Twenty-two adult patients underwent amputation of a limb affected by type-I complex regional pain syndrome at the University Medical Center Groningen from May 2000 to October 2008. Type-I complex regional pain syndrome was diagnosed according to International Association for the Study of Pain (IASP) criteria14 and the criteria described by Bruehl et al.1. All patients had long-standing, therapy-resistant type-I complex regional pain syndrome with a nonfunctional limb, unbearable pain, or life-threatening infections. As a result of allodynia, the patients could not tolerate touch of the affected limb. The duration of the pain syndrome was calculated from the first time it was documented in medical records. Treatment received prior to the consultation regarding the amputation was retrieved from the medical records.
Before amputation, all patients had been examined by a physician specializing in rehabilitation medicine, one of three psychologists or a psychiatrist, a physical therapist, and a vascular surgeon. The psychologist or psychiatrist assessed the patients for major psychopathology, to determine whether the patient had a realistic point of view about the possible beneficial and adverse effects of an amputation, and to determine whether there was a potential for rehabilitation. All of the health-care professionals discussed the indications for amputation and possible beneficial and adverse effects. An amputation was not performed if psychopathology was found. The professionals discussed the indications for, and possible effects of, amputation with the patient and weighed these effects against the patient’s expectations. The level of amputation was preferably proximal to the level of the signs of the complex regional pain syndrome, but it was also influenced by surgical possibilities, prosthetic design, and patient preference. All twenty-two patients were sent an invitation letter to participate in this qualitative retrospective study. Informed-consent forms were returned with use of a prepaid envelope. Once consent was given, a semistructured interview was scheduled.
Two questionnaires were sent prior to the interviews: the World Health Organization Quality of Life-BREF (WHOQOL-BREF)15 and the Groningen Questionnaire Problems after Arm Amputation (GQPAA)16 or the Groningen Questionnaire Problems after Leg Amputation (GQPLA)16. Current quality of life was evaluated with the WHOQOL-BREF, a twenty-six-item questionnaire covering four domains: physical health, psychological health, social relationships, and environment. The WHOQOL-BREF has good-to-excellent reliability and has performed well in preliminary validity tests15. WHOQOL-BREF scores were compared with Dutch norm values by calculating the 95% confidence intervals of differences in mean scores. The Groningen questionnaires assess the current use of a prosthesis, and the presence and frequency of impediments due to phantom limb pain and residual limb pain16.
Interviews were conducted in a hospital near the patient’s hometown, or at the patient’s residence if the patient preferred that. The interviews were conducted by a psychologist (E.S.) who had received extensive training in interviewing techniques and were recorded on tape. A physician (H.K.K.-S.) was present. Answers were recorded on a data sheet and compared afterward. Discrepancies between the psychologist’s and physician’s findings were resolved by discussion and by listening to the recorded interviews. In the invitation to participate, patients were assured that their responses would be handled with confidentiality and this assurance was repeated verbally prior to the interview. The physician had not been involved in the decision to amputate, and the psychologist had been involved in eight cases. The physician also had not been involved in the postsurgical rehabilitation, and the psychologist had been involved in one case.
Patients were asked to describe perceived changes in pain, mobility, activities of daily living, household tasks, work, hobbies, sports, relationships, intimacy, mood, physical appearance, worrying, sleep, and body scheme after amputation. Intensity of residual limb pain and phantom limb pain in the last two weeks prior to the interview was assessed by using a 100-mm visual analogue scale (VAS; 0 mm = no pain and 100 mm = unbearable pain). Assessment of recurrence of type-I complex regional pain syndrome in the residual limb was performed by the physician using the criteria recommended by Bruehl et al. for research purposes1.
The research protocol was approved by the local Medical Research Ethics Committee (METc 2009/117). Descriptive data analysis was performed with SPSS for Windows (version 16.0, SPSS, Chicago, Illinois).
Source of Funding
There was no external funding source for this study.
Twenty-one patients agreed to participate in the study: nineteen women and two men with a median age forty-six years (interquartile range [IQR], thirty-seven to fifty-one years). The median duration of the complex regional pain syndrome was six years (IQR, two to ten years). The median interval between the amputation and the study was five years (IQR, three to seven years). The inciting events, reasons for amputation, and level of amputation are summarized in Table I.
On average, the patients received 11.1 different types of treatment (range, three to nineteen) prior to the first consultation for amputation. Nineteen patients received differing combinations of exercise therapy, occupational therapy, manipulation, and partial immobilization. Seventeen patients had a sympathetic block or a sympathectomy. Sixteen patients received morphine. Fifteen patients received antidepressants. Twelve patients had electrotherapy, transcutaneous electrical nerve stimulation [TENS], or epidural spinal electrostimulation (ESES). Twelve patients received anticonvulsants, and nine received anti-anxiety agents.
Pain
A reduction in pain following amputation was reported by nineteen patients (90%; 95% confidence interval [CI], 71% to 97%), and eighteen of them reported a major reduction. Eighteen patients (86%; 95% CI, 65% to 95%) experienced residual limb pain, with the median intensity score on the VAS being 46 (IQR, 14 to 63). When the patients rated the impediment due to residual limb pain, the responses were equally divided among much or very much (33%), moderate (33%), and hardly any or none (33%). Seventeen patients (81%; 95% CI, 60% to 92%) experienced phantom limb pain, with a median intensity of 37 (IQR, 18 to 62). Six patients (29%) always experienced the phantom limb pain. Impediment due to phantom limb pain was rated as much or very much by seven of the seventeen patients, moderate by five, and hardly any or none by five.
Changes
Considering all changes, twenty patients (95%; 95% CI, 77% to 99%) reported an improvement in their lives. Seventeen patients (81%; 95% CI, 60% to 92%) reported an improvement in mobility and fourteen (67%; 95% CI, 45% to 83%), an improvement in sleep (Table II). More than half of the patients reported improvements in mood, physical appearance, washing oneself, clothing, and participation in work. Eight patients (38%; 95% CI, 21% to 59%) did not have any remaining symptoms of type-I complex regional pain syndrome. Six patients (29%) felt less understood after the amputation because the people in their environment had expected that all problems would be solved by the amputation. Prior to the amputation, sixteen patients referred to their affected limb as “that” limb, one patient referred to it as “my” limb, and it was not known how four patients referred to it. After the amputation, twenty patients (95%) referred to their residual limb as “mine.” One patient stated: “I have a leg again, instead of a paw.”
Level of Activity
Patients differed considerably with regard to their level of activity after the amputation. One patient was completely dependent on the care of others due to type-I complex regional pain syndrome in all four limbs before the amputation, and the amputation did not change this. Sports participation varied greatly, ranging from participation in Paralympic Games to an inability to participate in any sports activities.
Before amputation, three patients (14%) had a paid job, and three were part-time students. Afterward, eight patients (38%) were working and six were students (29%).
Quality of Life
Fourteen patients (67%; 95% CI, 45% to 83%) reported a good or very good quality of life. Four patients (19%; 95% CI, 8% to 40%) reported a poor or very poor quality of life. The mean scores on the WHOQOL-BREF in our study population were significantly lower than Dutch norm values in all domains except social relationships15 (Table III).
Satisfaction with Decision for Amputation
Eighteen patients (86%; 95% CI, 65% to 95%) stated that they would choose to undergo amputation again under similar circumstances. One female patient was not sure. She required the amputation because of a high probability of sepsis but did not want the amputation. Another patient could not pinpoint the exact reasons why she would not choose to have an amputation again, although she had problems with the fitting of her prosthesis and psychosocial problems after moving to another location. The third patient could not get used to the short upper residual limb, and her grandchildren were afraid of it.
Use of a Prosthesis
Prostheses were fitted and used regularly (at least eight hours a day) by ten (67%; 95% CI, 42% to 85%) of the fifteen patients with a lower-limb amputation; six of the prosthesis users had a knee disarticulation, and four had a transtibial amputation. Four patients with a transfemoral amputation and one with a knee disarticulation did not use a prosthesis. Four prosthesis users needed a walking aid outside. Three of them had a knee disarticulation; one of the three needed a forearm crutch, one used a cane, and one used a walking frame with wheels. The fourth patient had a transtibial amputation, and this patient sometimes used a cane and sometimes used a walking frame with wheels.
Four patients (three with a knee disarticulation and one with a transtibial amputation) could walk ≥1 km. Five patients (three with a knee disarticulation and two with a transtibial amputation) could walk 100 to 500 m. One patient with a transtibial amputation could walk <100 m.
Prior to nine of the fifteen lower-limb amputations, the expectation was that the patient would use a prosthesis based on his or her age, physical condition, comorbidities, and motor skills. All nine patients used a prosthesis daily. Another patient, who had not been expected to use a prosthesis because of a presumed lack of motivation, also used one daily. One of the six patients who had an upper-limb amputation had been expected, prior to the amputation, to use a prosthesis after the operation; however, that patient was not fitted with a prosthesis because of persistent residual limb pain. There had been doubts, before the operation, that two of the patients would use a prosthesis after their upper-limb amputation; one of these patients, who had a transradial amputation, used a prosthesis daily. None of the four patients who had a transhumeral amputation used a prosthesis.
Level of Amputation
Five patients said that they would have preferred more precise information about the level of amputation, and three patients found the amputation to be too proximal. One patient had a knee disarticulation, which she said that she would not have preferred because of the cosmetic issue of length differences between her legs while she was sitting. The vascular surgeon and the physician specializing in rehabilitation medicine reported that detailed information concerning these topics had been given.
Recurrence
Nine patients (43%) reported recurrence of the type-I complex regional pain syndrome in the residual limb, with one of them reporting that the recurrence was temporary. Four patients (19%) reported recurrence in another limb. All twenty-one patients allowed a physical examination, including the examiner touching the residual limb. On physical examination, four patients (19%; 95% CI, 8% to 40%) fulfilled the criteria of Bruehl et al.1: three of these patients (14%) had recurrence in the residual limb and one, in another limb. Another patient, with a lower-limb amputation, also had an upper-limb amputation in another hospital, three years after the first amputation, because of type-I complex regional pain syndrome. Thus, in total, there was a recurrence in five patients: in the residual limb in three (14% of the twenty-one) and in another limb in two (10%).
The patient who had the upper-limb amputation three years after the lower-limb amputation stated that both amputations improved her quality of life. She was able to walk while wearing a lower-limb prosthesis but did not use an upper-limb prosthesis. Two of the three patients who had recurrence in the residual limb reported that, despite the recurrence, they still had a major reduction in the level of pain and an increased mobility level after the amputation. The third patient indicated that the pain had worsened and the mobility level was reduced.
One patient required a shoulder disarticulation after a previous upper-limb amputation because of persistent pain that was unrelated to type-I complex regional pain syndrome.
Upper or Lower-Limb Amputation
There were no significant differences between the data for the group with an upper-limb amputation and those for the patients with a lower-limb amputation.
An amputation may positively contribute to the lives of patients with long-standing, therapy-resistant type-I complex regional pain syndrome. A reduction of pain as well as an improvement in mobility and sleep were reported by most patients in this study. Although they were not free from all symptoms, most patients were more actively participating in study, work, and sports activities. Quality of life was rated as good or very good by two-thirds of the patients. Twenty patients described their residual limb as “mine,” indicating that the residual limb was part of their body scheme again. A normal body scheme is a sign of recovery because most patients with type-I complex regional pain syndrome describe the affected limb as foreign to their body scheme17.
Despite the positive outcome, some patients still experienced symptoms of type-I complex regional pain syndrome (Table IV) and some reported a deterioration in some aspects of their lives (Table II). The results of the WHOQOL-BREF in comparison with Dutch norm values showed that type-I complex regional pain syndrome followed by an amputation had an impact on the physical health, psychosocial health, and environment domains. Despite several positive results, some patients had to deal with the adverse effects of an amputation. Some patients expressed that they felt less understood because people in their environment had expected all problems to be solved by the amputation.
Two-thirds of the patients with a lower-limb amputation and one of the six patients with an upper-limb amputation used a prosthesis regularly. Therefore, use of a prosthesis should be discussed with patients considering an amputation because of type-I complex regional pain syndrome. The results of a systematic review showed that about 48% of the patients who had a lower-limb amputation and about 23% of the patients who had an upper-limb amputation because of type-I complex regional pain syndrome used a prosthesis13. In general, use of a prosthesis is more likely after lower-limb amputations than after upper-limb amputations18. Although patients received extensive information about the level of amputation and possibilities of using a prosthesis, five patients said that they would have preferred more information. It may be that the patients did not remember all of the information that was provided19,20.
Recurrence at the time of follow-up examination was diagnosed in four patients. An additional patient had already undergone an amputation in another hospital because of recurrence of type-I complex regional pain syndrome in another limb. The recurrence rate was 14%, increasing to 24% if we take into account recurrence in another limb. This recurrence rate is low compared with the high rate (48%) reported in a recent systematic review13. However, that high recurrence rate was strongly influenced by the results of one study21. When that study was excluded from the systematic review, the recurrence rate dropped to 8%. Evidence-based guidelines state that there is insufficient evidence that amputation positively contributes to the lives of patients with therapy-resistant type-I complex regional pain syndrome8. Our study showed a considerable percentage of patients with a general improvement (95%) and with a major reduction in pain (86%). The results of our retrospective study may contribute to the discussion when an amputation is considered for a patient with long-standing, therapy-resistant type-I complex regional pain syndrome.
Amputation should be considered for therapy-resistant type-I complex regional pain syndrome only when the patient has no major psychopathology and has a realistic point of view about the possible beneficial and adverse effects of an amputation. Therefore, this decision should be made with great care, and amputation is suitable for only a small group of patients. In 2003 and 2004, 197 and 175 patients, respectively, had a lower-limb amputation at our institution. In that same period, eight patients with type-I complex regional pain syndrome had a lower-limb amputation.
Study Limitations
A limitation of this research is the small number of patients. The duration of the type-I complex regional pain syndrome prior to the amputation may have been underestimated, as we relied on documentation from other hospitals for the estimates. The results of this study are influenced by selection bias because the study included only those patients selected for amputation by the team of professionals. We do not know the quality of life of the patients who were refused amputation. Another limitation is the cross-sectional design of this study. We have no information about the quality of life or the factors prior to and immediately after the amputation. Several investigators have searched for a specific psychological profile of individuals with type-I complex regional pain syndrome but were unable to define one22-24. The cross-sectional design and small sample size in this study prevented investigation of predictors of outcomes. Patients were asked to recall their situation before the amputation, giving rise to a substantial risk of recall bias. Most patients reported improvements in their lives. It is possible that a patient “must” feel improved to justify the amputation. Eight patients had met the psychologist in the pre-amputation assessment, which might have biased their answers.
In order to evaluate determinants of the outcomes of amputation in cases of long-standing, therapy-resistant type-I complex regional pain syndrome, prospective documentation including diagnostic criteria is needed. Future research should also focus on evaluating the timing of amputation.
Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. None of the authors, or their institution(s), have had any financial relationship, in the thirty-six months prior to submission of this work, with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, no author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.