Debunking Complex Regional Pain Syndrome/Sudeck/Reflex Sympathetic Dystrophy

25 Debunking Complex Regional Pain Syndrome/Sudeck/Reflex Sympathetic Dystrophy

Francisco del Piñal

Abstract

The aim of this chapter is to demonstrate, that despite all our previous learning, we may have been misguided for many years as to the existence of the cryptic condition known as complex regional pain syndrome (CRPS), algodystrophy, Sudeck, or reflex sympathetic dystrophy. An in-depth study of each patient permits us to allocate each case in a named condition. The current arbitrary use of CRPS after surgery as “an unfortunate complication,” or labeling conditions as CRPS because the surgeon ignores the actual diagnosis, is a clear disservice to the patient and to the progress of medicine.

Keywords: CRPS, chronic pain, RSD, Sudeck

25.1 Introduction

“Convictions are more dangerous enemies of truth than lies.”

F. Nietzsche

CRPS—complex regional pain syndrome—(a.k.a. Sudeck atrophy, reflex sympathetic dystrophy [RSD], or algodystrohy) is an abnormal painful response after trauma or surgery, accompanied by vasomotor changes, and the lack of a plausible cause for its development. CRPS is an end-point diagnosis, whose main treatment is medical in a Pain Clinic.1^,²^,³^,⁴^,⁵^,⁶

The condition has evolved in its nearly 150 years of existence to include pathologies that were not initially part thereof, much depending on the moods of the age. What Silas Mitchell described as causalgia was the burning pain and vasomotor changes soldiers had in their limbs after sustaining major nerve trunk injuries. The condition had an evident inciting pathology, i.e., a nerve injury. This initial concept was somewhat distorted by Paul Sudeck in 1900, who extended the condition to cases with a similar clinical picture but caused by a minor or even a nontraumatic event (minor causalgia). Later, Leriche and Policard would attribute overactivation of the sympathetic nervous system as for the pathology resulting in the unusual clinical picture, hence the name Reflex Sympathetic Dystrophy. Recently, the taxonomy had to be revised in order to dodge the lack of sympathetic system involvement.⁷^,⁸ The new terms CRPS1 and CRPS2 emerged, parenthetically RSD for the former and causalgia for the latter.⁹ In other words, RSD/CRPS1/Sudeck were additions to what Mitchell already had described in 1870. In his favor, Sudeck did not have tools such as CT scan, MRI, arthroscopy, and the like at his disposal to hone his diagnosis, and modifying the saying: all cats may have looked gray in the darkness of those times.

It may be wiser for me not to say that I firmly believe that CRPS1 is a convenient condition which shelters bad doctoring and our frustrations when we are at a loss.¹⁰^,¹¹ Nevertheless, I am not alone in challenging the status quo. Several surgeons and neurologists¹²^,¹³^,¹⁴^,¹⁵^,¹⁶^,¹⁷^,¹⁸ have written about the abuse and misdiagnosis of this condition. We may differ in the underlying pathology: some blame the nerves; others think there is a deep-rooted psychiatric problem, or a psychological issue. Yet, the message of all these authors is the same: CRPS is not a condition, in itself, but more so a constellation of signs and symptoms that needs proper identification and consequently effective treatment.

So, despite the place it has been afforded in the literature, its long existence, the multitude of papers in top journals and chapters in reference books, I am sticking to my guns and once again reiterate: CRPS is a fabrication and as concocted should be radically excised from all medical practice.

25.2 The Weakness of the CRPS Concept

Reading the literature, in my quest to understand CRPS, I have come across several weaknesses/inconsistencies/biases:

●It is astonishing that with today’s medical advances,²^,³^,¹⁹^,²⁰ the condition still has no clear-cut clinical picture, no specific diagnostic tests, an unknown pathophysiology, and lacks curative treatment. Thus, it is a condition underpinned on clinical lore not on scientific grounds.

●The criteria for diagnosing CRPS are exceedingly lax (Budapest criteria or Veldman’s criteria).¹^,²¹^,²² Any painful condition with swelling and inflammation seems to fit under the umbrella. The most stringent criterion is the inability of the surgeon to provide an explanation for the patient’s clinical picture. This item assumes that all doctors have the same ability to diagnose, which at this point of my career I can only grade as hilarious. Contrarily, as I have written, the only association I have been able to find was an exponential increase in CRPS cases in proportion to the ignorance of the surgeon who diagnosed the case.¹¹ This assertion seems to have irritated a fair number of people, when in fact all I did was to write a finding. Fortunately, I am again not alone here and others claim CRPS diagnosis to be a consequence of “lazy medicine”²³ “by junior doctors who do not take into account the negative effect this diagnosis may entail.”²⁴ Nevertheless, as I will discuss below this is fueled by an erroneously taught urgency to rush into the diagnosis in order not to miss a case.

●Some of the scientific backing of the condition comes from the assembly of an inexistent continuum from real conditions (causalgia) and imaginary ones (RSD-Psychogenic hand). It is surprising that most studies on the incidence and outcomes currently quoted were performed on distal radius fractures (DRF) on a time when suboptimal treatments for DRF were dispensed (simply cast, or at best supplemented with K-wires).²⁵^,²⁶^,²⁷ In a long-term study, it is claimed that the “reduction” was unrelated to the presence of CRPS, while in the same study osteoarthritis was (p < 0.0001).²⁶ This contradicts what we know today, i.e., that osteoarthritis is directly related to intra-articular malunion.

●Most current papers are review articles and are written by the same authors-institutions (Chicago-Cleveland-London-Mainz-Nijmegen-Vanderbilt) and in our field (Bristol-Poznan-London) as the most productive centers. The reviews are preceded by editorials: heaping hallelujahs on the review writing. In such reviews,⁵^,⁶^,¹⁹ the dissident papers were systematically ignored, despite claiming in-depth reviews in PubMed. This is tantamount to pure denialism. Furthermore, the papers published in the higher impact journals are written by rehabilitation and pain doctors, when the only person who can decide if there is no explanation for a patient’s pain in the realm of the hand is the hand surgeon.

●Authorities on CRPS³^,⁴^,⁵^,⁶^,¹⁹^,²⁰^,²⁷^,²⁸^,²⁹ stress that early diagnosis is paramount to prevent the condition from evolving into the chronic stage. The review papers are full of claims such as: “better too much than too little,” “better to overdiagnose than miss a case,” “overdiagnosis is good,” etc. This places enormous pressure on the clinician to lower the threshold to diagnose the condition to avoid missing a single case. Yet, nobody has demonstrated that early diagnosis has any benefit on the outcome.

Premature diagnosis swells the figures with cases that are not “CRPS” and, not surprisingly, studies on the natural history of the condition have shown spontaneous resolution in most cases.25^,³⁰ In one of Zyluk’s papers, none of the 15/120 patients who satisfied criteria for CRPS 6 weeks after the accident did so at 1 year; the final incidence was less that 1%! Thus, early diagnosis, linked to early treatment, may give the impression that early treatment is highly effective, when in fact the patients may have been “cured” of a condition they had never had! In keeping with the above, all available treatments are ineffective in the chronic stage.⁴^,⁵^,⁶^,¹⁹^,²⁹^,³¹^,³² This chronic group probably represents the only “true” CRPS cases—please note true is in inverted commas.

●Due to the lack of specificity of the criteria used to diagnose CRPS (Budapest’s, Veldman’s) some patients allocated in CRPS, in theory an end-point diagnosis, had surgical cure. There are many examples of this in the literature.¹²^,¹³^,¹⁷^,³³^,³⁴^,³⁵^,³⁶^,³⁷ Unfortunately, most patients are not so lucky to find a surgeon who understands their claims, but are sent to the Pain Clinic, and there is no way back from there. This is not to say that the Pain Doctor is doing anything wrong. On the contrary, the mission of the Pain Doctor is to alleviate pain, not to know what the etiology of the pain is—this is the responsibility of the referral surgeon!

●This nonsensical sequence of events makes the number of CRPS cases soar to 50,000 a year in the United States alone.³⁸ Unfortunately, diagnosing CRPS lightly is not without its consequences. Psychiatrists, psychologists, and surgeons have warned that diagnosing CRPS has a deleterious effect on well-being and mental health, particularly of the most deprived population.³⁹ By being given this diagnosis, patients may find a reason for their complaints and actually become ill and unnecessarily medicalized.¹⁸^,²³^,²⁴^,⁴⁰ It is thus crucial to narrow the diagnosis down to only those (if any) who develop this condition.

25.3 The Series

The weaknesses discussed and the blown-up figures were quite insufficient evidence to remove CRPS from our armamentarium. It is irrefutable that there were patients who had signs and symptoms making them eligible under Veldman’s criteria, the Budapest Criteria, and who registered a high score on the CRPS severity score.²⁸ To unravel the conundrum, a prospective study was carried out. Beginning in January 2018 all patients attending the office for a second opinion having been diagnosed with and treated for CRPS were included. Our first purpose was to give them a fresh diagnosis and, if the patient accepted, to treat them surgically and assess the outcomes.

The study is still ongoing, so the breakdowns are under construction. Currently, there are 166 patients of whom 44 refused to be operated or failed to return to further follow-up. Some were disappointed or upset on being confronted with the fact they did not have CRPS. Some were sent to a psychiatric consultation, but never went. Sixteen good candidates for curative surgery, because they presented an irritative carpal tunnel syndrome, refused treatment counseled by their treating doctors (orthopaedic, rehabilitation, or pain doctors). I must admit that, albeit frustrating, there is nothing wrong with this choice, as papers and reference texts advise against surgery.³^,⁵^,⁶^,²⁰^,²⁷^,²⁹^,⁴¹^,⁴² Some of those who refused surgery were overt cases of malpractice, and yet surprisingly still preferred to be treated by the original surgeon, perhaps dissuaded by the cost of private care. A further 28 did not need any surgery, they were cases of overdiagnosis, some flare reactions, and at least four cases of misdiagnosis/malpractice.