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Case Reports
. 2011 Mar 15:2011:bcr0920092286.
doi: 10.1136/bcr.09.2009.2286.

Frey syndrome in neurofibromatosis 1

Laila Farah Ibrahim  1 , Clare Brenner, Joe McMenamin, David Webb
Affiliations
Case Reports

Frey syndrome in neurofibromatosis 1

Laila Farah Ibrahim et al. BMJ Case Rep. .

Abstract

A healthy 18-month-old girl presented with a history of intermittent hemifacial flushing when eating. Her symptom seemed to be exclusively triggered by chewing. Examination revealed cutaneous features of neurofibromatosis type 1 (NF1) and mild facial asymmetry. Imaging confirmed cerebral vacuolisation changes seen in NF1 and a left facial plexiform neurofibroma involving the parotid gland. This is the first reported case of Frey syndrome complicating NF1.

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Conflict of interest statement

Competing interests None.

Figures

Figure 1
Figure 1
(A) The child before eating. (B) Left-sided facial flushing bordered by the left pinna and extended to the midline across the forehead, down to the chin and along the line of the jaw.
Figure 2
Figure 2
Axial T2-weighted sequence (Siemens Magnetom Avanto 1.5T) showing subtle increased signal in the cerebellar white matter a little more obvious on the left than the right side.
Figure 3
Figure 3
Axial T1-weighted fat saturated post -gadolinium image (Siemens Magnetom Avanto 1.5T) showing enlargement and diffuse enhancement of the left parotid gland.
See this image and copyright information in PMC

References

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