed very strict criteria to define interstitial cystitis. However, it is now agreed that
if we all stick to the strict criteria of interstitial cystitis, two-thirds of patients that might benefit by
therapy for interstitial cystitis will not be diagnosed to have that disorder. The most important
concept concerning this diagnosis is to remember that patients with urinary tract infections will
have a positive urine culture. If patients repeatedly give symptoms that have been diagnosed to
be urinary tract infections, it is paramount that we establish that diagnosis through the use of
urine cultures, and if the patient repeatedly has negative cultures, we cannot say this patient
has recurrent urinary tract infections.
Somewhere between 500,000 and 800,000 patients in the United States have interstitial cystitis.
90% of these are women. 50% are unable to work full-time and 60% report severe dyspareunia.
The quality of life associated with this disorder is less than those patients on renal dialysis, and
interstitial cystitis is highly associated with a history of having undergone a previous
hysterectomy, having functional bowel
disease and vulvodynia [13]. The key
finding is frequent voids and by definition of
the International Continence Society,
voiding more than eight times in 24 hours
would be considered excessive unless fluid
intake is excessive. (Figure 6) The normal
functional volume of a bladder is 8 to 12
oz. per void, and if your patient is repeatedly demonstrating voided amounts of less than or
equal to 4 oz., this would be considered an obviously abnormal urolog. Key findings on physical
examination include a generalized hypersensitivity of the bladder base and trigone with an
Figure 6
Voids Per Day
Normal Subjects vs. IC Subjects
0
2
4
6
8
10
12
14
16
3 5 7 9 11 13 15 17 19 21
Numb er of Voids / Day
Normal Subjects N=48 IC Pat ients N=145
Courtesy of CL Parsons
otherwise relatively negative exam. Somewhere between 75% and 95% of patients with
interstitial cystitis are also found to have pelvic floor hypertonic disorders and therefore a careful
pelvic floor exam is necessary. Office testing that is beneficial in evaluating patients thought to
have interstitial cystitis includes a uroflow to assess voiding function (some 25% to 70% of
patients with interstitial cystitis have intermittent flow with significant hesitancy), a catheterized
urine sample obtained for a post void residual, a urinalysis, a microscopic examination to rule
out significant hematuria, and the performance of a urine culture to rule out an infection. Recent
data has demonstrated that 48% of patients that present with urgency/frequency symptoms
compatible with interstitial cystitis and who give a history of these symptoms starting at the
same time as a new sexual contact, are found to have Mycoplasma or Ureaplasma in their
vaginal vault [14] (this is a slight twist of the old data concerning Chlamydial urethritis in patients
found to have urethral syndrome). It is also important to note that standard Chlamydial tests will
test negative in a patient with Ureaplasma or Mycoplasma. This infectious process easily
responds to Azithromycin. Cystoscopy under general anesthesia is becoming an extremely
controversy tool to be used solely for the purpose of ruling in interstitial cystitis. A
cystourethroscopy in the office setting is important, particularly in older patients, to rule out a
bladder malignancy (found in 5 out of 202 patients with symptoms of urgency/frequency
syndrome), but the findings of petechial hemorrhage under general anesthesia is not diagnostic
of interstitial cystitis, and in fact patients with no evidence of interstitial cystitis at all will
demonstrate that cystoscopic finding when their bladder is over-distended.
Lowell Parsons in 1994 developed a simple test to look for a chemical hypersensitivity to the
infusion of potassium chloride into the bladder referred to as the KCl test or Parsons test. (See
appendix) He found that 70% of patients with the clinical diagnosis of interstitial cystitis had an
excessive amount of pain and/or urgency when potassium chloride was instilled into the bladder
as compared to saline (these substances are instilled in the bladder under a blinded condition
and patients are asked to grade their symptoms on a scale of 1 to 5. If the patient tests positive,
this represents a manifestation of a GAG layer deficiency with resultant tendency for acids,
potassium and other solutes to traverse through the normally protective GAG layer into the
submucosal layers thus causing many of the symptoms and neuropathic changes that are found
in interstitial cystitis. A very important finding was recently reported in OB/GYN (OB/GYN
2001;98:127) by Parsons et al. In this study, 134 patients with gynecologic diagnoses involving
pelvic pain syndromes (including the diagnosis of chronic pelvic pain, vulvodynia, endometriosis
and dyspareunia) were studied to determine if they had a positive KCl test. 79% to 100% of
patients tested were found to have a positive KCl test. When these patients were asked to fill
out a urologic questionnaire, 75% were found to have urologic symptoms. This test points out
that this GAG layer deficiency is commonly seen in most patients with any form of pelvic pain
disorder [15]. Thus, treatment should be directed towards this obvious GAG layer defect.
Treatment of interstitial cystitis (Figure 7)
includes simple behavioral modification
(maintaining a fluid intake of
approximately 64 oz. per day), low acid
diet, estrogen vaginal cream if the patient
is hypoestrogenic and the treatment of
pelvic floor muscle dysfunction that is so
commonly seen. Pharmacologic therapy
includes tricyclic antidepressants, the use
of antihistamines (especially with a
significant history of allergies), and the use of bladder anesthetics such as Pyridium or
Urimax to alleviate intermittent flares of bladder symptomatology. Intravesical heparin and/or
Figure 7
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Customized Treatment of
Interstitial Cystitis
Dietmodification
+
Self-help
+
Pelvic floor reelaxationn
+
Amitriptyline
+
Intravesical heparin /
oral Elmiron®
Allergic component
Antihistamine
Depression, sleep
deprivation
Antidepressant
Severe pain
Intravesical medication
(short-termrelief) and
opiods
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ICTTreatment AlsoTreat Appropriately
the use of oral Elmiron has been shown to have an extremely high success rate in alleviation
of bladder pain. As a follow-up to the previously mentioned data concerning gynecologic pain,
unpublished data demonstrates that gynecologic pain seems to markedly improve in patients
treated with Elmiron . If patients fail to respond to traditional therapies of interstitial cystitis, a
new modality that has been available since 1999 is sacral neuromodulation using InterStim .
The success rate for this type of neuromodulation has been found to be quite good in properly
selected patients. Antidotally, sacral neuromodulation has also been demonstrated to alleviate
significant functional bowel disease (both constipation and diarrhea varieties), vulvodynia, and
pelvic pain.
Once we understand the
neuropathic activation that occurs
in the sacral cords of our patients
with any form of prolonged pelvic
pain disorder, it should not be
surprising that these patients often
have coexisting symptoms. Pelvic
neuropathic hypersensitivity is a
new term that attempts to unify the
concepts of neuropathic up regulation and multisite involvement in our patients with
chronic pelvic pain. (Figure 8) A basic rule of management of any patient with chronic
Figure 8
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SUMMARY:
Pelvic Neuropathic Hypersensitivity
Sacral Cord
Neuropathic Activation
IC FBD
CPP PFTM
Vulvodynia
ANY
Bladder Insult
Neuroimmune
Cascade:
Histamine
Mast Cell activation
NGF
Substan ce P
NK 1
Bradykinin
APF
Secondary
PFM
Dysfunction
Pain &
Voiding
Dysfunction
Sensory Processing
Abnormality
Visceral/somatic
Pain Syndromes:
Visceral
Inflammatory/
Pain Stimuli
Primary
Sacral
Dysregulation/
PF Dysbehavior
Uroepithelial Damage
pain is therefore to identify each source of pain and to design therapy to treat each
component of the pain. Thus through the combination of behavioral modification for
bowel and bladder disorders, physical therapy for the myofascial problems including
trigger point injections, and pharmacologic intervention to try to down regulate the
neural cascade that has been initiated by the neuropathic response occurring within the
spinal cord, we can provide these patients with the best outcome possible [16,17]. The
initiation of early intervention is the key to preventing the development of these complex
chronic pelvic pain syndromes. It is important to try to identify the original end organ
insult, whether it is endometriosis, interstitial cystitis or pelvic muscle dysfunction, and to
treat that original insult it is still present. As stated earlier, one of the keys to these
complex syndromes is the treatment of all sources of pain and dysfunction. Yet, our
biggest challenge is to identify the characteristics that suggest the source of the pain
has left the end organ and has become centralized or neuropathic in origin. These
characteristics include symptoms of multisite visceral hyperalgesia and an allodynic
exam. When this has occurred, it is important that we design interventions that will not
further up regulate the sacral cord [18] (such as doing repeated surgeries), but instead
down regulate the neuropathic changes by decreasing the “volume of pain” that the
spinal cord sees so that the patient’s own modulating pathways can hopefully handle
what is left behind
.
