Dental root abnormalities in four children with PHACE syndrome

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 th...

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Bibliographic Details
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
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Summary: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.
ISSN:0736-8046
1525-1470
DOI:10.1111/pde.13818