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Complex regional pain syndrome, or CRPS, is a chronic pain condition which can develop spontaneously following trauma. It is associated, principally, with very significant persistent pain, that cannot be alleviated by over-the-counter painkillers. This persistent pain can spread over time, or in the presence of further injury.
Diagnosis is, commonly, a process of elimination, utilising a range of tests (including blood tests, bone tests, nerve conduction tests and imaging) to rule out other, more common, conditions as a cause of your symptomology.
There are several subtypes of CRPS. CRPS Type 1 occurs following illness or injury, although is not associated with specific nerve damage. CRPS Type 2, alternatively, is associated with damage to a specific nerve. Warm CRPS refers to a type characterised by a warm sensation on the skin, and is commonly experienced earlier in the condition’s course, whereas Cold CRPS is characterised by a cold sensation and is seen, generally, in long-term cases.
In addition to painful burning, stabbing or stinging sensation, CRPS can be accompanied by changes in skin colour and temperature, and by a markedly increased and painful sensitivity to any external stimuli on the skin’s surface (known as hyperalgesia). These can include changes in temperature, pressure, and tactile sensation. Pain may increase in severity over the course of days or weeks. These episodes are known as ‘flares’ and can be exacerbated by stress.
The affected leg may become dystonic, exhibiting tremor and spasm, and can also swell significantly and become stiff.
In addition, CRPS may also cause changes in your perception of the affected limb, including variation in perceived size. You may notice changes in sweating, including sweating asymmetry. Your nails may grow excessively quickly, or they may grow much more slowly and demonstrate structural changes such as the onset of new brittleness or grooving.
The constant painful experience of living with CRPS will typically have a very significant psychological impact, including the development of depression, anxiety and suicidal ideation.
The exact cause of CRPS is not clear.
It typically presents in the weeks following an injury and so is thought to be the result of an abnormal inflammatory or immune response to that injury in both the peripheral and central nervous system. Inflammatory proteins known as cytokines may contribute to the worsening of some symptoms. It is particularly associated with nerve trauma.
People with neuropathy, or other types of nerve degeneration, may be at greater risk of developing CRPS, since they present a reduced ability to repair nerves following injury. A similar inability is associated with smoking.
Complex regional pain syndrome has no known cure, although symptoms may be managed through a combination of therapies and lifestyle changes.
Analgesic medication: The core aim of non-surgical treatment is the reduction of pain. You will be referred to a pain specialist who will be able to prescribe painkilling medication. Topical analgesics can be used to reduce hypersensitivity, although typically, treatment will involve low-strength oral or intravenous medication, increasing in strength until it provides effective pain relief.
These can include, but are not limited to, non-steroidal anti-inflammatories, anticonvulsants like gabapentin and pregabalin, tricyclic antidepressants, and opioids such as morphine. Intravenous ketamine can be used in extreme cases. All have side effects, some of them very significant.
Spinal cord stimulation: where analgesic medication is unsuccessful, a spin cord stimulator may be fitted. This is inserted subcutaneously into the stomach or buttocks and connected to the nerves in your spine. It delivers low level electrical impulses to the spin which can generate a tingling sensation in the affected part of your body and thereby mask the CRPS pain. Commonly, it is necessary to complete a trial of spinal cord stimulation prior to having the implant fitted permanently.
Nerve block: nerve blocks can be used to temporarily or permanently disrupt pain signals from the affected nerves. They involve injecting anaesthetic close to the nerve to relieve pain. For upper limb CRPS, a stellate ganglion block is normally used, while for lower limb CRPS, a lumbar sympathetic nerve block. Anaesthetic may also be injected directly into the nerves of the affected area, including the sciatic and the femoral.
Physiotherapy: alongside more common exercises such as simple stretching and weightbearing, specialist techniques can be helpful in managing CRPS pain. Of these, desensitisation involves stimulating the skin with multitype materials and textures. This is done first close to, but not in, the affected area, before gradually being introduced. This will be extremely painful at first, but can result in a reduction in hyperalgesia over time.
Other therapies: mirror therapy can be used to trick the brain into associating CRPS with your healthy limb, potentially resulting in improvements in function and reductions in perceived pain. Transcutaneous electrical nerve stimulation (TENS) may ease pain by applying electrical impulses to nerve endings.
Surgical treatment options for CRPS are extremely limited, and include:
Sympathectomy: involves cutting or destroying specific sympathetic nerves considered to contribute to the experience of CRPS pain. While some patients demonstrate improvements in their pain scores, others experience no benefit or a worsening of their existing pain. It can also result in numbness, swelling and the formation of new pain.
Amputation and osseointegration: Amputation may be employed in severe cases of CRPS where the pain and immobility cannot be managed by any other treatment, or where the patient elects to complete the procedure.
Prof. Dr Al Muderis has successfully pioneered the use of amputation and osseointegration in the treatment of CRPS. In this case, following amputation, a titanium implant is inserted into the intramedullary canal of the bone in the affected limb to serve as a connection point for an exterior prosthetic.
The amputation is completed above the existing level of the CRPS pain to avoid the risk of post-amputation recurrence and is typically combined with Regenerative Peripheral Nerve Interfaces (RPNI) and Targeted Muscle Reinnervation (TMR) to provide severed nerves with a new signalling function and reduce the risk of phantom limb sensation or a worsening of prior CRPS pain.
Osseointegration, similarly, restores mobility and stability following amputation and prevents soft tissue contact with the prosthetic of the type known to risk CRPS recurrence in at-risk amputee. Among those CRPS patients who have completed osseointegration with Prof. Al Muderis, some have been able to withdraw from the use of analgesic medication altogether, while others have been able to reduce their dosage considerably.
For further inquiries or to arrange a consultation, please contact Professor Al Muderis’ office at +61 2 88829011 or book an appointment online.
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Professor Dr Munjed Al Muderis is an orthopaedic surgeon specialising in osseointegration, hip, knee and trauma surgery. He is a clinical professor at Macquarie University and The Australian School Of Advanced Medicine, a fellow of the Royal Australasian College of Surgeons and Chairman of the Osseointegration Group.