Thalamic stimulation as a treatment for primary erythromelalgia: technical case report.

OBJECTIVE AND IMPORTANCE: We report the use of bilateral thalamic stimulation in a case of primary erythromelalgia with immediate and important pain relief for 3 years. CLINICAL PRESENTATION: A 12-year-old boy experiencing primary erythromelalgia had a 4-year history of recurrent attacks of severe burning pain in both feet, accompanied by local reddening, swelling, and heating of the skin. The attacks were triggered by warmth and exercise. The pain was relieved only by elevation and cooling of the lower limbs, which he achieved by immersing his legs in a bucket of ice water, resulting in severe ulceration of the skin. INTERVENTION: Because of the gradual aggravation of the signs and symptoms and resistance of the patient's condition to several medical therapies, the patient received spinal cord stimulation. The implants were removed twice because of recurrent infection. Finally, the patient was treated with bilateral electrical stimulation of the ventral posterolateral thalamic nucleus, which resulted in important pain control until 3 years later. The patient was able to avoid water immersions, and all ulcerations disappeared. CONCLUSION: We conclude that thalamic stimulation was successful in this case of primary erythromelalgia.

Successful thoracoscopic sympathectomy for primary erythromelalgia in the upper extremities.

Erythromelalgia is known as a rare syndrome of unknown etiology, characterized by redness with burning pain, edema associated with increased skin temperature in the upper and/or lower extremities. Various treatments such as drug therapies and sympathetic blockade were reported. We report two cases including a 57-year-old woman and a 64-year-old woman, showing the successful clinical outcome by bilateral thoracoscopic sympathectomy.

[The surgical treatment of erythromelalgia by the destruction of the inlet areas of the posterior spinal nerve roots]. / Khirurgicheskoe lechenie éritromegalii metodom destruktsii vkhodnykh zon zadnikh koreshkov spinnogo mozga.

The paper presents a 13-year-old patient suffering from erythromelalgia in whom arm pain and burning ceased for 3 years after unilateral stereotactic cryodestruction of the thalamic ventrolateral nuclei. Following a relapse, reoperation on the other side turned out to be ineffective. Then the patient underwent destruction of the inlet areas of the spinal dorsal roots by breaking the cervical pain-conducting tracts. Pain and other autonomic manifestations of the disease came to an end. The authors give an anatomic and neurophysiological rationale for surgery.

Neurectomy for palliation of primary erythermalgia.

Ankle nerve crushing followed 6 months later by neurectomy was used for relief of a burning sensation and pain in a 32-year-old woman who had symptoms of unilateral primary erythermalgia. The patient continues to experience considerable improvement of the preceding symptoms and no complications from the insensate foot 1 year after neurectomy, although redness and tenseness of the ankle and the foot have persisted.

Stereotactic surgery of erythromelalgia.

Erythromelalgia is a very rare vascular disease affecting children and is accompanied by severe burning pain in the extremities. The disease is practically incurable. There are no recommendations in the literature concerning the possibility of managing the disease by stereotactic surgery. We operated on 3 children with severe pain due to erythromelalgia. The stereotactic destruction of VPL and CM was performed (in 2 cases on one side and bilaterally in 1). An excellent result with the disappearance of not only pain but all signs of the disease was noted in all cases (follow-up 8, 4.5 years and 6 months).

[Surgical treatment of erythromelalgia]. / Khirurgicheskoe lechenie éritromelalgii.

Two children (an 11-year-old boy and a 15-year-old girl) with erythromelalgia and uncontrollable pain in the lower extremities were treated by stereotaxic destruction of VPL and CM and (in one case) by partial rhizotomy. Pain in the extremities and the clinical manifestations of erythromelalgia disappeared after surgical treatment (follow-up periods of 5 and 2 years, respectively). The mechanism of the therapeutic effect of these operations is still not clear. Similar cases are not described in the literature.