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Dx Reflux Sympathetic Dystrophy Treatment:

Reflux Sympathetic Dystrophy

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. In 1864, Mitchell referred to this malady as causalgia, a Greek word meaning burning pain. Newer taxonomy refers to RSD as a type of complex regional pain syndrome (CRPS), which may develop after an initiating event such as trauma or surgery or may occur spontaneously. Under this classification, causalgia is a type of CRPS that develops after nerve injury. In patients with either of these conditions, sympathetic mediation of the pain (i.e., improvement with sympathetic blockade) may or may not be evident.

The 3 clinical stages of reflex sympathetic dystrophy (RSD) are acute, subacute, and chronic. The acute form lasts approximately 3 months. Pain, often burning in nature, is one of the first symptoms that initially limits function. Swelling, redness with vasomotor instability that worsens with dependency, hyperhidrosis, and coolness to the touch are common physical findings. Demineralization of the underlying bony skeleton begins because of disuse.

If the process is not arrested or reversed in the acute phase, the condition may progress to the subacute stage, which can last for up to 9 months. The patient develops persistent severe pain in the extremity and fixed edema that would have been reversible with elevation during the acute phase. The redness of the acute stage gives way to cyanosis or pallor and hyperhidrosis to dry skin. Loss of function progresses, both because of increased pain and fibrosis of the joints caused by chronic inflammation. In the hand, this leads to flexion deformity of the fingers. The skin and subcutaneous tissues begin to atrophy. Demineralization of the underlying bony skeleton becomes pronounced.

If the process continues, the chronic phase may develop approximately 1 year after disease onset. This stage may last for many years or can be permanent. Pain is more variable during this period. It may continue undiminished or abate. Edema tends to subside over time, leaving fibrosis around the involved joints. The skin is dry, pale, cool, and shiny. Flexion and extension creases are absent. Loss of function and stiffness are marked, and osteoporosis is extreme. In the upper extremity, this can manifest as a frozen shoulder and claw hand.

A thorough general history is strongly suggested. Maintaining a high index of suspicion is important because proper treatment requires rapid diagnosis and prompt therapy.

RSD commonly involves only one extremity. It is bilateral in approximately 25% of cases but is usually more prominent on one side.

*Usually constant and disproportionate to the precipitant injury
*May be exacerbated by ambient factors such as loud noises and emotional factors (eg, stress, light touch, active motion, passive motion)
*May be described as burning, cutting, searing, pressure, or tearing
*Usually begins locally but may progress to involve the entire extremity
*Possible evidence of prior increased sympathetic activity
*Cold hands
*Prior trauma, which may be trivial or significant (eg, Colles fracture), with or without diagnosable nerve injury
*Prior surgery
*Recent limb immobilization due to hemiplegic stroke, myocardial infarction
*Systemic disease such as diabetes

The pathogenesis of RSD is unknown....In patients with RSD, this sympathetic response continues unabated... Prolonged ischemia caused by the vasoconstriction produces more pain, establishing a reflex arc that promotes further sympathetic discharge and vasospasm... 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.

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.
*Sexual distribution is equal.
*The age of onset in most patients with RSD is 30-60 years, and the mean age is 49 years.
*RSD affects children and carries a much better prognosis than in adults.

Physical Perform a thorough physical examination followed by a focused examination of the involved extremity. Patients with RSD may present with suggestive physical findings that point to a presumptive diagnosis.
*Edema is always disproportionate to the severity of the precipitant injury or event.
*Pain, swelling, and color change may be more prominent with dependency in the early stages.
*Edema worsens rather than improves and extends beyond the region of initial concern. It evolves into a brawny, nonpitting edema that may progress to an intense fibrosis in all the joints of the extremity.
*Stiffness is more severe than expected and may be very distressing to the patient.
*Discoloration: May be dusky, cyanotic, pale, or red and may eventually lead to skin hypopigmentation
*Hyperhidrosis (early)
*Dry skin (late)
*Tenderness is initially localized but may progress to generalized tenderness. Exquisite tenderness, both periarticular and interarticular, is often present. Patients may exhibit allodynia (ie, pain with nonnoxious stimuli) and hyperpathia (ie, persistent pain after light pressure).
*Atrophy of the skin and subcutaneous fat pads
*Fibrosis of the palmar fascia
*Absence of extensor and flexor creases over joints
*Frozen shoulder, flexion deformities of the fingers, claw hand

Medical Care:
Thus, evidence-based treatment guidelines do not exist, and the approach depends largely on the specialty of the treating physician; not all patients respond to sympatholytic medications or to chemical or surgical sympathectomy...

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.

Medications: Anti-inflammatory medications and 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. 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.

Surgical Care
*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.

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. 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.

Medications Used in Treatments:
1. Corticosteroids: Prednisone
2. Non-steroidal anti-inflammatory drugs (NSAIDs)
3. Calcitonins: Calcitonin
4. Arteriolar Vasodilators:Clonidine/ Catapres®:
5. Guanethidine (Ismelin®): No longer available in the U.S.
6. Alpha Blockers: Phenoxybenzamine/ (Dibenzyline):
7. Alpha Blockers: Prazosin/ (Minipress®):

Suggested Links:
*N.H.S. Choices/ Complex Regional Pain
*post-thrombotic syndrome. Limb edema, pain and then bone scan confirmed RSD.

Reflex Sympathetic Dystrophy News and Research RSS Feed - Reflex Sympathetic Dystrophy News and Research

Reflex Sympathetic Dystrophy or Complex regional pain syndrome (CRPS) is a chronic pain condition. The key symptom of CRPS is continuous, intense pain out of proportion to the severity of the injury, which gets worse rather than better over time. CRPS most often affects one of the arms, legs, hands, or feet. Often the pain spreads to include the entire arm or leg. Typical features include dramatic changes in the color and temperature of the skin over the affected limb or body part, accompanied by intense burning pain, skin sensitivity, sweating, and swelling. Doctors aren’t sure what causes CRPS. In some cases the sympathetic nervous system plays an important role in sustaining the pain. Another theory is that CRPS is caused by a triggering of the immune response, which leads to the characteristic inflammatory symptoms of redness, warmth, and swelling in the affected area.

Two innovative techniques in the placement of an implanted spinal cord stimulator (SCS) are expected to reduce common complications at the implant site, according to new research revealed today.

*Scientists at the University of Liverpool are leading a -1.5 million project to study of an immunoglobulin, a type of drug which has been shown to ease complex regional pain syndrome (CRPS).

*Reduction in small-fiber nerves may underlie complex regional pain syndrome-I

*Researchers at Massachusetts General Hospital (MGH) have found the first evidence of a physical abnormality underlying the chronic pain condition called reflex sympathetic dystrophy or complex regional pain syndrome-I (CRPS-I).

*Reflex sympathetic dystrophy syndrome may affect more than 1.2 million Paula Abdul's story about her struggle with Reflex Sympathetic Dystrophy Syndrome (RSD), also known as Complex Regional Pain Syndrome (CRPS) highlights this complex and poorly understood disorder.

*Treatment is complex and often unsatisfactory, particularly if begun late. It includes drugs, physical therapy, sympathetic blockade, psychologic treatments, and neuromodulation. Few controlled trials have been done."

Suggested Links: Merck Manual: Complex Regional Pain Syndrome

*[Editor] The editor has a videotape interview with a 54 year old African-American woman who was successfully treated for RSD with the mixed androgen injections. Her recovery was witnessed from walking with a cane to being certified in Tae Kwon Do.

Alternative treatments in the Literature:

*Novel treatment using ketamine has shown success in reducing the severe pain caused by Complex Regional Pain Syndrome
*A novel treatment using a common anesthetic drug has shown success in reducing the severe pain caused by Complex Regional Pain Syndrome (CRPS), according to a study published in the September 2004 issue of Pain Medicine. [More]

*Low-dose infusion of ketamine completely relieves pain of complex regional pain syndrome.

*Limited, low-dose infusions of a widely used anesthetic drug may relieve the often intolerable and debilitating pain of Complex Regional Pain Syndrome (CRPS), a Penn State Milton S. Hershey Medical Center researcher found. [More]

*Efficacy and safety of high-dose vitamin C on complex regional pain syndrome in extremity trauma and surgery — systematic review and meta-analysis. Authors Shibuya N, Humphers JM, Agarwal MR, Jupiter DC

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