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Complex Regional Pain Syndrome

Mr Field has a special interest in this condition, having spent 18 months obtaining a Master of Surgery thesis in the subject in 1995.

Complex Regional Pain Syndrome (CRPS) is a clinical condition that generally follows a trauma of some kind. It presents as:
  • excessive pain (often out of proportion to the degree of injury)
  • loss of joint mobility (joint stiffness)
  • increased swelling
  • temperature, colour and sweating changes - generally referred to as vaso motor instability (VMI).


CRPS usually affects the extremities (the hand or the foot), but it has also been described in the shoulder, knee or hip. In fact, it is one of the causes of prolonged disability following both hand and lower limb injuries.

Possible causes of prolonged disability after hand injury could be

  1. Post traumatic arthritis
  2. Joint stiffness
  3. Cold intolerance
  4. Complex regional pain syndrome
  5. Tendon adhesions
  6. Nerve injury and the consequences thereof
  7. [Repetitive strain injury (RSI)]
RSI is in parentheses because, if it exists, it does not follow an injury per se. In addition, the condition is totally unique in having no physical signs.

Acutely, the condition involves the four cardinal symptoms - pain, stiffness, swelling and VMI. Later in the condition, atrophy of the skin and soft tissues may occur and this is associated with joint contractures and regional osteoporosis (thinning of the bone).

The trauma that commonly precipitates CRPS varies considerably. It may occur after a soft tissue or bony injury, or even following surgical procedures (also "trauma" - but hopefully more controlled!). There are many other conditions with which it has been associated.

The condition has many names. This is due to poor diagnosis in the past, and eponyms have served only to confuse matters. Historically, in 1901 Sudeck2, a radiologist, originally described an acute inflammatory bone atrophy following a herpes infection. Sudeck's atrophy is the term that has been applied to the condition for many years.

Alternative names for this condition

  1. Complex Regional Pain Syndrome Type 1
  2. Algodystrophy
  3. Sudeck's Atrophy
  4. Reflex Dystrophy
  5. Post-traumatic pain from osteoporosis
  6. Shoulder/Hand Syndrome
  7. Algo/neurodystrophy
The term Complex Regional Pain Syndrome has also been applied to the condition for some time, being first used by Evans in 1946. The name implies causation - the condition being some form of reflex response to whatever initiating factor is present. CRPS also implies involvement of the sympathetic nervous system - our flight or fight mechanism. Thus the condition involves peripheral blood flow changes (hence colour and temperature changes) and peripheral sweating. It is a useful term because we don't know the cause and we don't know who will get it - it is an individual 'reflex' reaction to the initiating event. However, the sympathetic nervous system has never been unquestionably proved to be involved in the condition.

Algodystrophy is, seemingly, a more sensible term. It does not involve causation and doesn't imply the involvement of any particular tissue - Algo being Greek for pain and Dystropica meaning Greek for wasting.

More recently the term Complex Regional Pain Syndrome Type 1 has been applied, generally by pain specialists (anaesthetists)3. This is the name now most commonly used. However, it is not ideal because unfortunately pain is only one element of the condition: sometimes swelling, VMI or stiffness can occur without pain, and they can be more disabling than the pain itself.


How common is it?

Incidence varies from 10 to 25% following a traumatic injury. (Most of the controlled studies have been undertaken following Colles' fracture of the wrist.) Experience suggests an incidence of 1-5% following any form of hand surgery performed by an experienced surgeon.

How long does it last?

There is only one long-term study of the outcome of CRPS following Colles' fracture (Bickerstaff, 1990)7. In that study the majority of symptoms resolved by 2 years. Certainly the finger stiffness in this study was still present to a high degree in cases at 2 years. Outcome is also heavily dependent on the degree of the condition. There is a very wide spectrum - from mild to significant. Cases are even presented for medico-legal opinion 5 years after the initiating event, and some symptoms have been found as late as 10 years after a Colles' fracture8.

Does the degree of injury affect the onset of CRPS?

It is certainly not true that the worse the injury the more likelihood there is of CRPS. In fact, some very minor injuries can cause it. It is part of the clinical syndrome that the pain involved in the condition is often out of proportion to the degree of injury.

Is CRPS associated with immobilisation in plaster casts or tight plaster casts?

No. There is no evidence that this is the case. In fact there is good evidence to the contrary.

Are there any other symptoms apart from the four cardinal ones?

There are other symptoms. Essentially these are movement disorders such as muscle spasms and weakness. There can be increased hair growth on the limb, as well as increased nail growth on the limb. In fact, the nails can become rigid and brittle.

Are CRPS patients psychologically unbalanced?

There is no evidence for this whatsoever. There is, however, good evidence that people who contract CRPS are psychologically no different from the rest of the population10. It is the author's belief that because medics know so little about the condition and how to treat it, and the patient is in so much pain, that this pain may alter their psychological profile.

How is the condition treated?

It is widely agreed that early recognition and treatment of the disorder is vital. Unfortunately treatments are limited - it is always difficult to treat a condition for which the cause is unknown. The mainstay of treatment is physiotherapy to maintain the range of movement of the joints, so preventing joint contractures at a later date. There is historical evidence that overactive physiotherapy can be harmful, although this is not proven scientifically. The 'scrub and carry' regime - essentially involving the patient using the hand to do these two activities - does appear to be beneficial.

Pain is generally addressed using simple analgesics or non-steroidal anti-inflammatories. However, blocking of the sympathetic nervous system may be of benefit. This can be done peripherally using a regional Guanethedine block or can be achieved centrally by blocking the sympathetic chain in the neck. Recently regional Guanethedine blockades have been found not to be beneficial, but central blockade can certainly help. The 'block' is generally first performed using a local anaesthetic. If this is beneficial, a more permanent blockade of the sympathetic system can be performed.

Various other medications have been tried in the form of Amitriptyline, Gabapentin and, more recently, vitamin C. None have been scientifically proven to be of benefit.

Are X-rays of any benefit in making the diagnosis?

Certainly late on in the condition there is a patchy osteoporosis seen on plain X-ray, which was described originally by Sudeck. Bone scans have been used in the past to diagnose the condition. However, the bone scan becomes hot in the 'delayed phase' in late CRPS and by this stage the condition is resistant to treatment i.e. its too late.the boat may have already been missed.

Which age groups are affected?

CRPS can occur in any age group, even in children. However, it is rare in children and also rare in old age. There is no sexual predilection.

Summary

Essentially CRPS is a rare condition that is difficult to diagnose. Diagnosis is made by clinical examination rather than any form of investigation. It is a syndrome with a spectrum of symptoms varying from mild to severe. If the patient is not treated early then significant joint contractures can occur, which can be very debilitating and permanent.

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Reference

  1. Dourly, P., Dehamma, Y. and Patten, S. (1981) Algodystrophy - A Diagnosis and Therapy of a Frequent Disease of the Locomotor's Apparatus Bringavelo. Ballin Heidelburg.
  2. Sudeck, P. (1900) Uber Die Akut Entzundliche Knochenatrophie. Arch. Klin. Chir., 147-56.
  3. International Conference of Pain (1981).
  4. Hoffman, B.P. (1953) Fractures of the distal end of the radius in the adult and child. Bull. Hosp. Joint Disease, 14, 114-24.
  5. Uber, P.G. (1980) Étude sur le wrist algodystrophique chez 500 hospitalise en milleur petique. MD Thesis, Universite de Paris.
  6. Field, J. and Atkins, R.M. (1997) Algodystrophy is an early complication of Colles' fracture. What are the implications? J. Hand Surg., 22B, 178-82.
  7. Bickerstaff, D.R. (1990) The Natural History of Post Traumatic Algodystrophy. MD Thesis.
  8. Field, J., Warwick, D. and Bannister, G.C. (1992) Features of algodystrophy at ten years following Colles fracture. J. Hand Surg., 17B, 318-20.
  9. Field, J. and Atkins, R.M. (1994) Algodystrophy is associated with tight plaster of Paris cast. J. Bone Joint Surg., 76B, 901-5.
  10. Field, J. and Gardner, V.F. (1996) Psychological distress associated with algodystrophy. J. Hand Surg., 21B, 6-100.