Frey syndrome‐like developmental dysautonomia in a child with PHACE syndrome. Issue 3 (26th February 2020)

• Record Type: Journal Article
• Authors: Kurian, Shannon R.; Siegfried, Elaine C.
• Is Part Of: Pediatric dermatology. Volume 37:Issue 3(2020)
• Page Start: 568
• Page End: 569
• Publication Date: 2020-02-26
• Subjects: developmental defects -- hemangiomas -- vascular malformation -- vascular tumors; Pediatric dermatology -- Periodicals; Children -- Diseases -- Periodicals; 618.925
• DOI: 10.1111/pde.14125
• Languages: English
• ISSNs: 0736-8046

Abstract

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.