Complex regional pain syndrome (CRPS), also described as reflex sympathetic dystrophy, is a syndrome that usually develops after an injury, is not limited to the distribution of a single nerve, and is apparently disproportionate to the injury. It is associated with edema, changes of blood flow, abnormal sudomotor activity in the region of pain, and allodynia or hyperalgesia. However, the use of these criteria in clinical studies is poorly described.1 In chronic stages, sequelae of CRPS are pain and impaired function.2
When the outcome is unpredictable, the indication for amputation is a matter of controversy.3–5 Refusing an amputation can have relatively good results.6 Prosthetic use is limited because of recurrence of CRPS, but most patients seem to be satisfied with the result of the amputation Table 1.4
A 44-year-old woman, known to have CRPS, consulted our multidisciplinary team (neurology, traumatology, and rehabilitation medicine) in 2001. She requested amputation of the right leg because of unbearable pain caused by CRPS (10 on the Visual Analogue Scale [VAS] for pain). Her medical record started in 1992. After mountain climbing, she complained about pain in her right knee, accompanied by swelling and purple coloring. Arthroscopy failed to reveal a diagnosis. In 1996, signs of CRPS appeared in her left shoulder, and in November 1999 she developed CRPS in the right arm after supraspinatus tendon avulsion.
At presentation, there was constant pain, numbness, and tingling from the foot to above knee level. The leg felt cold. Changes in temperature were described as pain. There was swelling and increased sudomotor activity. The leg was afunctional, with a knee flexion contracture (90°) and a fixed pes equinovarus. The CRPS was resistant to any medication.
We advised amputation at the transfemoral level because of the suspected improved mobility after amputation and the reduced arm and shoulder load when walking with crutches.
Patient continues to describe burning pain (5 to 6 on the VAS) during the day and at night, increasing with activity. Furthermore, she experiences phantom pain and phantom sensation. She does not wear the prosthesis because it aggravates the pain. She can walk small distances with two crutches, but most of the time she is wheelchair dependent. She is independent of personal care and does household activities, depending on the degree of pain.
She is married and has no children. Before the illness, she was a bus driver. No longer able to do the job, she became a volunteer at the call center of the Association of Patients with Reflex Sympathic Dystrophy.
Overall, she is satisfied with her quality of life (10 on a VAS for satisfaction).
A 34-year-old woman had CRPS type 1 of her right leg. Because of loss of functionality, pain, and infections, she consented to an amputation.
Her medical record started in 1994 with admission to a rehabilitation center by a neurologist. She then had a paralyzed leg of unknown cause. Neuropsychological examination revealed no abnormal personality or psychiatric disorders. She was dismissed with no gain in function.
In 2001, we saw her again in the rehabilitation department of our hospital. She had a painful (9 on the VAS) right leg with infected ulcers and no ambulatory function. After multidisciplinary examination and consultation (surgery, neurology, psychiatry, and rehabilitation medicine), a transfemoral amputation was recommended.
The patient reports burning pain (8 on the VAS) during the day and some moments during the night, increasing with activity (using her prosthesis). She also experiences phantom pain and phantom sensation. When she is using the prosthesis, the signs of dystrophy are aggravated. No dystrophy signs were recognized in other parts of the body. Pain is successfully treated by transcutaneous neurostimulation. The shape of the residual limb is normal, as are range of motion in the hip and hip muscle strength. She wears the prosthesis two times a day, 45 minutes each time. The walking distance with prosthesis is 2 to 3 kilometers with one elbow crutch. Except for decreased velocity, there is an acceptable gait pattern with a normal stance and swing phase. The prosthesis has a stance phase control system.
The rehabilitation program will continue until she can wear the prosthesis several hours a day. She is independent of personal care and can don and doff the prosthesis herself.
Work reintegration as a doctor’s assistant has begun. She is living with a friend in a wheelchair-adapted house, and she is doing her own household activities. Except for sitting while wearing the prosthesis, she is content (8 on the VAS) with the overall result.
A 31-year-old woman experienced a sports injury at the age of 13 years. After two arthroscopic procedures on the knee, she developed increasing signs of CRPS.
In 1999, neuropsychological examination revealed no disturbances. She wanted her leg to be amputated because of unbearable pain (10 on the VAS), no function (no strength in the leg muscles, Medical Research Council 0) and color change of the leg to purple. She had undergone several therapies without success.
In 2001, amputation at the transfemoral level was performed, followed by residual limb correction in 2004 because of bone overgrowth.
She sometimes has pain (2 on the VAS). The complaints are weather (rain) dependent. When wearing the prosthesis, she has no signs of dystrophy. There are no other signs of dystrophy elsewhere. The strength of the residual limb is normal, and there is a normal range of motion in the hip. She wears her prosthesis the entire day without using crutches. Walking distance is not limited, and stair climbing is no problem. There is a normal gait pattern with normal stance and swing phase.
She is independent of personal care. She can don and doff the C-leg prosthesis (computer-regulated knee mechanism) by herself.
Before the amputation, she worked fulltime as a canine beautician. Now she is working 25 hours a week as a veterinary surgeon assistant. She lives in a nonadapted house and does her own housekeeping. She is able to drive a car, and her hobbies are hiking, bicycling, and skiing. She is very satisfied (10 on the VAS) with the overall result.
It is well known that the indication for amputation in CRPS type 1 is rare, except in life-threatening infections. It is expected that most of the dystrophic aspects, especially pain, will not diminish after amputation. However, in these few cases, amputation was considered to be justified (relating to the international classification of functioning, disability, and health) to primarily improve the disability and overall health. The patients suffered for years without any positive prognosis or successful treatment and improvement. The pain diminished (substantially in two patients), and the quality of life appears to increase after amputation, even when a prosthesis is not worn.
1. Reinders MF, Geertzen JH, Dijkstra PU. Complex regional pain syndrome type 1: use of the International Association for the Study of Pain diagnostic criteria defined in 1994. Clin J Pain
2. Zyluk A. The sequelae of reflex sympathetic dystrophy. J Hand Surg
3. Thomas M, Fast A. Amputation for reflex sympathetic dystrophy. J Bone Joint Surg Br
4. Dielissen PW, Claassen AT, Veldman PHJM, Goris RJA. Amputation for reflex sympathetic dystrophy. J Bone Joint Surg Br
5. Eyres KS, Talbot IC, Harding ML. Amputation for reflex sympathetic dystrophy. Br J Clin Pract
6. Geertzen JH, Eisma WH. Amputation and reflex sympathetic dystrophy. Prosthet Orthot Int