A case of progressive hemifacial atrophy with Pourfour de Petit syndrome which was successfully treated by stellate ganglion block

We herein report a 31-year-old woman with progressive hemifacial atrophy. The atrophy at her left face began about ten years ago. She had been in a traffic accident one year before the onset of her facial atrophy. Neurological examination revealed anisocoria (right < left) and retraction of the left eyelid, which thus suggested the presence of Pourfour de Petit syndrome. The pupillary reaction to both cocaine and tyramine were reduced bilaterally. Thermography of the face showed slightly lower surface temperature on the left side. A facial thermal sweat test was normal. These findings indicated local hyperactivity of the sympathetic nervous system at the Th1-Th2 levels on the left side. A left stellate ganglion block effectively induced an accumulation of the subcutaneous tissue of her face on the left side. This is a very rare case in which local sympathetic hyperactivity is present and has caused progressive hemifacial atrophy.