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Reflex Sympathetic Dystrophy Treatment & Management

  • Author: Don R Revis, Jr, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Oct 16, 2015
 

Medical Care

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

For RSD affecting an upper extremity, 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. Guanethidine has been used, but was found to be no more effective than placebo, and was associated with significant adverse effects.[10]

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 adequate blockade has been achieved, ensure that the patient participates 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.

For lower-extremity RSD, a lumbar block is used.

A systematic review found insufficient evidence to draw firm conclusions regarding the efficacy or safety of sympathetic blockade with local anesthetic, but determined that the limited data available do not suggest the technique is effective for reducing pain in RDS.{ref1

Sympatholytic drugs

Sympatholytic drugs alone may be effective in early disease. In later stages of the disorder, sympatholytic drugs may be beneficial in combination with sympathetic block or sympathectomy.

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 suggested that intrathecal baclofen, a GABA-receptor agonist, relieved the dystonia and, in some cases, the hand pain in patients with RSD.[11] This suggests that GABA-ergic inhibitory pathways may also be important in the pathogenesis of RSD.

Other medications

Nonsteroidal anti-inflammatory drugs (NSAIDs) may provide some pain relief in patients with RSD. However, they are not effective in altering the skin changes or natural history of the process and thus play only a supportive role.

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[12] may also be effective in treating RSD.

Oral and intravenous bisphosphonates (eg, alendronate) have demonstrated benefit in early RSD.[13] In a randomized, double-blind, placebo-controlled trial, the aminobisphosphonate neridronate, administered intravenously, provided significant and persistent benefit in patients with RSD.[14] Neridronate has received orphan drug designation by the US Food and Drug Administration for treatment of RSD and other types of complex regional pain syndrome.

The anesthetic agent ketamine has shown promise in the treatment of RSD. However, the optimum dose and the route and timing of administration remain to be determined.{ref17

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Surgical Care

Surgical procedures used for RSD include the following:

  • Upper thoracic or lumbar sympathectomy
  • Chemical sympathectomy
  • Spinal cord stimulator implantation

Upper thoracic or lumbar sympathectomy 

Consider surgical sympathectomy 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. Choose sympathectomy early in the course of disease because once joint fibrosis develops, minimal functional improvement occurs. Pain relief, however, remains significant in late disease.

Considerations include the following:

  • Indications include disease duration of longer than 6 months and failure of permanent resolution despite five percutaneous sympathetic blocks.
  • The most significant improvement following surgical sympathectomy is pain relief, although circulation, range of motion, strength, and function usually improve somewhat
  • 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

For chemical sympathectomy, phenol or alcohol in injected 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

Epidural implantation of a spinal cord stimulator has been shown to provide significant, prolonged pain relief and functional improvement in RSD (hand or foot).[15, 16] In the United Kingdom, the National Institute for Health and Care Excellence (NICE) recommends spinal cord stimulation as a possible treatment for adults with chronic pain of neuropathic origin, including RSD, who have had      chronic pain for at least 6 months despite standard treatments and have had a successful trial of spinal cord stimulation as part of an assessment by a specialist team.[17]

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Consultations

Multiple consultants are often needed in challenging RSD cases, including the following:

  • An anesthesiologist who specializes in pain management and who is capable of performing sympathetic blocks
  • An internist/rheumatologist capable of supervising anti-inflammatory regimens
  • 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.

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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.

Other features of physical therapy include the following:

  • 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, and needs to be removed 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
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Contributor Information and Disclosures
Author

Don R Revis, Jr, MD Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine

Don R Revis, Jr, MD is a member of the following medical societies: American College of Surgeons, American Society for Aesthetic Plastic Surgery, American Society of Plastic Surgeons, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Robert E Wolf, MD, PhD Professor Emeritus, Department of Medicine, Louisiana State University School of Medicine in Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Affairs Medical Center

Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, Society for Leukocyte Biology

Disclosure: Nothing to disclose.

References
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