Systemic sclerosis: bilateral improvement of Raynaud's phenomenon with unilateral digital sympathectomy.

OBJECTIVE

To demonstrate that unilateral digital sympathectomy, in patients with Raynaud's phenomenon (RP) and systemic sclerosis (SSc), may result in bilateral resolution of RP and digital ulcerations.

METHODS

We report a case of SSc and RP that had bilateral benefits from unilateral digital sympathectomy. A computer-assisted Medline/PubMed search of the medical literature was performed for 1960 through June 2009 using the keywords sympathectomy, Raynaud's phenomenon, systemic sclerosis, CREST, and digital ulcers. These searches were also combined with text words unilateral, ipsilateral, bilateral, digital sympathectomy, selective sympathectomy, autonomic nervous system, hyperhidrosis, etiology, pathogenesis, hypothesis, and treatment. Only pertinent literature, primarily in the English language, was included.

RESULTS

The majority of patients with SSc have RP and many suffer from digital ulcerations. Medical and behavioral management may have limited benefit and surgical intervention can be considered in recalcitrant cases, although efficacy data are sparse. We describe a man with limited SSc who underwent unilateral digital sympathectomy but manifested bilateral benefit. To our knowledge, this is the first published report of contralateral response with this procedure. The patient ultimately demonstrated these digital benefits when stressed with extreme cold and hypoxia while mountaineering. Despite the onset of high-altitude sickness and cerebral edema, his fingers remained unaffected while other mountaineers sustained severe frostbite or died of hypothermia.

CONCLUSIONS

Selective unilateral sympathectomy in SSc, for RP with digital ulcerations, can result in bilateral benefits despite intense challenge with cold and hypoxia.