Auriculotemporal Frey syndrome not associated with surgery or diabetes: systematic review

Céline Betti,Barbara Goeggel Simonetti,Gabriel Bronz,Mario G Bianchetti,Gian P Ramelli,Sebastiano A G Lava,Marcel M Bergmann,Gregorio P Milani

doi:10.1007/s00431-022-04415-w

Abstract

Patients who undergo salivary gland, neck, or facelift surgery or suffer from diabetes mellitus often develop Frey syndrome (also known as auriculotemporal syndrome or gustatory sweating). Frey syndrome has been occasionally reported to occur in subjects without history of surgery or diabetes but this variant of Frey syndrome has not been systematically investigated. We searched for original articles of Frey syndrome unrelated to surgery or diabetes without date and language restriction. Article selection and data extraction were performed in duplicate. Our systematic review included 76 reports describing 121 individual cases (67 males and 54 females) of Frey syndrome not associated with surgery or diabetes. The age at onset of symptoms was ≤ 18 years in 113 (93%) cases. The time to diagnosis was 12 months or more in 55 (45%) cases. On the other hand, an allergy evaluation was performed in half of the cases. A possible cause for Frey syndrome was detected in 85 (70%) cases, most frequently history of forceps birth (N = 63; 52%). The majority of the remaining 22 cases occurred after a blunt face trauma, following an auriculotemporal nerve neuritis or in association with a neurocutaneous syndrome. The cause underlying Frey syndrome was unknown in 36 cases. Conclusion: Frey syndrome not associated with surgery or diabetes almost exclusively affects subjects in pediatric age and is uncommon and underrecognized. Most cases occur after forceps birth. There is a need to expand awareness of this pseudo-allergic reaction among pediatricians and allergists.What is Known:• Pre-auricular reddening, sweating, and warmth in response to mastication or a salivary stimulus characterize Frey syndrome.• It usually occurs after salivary gland surgery and in diabetes.What is New:• In children, Frey syndrome is rare, and most cases occur after a forceps-assisted birth.• In childhood, this condition is often erroneously attributed to food allergy.

Highlights

Frey syndrome not associated with surgery or diabetes almost exclusively affects subjects in pediatric age and is uncommon and underrecognized
The aims of this systematic review of the literature were to document demographics, presentation, diagnostic difficulties, and causes of Frey syndrome not associated with surgery or diabetes
We retained 76 reports describing 121 individual cases of Frey syndrome not associated with surgery or diabetes [5, 8–82]

Summary

Introduction

Mastication or a salivary stimulus may be followed by rapid reddening of the pre-auricular region, normally associated with local warmth, sweating, and general discomfort, and occasionally with shedding of tears or watery otorrhea [1]. Extended author information available on the last page of the article phenomenon was first described by M. The better characterization was made in 1923 by the Polish Jewish neurologist Łucja Frey-Gottesman (1889–1942). The condition is nowadays referred to as auriculotemporal (nerve) syndrome, gustatory sweating, or Frey syndrome [2, 3]. It typically and rather commonly occurs 6 to 18 months after salivary gland, neck, or facelift surgery [1], and in diabetes mellitus [4]

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