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Reflex Sympathetic Dystrophy

RDS

Reflex sympathetic dystrophy (RSD) is a form of a complex regional pain syndrome that affects the extremities. It is characterized primarily by pain, swelling and vasomotor changes.


Presentation

Reflex sympathetic dystrophy may occur in either the upper or lower limbs. The clinical course moves in stages:

  • Stage 1: The patients develops pain with an identifiable cause (it may not be found or apparent). There is throbbing pain, abnormal sensations like burning and allodynia with localized edema. The distribution of the pain is not compatible with a nerve region. There will be vasomotor disturbances with changes in temperature and skin colour. There may be radiological changes but they are minimal at this stage.
  • Stage 2: In this stage there is progression of soft tissue edema and skin thickening with associated muscle wasting. This occurs from 3 to 6 months after onset.
  • Stage 3: The third stage is characterized by limitation of movement, with severe contractures and trophic skin. The radiograph shows significant demineralization [8].
Lower Extremity Pain
  • This disorder should be considered in any painful pelvic girdle syndrome or lower extremity pain. The hip is involved in 88% of cases. Symptoms develop in the third trimester of pregnancy, between the 26th and the 34th weeks.[ncbi.nlm.nih.gov]
Regurgitation
  • He was a known case of mitral valve prolapse (MVP) with mitral regurgitation and complete heart block for which pacemaker was implanted 1 year back. Bilateral wrist X-ray was suggestive of pronounced demineralization (osteopenia) in the right hand.[ncbi.nlm.nih.gov]
Leukonychia
  • Unilateral leukonychia, Beau's lines, nailfold swelling, and nail clubbing have been an observed sequela of RSD. We present a case of a unilateral atypical trachyonychia occurring in the setting of RSD after traumatic fracture of a digit.[ncbi.nlm.nih.gov]
Oligoarthritis
  • Spondyloarthropathy was diagnosed based on oligoarthritis with sacroiliitis, presence of HLA B27, and a favorable response to non-steroidal antiinflammatory therapy.[ncbi.nlm.nih.gov]
Neuralgia
  • We propose that persistent RSD/CRPS-I is a post-traumatic neuralgia associated with distal degeneration of small-diameter peripheral axons.[ncbi.nlm.nih.gov]
  • .: Noradrenaline-evoked pain in neuralgia. Pain. 1995; 63:11-20. [10] Maihofner CM, Handwerker HOM, Neundorfer BM, et al.: Patterns of cortical reorganization in complex regional pain syndrome.[ainsworthinstitute.com]
  • In addition to causalgia, RSD is also known as Sudeck's atrophy, post-traumatic neuralgia, and shoulder-hand syndrome. All of these names are used interchangeably, adding to the confusion in diagnosing and treating RSD.[amputee-coalition.org]
  • In addition to causalgia, RSD is also known as Sudeck’s atrophy, post-traumatic neuralgia, and shoulder-hand syndrome. All of these names are used interchangeably, adding to the confusion in diagnosing and treating RSD.[amputee-coalition.org]
  • […] and other Cranial Neuralgias For discussion of Occipital Neuralgia, Glossopharyngeal Neuralgia, Nervus Intermedius (or Geniculate Neuralgia), and Vegal and Superior Laryngeal Neuralgia.[neurotalk.psychcentral.com]
Peripheral Neuropathy
  • Kim SH, Chung JM: An experimental model for peripheral neuropathy produced by segmental spine nerve ligation in the rat . Pain 1992, 50 :335–363. Google Scholar 27.[link.springer.com]
  • neuropathy, but particularly various musculoskeletal disorders, may be misdiagnosed as RSD (see Schott 4 ).[jnnp.bmj.com]
  • […] conditions: Bony or soft tissue injury Peripheral neuropathy, nerve lesions Arthritis Infection Compartment syndrome Arterial insufficiency Raynaud’s Disease Lymphatic or venous obstruction Thoracic Outlet Syndrome (TOS) Gardner-Diamond Syndrome Erythromelalgia[physio-pedia.com]
Hyperesthesia
  • Reflex sympathetic dystrophy is characterized by constant burning pain and hyperesthesia in an extremity. Lower extremities are usually affected. Pain is accompanied by swelling, sweating, vasomotor instability and sometimes trophic changes.[ncbi.nlm.nih.gov]
  • After arthrocentesis, dusky discoloration, edema, hyperesthesia, and decreased range of motion of the left knee and entire distal extremity were noted. Despite analgesia and physical therapy her symptoms worsened.[ncbi.nlm.nih.gov]
  • Case Reports First Online: 01 April 1992 Abstract We describe a 9 1/2 year old girl who suffered from severe recurrent pain and functional limitation in her right leg with hyperesthesia, hyperalgesia, color change and edema as the presenting symptoms,[link.springer.com]
  • Orthopedic Rehabilitation services Pain profilePAIN QUALIFIERS JOINT PAIN Aching 0 no pain Burning 1 mild pain with deep palpation Cramping Heaviness/fatigue 2 severe pain with deep palpation Numbness 3 severe pain with mild palpation Sharp/stabbing 4 hyperesthesia[slideshare.net]
  • Reflex sympathetic dystrophy is characterized by: Severe, chronic pain often described as stinging or burning Sensory abnormalities such as allodynia (pain due to a stimulus which does not normally provoke pain) or hyperesthesia (increased sensitivity[medifocus.com]
Complex Regional Pain Syndrome Type II
  • Review Topic QID: 4811 2 Complex Regional Pain Syndrome, type II 4 Complex Regional Pain Syndrome, type I ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (OBQ04.43) A 34-year-old laborer has her left foot crushed in a piece of farming[orthobullets.com]
  • Glossopharyngeal Neuralgia by Ssab 06-15-2018 02:50 PM 167 1,066 Reflex Sympathetic Dystrophy (RSD and CRPS) (15 Viewing) Reflex Sympathetic Dystrophy (Complex Regional Pain Syndromes Type I) and Causalgia (Complex Regional Pain Syndromes Type II)(RSD[neurotalk.psychcentral.com]
  • Transcutaneous Electrical Nerve Stimulation for the Management of Neuropathic Pain: The Effects of Frequency and Electrode Position on Prevention of Allodynia in a Rat Model of Complex Regional Pain Syndrome Type II. Phys Ther 2006;86:698-709.[physio-pedia.com]

Workup

There is no useful laboratory testing in this syndrome. Majority of the diagnosis will come from the history and physical exam. Plain radiographs will show pronounced demineralization of the skeleton affected by the pain and edema. This worsens as the disease progresses.

Bone radionuclide test may be useful in early disease, but findings are not specific for this condition. Magnetic resonance imaging (MRI) may be useful in identifying patients, as it gives useful information on the soft tissue and skin changes. CT scans may show multiple focal areas of osteoporosis.

Autonomic testing like resting skin temperature and resting sweat output may be done to document the vasomotor symptoms. An increased resting skin temperature is highly suggestive of this disease [9].

Treatment

There are two major therapies being used currently. There is insufficient data on other effective therapies and little randomized control studies.

  • Sympathetic blocks: A local anesthetic such as lidocaine or bupivacaine is injected into the stellate and upper dorsal sympathetic ganglia to block the efferent nerves. There should be a Horner's syndrome induced after this, which will disappear soon after (30 mins). The patient then undergoes intensive physiotherapy and occupational therapy. The patient may require 1 to 2 blocks per week until the condition is treated. For lower limbs a lumbar block is used [11].
  • Sympatholytic drugs in early disease may be effective. Regional infusions are usually effective [12] [13].

Other treatments such as non steroidal anti-inflammatory drugs (NSAIDs) may be used. Corticosteroids are highly effective in reducing pain and allowing for physical therapy. Surgical methods may be used and include thoracic or lumber sympathectomy. This is indicated in cases resistant to therapy. Surgery should be considered before there is significant joint deformity. Chemical sympathectomy with alcohol has also been done.

Prognosis

The course of the disease is variable, but if caught early and treated appropriately, many patients do not progress on to the later stages of the disease. There is little data on mortality, but morbidity may be significant.

Etiology

Reflex sympathetic dystrophy usually occurs after injury, which can be traumatic or postsurgical. In some cases there may be no identifiable incident. In some patients an event such as myocardial ischemia or a hemiplegic stroke, may be the inciting event. Common surgical procedures such as arthroscopy of the knee and other joints may be the cause in some patients. There also have been cases in diseases such as pancreatic and pancoast tumors [2] [3].

Epidemiology

35% of patients have no identifiable inciting event. There is an estimated 5% prevalence in people with a previous upper extremity injury. The syndrome was prevalent (12%) in patients with hemiplegic stroke, but this has significantly decreased with the advent of early mobilization. There is no gender or race predilection, but it has a peak incidence between 30 to 60 years [4].

Sex distribution
Age distribution

Pathophysiology

There appears to be a need of three conditions to be present for reflex sympathetic dystrophy to occur:

  • Abnormal sympathetic reflex
  • Persistent painful lesion 
  • Predisposition 

The true mechanism of this complex regional pain syndrome is unclear, but appears to involve the formation of a new reflex arc after the inciting event. The arc follows a sympathetic pathway and is then modulated by cortical centres and produces vascular changes. The pain occurs possibly due to the triggering event causing nerve damage with increased sensitivity to sympathetic neurotransmitters such as epinephrine.

There is thought to be prolonged and constant release of pain inciting peptides from peripheral nerves causing nerve inflammation with subsequent pain and allodynia. Neuropeptide Y and substance P have been implicated in this. There appears to be a genetic predisposition. A few human leukocyte antigen (HLA) associations have been found, a few examples include HAL-A3, and DR2 [5] [6].

Prevention

Quick physiotherapy and mobilization has been noted to reduce the incidence, especially in patient with hemiplegic strokes. Early mobilization is advised when possible after any intervention or injury of limb, to reduce the occurrence of this disease.

Summary

There are two types of a complex regional pain syndrome (CRPS):

  • Type 1, when the complex regional pain syndrome is not caused by an identifiable nerve lesion. Names include reflex sympathetic dystrophy, Sudeck's atrophy and reflex neurovascular dystrophy.
  • Type 2, when there is an identifiable lesion. It was previously known as causalgia [1]. 

Both types are chronic systemic diseases characterized by severe pain, swelling, and vasomotor dysfunction.

Patient Information

  • Definition: Reflex sympathetic dystrophy is a disease whereby there is a problem with a certain area of your body, usually a hand or leg. There is pain and abnormal sensation in a certain area with abnormal sweating and swelling
  • Cause: The cause is unknown, but it is thought that inciting events such as trauma and surgery may be the cause. In many people no cause is found. The triggering event causes there to be abnormal sensation in the affected area. 
  • Symptoms: The most common symptom is pain which can be burning or tingling. Some complain of throbbing pain which can also be severe. The affected area is sensitive to touch or cold, there is swelling and the skin become thick and rough. The muscles in the area become weak and smaller.
  • Diagnosis: Most of the diagnosis is made from asking you questions and examining you. They skin temperature and the amount of sweat in the area will be recorded. 
  • Treatment: The treatment involves blocking the nerves and physical therapy. You may also be given drugs to help with the pain and swelling.
  • Prevention: Quick physical therapy after an injury or stroke is advised to reduce the occurrence of the disease.

References

Article

  1. Stanton-Hicks M, Jänig W, Hassenbusch S, et al. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain 1995; 63:127.
  2. Pak TJ, Martin GM, Magness JL, Kavanaugh GJ. Reflex sympathetic dystrophy. Review of 140 cases. Minn Med 1970; 53:507.
  3. Sebastin SJ. Complex regional pain syndrome. Indian J Plast Surg. May 2011;44(2):298-307
  4. Goebel A. Complex regional pain syndrome in adults. Rheumatology (Oxford). Oct 2011;50(10):1739-50
  5. van Laere M, Claessens M. The treatment of reflex sympathetic dystrophy syndrome: current concepts. Acta Orthop Belg 1992; 58 Suppl 1:259.
  6. Kozin F. Reflex sympathetic dystrophy syndrome. Bull Rheum Dis 1986; 36:1.
  7. O'Brien SJ, Ngeow J, Gibney MA, et al. Reflex sympathetic dystrophy of the knee. Causes, diagnosis, and treatment. Am J Sports Med 1995; 23:655.
  8. Bonica JJ. Causalgia and other reflex sympathetic dystrophies. Postgrad Med 1973; 53:143.
  9. Todorović-Tirnanić M, Obradović V, Han R, et al. Diagnostic approach to reflex sympathetic dystrophy after fracture: radiography or bone scintigraphy? Eur J Nucl Med 1995; 22:1187.
  10. Cappello ZJ, Kasdan ML, Louis DS. Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. J Hand Surg Am 2012; 37:288.
  11. Cepeda MS, Carr DB, Lau J. Local anesthetic sympathetic blockade for complex regional pain syndrome. Cochrane Database Syst Rev 2005
  12. Perez RS, Kwakkel G, Zuurmond WW, de Lange JJ. Treatment of reflex sympathetic dystrophy (CRPS type 1): a research synthesis of 21 randomized clinical trials. J Pain Symptom Manage 2001; 21:511.
  13. Olcott C 4th, Eltherington LG, Wilcosky BR, et al. Reflex sympathetic dystrophy--the surgeon's role in management. J Vasc Surg 1991; 14:488.

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Last updated: 2018-06-21 19:55