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Complex Regional Pain Syndrome Glos Care Pathway Overview

This pathway has been produced to clarify the diagnosis and management of Complex Regional Pain Syndrome in Gloucestershire.

NOTE: This is interim guidance pending publication of the NICE chronic pain guideline GID-NG10069 publishing August 2020

Please click the relevant flowchart box to be taken directly to textual information

                    

Presentation

The word 'complex' reflects the variety of symptoms and signs that these patients can present with, in addition to pain. Symptoms usually begin within one month following the inciting event or immobilisation. As the signs and symptoms vary widely, patients can present to various specialities:

  • Orthopaedics - up to 25% of cases follow limb fractures and orthopaedic surgery. Patients may have been discharged but it may be necessary to re-refer to rule out organic pathology - e.g. compression of nerves by scar tissue.
  • Rheumatology, neurology and neurosurgery - having been referred to rule out other potential causes of pain.
  • Dermatologists - precipitating factors can be skin conditions, such as herpes zoster, vasculitis and leg ulcers. CRPS may also present with skin changes (see 'Signs', below).

Delay in diagnosis is common; thus, a thorough history and examination are crucial from the initial consultation.

The symptoms of CRPS vary in severity and duration. The characteristic symptom is that of pain - typically burning in nature and out of proportion to the severity of any injury. The affected area, which is not confined to a specific nerve distribution or dermatome, may have other features such as:

  • Sensitivity to touch.
  • Allodynia - perception of pain from a non-painful stimulus.
  • Hyperalgesia - painful stimuli provoke more pain than usual; mechanical and thermal hyperalgesia are especially common.
  • Swelling.
  • Abnormal vasomotor activity - spontaneous temperature changes, either warmer or cooler than the contralateral limb.
  • Abnormal sudomotor activity - spontaneous sweating.
  • Abnormal pilomotor activity - 'goosebumps'.

There are not always any objective findings in these patients. However, the following may occur:

  • About 80% of cases have temperature differences between opposite sides. They may be warmer or cooler and this may be a fluctuating sign (sometimes occurring within a few minutes) depending on room temperature, local temperature of the skin and emotional state. Occasionally, it may occur spontaneously. This may be associated with a change in skin colour.
  • Other skin changes include a shiny, dry or scaly appearance. Hair may be coarse initially, then become thin; nails become brittle and grow faster (initially), then slower; there may be associated rashes, ulcers or pustules which may become infected. Abnormal and spontaneous vasomotor, sudomotor and pilomotor activity also occur.
  • Hard, pitting oedema may occur diffusely over the painful region. There is often a well-demarcated boundary along the skin line - almost diagnostic of the condition, although similar findings occur when patients tie a band around the limb for comfort.
  • Movement may be limited, both because of the pain and because joints are often described as stiff (particularly with difficulty in initiating movements) or muscles weak. Disuse atrophy can ensue. Other muscular disorders include sudden and severe spasms, tremors and involuntary severe jerking and dystonia.

It is also important to look for psychological symptoms which may need to be treated.

Alternative or Additional Diagnoses

Exclude alternative or additional diagnoses including:

  • Infection
  • Fracture or soft-tissue injury
  • Compartment syndrome
  • Poorly placed splint / cast / fixation device
  • Arterial insufficiency: trauma, atherosclerosis or Burger’s Disease
  • Nerve entrapment / compression / neuropathy
  • Lymphatic or venous obstruction including DVT
  • Raynaud’s Disease
  • Self-harm
  • Thrombophlebitis
  • Thoracic outlet syndrome
  • Erythromelalgia
Initial Assessment / Diagnosis

The diagnosis is clinical and may be difficult, particularly in the early stages of the syndrome where there may be little, if any, objective evidence of a problem. The Royal College of Physicians recommends that doctors and other trained therapists use the 'Budapest criteria' to make a diagnosis.

Explanation of terms

‘Hyperalgesia’ is when a normally painful sensation (e.g. from a pinprick) is more painful than normal

‘Allodynia’ is when a normally not painful sensation (e.g. from touching the skin) is now painful

‘Hyperesthesia’ is when the skin is more sensitive to a sensation than normal

                                      

 

If A, B, C and D above are all ticked, please diagnose CRPS. If in doubt, or for confirmation, please refer to your local specialist.

Please note:

  • Distinction between CRPS type 1 (no nerve injury) and CRPS type 2 (major nerve injury) is possible, but has no relevance for treatment. As an exception, in surgical practice, in CRPS type 2, a nerve lesion can sometimes be directly treated (see surgical management section in Royal College of Physicians CRPS guidance)
  • If the patient has a lower number of signs or symptoms, or no signs, but signs and/or symptoms cannot be explained by another diagnosis, ‘CRPS-NOS’ (not otherwise specified), can be diagnosed. CRPS-NOS can also apply to patients with documented CRPS signs/symptoms in the past

In category 4, the decreased range of motion/weakness is not due to pain. It is also not due to nerve damage or a joint or skin problem. This is a special feature in CRPS and is due to a poorly understood communication between the brain and the limb. A helpful question to asses this feature is: ‘If I had a magic wand to take your pain away, could you then move your … (e.g. fingers)?’ Many patients will answer with ‘no’ to that question.

Around 10% of patients with CRPS cannot recall a specific trauma or may report that their CRPS developed with an everyday activity such as walking or typewriting. In some people CRPS can have a bilateral onset. In about 7% CRPS can spread to involve other limbs. Around 15% of CRPS patients do not improve after 2 years. It is appropriate to make the diagnosis of CRPS in these unusual cases.

Note: psychological findings such as anxiety, depression or psychosis do not preclude the diagnosis of CRPS.

Initial Primary Care Management

EARLY ACTION FOR ALL

If needed:

Core Physiotherapy/Hand Services

Please click on the locality below for more information.

                                                                        

Core Physiotherapy/Hand Services provide:

First line management using GHFT Pain Clinic Guidance and provide patient information

  1. Urgent physiotherapy/hand therapy for recent onset CRPS - based on assessment findings
  2. Patient Information: Explanation about condition, information leaflets details about support groups if available.
  3. Therapy Assessment: To identify physical, functional and psychological priorities for management. Suggested initial assessment tools include:
  1. Expert Advice: It is recommended that therapists discuss all cases with a colleague who has expertise in CRPS management.
  2. Effective ongoing analgesia to facilitate progress:
  • Via GP using GHT Pain Clinic guidance or suggesting that the GP can access timely, specific advice via the e-referral / Advice and Guidance route e.g. in patients with significant medical complexity, extremes of age or polypharmacy.
  • Via Pain Consultant- Referral in writing marked ‘URGENT -  CRPS’ addressed Dear Pain Consultant and sent to the Central Booking Office
  1. Psychological Assessment where indicated.  Suggested assessment tools include:
  1. For patients who fail to respond to appropriate therapy within 4 weeks, discuss with a colleague who has experience in CRPS and refer in writing, marking the referral ‘CRPS – URGENT’ to Pain Consultant.

NB within a 6 month ‘window’ from onset of symptoms, a day case infusion of bisphosphonates is a possibility.

When to Refer

Physiotherapy

All patients with a diagnosis of CRPS using the Budapest Criteria should be referred, or encouraged to self-refer, to Core Physiotherapy.

Pain Management

Patients with a diagnosis of CRPS using the Budapest Criteria and who have failed to respond to appropriate therapy (within 4 weeks) should be referred to Pain Management.

Secondary Care Management

Chronic Pain MDT (GHNHSFT)

Chronic Pain MDT provided by GHNHSFT includes Physiotherapy, Occupational Therapy, Nursing, Psychology and Consultants in Pain Medicine. Services include assessment of pain, education, advice and reassurance. Specialist Nurse can provide support for medication reduction if needed. Pain self-management interventions including Pain Management Programmes, Mindfulness Courses and 1:1 CBT and specialist exercise interventions available.

Some people with particularly complex pain presentations may benefit from referral to the tertiary pain management service at the Bath Centre for Pain Services www.bathcentreforpainservices.nhs.uk. In order to access funding from NHS England referrals must be sent from secondary care services demonstrating that all local options have been exhausted or are inappropriate.

Referrals are accepted via e-mail or letter primarily from MSK Interface services, Secondary Care and Mental Health but also from GPs.  Please include reason for referral, pain history, investigations performed and the results of these, PMH including relevant psychosocial history, previous medications tried and the response to them, current medications and allergies. Please use the referral form.

Ongoing Care

The long-term management of treatment-resistant CRPS should be shared between the primary care clinician, Physiotherapy team and chronic pain service and, where appropriate, specialist rehabilitation services.

Rehabilitation for patients with chronic CRPS: (defined as patients who have failed to respond to or progress with previous physiotherapy/hand therapy)

  1. Pain consultant will review and decide whether the patient requires onward referral to a specialist centre such as Bath Centre for Pain Services.
  2. Assessment within pain self-management. Either referral to a therapist with particular expertise  in CRPS management (Physio/Hand Therapist) or treatment within pain self-management depending on patient’s need.
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