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The overlooked disease: reflex sympathetic dystrophy.

Reflex sympathetic dystrophy (RSD) is a disease known by several different names, including causalgia, shoulder-hand syndrome, and post-traumatic spreading neuralgia.(1) It occurs when the arms or legs suffer injury or other trauma, even minor trauma like that associated with administering intravenous fluids or medication.(2) Patients with RSD suffer muscle and skin atrophy, lose mobility in the affected limb, and become extremely sensitive to touch--so sensitive, in fact, that the slightest contact can cause severe pain.

No one theory explaining the cause of RSD is widely accepted. One theory states that following injury, a reflex arc --a loop or circle involving various elements of the peripheral nervous system --is established. The result is that painful sensations are continuously activated.(3) Almost everyone at some time suffers trauma to an arm or leg, but no one knows why some patients develop RSD and others do not.

RSD is among the most frequently encountered problems in clinical medicine. Proper diagnosis and prompt therapy are critical if its devastating effects are to be avoided. Early recognition and treatment in most cases result in a speedy recovery. For optimal results, treatment should begin within six weeks after symptoms appear. Unfortunately, RSD, especially in its early stage, is often misdiagnosed or improperly treated, resulting in a prolonged or even permanent disability for the patient.

Clients may arrive in the attorney's office suffering from symptoms suggesting RSD. The attorney should send these clients to a specialist to make or rule out the diagnosis. A case that may include RSD should not be set for trial or settled until it is known whether the condition is present and, if so, what the long-term prognosis is. Many patients with advanced RSD become unemployable and never return to a normal, productive life, so the condition can have a great impact on future damages.

Stages of RSD

RSD progresses in stages. It begins gradually--hours, days, or weeks after the initial injury. The length of the stages varies considerably; they last anywhere from several weeks to many years. The medical literature describes three stages:

Stage I: The Acute Stage. The pain has a burning, throbbing, or aching quality. It is aggravated by physical contact (no matter how slight), movement, or emotional disturbances. It is painful for an affected arm to hang vertically at the side of the body. In all cases, the pain is much more severe than what would be expected from the injury.

Even a light covering like a sheet or blanket cannot be tolerated. Sleep is difficult and frequently disturbed. The skin may be warm and dry or wet and clammy. The hands or feet may be swollen. The patient may be highly emotional.

In cases involving an injured arm, the patient holds the hands and fingers in a slightly flexed position. The elbow is flexed, and its motion is often unaffected. The grip is weak. An affected leg is also weak, and the patient may use a cane or crutch to walk.

Stage II: The Dystrophic Stage. Pain in the affected limb is constant and increased by any stimulus. Skin temperature previously elevated begins to fall, and blood flow is diminished. The skin begins to thin out or atrophy. It is pale, taut, and shiny. The patient loses hair, and nails become brittle, ridged, and cracked. The subcutaneous tissue begins to atrophy.

The joints--especially hand joints in arm injury cases--become stiff. The patient refrains from moving the affected arm or leg as much as possible. X-rays may reveal patchy osteoporosis.

Because the inital injury appears to be too minor to cause the patient's severe symptoms, the physician and patient may begin to believe that the cause of the problem is mental rather than physical. Indeed, the physical pain of RSD has a profound effect on the patient's psychological and emotional well-being. But the physician must treat the disease to obtain positive psychological and emotional results.

Stage III: The Atrophic Stage. The pain is often intractable and widespread, involving the entire limb. The skin continues to atrophy and becomes increasingly smooth, glossy, and taut. Flexion contractures develop, making the patient unable to fully extend the limb. In arm injury cases, the muscles of the hand atrophy, and the range of motion of the fingers and wrist are severely limited. Muscle atrophy and reduced range of motion also occur in leg cases, and the patient often must use a cane, walker, or wheelchair.

The effects of RSD at this stage are mostly irreversible. The patient is left with a useless extremity; constant, severe pain; and severe emotional instability.


Symptoms range from mild to severe. In its early stage, the condition may be difficult to diagnose, but full-blown RSD should be obvious even to physicians who ordinarily do not care for patients with this problem. No competent physician could fail to consider the possibility of RSD when a patient has pain in an arm or leg that is out of proportion to the injury, combined with swelling, skin color changes, temperature changes in the affected extremity, and marked hypersensitivity of the skin.

The following studies can help the physician make a proper diagnosis:

* Thermography. This test measures the skin temperature of affected and unaffected limbs. The temperature of a limb with RSD in the early stages of the disease will be about 2 degrees centigrade higher than an unaffected limb.(4) In the later stages, the affected limb will have a lower temperature than the unaffected limb.

* Three-phase radionuclide bone scanning. In this test, tiny amounts of radioactive material are injected into a vein to be distributed throughout the body. If RSD is present, the tissues around the affected joint will pick up the injected material. This method has confirmed RSD in the hand as early as two weeks after the initial injury.(5)

* Sympathetic neural blockade. In this procedure, also called a ganglion block, a local anesthetic is injected to block the sympathetic nerves that are an important part of the reflex are. A proper block will eliminate or significantly decrease the pain in the affected arm or leg. If the pain is not affected, it is unlikely that the patient has RSD.

Once the diagnosis is established, prompt and proper treatment may begin. Treatment focuses on two principles: (1) increasing the limb's range of motion by decreasing swelling and preventing fibrosis (tissue scarring and development of flexion contractures) and (2) managing pain with paraffin baths, cold packs, local anesthetics, analgesics, and ganglion blocks before range-of-motion exercises.

Physical therapists use active and passive range-of-motion exercises in treating patients. In an active exercise, the patient moves a joint using his or her own muscle power. In a passive exercise, the therapist moves a joint while the patient keeps the muscles relaxed.

The goal of therapy is to prevent chronic, intractable RSD. Early, vigorous physical therapy with range-of-motion exercises is necessary to stop the condition's progress, but pain must be relieved before therapy begins. Otherwise, the therapy actually helps establish chronic RSD rather than cure it.

In the later stages of the disease, treatment becomes more varied: The physician may try using electrical stimulation, steroids, or a variety of other treatments --some of them experimental.(6) These treatments are generally less effective in returning the patient to normal, which is why diagnosis and proper treatment in the early stages are crucial.

Case Reports

The following cases illustrate the evolution of RSD in individual patients.

Case 1: A 21-year-old woman received intravenous sedation through a needle inserted in the back of her right hand when she had teeth extracted. She returned to the oral surgeon's office 25 days later complaining of a shooting pain in her right index and middle fingers and the back of her right hand. She had difficulty bending her fingers.

There was no evidence of infection or inflammation of the vein. Because the patient worked as a cashier, the oral surgeon believed that tendonitis might have developed. She was advised to apply moist heat, to rest the hand, and to keep it elevated.

At her next appointment, the patient reported that she was still experiencing the same symptoms. The oral surgeon referred her to an orthopedic hand surgeon, who diagnosed RSD.

The patient began intense physical therapy. The therapist noted swelling of the fingers and hypersensitivity to touch. Thermography indicated that the hand's skin temperature was elevated, consistent with RSD. Ganglion blocks did not produce long-lasting pain relief.

The patient was referred to a pain management center for psychological evaluation and counseling for her chronic pain. She withdrew from the physical therapy program against her doctor's advice and never returned for follow-up care.

Case 2: During a fight in a pub, a 25-year-old man suffered an inch-long cut between the first and second fingers of his right hand. The cut was treated with stiches, which were removed at the appropriate time.

A month after the injury, the patient complained of numbness in the area of the scar and disabling pain in the hand. He reported that exposure to cold increased the pain. His doctor noted that the hand was swollen and sweated excessively. The doctor contemplated an RSD diagnosis but considered depression more likely and prescribed an antidepressant medication.

The pain intensified and spread to the right shoulder. A ganglion block was ineffective for long-lasting pain relief Surgery relieved pain in the scar only,

The patient's condition deteriorated over the next year. Muscle and skin atrophy developed. Repeated ganglion blocks and steroid treatment were ineffective. An X-ray of the right hand revealed patchy osteoporosis of several bones. A bone scan showed reduced uptake of the injected radioactive material in the same areas.

More than five years after the injury, the patient had not returned to work. Chronic pain, severe muscle atrophy, and flexion contractures made his right hand useless. Amputation was advised, but the patient refused.

Case 3: A 32-year-old woman injured her right shoulder in a car collision. Later that day, she experienced severe pain in her right arm. Her right shoulder and upper arm were tender to touch. She went to a local hospital, where she was examined and X-rays were taken. Cold compresses and acetaminophen for pain were advised.

Over the next several days, she complained about severe burning pain over her entire right arm. The pain was exacerbated by even the slightest touch--from a breeze or a bed sheet, for example. When she called the emergency room, she was advised to continue using warm compresses and acetaminophen.

Over the next several months, she visited several doctors and was diagnosed as suffering from RSD. Her shoulder was injected with local anesthetics and steroids, and she was given narcotics to control her pain.

She eventually came under the care of a pain specialist, who tried various treatments. Finally, a morphine pump was implanted to deliver morphine directly into the cerebrospinal fluid. It is unlikely that this patient will ever return to any semblance of a normal life.

These examples show how an initial trauma--ranging from the administration of I.V. sedation to injuries received in a fight or a car crash--can lead to RSD. In the first case, although there was no medical negligence associated with the surgery, the attorney must investigate the client's subsequent medical care to determine whether the possibility of RSD was considered early, whether a proper referral was made, and whether proper and timely treatment was initiated. In the second and third cases, an alert attorney will realize that RSD is a major part of the client's injury. In each case, the condition has a significant impact on future damages.

Legal Considerations

An RSD case reaches the lawyer over one of two separate routes. Sometimes RSD develops after an initial injury caused by an intentional or negligent act. In other cases, the initial injury was neither intentionally nor negligently caused, but the client develops RSD because the treating physician missed its early signs and failed to make a prompt diagnosis or made the diagnosis but did not provide proper treatment.

The first scenario suggests a straightforward liability issue, such as an injury due to a car crash, fall, or battery, with RSD developing from the injury. Any subsequent negligence by a health care provider is linked causally to the original tort, and the resulting injury is part of the plaintiff's damages. It is crucial for the lawyer to understand the potential for RSD to significantly affect damages. An expert experienced in RSD diagnosis and treatment is essential to help establish present and future damages.

The second scenario suggests medical negligence. In these cases, the lawyer

must have a basic understanding of RSD and its causes. The attorney should focus on three areas of special importance: the evolution of the disease and its prognosis, proof of causation, and damages.

An attorney familiar with the early signs of RSD should question the client (without prompting) about when the symptoms first appeared. Once the client gives this information, the lawyer should ask these questions:

* Can you identify relatives, friends, neighbors, or work associates who can attest to that time frame?

* Did you tell the treating physician about the symptoms? When? Can others testify to this fact?

* Did the physician explain what the cause of the symptoms might be? If so, what was that explanation?

* If you called the physician's office complaining of RSD symptoms, was it a long-distance call? Does the physician's office keep a telephone log?

* Were you given prescriptions for pain medication? What pharmacy has these records?

* Did you have physical therapy? Did you complain of excessive pain during therapy?

The goal is to establish a clear and corroborated chronology detailing the earliest appearance of symptoms and when they were reported to the treating physician. At this point, a differential diagnosis including RSD as one possible cause should have been made.

The next step is to determine when the RSD diagnosis was actually made. A detailed history should help determine whether the condition progressed from Stage I to Stage II or even Stage III between the time the diagnosis should have been considered and the time it was made.

The inquiry next focuses on treatment. The attorney should investigate two separate areas: the treatment prescribed for the underlying injury and the treatment specifically prescribed for the RSD. The following example illustrates how physicians often focus on treating the underlying injury.

A person suffers a dislocated shoulder. After initial treatment and six weeks of immobilization, the patient is sent to physical therapy for active and passive range-of-motion exercises. Severe pain in the shoulder interferes with the therapy, and the therapist notifies the orthopedic surgeon. The surgeon orders hot and cold compresses, passive range-of-motion exercises, and codeine for pain. He tells the patient that he must persist with the therapy or the shoulder will not heal.

The physician and the therapist in this example are focusing on the treatment ordinarily needed to rehabilitate a dislocated shoulder. They have not considered the possibility that the patient may be developing the early signs of RSD. Indeed, they may be making matters worse because pain during physical therapy reinforces RSD.

The attorney must pursue in detail these areas of inquiry:

* What treatment was prescribed? When? By whom?

* Was physical therapy initiated? When? What were the elements of the program?

* Was the pain relieved before each therapy session? What method was used (oral medication, local anesthetic)?

* Did the treatment help restore function and alleviate pain?

* Did the client follow the physician's recommendations, keep all appointments, and actively participate in his or her care and treatment?

* Did the client require external electrical stimulation?

* What is the present stage of the RSD?

* What is the client's present treatment program?

* Is the client on narcotic medications for pain?

* Have any ganglion blocks been performed?

* Has an electrical stimulator been placed on the spinal cord so stimulation can be applied internally?

* Has an implantable morphine pump been considered?

* What is the client's present psychological and emotional status? Has the client required the services of a psychiatrist, psychologist, or other therapist?

* How has the RSD affected the client's family? Have family members required counseling because of it?

* Has the client returned to his or her pre-injury job?

* Has the client entered a job retraining program?

* Is the client now totally or partially disabled?

The answers to these questions will help the attorney determine whether the client received proper medical care and the extent of the client's damages.

It is important for the attorney to appreciate that RSD cases always involve an element of permanent disability, resulting either from the disease itself or from the treatment. If, for example, the client must use narcotics on an ongoing basis, he or she may be unable to perform the normal tasks and activities of daily living.

We have seen two cases in which the clients had preexisting diagnoses of RSD associated with earlier trauma. They sustained subsequent injuries to the same or other parts of the body. In these cases, the attorney must be alert to how the more recent trauma has been super-imposed on the preexisting illness, further compromising the client's ability to perform ordinary work and leisure activities.

For example, one client who had been diagnosed with RSD and was being treated with morphine was reinjured in a motor vehicle collision. Previously, the client had been able to work part time at home. With the additive effect of a back injury from the collision, the client was rendered totally disabled.

A more typical scenario appeared in a case involving a young punch press operator whose hand was crushed in the workplace. Surgery and therapy produced a good return in terms of mechanical function of the hand and wrist. However, the client began to suffer chronic pain and eventually lost the use of his hand. Over the course of a year, the symptoms progressed to the other arm. In the ensuing products liability and workers' compensation actions, settlements were based on damages related to the client's total disability as a result of reflex sympathetic dystrophy.

RSD is little understood by most physicians and lawyers. Physicians who do not understand it cannot properly care for their patients, and lawyers who do not understand it cannot properly represent their clients' interests. Increased awareness is necessary for early detection of the disease and for lawyers to win adequate compensation for their clients who suffer the ravages of the full-blown disorder.


(1) Robert J. Schwartman & Toni L. McLellan, Reflex Sympathetic Dystrophy, 44 ARCHIVES NEUROLOGY 555 (1987); Pedro L. Escobar, Reflex Sympathetic Dystrophy, 15 ORTHOPAEDIC REV. 646 (1986). (2) Thomas R. Brock, Reflex Sympathetic Dystrophy Linked to Venipuncture: A Case Report, 47 J. ORAL & MAXILLOFACIAL SURGERY 1333 (1989); Baruch Gold ct al., Reflex Sympathetic Dystrophy Following Minor Trauma, 25 ISRAEL J. MED. SCI. 107 (1989); Tomas J. Silber & Massoud Majd, Reflex Sympathetic Dystrophy Syndrome in Children and Adolescents, 142 AM. J. DISEASES CHILDREN 1325 (1988). (3) See William J. Roberts, A Hypothesis on the Physiological Basis for Causalgia and Related Pains, 24 PAIN 297 (1986). (4) Daniel L. Hodges & Thomas J. McGuire, Burning and Pain After Injury, POSTGRADUATE MED., Feb. 1, 1988, at 185, 187. (5) A. Constantinesco, Three Phase Bone Scanning as an Aid to Early Diagnosis in Reflex Sympathetic Dystrophy of the Hand, 5 ANNALES DE CHIRURGIE DE LA MAIN 93 (1986); N. Fano & C. Holm, Bone Scintingraphy in Post-Traumatic Reflex Dystropky, 17 SCANDINAVIAN J. RHEUMATOLOGY 455 (1988). (6) For a discussion of treatment methods in late-stage RSD, see Salim Y. Ghostine ct al., Phenaxybenzamine in the Treatment of Causalgia, 60 J. NEUROSURGERY 1263 (1984); J.G. Hannington-Kiff, Relief of Causalgia in Limbs by Regional Intravenous Guanethidine, 1979-2 BRIT. MED. J. 367.

Victor R. Scarano, M. D., is a partner in the Philadelphia of Shrager, McDaid, Loftus, Flum & Spivey David S. Shrager, a former ATLA president, is a partner in the same firm.
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Title Annotation:Medical Negligence
Author:Shrager, David S.
Date:May 1, 1994
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