Chemical lumbar sympathectomy in the treatment of recalcitrant erythromelalgia.

Abstract

OBJECTIVE:

Erythromelalgia is highly disabling and treatment is often very challenging. There have been solitary case reports that it might benefit from sympathectomy. This study sought to evaluate the short-term and long-term efficacy of chemical lumbar sympathectomy (CLS) for treatment of recalcitrant erythromelalgia and try to identify a CLS-responsive subset.

METHODS:

Patients with recalcitrant erythromelalgia were recruited from a tertiary hospital over a 10-year period. L3 to L4 CLS was performed using 5% phenol. The pain intensity score (visual analog scale [VAS] 0-10) was assessed before CLS and at 1 day, 1 week, 3 months, 6 months, 1 year, and 2 years after CLS. A VAS decrease of 90%-100% is defined as complete response, 60%-89% as major partial response. Relapse was defined by a return of a VAS score of 5 or higher. SCN9A gene mutations were screened.

RESULTS:

Thirteen patients were enrolled, with a median age of 15 years. The mean follow-up was 6.2 ± 3.8 years. SCN9A gene mutation was identified in five patients having family histories. The VAS was 8.2 ± 2.0 at baseline; it decreased to 4.9 ± 2.7 at 1 day and 1.9 ± 3.0 at 1 week after CLS. Nine patients (69.2%) achieved complete response at 1 week after CLS, including three patients with SCN9A gene mutation. Among the three complete response patients having the gene mutation, two reverted to major partial response and one relapsed at 2 years after CLS. Among the six complete response patients without mutation, five maintained complete response and one relapsed. Among the four patients who did not achieve complete response, one patient died at 3.5 months and one patient had an amputation performed at 4 months after CLS.

CONCLUSIONS:

CLS provides a valid option for the treatment of recalcitrant erythromelalgia. It takes about 1 week to achieve full efficacy. Relapse may occur, especially in patients with an SCN9A gene mutation.

• Authors: Wen-Hui Wang, Long Zhang, Guo-Xiang Dong, Ting-Ting Sun, Zhi-Miao Lin, Yong Yang, Xuan Li
• Journal: Journal of vascular surgery (J Vasc Surg) Vol. 68 Issue 6 Pg. 1897-1905 (12 2018) ISSN: 1097-6809 [Electronic] United States
• PMID: 30126782 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

Chemical References

• NAV1.7 Voltage-Gated Sodium Channel
• SCN9A protein, human

Topics

• Adolescent
• Amputation, Surgical
• Child
• DNA Mutational Analysis
• Erythromelalgia (diagnosis, genetics, physiopathology, therapy)
• Female
• Humans
• Lumbar Vertebrae (innervation)
• Male
• Middle Aged
• Mutation
• NAV1.7 Voltage-Gated Sodium Channel (genetics)
• Pain Measurement
• Prospective Studies
• Recurrence
• Remission Induction
• Sympathectomy, Chemical (adverse effects, methods)
• Time Factors
• Treatment Outcome
• Young Adult