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Therapy-Resistant Complex Regional Pain Syndrome Type I: To Amputate or Not?
Marlies I. Bodde, MD1; Pieter U. Dijkstra, PhD1; Wilfred F.A. den Dunnen, MD, PhD1; Jan H.B. Geertzen, MD, PhD1
1 Center for Rehabilitation, Department of Rehabilitation Medicine (M.I.B., P.U.D., and J.H.B.G.), and Department of Pathology and Medical Biology (W.F.A.d.D, University Medical Center Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands. E-mail address for M.I. Bodde: m.i.bodde@rev.umcg.nl. E-mail address for P.U. Dijkstra: p.u.dijkstra@rev.umcg.nl. E-mail address for W.F.A. den Dunnen: w.f.a.den.dunnen@path.umcg.nl. E-mail address for J.H.B. Geertzen: j.h.b.geertzen@rev.umcg.nl
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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.

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Investigation performed at the University Medical Center Groningen, Groningen, The Netherlands
A commentary by Richard J. de Asla, MD, is linked to the online version of this article at jbjs.org.

Copyright © 2011 by The Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2011 Oct 05;93(19):1799-1805. doi: 10.2106/JBJS.J.01329
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Abstract

Background: 

Amputation for the treatment of long-standing, therapy-resistant complex regional pain syndrome type I (CRPS-I) is controversial. An evidence-based decision regarding whether or not to amputate is not possible on the basis of current guidelines. The aim of the current study was to systematically review the literature and summarize the beneficial and adverse effects of an amputation for the treatment of long-standing, therapy-resistant CRPS-I.

Methods: 

A literature search, using MeSH terms and free text words, was performed with use of PubMed and EMBASE. Original studies published prior to January 2010 describing CRPS-I as a reason for amputation were included. The reference lists of the identified studies were also searched for additional relevant studies. Studies were assessed with regard to the criteria used to diagnose CRPS-I, level of amputation, amputation technique, rationale for the level of amputation, reason for amputation, recurrence of CRPS-I after the amputation, phantom pain, prosthesis fitting and use, and patient functional ability, satisfaction, and quality of life.

Results: 

One hundred and sixty articles were identified, and twenty-six studies with Level-IV evidence (involving 111 amputations in 107 patients) were included. Four studies applied CRPS-I diagnostic criteria proposed by the International Association for the Study of Pain, Bruehl et al., or Veldman et al. Thirteen studies described symptoms without noting whether the patient met diagnostic criteria for CRPS-I, and nine studies stated the diagnosis only. The primary reasons cited for amputation were pain (80%) and a dysfunctional limb (72%). Recurrence of CRPS-I in the stump occurred in thirty-one of sixty-five patients, and phantom pain occurred in fifteen patients. Thirty-six of forty-nine patients were fitted with a prosthesis, and fourteen of these patients used the prosthesis. Thirteen of forty-three patients had paid employment after the amputation. Patient satisfaction was reported in eight studies, but the nature of the satisfaction was often not clearly indicated. Changes in patient quality of life were reported in three studies (fifteen patients); quality of life improved in five patients and the joy of life improved in another six patients.

Conclusions: 

The previously published studies regarding CRPS-I as a reason for amputation all represent Level-IV evidence, and they do not clearly delineate the beneficial and adverse affects of an amputation performed for this diagnosis. Whether to amputate or not in order to treat long-standing, therapy-resistant CRPS-I remains an unanswered question.

Level of Evidence: 

Therapeutic Level IV. See instructions to Authors for a complete description of levels of evidence.

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    References

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    MI Bodde, PU Dijkstra, WFA den Dunnen and JHB Geertzen
    Posted on December 08, 2011
    Response to Frolke et al and Van Ek et al
    University Medical Center Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands

    We would like to thank authors of both letters (Frolke et al and Van Ek et al) for critically reading our article and their comments. Both groups state that amputation in case of longstanding therapy-resistant CRPS-I should be discouraged or even that it should be considered bad practice. First of all, we would like to specifically state that, as already mentioned in our article, based on the current literature on amputation in longstanding and therapy-resistant CRPS-I conclusions cannot be drawn since previous studies do not clearly delineate the beneficial and adverse effects of an amputation performed for this diagnosis. Secondly, we agree with the above mentioned authors that all therapies should have been tried before amputation is considered in longstanding and therapy resistant CRPS-I (also stated in our review). However, if patients have tried all other treatment options unsuccessfully and ask a clinician to consider an amputation the clinician should be able to give advice based on available evidence, good clinical practice and patient preference. In their critical comments Frolke et al and Ek et al present results which have not been published. We encourage them to publish these results in order for clinicians to make better informed decisions. Both groups state that PEPT may prevent the situation in which CRPS-I turns to a stage that the patient asks for an amputation. Frolke et al claim that ‘unrestricted mobilization with prosthesis contributes highly to quality of life´. Of course, if patients with an amputation use a prosthesis, they will rate a higher quality of life. The opposite cannot merely be stated. It is not proven that quality of life in this patient group does not benefit from amputation if a prosthesis is not used. We report use of prostheses in 23% of upper-limb amputees and 48% of lower-limb amputees, which indeed is lower than in other patient groups but it does not mean that patients with unbearable pain rate their quality of life lower after the amputation because they cannot use a prosthesis. In their comment Ek et al note that a complete recovery occurred in 47%, a partial recovery in 43% of the patients. We found in their paper (figure 1) that 106 patients were included. After the program 18 patients with upper limb involvement and 31 patients with lower limb CRPS-1 had recovered fully ((18+31)/106 = 46%) However, that same study shows a small group of patients (9/106 = 8%) with either no change (n=5) or drop out of the study (n=4) due to too strenuous interventions or the experience of too much pain. (Two patients were lost to follow-up.) It is for the purpose and wellbeing of this small group of patients that we have written this systematic review. Even with PEPT as a new, promising therapy, patients with therapy-resistant CRPS-I may remain. Other patients suffering from CRPS-I have life threatening infections of the extremity. In both cases amputation should not necessarily be considered bad practice as is so strongly stated by these authors. For the conclusions of our systematic review we included only published data. Results not reported in literature, could obviously not be used. As stated before poor reporting on beneficial and adverse effects of amputation in CRPS-I patients left us with no other conclusion that at present, whether to amputate or not in order to treat long-standing, therapy-resistant CRPS-I remains an unanswered question. We encourage all researchers in the field of CRPS-I treatment, especially those involved with PEPT, to publish their results in detail. We never stated that amputation is the best option for long-standing therapy-resistant CRPS-I. We do advise clinicians, in case of long-standing therapy-resistant CRPS-I to make a decision based on available evidence (which is quite limited), good clinical practice and patient preference. Amputation may still be the last treatment option available when all other treatments have failed.

    Jan Willem Ek, Frank Klomp, Robert van Dongen, Jan van Egmond
    Posted on November 29, 2011
    Amputating patients with CRPS type 1 is bad practice.
    Department of Rehabilitation Medicine, Bethesda Hospital, Hoogeveen and Institute for Anesthesiology, Radboud University, Nijmegen, Netherlands

    Bodde et al.[1]conclude that it is an unanswered question if amputation is an option for therapy-resistant CRPS type 1. We agree with Frolke and Van de Meent that amputation is no option. Therapies which seem to impact at the higher levels of the central nervous system[2,3,4,5], brain research[6,7,8,9] and the symptoms of long lasting CRPS type 1[10,11,12], point in the direction of an origin in the brain. If the origin of the pain is in the brain, amputation will not solve the problem. The brain hypothesis explains also the failure of therapy’s directed at the symptoms in the periphery (dmso crème, fluimicil, mannitol) or at a slightly higher level in the nervous system (baclofen, ketamine, sympathetic blockade)[13,14] . Effects of a treatment directed at the function and neglecting the pain (Pain Exposure Physical Therapy or PEPT) are positive and even patients who were considering amputation improved markedly [4]. Bodde et al.[1] cite this article. Their interpretation of the results, however, is incomplete since they state that only 46% of the patients would have improved if more stringent criteria for functional improvement had been applied. In the study 106 patients were included, the mean duration of the CRPS type 1 (IASP-criteria) was 55 month and the patients received more than 3 different treatments. A normal function and thus full recovery of the hand/arm function was defined as a Radboud Skills Test limitation score < 3. A partial recovery of the arm/hand was defined as an improvement of the Radboud Skills Test limitation score. A normal function and full recovery for the leg/foot was defined as being able to walk either more than 4km or more than 1 hour, without crutches. Partial recovery was defined as an improvement of the walking-distance, however, less than 4 km. Applying this criteria on both groups taken together show a complete recovery in 47 %, a partial recovery in 43 % and no recovery (including the patients lost to follow up) in 10 %. We are aware that it is a case-series with all the caveats that belong to such a design. The mean duration of the disease, however, was 55 months and most patients had been treated with three or more different therapies without long-lasting success. A follow up study of the results after 4 to 6 years shows promising results (publication in preparation, van Egmond et al.). Van de Meent et al.[5] confirmed the results of the case-series in a multiple single case study design. A lot of research has to be done, but the results of the PEPT therapy and other treatments directed at the function of the limbs and brain plasticity underscore the possibility of preventing a possible iatrogenic syndrome. With today’s knowledge we therefore consider it as bad practice to amputate a patient with (chronic) CRPS type 1. REFERENCES: [1]Bodde MI, Dijkstra PU, den Dunnen WF, Geertzen JH. Therapy-resistant complex regional pain syndrome type 1: to amputate or not? J Bone Joint Surg Am. 2011;93(19):1799-805.[2]Moseley GL. Graded motor imagery is effective for long-standing complex regional pain syndrome: a randomised controlled trial. Pain 2004;108: 192–98. [3]De Jong JR, Vlaeyen JWS, Onghena P, Cuypers C, den Hollander M, Ruijgrok J. Reduction of pain-related fear in complex regional pain syndrome type 1: The application of graded exposure in vivo. Pain 2005; 16: 264–75. [4]Ek JW; van Gijn JC; Samwel H; van Egmond J; Klomp FP; van Dongen RT. Pain exposure physical therapy may be a safe and effective treatment for longstanding complex regional pain syndrome type 1: a case series. Clin Rehabil. 2009;23:1059-66. [5]Van de Meent H, Oerlemans M, Bruggeman A, Klomp F, van Dongen R, Oostendorp R, Frölke JP. Safety of 'pain exposure' physical therapy in patients with complex regional pain syndrome type 1. Pain. 2011;152(6):1431-8. [6]Maihöfner CA, Handwerker HO, Neundorfer B, Birklein F. Cortical reorganization during recovery from complex regional pain syndrome. Neurology 2004; 63: 693–701. [7]Pleger B, Tegenthoff M, Ragert P et al. Sensorimotor returning in complex regional pain syndrome parallels pain reduction. Ann Neurology 2005; 57: 425–29.[8]Maihöfner C, Forster C, Birklein F, Neundofer B, Handwerker HO. Brain processing during mechanical hyperalgesia in complex regional pain syndrome: a functional MRI study. Pain 2005;114:93-103. [9]Geha, P.Y., Baliki, M.N., Harden, R.N., Bauer, W.R., Parrish, T.B., & Apkarian, A.V. (2008). The brain in chronic CRPS pain: abnormal gray-white matter interactions in emotional and autonomic regions. Neuron, 60, 570–581. [10]Janig W, Baron R. Complex regional pain syndrome: mystery explained? Lancet Neurol 2003;2:687-97. [11]Mc Cabe C.S., Haigh R.C., Halligan P.W., Blake D.R., Referred sensations in patients with complex regional pain syndrome type 1. Rheumatology 2003 (Oxford) 1067-73. [12]Fretlloh J, Huppe M, Maier C. Severity and specificity of neglect-like symptoms in patients with complex regional pain syndrome (CRPS) compared to chronic limb pain of other origins.Pain2006;124:184-9. [13]Cepeda MS, Carr DB, Lau J. Local anesthetic sympathetic blockade for complex regional pain syndrome. Cochrane Database Syst Rev. 2005 Oct 19;(4):CD004598. [14]Tran de QH, Duong S, Bertini P, Finlayson RJ. Treatment of complex regional pain syndrome: a review of the evidence. Can J Anaesth. 2010 Feb;57(2):149-66.

    Jan Paul Frölke and Henk van de Meent
    Posted on November 07, 2011
    Do not amputate for CRPS-1
    Radboud University Nijmegen Medical Center, The Netherlands

    In the article “Therapy-Resistant Complex regional Pain Syndrome Type 1: To Amputate or Not” (2011;93:1799-805), Bodde et al. reviewed the literature on amputations for complex regional pain syndrome type 1 (CRPS-1) in 107 patients who underwent an amputation. They concluded that it remains unclear whether amputations for this condition is beneficial or not [1]. Surprisingly, this statement is made despite the authors' observations that only 17% of the patients were able to use the prosthesis, while unrestricted mobilization with prosthesis contributes highly to quality of life. In 1995, a study was performed in our department on 28 patients who underwent amputations for CRPS-1 [2]. In this study, 18 patients were analyzed after lower limb amputation for CRPS-1. Satisfactory use of the prosthesis occurred in only 2 patients. In view of these disappointing results, since 2006 we changed our treatment strategy in patients with long-standing, therapy resistant CRPS-1. This treatment is called ‘Pain Exposure’ physical therapy (PEPT) [3] and has also shown to be safe and effective in patients with acute CRPS-1 [4]. PEPT is based on progressive loading exercises and management of pain-avoidance behavior without the use of specific CRPS-1 medication or analgesics. We reviewed our percentage of amputations after the introduction of this new treatment strategy and found only one patient who refused PEPT and underwent an upper limb amputation, from 723 patients at our institution who were evaluated for CRPS-1 since 2006. Compared to a similar cohort in the former study [2] this is a highly significant difference (1/723 vs 19/829; p<0,0005). Based on our results, we would therefore strongly recommend discouraging amputations for CRPS-1. Amputations for CRPS-1 are serious disabling interventions and can be avoided with current new treatment strategies. REFERENCES [1] Bodde MI, Dijkstra PU,Den Dunnen WFA, Geertzen JHB. Therapy-Resistant Complex Regional Pain Syndrome Type I: To Amputate or Not? J Bone Joint Surg Am. 2011;93(19):1799-1805. [2] Dielissen PW, Claassen AT, Veldman PH, Goris RJ. Amputation for reflex sympathetic dystrophy. J Bone Joint Surg Br. 1995;77(2):270-3. [3] Ek JW, van Gijn JC, Samwel H, van Egmond J, Klomp FP, van Dongen RT. Pain exposure physical therapy may be a safe and effective treatment for longstanding complex regional pain syndrome type 1: a case series. Clin Rehabil. 2009;23(12):1059-66. [4] van de Meent H, Oerlemans M, Bruggeman A, Klomp F, van Dongen R, Oostendorp R, Frölke JP. Safety of 'pain exposure' physical therapy in patients with complex regional pain syndrome type 1. Pain. 2011;152(6):1431-8.

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