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Tooth Enamel Hypoplasia in PHACE Syndrome
Pediatric Dermatology
  • Yvonne E. Chiu, Medical College of Wisconsin
  • Dawn H. Siegel, Medical College of Wisconsin
  • Beth A. Drolet, Medical College of Wisconsin
  • Brian D. Hodgson, Marquette University
Document Type
Format of Original
4 p.
Publication Date
Individuals with PHACE syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe syndrome) have reported dental abnormalities to their healthcare providers and in online forums, but dental involvement has not been comprehensively studied. A study was conducted at the third PHACE Family Conference, held in Milwaukee, Wisconsin, in July 2012. A pediatric dentist examined subjects at enrollment. Eighteen subjects were enrolled. The median age was 4.2 years (range 9 mos–9 yrs; 14 girls, 4 boys). Eleven of 18 patients had intraoral hemangiomas and five of these (50%) had hypomature enamel hypoplasia. None of the seven patients without intraoral hemangiomas had enamel hypoplasia. No other dental abnormalities were seen. Enamel hypoplasia may be a feature of PHACE syndrome when an intraoral hemangioma is present. Enamel hypoplasia increases the risk of caries, and clinicians should refer children with PHACE syndrome to a pediatric dentist by 1 year of age.

Pediatric Dermatology, Vol. 31, No. 4 (July/August 2014): 455-458. DOI.

Citation Information
Yvonne E. Chiu, Dawn H. Siegel, Beth A. Drolet and Brian D. Hodgson. "Tooth Enamel Hypoplasia in PHACE Syndrome" Pediatric Dermatology (2014) ISSN: 0736-8046
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