With a brief glance at the image and the title, what is the spot diagnosis?
A 2-year-old boy was referred to the otolaryngology clinic with complaints of recurrent right cheek’s redness/flushing during eating for the past 18 months. His mother first noticed this when he started weaning at the age of 6 months (Panel B). The redness is not associated with pain, itching, sweating, fever, or any other symptoms. The boy was born at full term, via forceps-assisted vaginal delivery.
Panel C of the attached image shows the child after eating at 18 months of age, whereas panel D shows the picture of the child when he is not eating. Note that the flushing is only during eating.
Past surgical and medical history were unremarkable. There were no notable allergies to food or nuts, and the skin allergen testing was also negative.
To clinically confirm the suspected diagnosis, the child was given strawberries to eat in the clinic, the flushing reappeared.
A diagnosis of Frey’s syndrome was made.
Frey’s syndrome is also known as ‘Auriculotemporal nerve syndrome.’ It is damage to the parasympathetic nerve fibers within the parotid gland followed by aberrant reinnervation manifested by gustatory flushing and/or sweating.
In simpler words, it is aberrant reinnervation of the parasympathetic and sympathetic nerves after any injury/trauma to the nerves. To make it even simpler, the responses, such as flushing and sweating, previously controlled by sympathetic nerves, now respond to parasympathetic stimulation such as eating (mastication and swallowing).
This aberrant regeneration of the nerves is responsible for flushing whenever the boy eats.
Damage to the nerve fibers can occur during parotid gland surgery, facial trauma, infections such as herpes zoster, and forceps-assisted vaginal delivery.
Diagnosis of Frey’s syndrome is clinical; usually, no fancy tests are required!
Frey’s syndrome usually doesn’t require treatment. The child in the discussion here was provided no specific treatment, his condition persisted even at the age of three.
There is no recommended treatment for it. However, spontaneous remission has been reported in children as they grew older. If the patient’s symptoms are excessive and uncomfortable, then medical treatments, such as topical anticholinergic ointments (scopolamine, glycopyrrolate), topical αlpha agonist (clonidine), and botulinum toxin injections can be offered to the patient. None of these medical management approaches is a definitive cure. These may subside or dampen the symptoms for a temporary period.
Is Frey’s syndrome dangerous or fatal?
The answer is no!
Undeniably, it is uncomfortable and embarrassing for the affected individual, which may affect the suffer’s self-confidence in the long run, but it has no known associated medical adversities.
References
Alexander Smith, M. C. (2020, April 09). Frey’s Syndrome. Retrieved from The New England Journal of Medicine: https://www.nejm.org/doi/full/10.1056/NEJMicm1809117
Caulley, L., & Hong, P. (2013). Pediatric auriculotemporal nerve (Frey) syndrome. CMAJ : Canadian Medical Association journal = journal de l’Association medicale canadienne,185(6), 504.
