Amputation in CRPS: a no-no or reasonable in certain circumstances? A medical & legal controversy.

This is an area where strongly held opinions are not underpinned by strong evidence. In response to papers reporting this treatment, it has been said categorically that amputation should never be considered in intractable CRPS (an increasingly untenable position as evidence accumulates IMHO).

My personal opinion is that there are some rare cases where it is appropriate in intractable CRPS cases, after referral from the Pain Service. My experience has been limited to just a few cases, but the decision was not regretted over the period of follow-up.

All of the papers below are best read in their entirety of course, and are easily available. There is no pretence that these are the only papers on the subject, but they illustrate the controversy well. Within them, one can discern the evolution of a concept of which patients and under which circumstances the operation has the best chances of success.

Dielissen et al, ‘AMPUTATION FOR REFLEX SYMPATHETIC DYSTROPHY’. J Bone Joint Surg (Br] 1995:77-B:270-3.

n=28, 34 amputations in 31 limbs.

2 patients were relieved of pain by amputation, (unpredictably). 10 of 14
patients were cured of infection and 9 of 15 patients had improvement of residual function.

In 28 of the amputations, RSD recurred in the stump, especially after amputation at a level which was not free from symptoms.

Because of recurrence of RSD in the stump or severe hyperpathia only two patients wear a prosthesis. Despite this 24 patients were satisfied with
the results.

This paper drew a strong response (Thomas & Fast, J Bone Joint Surg [Br] 1995:77-B:836-7): ‘We agree with the authors’ conclusion that amputation is not a reasonable treatment for RSD, but feel that a stronger condemnation of such practice is essential….The results in all cases were equivocal at best, and we consider that these case reports provide an argument against the use of amputation for RSD, and that there is no indication…….We feel that the patients studied by Dielissen et al had amputations despite compelling reasons for not performing such surgery. One hopes that such a study need never again be published by the Journal.’

However, there was to be no closing of Pandora’s box. These patients are in desperate straights, and will grasp at any possibility of even partial alleviation of their suffering – as would we all in similar circumstances.

Krans-Schreuder et al, ‘Amputation for Long-Standing, Therapy-Resistant Type-I Complex Regional Pain Syndrome’.J Bone Joint Surg Am. 2012;94:2263-8 d http://dx.doi.org/10.2106/JBJS.L.00532

n=21. Median duration CRPS 6 years, follow-up 5 years. Indications: pain (5), recurrent infection (14) or to improve residual
function (15).

20 patients reported improvement; 19 reduction in pain; 17 improved mobility; 14 improvement in sleep. 18 would make the same decision again in the same circumstances. 10/15 lower limb amputees used a prosthesis, 1/6 upper limb amputees did so. Recurrence of CRPS in limb residua occurred in 3, and in another limb in 2.

Midbari et al, ‘Amputation in patients with complex
regional pain syndrome. A COMPARATIVE STUDY BETWEEN AMPUTEES AND NONAMPUTEES WITH INTRACTABLE DISEASE’.Bone Joint J, 2016;98-B:548–54.

n=38. 50% in each matched group. CRPS to amputation interval mean 5.2 years; follow-up 6.6 years. Assessment: Short-Form (SF) 36, Short Form
McGill Pain questionnaire (SF-MPQ), Pain Disability Index (PDI), the Beck Depression Inventory (BDI) and a clinical demographic questionnaire. Retrospective, post hoc matched comparator group with CRPS.

Admittedly this study construct is not perfect, but in view of the rarity of the problem it is difficult in reality to construct a ‘perfect study’.

Amputation carried out through healthy skin, a number of cm above the affected area. This cis probably an important point that may explain the improved outcomes.

The amputation group showed better results in: median pain intensity, median SF-MPQ, median BDI, & 6 of 8 SF-36 domains.

The authors commented:

Amputation should be considered as a form of treatment for patients with intractable CRPS.

Midbari & Eisenberg. ‘Is the Pain Medicine Community Reluctant to Discuss Limb Amputation in Patients with Intractable Complex Regional Pain Syndrome?’ Pain Medicine 2017; 18: 1406–1407 doi: 10.1093/pm/pnw289

This is an interesting letter to the Editor, commenting on the difficulty in publishing any article about CRPS & amputation in a pain journal, observing that all the publications are in orthopaedic journals.

‘After receiving those comments, we searched the literature on CRPS and amputation again. Surprisingly (or not), none of the published papers on this topic has been published in the pain literature. Rather, all papers
have been published in orthopedic/rehabilitation journals. So what does this mean? With no doubt, the outcome of amputation in patients with CRPS is not the main issue here because, clearly, not much is known about that. The main matter, in our minds, is reluctance of the pain medicine community to even bring up for discussion amputation as treatment option for intractable CRPS. This reluctance can simply reflect a belief that amputation does not improve outcome. However, it can also mean admitting that for these patients all efforts have failed and pain medicine has nothing else to offer.’

It is interesting to speculate whether, in the UK, a doctor is fulfilling the duty to discuss all reasonable options with the patient, if this option is not discussed with a patient with intractable CRPS when amputation is technically feasible. Perhaps a legal reader could comment on this?

Ayyaswamy et al, ‘Quality of life after amputation in patients with
advanced complex regional pain syndrome: a systematic review’. EOR | volume 4 | September 2019. DOI: 10.1302/2058-5241.4.190008
.

8/11 studies supported amputation as an option for selected unresponsive cases. 66% experienced improvement in QoL, 37% were able to use a prosthesis, 16% had a decline in QoL, for 12% no clear details were given.

Risks: phantom limb pain 65%, recurrence of CRPS 45%, & stump pain 30%. 2/3 were satisfied.

Shrier et al, ‘Psychosocial factors associated with poor outcomes after amputation for complex regional pain syndrome type-I.’ PLOS One. Published: March 13, 2019 https://doi.org/10.1371/journal.pone.0213589

4 psychosocial factors were associated with a poor outcome:

  • change in the worst pain in the past week was associated with poor social support
  • intensity of pain before amputation
  • Being involved in a lawsuit prior to amputation was associated with a recurrence in the residual limb (Bruehl criteria).
  • A psychiatric history was associated with recurrence somewhere else (Bruehl criteria)

Shrier et al, ‘Decision making process for amputation in case of therapy resistant complex regional pain syndrome type-I in a Dutch specialist centre’. Medical Hypotheses. Volume 121, December 2018, Pages 15-20

These authors describe a structured decision-making & consent process:

After referral of the patient with the request to amputate the affected limb, it is checked if

  • the diagnosis CRPS-I is correct,
  • duration of complaints is more than 1 year,
  • all treatments described in the Dutch guidelines have been tried
  • consequences of an amputation have been well considered by the patient
    • Thereafter the patient is assessed by a multidisciplinary team (psychologist, physical therapist, anesthesiologist-pain specialist, physiatrist and vascular surgeon).
    • During a multidisciplinary meeting professionals summarize their assessment.
    • Pros and cons of an amputation are discussed, taking into account level of amputation and expectations about post amputation functioning of patient and team.
    • Based on assessments and discussion a consensus based decision is formulated and the patient is informed.
    • If it is decided that an amputation is to be performed, the amputation will follow shortly.
    • If it is decided not to amputate, the decision is extensively explained to the patient.

It should be noted that a UK consent process would need more autonomous patient choice, compared to the described process above. However the preliminary review of diagnosis & treatments given, multidisciplinary assessment & options review with likely outcomes/uncertainty acknowledgement could certainly be part of a UK process.

Hoellwarth et al, ‘Amputation With Osseointegration for Patients With Intractable Complex Regional Pain Syndrome. A Report of 3 Cases’JBJS Case Connector: January-March 2021 – Volume 11 – Issue 1 – p e20.00267
doi: 10.2106/JBJS.CC.20.00267

A short report describing success in transfemoral amputation followed by an osseo-integrated prosthesis. 2 gained independent ambulation, one remains on crutches. ‘One uses no pain medication, one is weaning off, and one requires a reduced regimen after revision nerve innervation’.

Summary

Amputation should only be considered (but it should be considered not omitted) as a last resort for established severe Chronic Regional Pain Syndrome (CRPS), after regular pain clinic modalities have been unsuccessful.

Amputation should be carried out through normal skin above the level of symptoms.

Certain psychosocial circumstances may predict a poor outcome, and therefore should be avoided if possible.

Patients are more likely to experience improvement in quality of life than to experience deterioration.

An osseo-integrated prosthesis is an interesting new development that may increase comfort & acceptability. Evidence for this is small in numbers and has limited follow-up.

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