RAYNAUD'S Disease is a vasospastic disorder affecting primarily the distal resistance vessels. The disease is typically characterized by the abrupt onset of digital pallor or cyanosis in response to cold exposure or stress. Raynaud's Disease may occur independently or be associated with other conditions (systemic lupus erythematosus and scleroderma) and connective tissue diseases. Initial symptoms may include a burning sensation in the affected area accompanied by allodynia and painful paresthesias with vasomotor (cold, cyanotic) changes. Ultimately, as the ischemia becomes more chronic, this condition may progress to amputation of the affected digits.
The most common indication for spinal cord stimulation in the United States is for chronic painful neuropathies. However, in Europe, spinal cord stimulation is frequently used to treat ischemic conditions, such as peripheral vascular disease and coronary occlusive disease.1Although technically an off-label indication in the United States, this practice is supported by many published studies. There have also been case reports of its use in other diseases resulting in arterial insufficiency to the extremities, such as thromboangiitis obliterans (Buerger's Disease),2but its use in Raynaud's Disease is relatively underreported.3
This case describes the use of cervical spinal cord stimulation to treat refractory digital ischemia in a patient with advanced Raynaud's Disease.
The Vascular Surgery Service consulted the inpatient Acute Pain Service in April, 2003 to evaluate and treat a 51-yr-old female with a long history of Raynaud's Disease. She complained principally of bilateral hand pain (described as 8 of 10 on a verbal analog numeric rating scale) and symptoms of ischemia. Her disease had progressed to the point that she had already had an amputation at the distal phalanx of her left index finger. She had distal ulcerations of several of the digits and had dystrophic nails.
She complained of cold intolerance, allodynia, and hyperalgesia of the fingers but also had similar symptoms, to a lesser extent, in the lower extremities. She had been a long-time smoker but had discontinued smoking several months before this consultation. Her pain was poorly controlled with high-dose opioids (methadone 80 mg by mouth every 8 h), as well as gabapentin. She had not responded to tricyclic antidepressants, nonsteroidal antiinflammatory drugs, anticoagulants, or antihypertensive agents. In addition, she had a history of scleroderma, fibromyalgia, and bilateral carpal tunnel syndrome.
Examination of the patient's hands revealed multiple nonhealing ulcers, dystrophic nails, and extreme pallor of all digits. She had severe allodynia, hyperalgesia, and hyperpathia of these digits without extension to the hand or arms. Radial artery pulses were normal (fig. 1).
Therapeutic considerations included recurrent amputation, sympathetic nerve block, surgical sympathectomy, and spinal cord stimulation. The goal of our consultation was to find a treatment plan that would avoid amputation. Sympathetic nerve blocks have a short duration of action and would not have addressed the long-term problem. Furthermore, both sympathetic nerve blocks and surgical sympathectomy lack long-term studies indicating positive long-term outcomes.4Therefore, we elected a trial of cervical spinal cord stimulation.
This procedure was performed in two phases. In phase I, a single spinal cord stimulator lead (Pisces Quad; Medtronic®, Minneapolis, MN) was placed in the midline. Immediately, the patient noticed a dramatic improvement in circulation and pain in her hands along with paresthesia from the stimulator in the lateral aspects of both arms and forearms and in the entire hand bilaterally. Her allodynia was improved, and her pallor was eliminated.
Because of the substantial improvement in subjective pain, functional capacity, and objective signs of perfusion, bilateral spinal cord stimulator leads (Pisces Quad; Medtronic®) were placed in the posterior epidural space, with the zero electrodes positioned at the C4 level (fig. 2). This resulted in a larger area of paresthesia in both hands. The spinal cord stimulator implanted pulse generator (Synergy; Medtronic®) was subsequently placed in the abdominal subcutaneous tissue. The patient developed a mild cellulitis postoperatively, which responded well to oral antibiotic (cephalexin). She had an uneventful further recovery.
Six weeks after the completion of implantation the patient had a completion amputation of the two distal phalanges of the left index finger as a result of a nonhealing ulcer of the remnant of this digit. Aside from that preexisting ulcer, there was substantial improvement in the objective signs of perfusion and healing of other ulcers and nail dystrophy in the subsequent months.
Five months after the implantation, the patient rated her pain at 2 of 10 and was able to use her hands. She had had no subsequent surgical care or complications associated with her spinal cord stimulator. She was very satisfied with her pain control and functional ability with her hands (fig. 3). She continued on her other medications (with a slight reduction in methadone to 70 mg by mouth every 8 h) because of pain from her other conditions; however, she did not have further hospitalizations for this condition.
At 10 months after implantation, she had some recurrence of ischemia in the right distal index finger, which was previously her most serious area of ischemia. One year after implantation, this finger was amputated at the distal phalanx of this digit. She has had no subsequent digital ischemia, nor has she had recurrent pain outside of this area. Subsequent to this surgery, she has decreased her oral analgesic medications and continues to be active.
Before spinal cord stimulator implantation, this patient had increasingly severe pain and signs of ischemia for approximately 8 yr. Subsequent to implantation, with the exception of her most severely diseased digit, both the subjective complaints of pain and objective signs of ischemia have resolved and have maintained their resolution for more than 1 yr. She has had no other significant changes in medical therapy over that time aside from her surgical treatment.
Spinal cord stimulator settings from her most recent reprogramming session are available for review in the Appendix.
The patient's previous history of her history of cigarette smoking predisposed her to vascular disease. However, treatment of the underlying scleroderma and smoking cessation had not improved her symptoms of digital pallor and pain. Her disease progressed to the point of having serial amputation. Introduction of spinal cord stimulation improved her pain control and objective signs of microvascular circulation to the extent that no further amputations have been required except for a completion amputation of a digit already beyond salvage.
There has been substantial controversy regarding the use of spinal cord stimulation in ischemic extremity disease. However, current information favors the use of this treatment in inoperable ischemic extremity disease. There have been a large number of prospective studies, as well as a systematic Cochrane Review, indicating the appropriateness of spinal cord stimulation in this scenario.5However, the studies focus primarily on atherosclerotic vascular disease. Although the exact mechanism of action of spinal cord stimulation in ischemic extremity disease is still uncertain, it most certainly affects peripheral vasoconstriction and therefore should be effective in vasospastic disease, such as Raynaud's Disease.
The present case demonstrates effective use of cervical spinal cord simulation in a case of severe ischemic extremity disease and represents an opportunity for an area of further study.