Pelvic and Sympathetic Mediated Pain (CRPS)

You have been referred to the Interventional Pain Medical Group because pain should no longer be an option. Reproductive, gastrointestinal, urologic, sympathetic, musculoskeletal and neuromuscular disorders may cause or contribute to chronic pelvic pain. Your physician has diagnosed abnormal function of the sympathetic (autonomic) nervous system.

Sympathetic pain is a very complicated syndrome. The terminology used for all sympathetic pain is called Complex Regional Pain Syndrome (CRPS), which includes Reflex Sympathetic Dystrophy (RSD), Causalgia, Sympathalgia, Sympathetically Medicated Pain and Sympathetically Independent Pain.

At the Interventional Pain Medical Group, your pain physician will perform a comprehensive evaluation, diagnosis and formulate a treatment plan.

Making the Diagnosis of RSD/CRPS

The diagnosis of RSD/CRPS can be made in the following context. A history of trauma to the affected area associated with pain that is disproportionate to the inciting event plus one or more of the following:

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• Abnormal function of the sympathetic nervous system
  
• Swelling
  
• Movement disorder
  
• Changes in tissue growth (dystrophy and atrophy

Thus patients do not have to meet all of the clinical manifestations listed above to make the diagnosis of RSD/CRPS. The new CRPS classification system acknowledges this fact by stating that some patients with RSD/CRPS may have a third type of CRPS by categorizing it as "otherwise not specified". There seems to be a small group of patients whose pain following trauma resolves over time, leaving the patient with a movement disorder. The pain and symptoms of RSD/CRPS may exceed both the magnitude and duration of symptoms expected from the normal healing process expected from the inciting event. Similarly, the RSD/CRPS diagnosis is precluded by the existence of known pathology that can be explained by the observed symptoms and degree of pain. There are "grades" of this syndrome described in the literature with symptoms ranging from minor to severe.

The left hand and forearm of a 37-year-old man with complex regional pain syndrome (CRPS 1) reveals swelling and color change characteristic of the condition. CRPS arose after a work-related injury.

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Clinical Features of RSD/CRPS

1. Pain – The hallmark of RSD/CRPS is pain and mobility problems out of proportion to those expected from the initial injury. The first and primary complaint occurring in one or more extremities is described as severe, constant, burning and/or deep aching pain. All tactile stimulation of the skin (e.g. wearing clothing, a light breeze) may be perceived as painful (allodynia). Repetitive tactile stimulation (e.g. tapping on the skin) may cause increasing pain with each tap and when the repetitive stimulation stops, there may be a prolonged after-sensation of pain (hyperpathia). There may be diffuse tenderness or point-tender spots in the muscles of the affected region due to small muscle spasms called muscle trigger points (myofascial pain syndrome). There may be spontaneous sharp jabs of pain in the affected region that seem to come from nowhere (paroxysmal dysesthesias and lancinating pains).


2. Skin changes - skin may appear shiny (dystrophy-atrophy), dry or scaly. Hair may initially grow coarse and then thin. Nails in the affected extremity may be more brittle, grow faster and then slower. Faster growing nails is almost proof that the patient has RSD/CRPS. RSD/CRPS is associated with a variety of skin disorders including rashes, ulcers and pustules. 8 Although extremely rare, some patients have required amputation of an extremity due to life-threatening reoccurring infections of the skin. Abnormal sympathetic (vasomotor changes) activity may be associated with skin that is either warm or cold to touch. The patient may perceive sensations of warmth or coolness in the affected limb without even touching it (vasomotor changes). The skin may show increased sweating (sudomotor changes) or increased chilling of the skin with goose flesh (pilomotor changes). Changes in skin color can range from a white mottled appearance to a red or blue appearance. Changes in skin color (and pain) can be triggered by changes in the room temperature, especially cold environments. However, many of these changes occur without any apparent provocation. Patients describe their disease as though it had a mind of its own.


3. Swelling - pitting or hard (brawny) edema is usually diffuse and localized to the painful and tender region. If the edema is sharply demarcated on the surface of the skin along a line, it is almost proof that the patient has RSD/CRPS.  However, some patients will show a sharply demarcated edema because they tie a band around the extremity for comfort.  Therefore, one has to be certain that the sharply demarcated edema is not due to a previously wrapped bandage around the extremity.
   
   
   
   
4. Movement Disorder - Patients with RSD/CRPS have difficulty moving because they hurt when they move. In addition, there seems to be a direct inhibitory effect of RSD/CRPS on muscle contraction. Patients describe difficulty in initiating movement, as though they have "stiff" joints. This phenomena of stiffness is most noticeable to some patients after a sympathetic nerve block when the stiffness may disappear. Decreased mobilization of extremities can lead to wasting of muscles (disuse atrophy). Some patients have little pain due to RSD/CRPS but instead they have a great deal of stiffness and difficulty initiating movement. 6 Tremors and involuntary severe jerking of extremities may be present. Psychological stress may exacerbate these symptoms. Sudden onset of muscle cramps (spasms) can be severe and completely incapacitating. Some patients describe a slow "drawing up of muscles" in the extremity due to increased muscle tone leaving the hand-fingers or foot-toes in a fixed position (dystonia).


5. Spreading Symptoms - Initially, RSD/CRPS symptoms are generally localized to the site of injury. As time progresses, the pain and symptoms tend to become more diffuse. Typically, the disorder starts in an extremity.   However, the pain may occur in the trunk or side of the face. On the other hand, the disorder may start in the distal extremity and spread to the trunk and face. At this stage of the disorder, an entire quadrant of the body may be involved. Maleki et. al. recently described three patterns of spreading symptoms in RSD/CRPS: 9
1. A "continuity type" of spread where the symptoms spread upward from the initial site, e.g. from the hand to the shoulder.
2. A "mirror-image type" where the spread was to the opposite limb.
3. An "independent type" where symptoms spread to a separate, distant region of the body. This type of spread may be related to a second trauma


6. Bone changes – X-rays may show wasting of bone (patchy osteoporosis) or a bone scan may show increased or decreased uptake of a certain radioactive substance (technecium 99m) in bones after intravenous injection.


7. Duration of RSD/CRPS – The duration of RSD/CRPS varies, in mild cases it may last for weeks followed by remission; in many cases the pain continues for years and in some cases, indefinitely. Some patients experience periods of remission and exacerbation. Periods of remission may last for weeks, months, or years.

The pain you experience may be increasingly severe. It affects people of all ages and has physical, emotional and psychological ramifications. Your pain may have been triggered by trauma or injury, minor surgery, infection, cystitis or endometriosis. The initial injury heals but the painful condition may progress and in time cause severe disability secondary to pain. Symptoms include intolerance to any touch or pressure, swelling, redness, sweating, skin changes, coldness, color changes, osteoporosis. In advanced stages, the pain can be debilitating. Due to the untreated pain syndrome, depression may overwhelm the patient’s life.

Treatment needs to be early, aggressive and provided with a multi-disciplinary approach to avoid lifelong complications!

The Interventional Pain Medical Group can help your doctor treat your sympathetic pain by coordinating medical treatments, providing psychological counseling and diagnostic and therapeutic injections specific to your pain. These may include sympathetic blocks (i.e. stellate, gasserian, celiac, lumbar, hypo-gastric, ganglion impars), epidural injections, regional blocks, intravenous sympathetic blocks and implantable pump and stimulator techniques. Specifics of risk vs. benefit will be discussed in greater detail with your pain specialist.

Injection therapy works to interrupt and reset the abnormally functioning sympathetic nervous system. Early aggressive treatment leads to the greatest success and prevents progression of the disease. In some cases, pain is completely relieved after the first injection. Success varies with the duration and stage of the sympathetic and pelvic pain.

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COMPLEX REGIONAL PAIN SYNDROME RIGHT LOWER LEG There is significant temperature difference between the painful leg and the non-painful leg.

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