Reflex Sympathetic Dystrophy Treatment & Management

The natural history of reflex sympathetic dystrophy (RSD) is variable and unpredictable, the pathogenesis is unknown, and few controlled treatment trials exist. Thus, evidence-based treatment guidelines do not exist, and the approach depends largely on the specialty of the treating physician. Even if a disturbance in sympathetic nervous system function is important in the development of the clinical syndrome, not all patients respond to sympatholytic medications or to chemical or surgical sympathectomy.

Clinical experience teaches that early recognition and treatment are necessary to avoid permanent disability and that the effectiveness of treatment is limited once the patient has reached the chronic fibrotic stage. Certainly, the incidence and severity of RSD can be greatly reduced by initiating prophylactic measures in situations that are known to be triggers (eg, hemiplegic stroke, Colles fracture). These measures include immediate and aggressive mobilization of the involved extremity with passive and then active range-of-motion exercises. Similarly, in patients with established RSD, physical and occupational therapy are key components of any therapeutic regimen.

Two major approaches to the medical treatment of early RSD exist: sympathetic blockade and anti-inflammatory therapy. Although these are not mutually exclusive, the order of usage is generally specialty-dependent, with anesthesiologists/surgeons starting with the former and internists/rheumatologists starting with the latter.

• Sympathetic block (upper extremity, stellate; lower extremity, lumbar)

  • Ensure that this is performed by a trained individual.
  • Inject a local anesthetic into the stellate and upper dorsal sympathetic ganglia to block the efferent sympathetic impulses from the involved extremity. Lidocaine or bupivacaine, with or without epinephrine, is usually used.
  • This procedure warms the skin, inhibits sweating, and causes flushing.
  • A successful blockade is indicated by the development of ipsilateral Horner syndrome, ie, ptosis, miosis, and enophthalmos.
  • Symptoms usually abate within 30 minutes, confirming the diagnosis. Once the patient is adequately blocked, ensure their participation in hand therapy. Although the interruption lasts only a few hours, the benefits may persist for several days.
  • Use 1-2 blocks per week. An average of 4-5 blocks is required to permanently relieve symptoms. For symptoms that are not adequately relieved after 4-5 blocks, institute a continuous stellate blockade via a subcutaneously placed catheter or conduct an operative sympathectomy.
• Sympatholytic drugs

  • Sympatholytic drugs may be efficacious when used alone in early disease.
  • Sympatholytic drugs may be beneficial in combination with sympathetic block or sympathectomy in later stages of the disorder.
  • Regional intravenous sympathetic blockade with sympatholytic drugs, such as phenoxybenzamine, using a Bier block–like procedure may be helpful, but results have varied. This is most useful in early disease.
  • A randomized study performed in 2000 suggested that intrathecal baclofen, a GABA-receptor agonist, relieved the dystonia and, in some cases, the hand pain in patients with RSD.^[8] This suggests that GABA-ergic inhibitory pathways may also be important in the pathogenesis of RSD.
• Anti-inflammatory medications (corticosteroids, calcitonin)

  • Although nonsteroidal anti-inflammatory drugs (NSAIDs) may provide some symptomatic pain relief in patients with RSD, they are not effective in altering the skin changes or natural history of the process and thus play only a supportive role. On the other hand, a course of high-dose corticosteroids, eg, prednisone 30-40 mg/d tapering over 2-4 weeks, can dramatically reduce pain, swelling, and stiffness. This enables the institution of an aggressive physical-therapy program. In general, corticosteroids are of most value in early RSD (acute and subacute) when the bone scan shows increased uptake in the involved extremity.
  • Calcitonin is not an anti-inflammatory medication per se but has been reported to reverse the inflammatory changes and reduce pain in early RSD, especially in patients with hyperdynamic blood flow. Subcutaneous injections of 100-160 units are administered daily for 4-8 weeks, then every other day for 3-6 weeks. A few reports suggest that intranasal calcitonin^[9] and oral bisphosphonates may also be effective in treating RSD.

• Upper thoracic or lumbar sympathectomy

  • Consider this procedure if the relief achieved with sympathetic blockade and anti-inflammatory therapy has not permanently resolved the RSD and relapse has occurred despite continuing treatment. These procedures are reserved for patients who have had an initial response to sympathetic blockade and are thus likely have a sympathetically mediated process.
  • Indications include disease duration of longer than 6 months and failure of permanent resolution after 5 percutaneous sympathetic blocks are performed.
  • The most significant improvement following surgical sympathectomy is pain relief, although circulation, range of motion, strength, and function are usually somewhat improved.
  • Choose sympathectomy early in the course of disease because, once joint fibrosis develops, minimal functional improvement occurs. Pain relief, however, is still significant in late disease.
  • If surgery of the involved extremity is required, perform it after the sympathectomy.
  • Ensure that a surgical sympathectomy is performed by an adequately trained individual.
• Chemical sympathectomy

  • Inject phenol or alcohol to ablate the sympathetic chain.
  • Perform this only if the patient is at a very high surgical risk for hoarseness from a recurrent laryngeal nerve injury, lung injury, or permanent Horner syndrome.
• Spinal cord stimulation: In 2000, a controlled trial demonstrated the efficacy of epidural implantation of a spinal cord stimulator in pain relief and functional improvement in RSD (hand or foot) compared with physical therapy alone.^{[10, 11]}

• Multiple consultants are often needed in challenging RSD cases, including an anesthesiologist who specializes in pain management and who is capable of performing sympathetic blocks, an internist/rheumatologist capable of supervising anti-inflammatory regimens, and a surgeon who is capable of performing surgical sympathectomy.
• Consultation with a physical therapist and occupational therapist (hand therapist) is important to institute aggressive exercise programs.

• Physical and occupational therapy

  • As discussed above, an aggressive range-of-motion exercise program is an essential part of RSD management. However, especially after sympathetic block or sympathectomy, this may have to be a graduated regimen, with patient-directed passive range of motion to tolerance and, later, active range of motion. When appropriate (eg, in patients with hemiplegia), the entire extremity requires attention.
  • Following the stellate block or sympathectomy, hand therapy may proceed without causing further pain. Ensure that the therapist does not cause pain, usually by avoiding application of passive motion. Patients can safely apply passive motion because they know when motion becomes painful.
  • Massage of the involved extremity is an important component of therapy.
  • The patient requires close follow-up, education, and encouragement to maintain an exercise routine.
• Heat therapy relaxes muscle spasms, improves motion, and relieves pain.
• Judicious use of splinting with Thermoplast and Velcro straps in the balanced hand position may help to prevent shortening of the collateral ligaments of involved joints and may provide intermittent pain relief. The splint must be comfortably fit. Remove it frequently throughout the day for exercise and massage.
• Record routine measurements of the patient's strength and range of motion. This provides encouragement to the patient and facilitates communication between the therapist and the treating physician.