Dental root abnormalities in four children with PHACE syndrome

• Published in: Pediatric dermatology 2019-07, Vol.36 (4), p.505-508
• Main Authors: Youssef, Molly J., Siegel, Dawn H., Chiu, Yvonne E., Drolet, Beth A., Hodgson, Brian D.
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - diagnosis; Aortic Coarctation - diagnosis; Case reports; Child; Child, Preschool; Children; Dental roots; Dentition; developmental defects; Eye Abnormalities - diagnosis; Female; Hemangioma; hemangiomas/vascular tumors; Humans; Male; Molars; Monitoring, Physiologic - methods; Neurocutaneous Syndromes - diagnosis; Radiography, Panoramic - methods; Rare Diseases; Risk Assessment; Sampling Studies; Sarcoma; Teeth; Tooth Abnormalities - diagnostic imaging; Tooth Root - abnormalities; Tooth Root - diagnostic imaging; Vein & artery diseases
• ISSN: 0736-8046; 1525-1470
• DOI: 10.1111/pde.13818

PHACE(S) syndrome is a condition characterized by posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe. We present four children with PHACE(S) syndrome who have absence of or severe malformation of the roots of their permanent first molars (PFMs). Root abnormalities in the children's molars were bilateral and not restricted to the segments affected by cutaneous hemangioma. The reason for root abnormalities is unknown, but given the rarity of these findings in healthy children, it is likely an additional dental manifestation of PHACE syndrome. The absence of functional roots in the PFMs can result in significant consequences. Therefore, we recommend a panoramic dental radiograph during transitional dentition for children with PHACE syndrome to screen for dental root abnormalities.