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Reflex Sympathetic Dystrophy

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

Background

Reflex sympathetic dystrophy (RSD) is a clinical syndrome of variable course and unknown cause characterized by pain, swelling, and vasomotor dysfunction of an extremity. This condition is often the result of trauma or surgery. Limb immobility may lead to RSD; RSD in a hemiplegic upper limb after stroke is often termed shoulder-hand syndrome. RSD may also develop in the absence of an identifiable precipitating event.

Newer taxonomy categorizes RSD as type 1 complex regional pain syndrome (CRPS), which occurs in the absence of definable nerve injury.[1] Type 2 CRPS, causalgia, develops after nerve injury; the term causalgia was coined by Mitchell in 1864 and derives from the Greek for burning pain. In patients with either type 1 or type 2 CRPS, sympathetic mediation of the pain (ie, improvement with sympathetic blockade) may or may not be evident.

RSD is largely a clinical diagnosis (see Presentation and DDx). Two major approaches to the treatment of early RSD are sympathetic blockade and anti-inflammatory therapy. Surgical sympathectomy may be considered in patients with refractory RSD that had initially responded to sympathetic blockade. Spinal cord stimulation is another surgical option. (See Treatment and Medication.)

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Pathophysiology

The pathogenesis of RSD is unknown. Three conditions are deemed important in the development of RSD: a persistent painful lesion, a predisposition or susceptibility to developing RSD, and an abnormal sympathetic reflex. Susceptibility factors are unknown and may include genetic predisposition (HLA typing)[2, 3, 4] and, in some patients, a tendency toward increased sympathetic activity. This includes cold hands, hyperhidrosis, or a history of fainting.

Healthy individuals undergo a sympathetic response to injury, with vasoconstriction designed to prevent blood loss and swelling. This initial response soon subsides and gives way to vasodilatation and increased capillary permeability, allowing tissue repair.

In patients with RSD, this sympathetic response continues unabated. The reasons for the perpetuation of the response are unknown but may be related to central dysregulation of nociceptive impulses. This dysregulation may be mediated by wide dynamic range neurons in the spinal cord. Prolonged ischemia caused by the vasoconstriction produces more pain, establishing a reflex arc that promotes further sympathetic discharge and vasospasm. This is compounded by the local response to trauma, with liberation of substantial amounts of proinflammatory mediators, such as histamine, serotonin, and bradykinin. The result is a swollen, painful, stiff, nonfunctioning extremity. At least partial sympathetic mediation of this phenomenon is likely because of the ability of sympathetic nerve blockade to relieve pain and other features of RSD in some patients.

Numerous studies have reported altered brain function in RDS. Researchers have also documented structural alterations in the brain. Pleger et al reported that magnetic resonance imaging (MRI) in patients with type 1 complex regional pain syndrome (RDS) showed altered gray matter structure in dorsomedial prefrontal cortex, as well as increases in gray matter density in the motor cortex contralateral to the affected limb, which were inversely related to decreased white matter density of the internal capsule within that brain hemisphere.[5]

A study by Barad that used structural MRI found that compared with controls, patients with complex regional pain syndrome had decreased gray matter volume in several pain-affect regions (including the dorsal insula, left orbitofrontal cortex, and several aspects of the cingulate cortex) and increased gray matter volume in the bilateral dorsal putamen and right hypothalamus.[6]

Lee et al found that the right dorsolateral prefrontal cortex and left ventromedial prefrontal cortex were significantly thinner in patients with CRPS than in healthy controls. In addition, CRPS patients had longer stop-signal task reaction times and made more perseveration errors on the Wisconsin Card Sorting Test.[7]

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Etiology

RSD is usually posttraumatic or postsurgical; however, it can occur in a previously healthy extremity with no known trigger.

Penetrating wounds that can lead to RSD include the following:

  • Lacerations
  • Abrasions
  • Venipuncture
  • Intramuscular injection of medication or illicit drugs
  • Gunshot wounds

 

Other traumatic causes of RSD include the following:

  • Crush injuries and blunt trauma
  • Neck or shoulder injuries
  • Acute traumatic carpal tunnel syndrome
  • Chest trauma
  • Sprain, fracture, or dislocation

Postsurgery RSD has been reported after the following procedures:

  • Carpal tunnel release
  • Dental extractions
  • Cervical rib resection
  • Fracture repair (Colles fracture)
  • Arthroscopy

Local disorders associated with RSD include the following:

  • Nerve compression syndromes
  • Arthritis
  • Tissue ischemia
  • Stenosing tenosynovitis

Systemic disorders associated with RSD include the following:

  • Myocardial infarction
  • Stroke
  • Pancoast tumor
  • Pancreatic cancer
  • Herpes zoster

Race-, Sex-, and Age-related Demographics

No racial predilection exists for RSD. Sexual distribution is equal. Although RSD can occur in children, the age of onset in most patients RSD is 30-60 years, and the mean age is 49 years.[8]

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Epidemiology

Frequency

United States

An estimated 5% of patients who experience trauma to the upper extremity develop RSD, although this figure is not known with certainty because of confusion over the diagnosis. Extremity immobilization can trigger RSD. Without prophylactic measures (active physical therapy), RSD can develop in 12-20% of people who experience a hemiplegic stroke.

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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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  2. Kemler MA, van de Vusse AC, van den Berg-Loonen EM, et al. HLA-DQ1 associated with reflex sympathetic dystrophy. Neurology. 1999 Oct 12. 53(6):1350-1. [Medline].

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