Anesth Analg 1999;88:865
© 1999 International Anesthesia Research Society
REGIONAL ANESTHESIA AND PAIN MANAGEMENT
Endoscopic Thoracic Sympathectomy for Primary Erythromelalgia in the Upper Extremities
Toshiya Shiga, MD, PhD*,
Atsuhiro Sakamoto, MD, PhD*,
Kiyoshi Koizumi, MD, PhD , and
Ryo Ogawa, MD, PhD*
*Department of Anesthesiology and
Second Division of Surgery, Nippon Medical School, Tokyo, Japan
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Introduction
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Erythromelalgia, a rare syndrome of unknown cause, is characterized by burning pain, redness, edema, and increased skin temperature in the lower, upper, or both extremities (1,2). In addition to drug therapies (2–5), sympathetic blockade is effective in improving the symptoms (6–8). We report the successful outcome of treating primary erythromelalgia of the upper extremities with bilateral thoracic sympathectomy using a video-assisted thoracic surgical (VATS) technique.
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Case Report
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A 42-yr-old woman had been suffering from redness in distal site of both fingers since 1989. During the 2 yr before diagnosis of erythromelalgia, she began to notice severe burning pain in the fingers associated with edema. Her pain worsened after exertion that involved her hands but was alleviated markedly when she immersed them in cool water. Attacks of burning pain often occurred at night and sometimes lasted until early morning. Peripheral pulses were normal. Mechanical allodynia was absent in the affected areas. A diagnosis of erythromelalgia was made by a dermatologist she first consulted. Laboratory examination exhibited no symptoms of hypertension, diabetes, rheumatoid arthritis, systemic lupus erythematous, or gout. Red blood cell and platelet counts were within normal limits. She had no family history of relevant conditions. Thermography showed marked hyperthermia in the affected areas. Histopathological examination by punch skin biopsy from an affected area revealed thickening of the capillary blood vessel walls in the superficial epidermis, probably due to blood congestion; however, the findings were nonspecific. She received aspirin from a dermatologist but had no improvement in the symptoms and suffered side effects of nausea and vomiting. Therefore, the aspirin was discontinued, and the dermatologist consulted with us. Thoracic epidural blockade and stellate ganglion blockade afforded marked (Figure 1) but transient (a few days) pain relief. Because the patient desired lasting pain relief, we chose endoscopic thoracic sympathectomy (ETS) to attempt a more permanent sympathetic blockade.
First, the patient underwent right ETS. In the operating room, an epidural catheter was inserted 2–3 cm cephalad at the T3 level. Anesthesia was induced with propofol (2 mg/kg), followed by vecuronium (1 mg/kg). Her trachea was intubated with a double-lumen endotracheal tube for differential ventilation. Anesthesia was maintained with sevoflurane, O2, and nitrous oxide (fraction of inspired oxygen [FIO2] 0.33), combined with intermittent 1% mepivacaine injection of approximately 3–6 mL via the epidural catheter. FIO2 was set at 1.0 during one-lung ventilation. The VATS procedure was performed using the standard technique (9). The sympathetic ganglia T2 and T3 over the second and third ribs were resected under thoracoscopic vision. The patient’s symptoms began to improve immediately after surgery. One week later, left ETS was performed in the same manner. Postoperative histopathological examination of resected samples confirmed the evidence of ganglia. Compensatory sweating appeared in the trunk after surgery. No major complications, such as Horner’s syndromes or pneumothoraces, developed. A few days after surgery, the epidural administration of local anesthetic was discontinued, as the patient had almost no more postoperative pain. Postoperative findings of thermography remained the same as those at admission. The patient was discharged 1 wk after the second surgery. At her 6-mo follow-up examination, she remained pain-free.
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Discussion
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Erythromelalgia can sometimes be confused with other disorders such, as complex regional pain syndrome (CRPS; formerly defined as reflex sympathetic dystrophy or causalgia) (10). Our patient had no history of recent trauma in the upper extremities, and her symptoms failed to meet the diagnostic criteria of CRPS (11). Because immersion of the affected fingers in ice water relieved the pain, it was easy to discriminate erythromelalgia from the above disorders. Furthermore, typical thermographic findings and histopathology confirmed the diagnosis of erythromelalgia (10,12). Erythromelalgia is classified into primary and secondary forms (10); in this case, primary erythromelalgia was diagnosed by the dermatologist by excluding any secondary cause.
This is the first report concerning the treatment of erythromelalgia by bilateral thoracic sympathectomy using the VATS technique. Over the past decade, ETS has become an established treatment for hand or axillary hyperhidrosis (9,13). However, extensive use of ETS for the treatment of chronic pain has not been well documented compared with its utility for treating hyperhidrosis; therefore, the decision to use ETS in such cases should be made restrictively. To determine whether ETS is indicated, a placebo-controlled diagnostic test using a temporary sympathetic blockade is mandatory. Because our patient showed a good response to this test, as assessed by using a visual analog scale, her pain seemed to be sympathetically maintained. Thus, permanent sympathetic blockade was considered appropriate. Several options were proposed. First, conventional chemical sympathetic blockade guided by roentgenography was considered because it is relatively noninvasive; however, its technical difficulty made us hesitate to choose it. Second, we determined that continuous infusion of epidural local anesthetics seemed to result in only transient pain relief. In our patient, sympathetic blockade with a single dose of local anesthetic was no longer effective beyond 3 days. Third, taking the risk-benefit ratio into consideration, a bilateral open thoracotomy procedure was considered too invasive. Therefore, we chose ETS because it is less invasive and more effective than these other techniques.
A concern may be raised about recurrence. Our patient has had no recurrence of symptoms 6 mo postsurgery. However, this observation time is too short to make conclusions with regard to long-term results. There are poor data on the long-term results of ETS in patients with chronic pain. Two groups reported the long-term recurrence of Raynaud’s syndrome treated with ETS to be approximately 50%–60% (14,15), whereas that of hyperhidrosis is almost 0%. In our patient, a much longer-term follow-up is required to rule out recurrence. Postoperative thermography was not significantly different from preoperative thermography, despite the pain relief and improvement in edema and redness. This suggests that sympathetic blockade is a nonspecific therapy for this disease, although erythromelalgia is considered to be sympathetically related.
We presented early successful results in the treatment of primary erythromelalgia with ETS and believe that this therapy may be effective for continuous pain relief and improvement in edema and redness.
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Footnotes
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Address correspondence and reprint requests to Toshiya Shiga, MD, PhD, Department of Anesthesiology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan. Address e-mail to shiga/anesth@nms.ac.jp.
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References
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Introduction
Case Report
) administration of local anesthetics with those after (
) administration. ***P < 0.001 versus before administration of local anesthetics.
