Case of the Month #37: Complex Regional Pain Syndrome by Dr Sunil Dasari

An integrated interdisciplinary approach is recommended, using the four pillars of treatment for CRPS:

• Physical and Vocational rehabilitation, 
• Pain relief-Medications and interventions, 
• Psychological interventions, 
• Patient information and education to support self management.
   

Prompt diagnosis and early treatment are considered best practice to avoid secondary physical problems associated with disuse of the affected limb and the psychological consequences of living with undiagnosed chronic pain^3.

Practitioners can support patients by providing a clear diagnosis, information and education about the disease, helping to set realistic goals and, where possible, involving the patient’s partner and/or other family members³.

• Physical/occupational/psychological therapy¹: multidisciplinary approach including rehabilitation with physical (PT), occupational (OT), and psychological therapy. Because of the extremity pain in CRPS, patients tend to avoid the use of the affected limb. With the use of PT and OT, the goal is to have the patient improve the functionality and the range of motion of the extremity and achieve reduction in pain and increased mobility.
  Patients will tend to avoid the use of the affected limb and generate fear associated with the pain in the affected limb. With severe pain, patients will take on significant emotional stress. Psychological therapy can be helpful in this regard to assist patients with coping mechanisms for pain, relaxation training, thermal biofeedback, and graded exposure therapy. The role of PT/OT and psychological therapy is to improve functionality, mobility, quality of life, and the ability to manage the patient’s own pain. In CRPS, these therapies should be utilised early and are considered by many pain clinicians to be first-line treatment¹.
   
• Neuropathic pain medications: Using neuropathic pain medications to treat CRPS is based on their usefulness in treating other neuropathic conditions. RCTs in paediatric age groups with CRPS have shown improvement in pain and sleep with Amitriptyline and Gabapentin¹.
   
• Anti-inflammatory medications: Non-steroidal anti-inflammatory medications with their anti-inflammatory action have a role in the acute phase of the syndrome¹.
   
• Bisphosphonates: Pamidronate (single 60 mg intravenous dose) should be considered for suitable patients with CRPS less than 6 months in duration as a one-off treatment³.
   
• Interventions: Spinal cord stimulator - There is evidence to support the use of SCS in CRPS^5,6.  They should be considered early and not as a therapy of last resort. Dorsal Root Ganglion (DRG) stimulation¹ may prove to be the more superior neuromodulation option for CRPS, as DRG can target specific painful areas of limbs. Intravenous regional sympathetic blocks (IVRSB) with guanethidine should not be used routinely³.
   

CRPS may never fully resolve, and it often severely reduces patients’ quality of life and may be associated with increased psychological distress. A diagnosis of CRPS can be made in patients who have only had minor soft tissue injury. It may even occur without a traumatic event. Physiotherapy and/or occupational therapy, unless contraindicated, should be initiated immediately when CRPS is suspected. Initiate early treatment with simple analgesic and neuropathic agents such as amitriptyline, nortriptyline, duloxetine, gabapentin or pregabalin.

Amputation should not be used to provide pain relief in CRPS³. Amputation may be considered in rare cases of intractable infection of the affected limb. Surgery should be avoided on a CRPS-affected limb where possible and be deferred where it cannot be avoided until one year after the active process has resolved. Surgery may be indicated in CRPS type 2 when there is an identifiable remediable nerve lesion (eg certain cases of neuropathic pain due to either nerve compression by scar tissue, neuroma formation or perioperative nerve injury, such as through a needle stitch) but should be undertaken only when, on balance, the expected benefit from pain reduction outweighs the risk of exacerbation. 

In the pain clinic, he was educated about complex regional pain syndrome (CRPS) and how physiotherapy can help to improve functionality, weight bearing, mobility and self-management. The patient was referred to physiotherapy and was started on Pregabalin to help with the neuropathic pain. An early discussion about consideration of a Spinal cord stimulator was initiated. The patient was referred for mental health and psychology support for his depression and anxiety. The patient’s family was involved with the discussions and management plans to support him. He attended physiotherapy sessions which has shown improvement in functionality, weight bearing and decreased pain scores. The patient understands that it is slow process of recovery and is setting realistic goals.