Formerly Known as Reflex Sympathetic Dystrophy (RSD)

What is Complex Regional Pain Syndrome (CRPS)?

Diagnosis of CRPS

There is no single test to confirm a diagnosis of CRPS. The diagnosis is primarily through observation of signs and symptoms.  Patients must be examined by a qualified physician who does a thorough history and physical examination.  X-rays, MRI, EMG/NCV, bone scans, thermography, or pain imaging where available may be helpful.  Consultation with other specialists may be needed, and a pain clinic is often recommended.

• Occupational/Physical Therapy. An increasing exercise program to help with motion may help preserve or restore mobility and function to the affected hand.  Overall aerobic conditioning is very important to improve coping ability, sleep, and pain control.
  Psychotherapy. CRPS can have profound psychological effects on patients and their families. Many with CRPS have depression, anxiety, or post-traumatic stress disorder. A psychologist or psychiatrist may be able to improve coping ability and motivation as well as detect and address any substance dependency issues.
  
  
• Nerve Blocks. Many patients experience significant relief from nerve blocks, in which local anesthetic is injected to numb nerves. By relieving pain, blocks can enable more effective therapy, improve mood, and improve level of activity.   Stellate ganglion blocks may be used to numb the stellate ganglion, which is a cluster of sympathetic nerves at the base of the neck, in an effort to reduce the over-activity of the sympathetic nerves seen in CRPS (see Figure 1).
  
  
• Medications. Many different drugs are used to treat CRPS and associated conditions, such as sleep disorders, depression, and anxiety.  Medications may include topical analgesics, anti-seizure drugs, antidepressants, corticosteroids, muscle relaxants, opioids, and sleeping medications.
  
  
• Surgery. If the CRPS is from a compressed nerve, such as with carpal tunnel syndrome, then surgery to release pressure on the nerve may be needed (e.g., carpal tunnel release). Rarely, an operation known as sympathectomy is used to divide the sympathetic nerves in patients who are helped by nerve blocks, and its use is controversial.
  

Other options include spinal cord stimulation and intrathecal drug pumps, in which pain medications are injected continuously into the space around the spinal cord.  Deep brain stimulation and Electrotherapy (ECT) have also been used, but new therapies continue to emerge.

Prognosis

Figure 1:  Stellate ganglion block

References

1.  de Mos M, de Bruijn AG, Huygen FJ, Dieleman JP, Stricker BH, Sturkenboom MC.  The      incidence of complex regional pain syndrome: a population-based study.  Pain. 2007;129(1-2):12-20.
 
2. Sandroni P, Benrud-Larson LM, McClelland RL, Low PA. Complex regional pain syndrome type I: incidence and prevalence in Olmsted county: a population-based study. Pain. 2003;103:199–207.

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The large joints in the hand at the base of each finger are known as the metacarpophalangeal (MP, or MCP) joints (see Figure 1). They act as complex hinge joints and are important for both power grip and pinch activities.

Arthritis is the wearing away of the cartilage at a joint. Cartilage is the coating layer of tissue on the end of a bone that acts as a shock-absorber.  Loss of cartilage can lead to joint destruction and a shift in the finger position towards the small finger side, which is called ulnar drift (See Figure 2).  When arthritis affects the MP joints, the condition is called MP joint arthritis.

Causes

Treatment

There are many treatments available depending on the amount of pain and loss of function. Medication (prescribed by an arthritis doctor or rheumatologist) can be very helpful in relieving pain and preventing worsening joint destruction. Sometimes joint injections of a steroid medication can also help.

Figure 1: Finger Joints

Figure 2:  Ulnar drift (fingers point towards little finger side)

Figure 3:  X-ray of MP joint arthritis. The joint on the right has no space between the bones when compared to the joint on the left because of cartilage loss

Figure 4:  X-rays of silastic finger joint replacements

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Ganglion cysts are very common lumps within the hand and wrist that occur adjacent to joints or tendons.  The most common locations are the top of the wrist (see Figure 1), the palm side of the wrist, the base of the finger on the palm side, and the top of the end joint of the finger (see Figure 2). The ganglion cyst often resembles a water balloon on a stalk (see Figure 3), and is filled with clear fluid or gel.

Causes

The cause of these cysts is unknown although they may form in the presence of joint or tendon irritation or mechanical changes. They occur in patients of all ages.

These cysts may change in size or even disappear completely, and they may or may not be painful. These cysts are not cancerous and will not spread to other areas.

Diagnosis  
The diagnosis is usually based on the location of the lump and its appearance. They are usually oval or round and may be soft or very firm. Cysts at the base of the finger on the palm side are typically very firm, pea sized nodules that are tender to applied pressure, such as when gripping. Light will often pass through these lumps, (trans-illumination) and this can assist in the diagnosis. Your physician may request x rays in order to look for evidence of problems in adjacent joints.  Cysts at the far joint of the finger frequently have an arthritic bone spur associated with them, the overlaying skin may become thin, and there may be a lengthwise groove in the fingernail just beyond the cyst.

Treatment 
Treatment can often be non-surgical. In many cases, these cysts can simply be observed, especially if they are painless, as they frequently disappear spontaneously. If the cyst becomes painful, limits activity, or is otherwise unacceptable, several treatment options are available. The use of splints and anti-inflammatory medication can be prescribed in order to decrease pain associated with activities. An aspiration can be performed to remove the fluid from the cyst and decompress it. This requires placing a needle into the cyst, which can be performed in most office settings. Aspiration is a very simple procedure, but recurrence of the cyst is common. If non-surgical options fail to provide relief or if the cyst recurs, surgical alternatives are available. Surgery involves removing the cyst along with a portion of the joint capsule or tendon sheath (see Figure 3). In the case of wrist ganglion cysts, both traditional open and arthroscopic techniques usually yield good results. Surgical treatment is generally successful although cysts may recur. Your surgeon will discuss the best treatment options for you.

Figure 1:  Ganglion top side (dorsum) wrist

Figure 2:  Ganglion end joint of finger (mucous cyst)

Figure 3:  Cross-section of wrist showing stalk (or root) of ganglion.

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Patients with de Quervain syndrome have painful tendons on the thumb side of the wrist.  Tendons are the ropes that the muscle uses to pull the bone.  You can see them on the back of your hand when you straighten your fingers.  In de Quervain syndrome, the tunnel (the first extensor compartment; see Figure 1A-B) where the tendons run narrows due to the thickening of the soft tissues that make up the tunnel. Hand and thumb motion cause pain, especially with forceful grasping or twisting.

Causes

Doctors are not sure  what causes de Quervain syndrome.  Patients often describe a feeling of inflammation, but studies have shown that the process is not inflammatory.  People of all ages get it.  When new mothers develop de Quervain syndrome, it typically appears 4 to 6 weeks after delivery.  The old theory that it was caused by wringing out cloth diapers has been replaced by concerns about holding the baby, but changes in hormones and swelling seem more probable.

Treatment

• A splint that stops you from moving your thumb and wrist.
• Tylenol or aspirin type medications (e.g., ibuprofen).

Treatments that attempt to change the course of the disease include:

• A cortisone-type of steroid injection into the tendon compartment.  It has not been clearly established that these injections change the course of the disease and response to the injection varies.
• Surgery to open the tunnel and make more room for the tendons.

Figure 1A:  The first dorsal compartment. There are six compartments on the dorsal, or back, side of the wrist. The first and third compartments house tendons that control the thumb.

Figure 1B:  A drawing of the first dorsal compartment.

Figure 2A and B:  Pain with this motion (a hammering motion with the thumb in the fist) is characteristic of de Quervain syndrome.

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Dupuytren’s disease is an abnormal thickening of the tissue just beneath the skin known as fascia. This thickening occurs in the palm and can extend into the fingers (see Figure 1). Firm pits, nodules and cords may develop that can cause the fingers to bend into the palm (see Figure 2), in which case it is described as Dupuytren’s contracture. Although the skin may become involved in the process, the deeper structures—such as the tendons—are not directly involved. Occasionally, the disease will cause thickening on top of the finger knuckles (knuckle pads), or nodules or cords within the soles of the feet (plantar fibromatosis).

Causes 
The cause of Dupuytren’s disease is unknown but may be associated with certain biochemical factors within the involved fascia. The problem is more common in men over age 40 and in people of northern European descent. There is no proven evidence that hand injuries or specific occupational exposures lead to a higher risk of developing Dupuytren’s disease.

Signs and Symptoms
Symptoms of Dupuytren’s disease usually include lumps and pits within the palm. The lumps are generally firm and adherent to the skin. Thick cords may develop, extending from the palm into one or more fingers, with the ring and little fingers most commonly affected. These cords may be mistaken for tendons, but they actually lie between the skin and the tendons. These cords cause bending or contractures of the fingers. In many cases, both hands are affected, although the degree of involvement may vary.

The initial nodules may produce discomfort that usually resolves, but Dupuytren’s disease is not typically painful. The disease may first be noticed because of difficulty placing the hand flat on an even surface, such as a tabletop (see Figure 3). As the fingers are drawn into the palm, one may notice increasing difficulty with activities such as washing, wearing gloves, shaking hands, and putting hands into pockets. Progression is unpredictable. Some individuals will have only small lumps or cords while others will develop severely bent fingers. More severe disease often occurs with an earlier age of onset.

Treatment

In mild cases, especially if hand function is not affected, only observation is needed.  For more severe cases, various treatment options are available in order to straighten the finger(s).  These options may include collagenase injection, needle aponeurotomy or open surgery.  Collagenase injection is a technique where a small amount of medicine is injected into the Dupuytren's tissue, weakening it so that the finger can be manipulated manually to make it straighter.  Needle aponeurotomy is a method where a needle is placed through the skin and used to cut the Dupuytren's tissue.  Both collagenase injection and needle aponeurotomy are office procedures.  Your hand surgeon can describe these options in more detail, including potential risks and benefits, to help you decide what treatment method is best for you.  Specific surgical considerations include the following:

• The presence of a lump in the palm does not mean that surgery is required or that the disease will progress.
• Correction of finger position is best accomplished with milder contractures or contractures that affect the base of the finger. Complete correction sometimes can not be attained, especially of the middle and end joints in the finger.
• Skin grafts are sometimes required to cover open areas in the fingers if the skin is deficient.
• The nerves that provide feeling to the fingertips are often intertwined with the cords.
• Splinting and hand therapy are often required after surgery in order to maximize and maintain the improvement in finger position and function.

Figure 1, 2: Advanced case of Dupuytren's with pits, nodules and cords leading to bending of the finger into the palm

Figure 3: Table top test

Figure 4: Treatment Diagram

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