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Pudendal Neuralgia: Symptoms & Causes

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0% found this document useful (0 votes)
38 views10 pages

Pudendal Neuralgia: Symptoms & Causes

Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
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Pudendal N euralgia

Waseem Khoder, MD, Douglass Hale, MD, FACOG, FACS*

KEYWORDS
 Pudendal neuralgia  Pudendal nerve entrapment  Nantes criteria
 Pudendal nerve block

KEY POINTS
 Pain is most often unilateral and increases with sitting.
 Diagnosis is made clinically. Nantes criteria can be helpful in making the diagnosis.
 Treatment options include physical therapy, medications, pudendal nerve blocks, and
surgical decompression.

SYMPTOMS OF PUDENDAL NEURALGIA

Pudendal neuralgia is a painful neuropathic condition, involving the dermatome of the


pudendal nerve.1 Amarenco and colleagues2,3 described pudendal neuralgia first in
1987. Patients with pudendal neuralgia usually present with burning pain in the dis-
tribution of the pudendal nerve. The pain is localized to the vulva, vagina, clitoris,
perineum, and rectum in females and to the glans penis, scrotum, perineum, and
rectum in males.1 The pain can involve the entire area innervated by the pudendal
nerve or affect a smaller region involving only a particular branch. In these cases,
the pain is restricted to the terminal branches and may involve only the clitoris, the
vulva/vaginal area alone, or the rectum alone.4 Patients with pudendal neuralgia
may have associated symptoms such as urinary frequency and urgency, symptoms
of painful bladder syndrome, and dyspareunia.5,6 The classic presentation of puden-
dal neuralgia is unilateral pain. However, bilateral pudendal neuralgia has been
reported.7
Patients may have significant hyperalgesia (increased sensitivity and significant pain
to mild painful stimulus), allodynia (pain in response to nonpainful stimulus), and par-
esthesias (sensation of tingling, pricking, or numbness).7 Typically, symptoms are

The authors have nothing to disclose.


Division of Female Pelvic Medicine and Reconstructive Surgery, Department of Obstetrics and
Gynecology, Indiana University School of Medicine, 1633 North Capitol Avenue, Suite 436, Indi-
anapolis, IN 46202, USA
* Corresponding author.
E-mail address: dhale@iuhealth.org

Obstet Gynecol Clin N Am 41 (2014) 443–452


http://dx.doi.org/10.1016/j.ogc.2014.04.002 obgyn.theclinics.com
0889-8545/14/$ – see front matter Ó 2014 Elsevier Inc. All rights reserved.
444 Khoder & Hale

present when patients are sitting down and are much less severe or even absent when
they lying down or standing.4 Previous reports have shown that patients have signif-
icantly less pain when sitting on a toilet seat versus a chair. This phenomenon is
believed to be associated with descent of the levator ani and less compression
applied to the pudendal nerve. Patients usually awaken in the morning with minimal
or no symptoms; however, the pain increases as the day progresses. Patients may
report the sensation of having a foreign body in the vagina or feel as though they
are sitting on an object. This pain may lead to some patients favoring a certain side
while sitting, something that may be clinically observed by the clinician entering the
examination room.8

ANATOMY

The pudendal nerve consists of sensory, motor, and autonomic nerve fibers. Sensory
nerve cell bodies are located in the dorsal root ganglia of the sacrum, S2–S4. Anterior
horn cells are located in the ventral horn of the sacral spinal cord (S2–S4) in a region
called Onuf’s (Onufrowicz) nucleus.9 The nerve forms in the sacral plexus and comes
to lie medially and caudally in relation to the trunk of the sciatic nerve. Passing laterally,
it enters the gluteal region in the infrapiriform canal and then traverses the greater
sciatic foramen.1 Accompanied by its artery, usually situated cranial to the nerve, it
is also surrounded by veins.
The pudendal bundle passes around the termination of the sacrospinous liga-
ment just before its attachment to the ischial spine. At this level, the pudendal
nerve is situated between the sacrospinous ligament ventrally and the sacrotuber-
ous ligament dorsally. In rare cases, the nerve may travel between split layers of the
sacrotuberous ligament. The nerve then passes ventrally, medially, and caudally
and enters the perineal region via the lesser sciatic foramen. It lies lateral to the
plane of the levator ani muscle traveling within a duplication of fascia on the medial
surface of the obturator internus muscle, which forms the Alcock canal (Fig. 1).10
The canal contains the pudendal nerve and vessels embedded in loose areolar
tissue.11 Most often, the 3 branches of the neurovascular bundle arise inside the
canal: the inferior rectal nerve, the perineal nerve, and the dorsal nerve of the
clitoris.12
The inferior rectal nerve supplies the integument around the anus and communi-
cates with the perineal branch of the posterior femoral cutaneous nerve and its termi-
nal branch, the labia majora nerve. The inferior rectal branch provides sensation to the
distal aspect of the anal canal and to the perianal skin. This branch also provides mo-
tor innervation to the external anal sphincter.
The perineal nerve has a deep motor portion and 2 superficial sensory branches, the
medial and lateral posterior labial nerves. This nerve provides sensation to the peri-
neum and the ipsilateral labia majora. It also provides motor innervation to the trans-
verse perinei muscle, the bulbospongiosus, the ischiocavernosus, and the sphincter
urethrae and possibly contributes some branches to the levator ani muscles.13 This
branch emerges at the posterior part of the Alcock canal.12
The dorsal nerve of the clitoris is the terminal and most superficial branch of the
pudendal nerve, found at the level of the symphysis pubis. The nerve is an afferent
nerve that carries sensory information from the clitoris. Emerging from the dorsal
aspect of the erectile organs, it travels in the infrapubic region and enters the Alcock
canal where it joins the nerve trunk.1
Although the anatomy of the pudendal nerve is well outlined, great variation may
exist, especially within the ischiorectal fossa, after its branches exit from Alcock
Pudendal Neuralgia 445

Fig. 1. The pudendal nerve (marked by a needle) when passing in the Alcock canal along the
obturator internus muscle. 1, Coccyx; 2, sacrotuberous ligament; 3, ischial tuberosity; 4,
anus; 5, piriformis muscle; 6, iliococcygeus muscle.

canal.14 As the branches of the nerve run relatively superficially through the pelvis,
they are vulnerable to injury.

CAUSES OF PUDENDAL NEURALGIA

Causes can be classified into mechanical, infectious, and immunologic.15 The


mechanical compromise may result from surgical procedures, trauma, or childbirth.16
Mechanical compression has often been referred to as entrapment, similar to carpal
tunnel syndrome.17,18 This entrapment may be caused by pelvic floor muscle spasm,
pressure from surrounding ligaments (sacrospinous, sacrotuberous), or scar tissue
from trauma or surgeries involving the surrounding areas.
A common cause cited for entrapment is prior surgical procedure for prolapse or in-
continence, where entrapment may be caused by mesh or suture.19–21 Examples of
procedures that have the potential to cause injury to the nerve are those that involve
sacrospinous ligament fixation for the treatment of vaginal vault prolapse.22,23
Because the nerve runs inferior to the sacrospinous ligament, there is the potential
for entrapment of the nerve if the suture is misplaced.
Entrapment from inflammation and scarring can occur in patients with a prior history
of pelvic trauma, falls injuring the back or buttocks, as well as traumatic insertion of
foreign objects into the rectum or vagina. This scarring may occur around the ischial
spine, between the sacrotuberous and sacrospinous ligaments, along the falciform
process of the sacrotuberous ligament, or by compression of the nerve as it courses
through Alcock canal.
Other causes may include herpes simplex infection, compression or inflammation
from tumors, endometriosis, cycling, squatting exercises, and chemoradiation.24–27
446 Khoder & Hale

NERVE INJURY TYPES

There are 3 common mechanisms of nerve injury:


1. Compression injuries are most common and can occur as acute or chronic
lesions. The severity of damage is related to the magnitude as well as the duration
of the trauma. Blood supply to the nerve is compromised, which may result in
demyelination injury, and the resulting functional disorder may range from slight
paresthesias and/or motor weakness to complete sensory loss and/or muscle
paralysis.28
2. Stretch injuries can also occur and are commonly related to childbirth. As little as
10% stretch in relation to the length of the nerve can lead to damage.29
3. Transection injuries occur far less commonly, but are also most difficult to treat.28
Regeneration rates, including remyelination, have been reported to be around
1 mm/d in clinical situations, with further diminishing rates over time. These rates
vary depending on the extent of injury, as well as the type, with higher rates of regen-
eration in compression injuries and lower rates in transection injuries.30
Classification of nerve injuries31:
Neurapraxia: trauma causes destruction of the myelin sheath, without affecting the
axons or causing rupture of the surrounding connective tissue. This local
conduction blockage resolves normally in less than 12 weeks as the nerve
remyelinates.
Axonotmesis: trauma causes destruction of the myelin sheath and downstream
Wallerian degeneration. The encapsulating connective tissue (endoneurium) is
preserved, thus serving as a guide for proximal-distal axonal regrowth. Recovery
is slow (1 mm/d) and usually complete.
Neurotmesis: there is a full transection of the nerve, with disrupted continuity in all
the layers and downstream Wallerian degeneration. Surgical intervention to rees-
tablish continuity is required, or nerve regrowth may result in a proximal
neuroma.

DIAGNOSIS

History and physical examination are the most important components leading to diag-
nosis. Causes that can present with a similar constellation of symptoms must be
excluded. These causes include painful bladder syndrome, vulvodynia, levator
myalgia, piriformis syndrome, coccydynia, cauda equina syndrome, and neuralgias
of other nerves such as the obturator, genitofemoral, or ilioinguinal nerves.
Detailed history should elicit the pain characteristics including onset, type, duration,
aggravating and alleviating factors, and frequency. Examination should include
inspection for any lesions in the perineum, vulva, and vagina; palpation and Q-tip
examination to rule out vulvodynia; and bimanual examination focusing on pelvic floor
muscles. In addition to the levator muscles, the piriformis and the obturator internus
and externus should be thoroughly evaluated. Palpation on the ischial spine or puden-
dal nerve that produces paresthesias or pain is referred to Tinel’s sign. Some patients
start favoring one side of their pelvis while sitting, which can be discretely observed
during history and physical examination.

Nantes Criteria for Diagnosis of Pudendal Neuralgia


In 2008, Dr Labat4 published the Nantes criteria for the diagnosis of pudendal
neuralgia.
Pudendal Neuralgia 447

This article gave some structure to making this diagnosis. According to these
criteria, a patient must exhibit all 5 characteristics, without any symptoms of the exclu-
sion criteria.4

Inclusion criteria

Nantes criteria for the diagnosis of pudendal neuralgia

Pain in the area innervated by the pudendal nerve extending from anus to clitoris
Pain is more severe when sitting
Pain does not awaken patients from sleep
Pain with no objective sensory impairment
Pain relieved by diagnostic pudendal block

Data from Labat JJ, Riant T, Robert R, et al. Diagnostic criteria for pudendal neuralgia by
pudendal nerve entrapment (Nantes criteria). Neurourol Urodyn 2008;27(4):306–10.

Exclusion criteria
Pain located exclusively in the coccygeal, gluteal, pubic, or hypogastric area
(without pain in the area of distribution of pudendal nerve)
Pruritus
Exclusively paroxysmal pain
Abnormalities on any imaging test (magnetic resonance imaging [MRI], computed
tomography [CT], and others) that might explain the pain

Complementary diagnostic criteria


Pain characteristics: burning, shooting, stabbing, numbing
Allodynia or hyperesthesia
Sensation of foreign body in the rectum or vagina (sympathalgia)
Pain is progressively worse throughout the day
Pain is predominantly unilateral
Pain is triggered by defecation
Significant tenderness around the ischial spine on vaginal or rectal examination
Abnormal neurophysiology testing (pudendal nerve motor latency testing) in nullip-
arous women

Additional Testing
There are no imaging studies that diagnose pudendal neuralgia; however, MRI and CT
may assist in excluding other causes of the pain.
Neurophysiology tests such as pudendal nerve terminal motor latency (PNTML) test
and electromyography (EMG) may serve as complementary diagnostic measures.32
However, they are not specific for patients with pudendal neuralgia. These tests can
give abnormal results in multiple types of nerve injuries. This fact limits the utility of
these tests when performed in multiparous women because of the high incidence of
asymptomatic stretch and compression nerve injuries in this group of women.
In addition, PNTML test is a good test of demyelization and crushing but not of nerve
fiber loss. The PNTML test examines only the motor function of the nerve and cannot
provide any direct evidence of sensory nerve damage.33 Therefore, an abnormal result
448 Khoder & Hale

of PNTML test indicates that the pudendal nerve is affected, but it is not specific for
pudendal neuralgia. Conversely, a normal reading does not rule out pudendal neural-
gia because only the motor nerves are being evaluated. During PNTML testing, the
nerve is stimulated electrically and special electrodes measure the speed of the stim-
ulus transmission. This speed includes the time for transmission along the axon from a
designated spot on the nerve, transmission across the neuromuscular junction, and
lastly, muscle contraction. If the latency in longer than 2.2 ms, nerve damage may
be present. It should be noted that the PNTML measures only the largest, fastest con-
ducting nerves. This measurement may remain normal despite extensive impairment
of smaller nerves.34
EMG and single-fiber EMG with fiber density measurements are better able to docu-
ment neuropathy compared with latency tests. However, this testing only evaluates
the motor function of the nerve and can be quite uncomfortable and painful. To get
useful results, multiple needle placements are required for recording data from the
muscle. It is recommended that at least 20 sites be assessed and the results be aver-
aged. The use of local anesthetic would adversely affect results.35

TREATMENT
Conservative Management
Physical therapy has been considered the first-line treatment by many investigators.
Multiple stretches and exercises have been described to help reduce pain levels.
Patients are preferably treated by therapists who specialize in pelvic floor therapy.
Most of these patients have muscle spasm as a primary or secondary reaction. As
such, therapists should address muscle imbalances, spasm, and other dysfunctions.8
Therapists focus on palpation and manual techniques, posture, range of motion, and
strength of the pelvis, back, and hips. Therapists have a variety of techniques to manu-
ally release the muscle spasm and help lengthen the muscles.36 Therapy is most often
applied through the vagina but can also be applied through the rectum.
Electrical stimulation and biofeedback have been used to assist therapists with
treatment. Patients are given a home exercise regimen that includes relaxation and
lifestyle modifications to continue the benefits gained during office sessions. In cases
of muscle spasm refractory to physical therapy, botulinum toxin has been used.37
Medications can also be used for first-line treatment, including muscle relaxants
and neuromodulators. Some of the medications commonly used are gabapentin, pre-
gabalin (75 mg orally twice daily), cyclobenzaprine, and tricyclic antidepressants.
Local medications, such as intravaginal diazepam (5-mg tablets up to 3 times daily
or compounded suppositories), have also been applied; however, studies conflict
on outcomes. Some studies mention using rectal belladonna and opium suppositories
as possible adjuncts.38

Pudendal Nerve Block


Multiple approaches have been used for pudendal nerve block including transperineal,
transgluteal, transrectal, and most often in women, a transvaginal approach. The
ischial spine is used as a landmark to identify the injection site. CT-guided approaches
have been reported in many studies. Investigators reported that using CT improves the
accuracy of locating the pudendal nerve.39 Another study reported fluoroscopy to aid
in injection because the needle was placed on the tip of the ischial spine.40 Magnetic
resonance (MR) neurography has been used for the evaluation of some nerve
compression syndromes including carpel tunnel syndrome and also applied to facili-
tate ulnar nerve release surgeries. It has been shown that MR neurography is as
Pudendal Neuralgia 449

effective as needle EMG for identifying patients who are helped by surgical treatment.
Filler41 used MR neurography and open MR image-guided injections in a nonrandom-
ized study in patients to distinguish different entrapment locations of the pudendal
nerve.
Multiple injection protocols have been described using local anesthetics, steroids,
or a combination of the two. One series offered a series of 3 CT-guided injections,
each 6 weeks apart, using a combination of bupivacaine 0.5%, 5 mL, and triamcino-
lone 40 mg/mL, 2 mL.38
Another injection protocol described a set of 3 injections, each 1 week apart. The
first injection is bupivacaine (3 mL of 0.25% solution) only with the next 2 injections
being a mixture of methylprednisolone (1 mL 40 mg/mL solution) and bupivacaine
(3 mL 0.25% solution), if the first one has provided relief to the patient. Immediate
pain relief within minutes of therapy has been reported, and effects may last up to
6 weeks. Patients may benefit from repeated nerve blocks, and therapy needs to be
individualized.42

Surgical Pudendal Nerve Decompression


Decompression is offered to patients when entrapment is suspected and pudendal
nerve blocks have provided minimal or no relief. Approaches to decompression of
an entrapped pudendal nerve include transgluteal, which is performed in the prone
position, and those that may be approached from the lithotomy position. The latter
includes the transperineal (also described as para-anal) and transvaginal
approaches.
The transperineal approach involves opening Alcock canal through the ischiorectal
fossa and releasing the nerve from its entrapment. Shafik6 reported pain relief in 9 of
11 patients with pudendal neuralgia and vuvlvodynia. Beco and colleagues43 used
bilateral decompression through this approach for perineal pain and reported a
57% success rate.
The transgluteal approach uses a gluteal incision with the fibers of the gluteus
muscle being separated longitudinally to reach the sacrotuberous ligament. The
pudendal nerve is then identified after freeing and dividing the sacrotuberous liga-
ment. The pudendal nerve is then decompressed along its entire length, from the pir-
iformis muscle to Alcock canal. The fascia of the obturator internus muscle is incised,
and the nerve is freed. The most common locations for pudendal nerve entrapment, as
reported in the literature, are between the sacrospinous and sacrotuberous ligaments
or the falciform process of sacrotuberous ligament.44
Hibner and colleagues38 described their technique for pudendal nerve decompres-
sion in patients whom pudendal neuralgia began after sacrospinous ligament suspen-
sion or mesh-augmented surgery for prolapse. In these instances, suture or mesh
material is often found directly entrapping the nerve. A segment of Neuragen (Origin
Biomed Inc, Halifax, Canada) was used as a nerve-protecting tubing to prevent
rescarring of the nerve. The nerve was also saturated with a platelet-rich plasma
matrix, which has been shown to promote nerve healing in other nerve surgeries.45
The sacrotuberous ligament was repaired with a graft of cadaveric gracilis tendon after
placing a pain pump catheter along the course of the nerve. The catheter is removed at
20 days, and physical therapy started.38
Robert and colleagues46 published the results of a prospective, randomized
controlled trial that compared transgluteal decompression with nonsurgical treatment
and repetitive pudendal blocks. A total of 32 patients were included in this study (16 in
each group). After 1 year of treatment, 71.4% of the surgery group compared with
13.3% of the nonsurgery group showed improvement.
450 Khoder & Hale

According to the literature, after surgical decompression, approximately 40% of pa-


tients are pain free, 30% have some improvement in pain, and 30% patients neither
show improvement nor worsening.46

SUMMARY

Pudendal neuralgia is a painful condition affecting the nerve distribution of the puden-
dal nerve. The Nantes criteria give some structure in making the diagnosis for this frus-
trating condition. More research is needed to clarify the best diagnostic and treatment
methods for this condition. Until then, a step-ladder approach to therapy, as
described, is suggested when treating these patients.

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