Michael is a 47-year-old construction worker presenting for follow-up evaluation for complex regional pain syndrome (CRPS). While managing a construction site 3 years ago, Tom fell approximately 9 ft from a rooftop, sustaining a tibial plateau fracture to his right leg. He underwent an open reduction and internal fixation. His post-operative recovery and rehabilitation were going well until 3 months following the accident, when he began to experience excruciating pain in his entire right leg. He described the pain as intensely burning as if it were on fire and that the leg had become sensitive to the slightest touch. He was unable to sleep because of the pain he experienced when the bed linen would touch his leg and wake him up. He states that he also began to notice that the leg would swell and change color (sometimes reddish, other times mottled following exposure to cold) and that he no longer had any hair on his right leg. He was seen by a number of physicians and received numerous diagnoses until last year, when a neurologist diagnosed him with CRPS.
What are the possible conditions? How would you make the diagnosis? The diagnosis of CPRS is clinical. Michael's problems started following the injury and the surgery that followed it - therefore it is likely CRPS type I. However, certain conditions can mimic it. These include neoplasms, arthritis (septic, degenerative, gout, SLE), avascular bone necrosis, conversion/self-harm, and dis-/non-use. Certain tests, such as quantitative sensory testing, autonomic function tests, temperature measurement, vascular flow measurements, and imaging studies (bone scintigraphy, bone densitometry, CT, MRI), can be performed to rule these out.
Michael tried various medications to control the pain including tramadol, duloxetine, carbamazepine, gabapentin, and oxycodone. He reports that none of these treatments sufficiently controlled his pain and he is not using them anymore. He was also seen by a pain specialist who performed several nerve blocks that helped with the pain but only temporarily. He is very frustrated with his condition, he states that he can no longer work because of the pain, and he is afraid that he will soon lose his home. He admits that he is irritable at times and feels depressed occasionally, but denies suicidal ideation. His medical history is otherwise unremarkable.
What is your next step?
You have to take a detailed history and perform a systematic physical examination in order to confirm the diagnosis of CRPS and to rule out other diseases. His conditions might have changed since he saw his previous attending physician. Depending on your assessment, you may need to order further investigations as necessary.
His average pain score is 6-7 on a scale of 0-10. It can go up to 10 "on a bad day" and at night. His physical examination is notable for edema of the right leg to the level of the knee, associated with red reticulation of the skin and apparent hair loss. The right leg and foot are cooler than the left, but popliteal and dorsalis pedis pulses are intact bilaterally. He has widespread allodynia and hyperalgesia on clinical examination with preservation of muscle mass, tone, and strength. Deep tendon reflexes are present in all extremities and found to be normal.
What are the options now available to help Michael?
Michael has been suffering from CRPS for the last few years. He has failed to respond to pharmacotherapy and other forms of treatment. He is in distress. Unfortunately, the available options are limited. Spinal cord stimulation is one option, but it is an invasive procedure. The other option is to undergo a pain management program which is not a cure in its own merit.
Following an extensive review of his past medical history, prior treatment regimens, results of investigations, and consultation with a psychologist, Michael agrees to a trial of neuromodulation with a spinal cord stimulator. One week later Tom is seen in the operating room by an interventional pain specialist who, under local anesthesia, inserts a percutaneous epidural electrode array and connects it to an external pulse generator. Tom is allowed to go home following the procedure and is asked to return in 5 days for follow-up. Five days later Tom returns to the office and reports that his pain is much better and that its intensity has been reduced by 70%. He is able to play with his children and work around the house. He reports that the swelling has subsided and the leg no longer changes color in the cold. He is pleased with the trial and is anxious to proceed to a permanent implantation.
Following permanent implantation of the spinal cord stimulator, Michael experiences a greater than 50% reduction is his pain intensity and is now able to perform all of his activities of daily living. He also reports being able to sleep much better at night.
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