Frey syndrome-like developmental dysautonomia in a child with PHACE syndrome.

Frey syndrome-like developmental dysautonomia in a child with PHACE syndrome.:

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Frey syndrome-like developmental dysautonomia in a child with PHACE syndrome.

Pediatr Dermatol. 2020 Feb 26;:

Authors: Kurian SR, Siegfried EC

Abstract

PHACE syndrome classically presents with a large, segmental facial infantile hemangioma (IH) associated with structural and vascular abnormalities involving the head and neck, heart, and eyes. We evaluated an infant who presented with ptosis caused by a clinically subtle, deep right-sided periorbital IH identified on MRI that also incidentally revealed hypoplasia of the right common carotid and right internal carotid arteries, supporting a diagnosis of PHACE syndrome. She subsequently developed acute-onset, transient right-sided facial erythema without anisocoria, triggered by feeding and emotional stress. We believe this represents a Frey syndrome-like developmental dysautonomia, previously unreported in association with PHACE syndrome, suggesting an associated defect in neurovascular embryogenesis.

PMID: 32101632 [PubMed - as supplied by publisher]