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Dental root abnormalities in four children with PHACE syndrome. Pediatr Dermatol 2019 Jul;36(4):505-508



Pubmed ID


Pubmed Central ID




Scopus ID

2-s2.0-85063688224   5 Citations


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.

Author List

Youssef MJ, Siegel DH, Chiu YE, Drolet BA, Hodgson BD


Yvonne E. Chiu MD Vice Chair, Professor in the Dermatology department at Medical College of Wisconsin
Brian Hodgson DDS Assistant Professor in the Developmental Sciences-Dental School department at Marquette University

MESH terms used to index this publication - Major topics in bold

Abnormalities, Multiple
Aortic Coarctation
Child, Preschool
Eye Abnormalities
Monitoring, Physiologic
Neurocutaneous Syndromes
Radiography, Panoramic
Rare Diseases
Risk Assessment
Sampling Studies
Tooth Abnormalities
Tooth Root