Sunday, February 16, 2014

Pudendal Neuralgia & Vulvodynia

Pudendal Neuralgia:

What is it?

"Pudendal Neuralgia is a painful neuropathic condition that is caused by inflammation of the pudendal nerve."


"The main symptom of pudendal neuralgia (PN) and pudendal nerve entrapment (PNE) is pain in one or more of the areas innervated by the pudendal nerve or one of its branches. These areas include the rectum, anus, urethra, perineum, and genital area. In women this includes the clitoris, mons pubis, vulva, lower 1/3 of the vagina, and labia. In men this includes the penis and scrotum. But often pain is referred to nearby areas in the pelvis. The symptoms can start suddenly or develop slowly over time. Typically pain gets worse as the day progresses and is worse with sitting. The pain can be on one or both sides and in any of the areas innervated by the pudendal nerve, depending on which nerve fibers and which nerve branches are affected. The skin in these areas may be hypersensitive to touch or pressure (hyperesthesia or allodynia).
Possible symptoms include burning, numbness, increased sensitivity, electric shock or stabbing pain, knife-like or aching pain, feeling of a lump or foreign body in the vagina or rectum, twisting or pinching, abnormal temperature sensations, hot poker sensation, constipation, pain and straining with bowel movements, straining or burning when urinating, painful intercourse, and sexual dysfunction – persistent genital arousal disorder (genital arousal without desire) or the opposite problem - loss of sensation.
It is not uncommon for PN to be accompanied by musculoskeletal pain in other parts of the pelvis such as the sacroiliac joint, piriformis muscle, or coccyx. It is usually very difficult to distinguish between PN and pelvic floor dysfunction because they are frequently seen together. Some people refer to this condition as pelvic myoneuropathy which suggests both a neural and muscular component involving tense muscles in the pelvic floor.
Other Possible Symptoms
  • The chief symptom is pain in the area innervated by the pudendal nerves such that sitting becomes intolerable.
  • The pain may be lessened when sitting on a toilet seat or a doughnut pillow as this lessens the pressure on the pudendal nerve. Most people simply have to avoid sitting because it is impossible to find a cushion that relieves pain in all areas.
  • The pain is often not immediate but delayed and continuous and stays long after one has discontinued the activity that caused the pain (stop sitting, cycling, sex...).
  • Often the pain is lower in the morning upon awakening and increases throughout the day.
  • There may be extreme pain or tenderness along the course of the nerve when the nerve is pressed on via the vagina or rectum.
  • Pain in perineum.
  • Pain after orgasm.
  • Loss of sensation with difficulty achieving orgasm.
  • Strange feeling of uncomfortable arousal without sexual desire.
  • Intolerance to tight pants or elastic bands around the legs.
  • Friction and feeling of inflammation along the course of the nerve when walking for too long or running.
  • Constant pain even with standing or lying down.
  • Problem with urinary retention after urination. Need to push to empty bladder. Harder to detect the feeling of urine when passing through the urethra.
  • Urethral burning with or after urination
  • Feeling like the bladder is never empty or feeling the need to urinate even when the bladder is empty.
  • Urinary frequency.
  • Pain after bowel movement. Sometimes sufferers also report pain prior to and during the bowel movement.
  • Painful muscles spasms of the pelvic floor after bowel movement.
  • Constipation.
  • Sexual problems. Men complain of a diminution of sensations. Pain after ejaculation is common. For women pain during and after intercourse is often reported.
  • Scrotum/Testicular pain is possible. The testicle itself is innervated by another nerve however the difference in pain from scrotum/testicle can be hard to detect.
  • Buttock sciatica and everything that goes with it: numbness, coldness, sizzling sensation in legs, feet, or buttock. This is more often due to a reaction of the surrounding muscles to the pain in the pelvic region. It could also be from "cross talk" of the nerves.
  • Low back pain resulting from radiation of the pain.
  • The symptoms can be unilateral or bilateral. If the entrapment is only on one side, the pain can also be reflected to the other side.
  • Some people develop conditions such as complex regional pain syndrome and even post-traumatic stress disorder after prolonged or severe pain."
"The diagnosis is usually made based on the patient’s symptoms, history, and exclusion of other illnesses such as infection or tumor. While no test is 100% accurate some of the more commonly used tests are the pudendal nerve motor latency test (PNMLT), electromyography (EMG), diagnostic nerve blocks, 3T MRI using special software and settings, and magnetic resonance neurography (MRN). Pudendal neuropathy can occur in men or women although about 2/3 of patients are women. It is considered rare and many doctors are just now becoming aware of this illness. Sometimes it is referred to as cyclist’s syndrome, pudendal canal syndrome, or alcock’s syndrome. Pudendal neuropathy can have similar symptoms to another disease or be misdiagnosed as another disease. Those most often associated with or confused with PN are chronic non-bacterial prostatitis, levator ani syndrome, proctalgia fugax, interstitial cystitis, vulvodynia, vestibulitis, chronic pelvic pain syndrome, hemorrhoids, piriformis syndrome, coccydynia, ischial bursitis, idiopathic (of unknown cause) orchialgia, or idiopathic prostadynia.
"The final diagnosis of pudendal neuralgia is based on a person having several or all of these criteria:
  1. Typical PNE symptoms
  2. An abnormal electro physiological test
  3. A positive response to the nerve block
  4. A distinct abnormality on a 3T MRI or an MRN
  5. Pain elicited upon pressing along the course of the nerve
  6. Elimination of other diseases being the cause"
For more exact information (like what the physical exam should include, etc.) see:
"There are numerous possible causes for pudendal neuropathy. Some of the possible causes are an inflammatory or autoimmune illness, frequent infections, tension on the nerve, a nerve entrapment similar to carpel tunnel syndrome, or trauma to the nerve from an accident/fall, exercise, childbirth, prolonged sitting, or surgery. Sometimes there is no apparent explanation and some doctors have theorized that the problem can be hereditary due to a musculoskeletal predisposition. Occasionally the problem originates in the spine or sacral area rather then the peripheral pudendal nerve.
Pudendal neuralgia can be caused by inflammation of the nerve or by mechanical damage/trauma to the nerve. Sometimes the pain develops slowly and is almost imperceptible at first, sometimes preceded by paresthesia in the area innervated by the pudendal nerve. Paresthesia is a “pins and needles” sensation or a feeling of prickling, numbness, and tingling.
Many people however recall one event in particular as the beginning of their symptoms. Some recall the feeling of a lightning electrical shock after a bad move. Some people report their symptoms started after direct shock like a fall on the buttock or a car accident.  Others report pain after a sacral surgery such as a sacroiliac joint fusion resulting in a tilted pelvis or a pelvic surgery such as a sacrospinal fixation. Sometimes there is direct trauma to the nerve either from retractors or misplaced sutures. Pelvic surgery such as a hysterectomy may trigger pudendal neuralgia even though the nerve was not touched directly.  One theory is that the nerve can undergo a stretch injury if the body is in a certain position for a long period of time during surgery. Sometimes women develop pudendal nerve pain immediately following childbirth and while often this eventually subsides, for some women the pain does not go away. Women with severe endometriosis may develop scarring or inflammation if the endometriosis settles on the nerve. 
Prolonged sitting at work and frequent long drives are a common cause of compression to the nerve. Sports involving repetitive hip flexion like heavy weight lifting may cause enlarged or strained ligaments or enlarged muscles that impinge on the nerve. Some young athletes have been shown to have an elongated ischial spine, a bone that protrudes into the pelvis near the pudendal nerve. Cycling is a leading favorable risk factor for the development of the condition. In the sports medicine community it is sometimes called “cyclist syndrome”.
One hypothesis suggests that people who have PN were predisposed to have it and something occurred that triggered it. Other people who are predisposed may never develop the condition if they never engage in an activity or experience an incident that triggers it. For instance, someone who is predisposed to PN may take up weightlifting and consequently develop PN while another person who is predisposed but does not weight lift will not develop PN.
Tight muscles, tendons, or enlarged ligaments can lead to constant friction on the nerve or if the pelvis is out of alignment there may be undue pressure on the nerve. For some, the pudendal nerve can follow an irregular path or they may naturally have a tight space between the ligaments at the ischial spine or in the alcock’s canal. Some doctors have seen PN run in families, with several members in successive generations developing PN. Some people tend to form excessive scar tissue and this may lead to entrapment of the nerve. Certain autoimmune or inflammatory illnesses have been linked to pudendal neuralgia.
However, sometimes the cause remains unknown."


What Is It?
"Vulvodynia, simply put, is chronic vulvar pain without an identifiable cause. The location, constancy and severity of the pain vary among sufferers. Some women experience pain in only one area of the vulva, while others experience pain in multiple areas. The most commonly reported symptom is burning, but women’s descriptions of the pain vary. One woman reported her pain felt like “acid being poured on my skin,” while another described it as “constant knife-like pain.”
There are two main subtypes of vulvodynia, which sometimes co-exist:

Vulvar Vestibulitis Syndrome
(aka Provoked Vestibulodynia)

As shown in the diagram on the right, vulvar vestibulitis syndrome (VVS) is characterized by pain limited to the vestibule, the area surrounding the opening of the vagina. It occurs during or after pressure is applied to the vestibule, e.g., with sexual intercourse, tampon insertion, a gynecologic examination, prolonged sitting and/or wearing fitted pants.

VVS is further classified as Primary or Secondary. Women with Primary VVS have experienced vestibular pain since the first attempt at vaginal penetration. Women with Secondary VVS have experienced pain-free sexual intercourse prior to the development of pain.

Generalized Vulvodynia

For women with generalized vulvodynia (GV), pain occurs spontaneously and is relatively constant, but there can be some periods of symptom relief. Activities that apply pressure to the vulva, such as prolonged sitting or simply wearing pants, typically exacerbate symptoms.

Some women experience pain in a specific area, e.g., only in the left labia or near the clitoris, while others experience pain in multiple areas, e.g., in the labia, vestibule, and clitoris. In the latter group, pain may also occur in the perineum and inner thighs, as demonstrated in the diagram on the right.

Learn more by viewing NVA’s Online Teaching Program."
"Pain is the most notable symptom of vulvodynia, and can be characterized as a burning, stinging, irritation or sharp pain that occurs in the vulva, including the labia and entrance to the vagina. It may be constant, intermittent or happening only when the vulva is touched, but vulvodynia is usually defined as lasting for at least three months. The pain is usually found around the urethra and at the top of the legs and inner thighs, and it can be either intermittent or constant. Symptoms may occur in one place or the entire vulvar area.

The pain is usually described as a burning, stinging, itching, irritating or a raw feeling. Sexual intercourse, walking, sitting or exercising can make the pain worse. It can be present in the labia majora and/or labia minora. Sometimes it affects the clitoris, perineum, mons pubis and/or inner thighs. The pain may be constant or intermittent, and it is not necessarily initiated by touch or pressure to the vulva. The vulvar tissue may appear inflamed, but in most cases there are no visible findings. Vulvodynia usually starts suddenly and may last for months to years. Although it isn't life-threatening, the pain may make one cut back on some normal activities. It can also make one upset or depressed. It might even cause problems in one's relationship with spouse or partner, because it can make sexual intercourse painful."

"The best tool for making a diagnosis of vulvodynia is your ears — listen to what your patient is telling you! As part of the patient's medical history, make sure you note any association between the onset or exacerbation of symptoms and life changes/stressors, changes in medical status, surgeries, and hormonal changes, including childbirth, lactation, and menopause. Physical examination should include evaluation for infection, inflammatory process, and vulvar dystrophies.
Vulvodynia may present as generalized on the vulva or localized within the vestibule. Q-tip testing is very important in making the appropriate diagnosis of vulvodynia. Note if sensitivity is present on the vulva or within the vestibule at the Skene's and Bartholin glands. Use a 0- to 10-point rating scale, with 0 being no pain/symptoms and 10 being the worst level of pain/symptoms. If a diagnosis of vulvodynia is made, Q-tip testing is helpful as an objective measure of level of discomfort (and hopefully improvement) over time.
Vulvodynia is ultimately a diagnosis of exclusion after all other potential causes have been ruled out and symptoms have persisted for at least 6 months."
"The initial treatment for any woman presenting with vulvar symptoms is to institute vulvar skin-care guidelines. These are designed to remove any contact irritants to the vulva, such as scented soaps, detergents, hot water, shaving, and washcloths.

"Neuropathic pain medications are the mainstay of treatment for vulvodynia. These alter the perception of pain by blocking reuptake transmitters, norepinephrine, and serotonin.
My first-line therapy is normally the tricyclic antidepressants, including amitriptyline, nortriptyline, and desipramine. I use amitriptyline primarily, which has a 60% response rate. It is generic and readily available at minimal cost. Fatigue is the primary side effect at the low doses used for treatment. Most patients develop a tolerance for this over time.
My next line of treatment is the anticonvulsants. Gabapentin can be used individually or in combination with amitriptyline. More than 60% of patients have shown significant improvement when prescribed gabapentin. I have also used pregabalin, which has been associated with results similar to gabapentin.

"Infrequently, I use amitriptyline 2% with baclofen 2% as a topical treatment, but that preparation must be compounded and has greater costs. If the patient has concurrent depression, I will also use a selective serotonin–norepinephrine reuptake inhibitor, such as duloxetine. I avoid most topical medications because they serve as a contact irritant over time and offer little symptom resolution. I have commonly seen women being prescribed topical lidocaine. This can become a contact irritant with routine use, but it can provide emergency relief to break the pain cycle. 

"Many women with vulvodynia have increased resting tone, poor strength, and/or irritability of muscles. In those patients, biofeedback therapy can be very helpful. Biofeedback has a success rate of 60% to 80%. Physical therapy with a therapist trained in the pelvic-floor musculature can be very helpful, either alone or in conjunction with biofeedback. It is important not to start physical therapy or biofeedback until the vestibular Q-tip testing has improved (i.e., the gland scores have decreased). Starting these therapies too soon will likely inhibit results and give woman a sense of failure.         

"Since stress plays a role in vulvodynia, any stress-reduction technique, such as meditation and yoga, can be used. Some have found acupuncture helpful. Psychotherapy can be useful because this is a chronic-pain state and women often suffer with depression or relationship problems. It is especially important to let patients know that you understand their problem is a real and debilitating condition, not just "in their heads."

"The last option for women suffering from localized vulvodynia is vestibulectomy. This should be considered only after all other options have failed. "

One extra note:
"It is thought that there can be overlap between vulvodynia and IC. Studies suggest that the prevalence of concurrent IC and vulvodynia ranges from 12% to 68%. Both IC and vulvodynia are syndromes of the urogenital sinus, including pelvic-floor muscle dysfunction, inflammatory changes with activation of mast cells, increased angiogenesis, and neural hyperplasia."