Tooth Enamel Hypoplasia in PHACE Syndrome
Article first published online: 11 JUN 2014
© 2014 Wiley Periodicals, Inc.
Volume 31, Issue 4, pages 455–458, July/August 2014
How to Cite
Chiu, Y. E., Siegel, D. H., Drolet, B. A. and Hodgson, B. D. (2014), Tooth Enamel Hypoplasia in PHACE Syndrome. Pediatric Dermatology, 31: 455–458. doi: 10.1111/pde.12361
- Issue published online: 14 JUL 2014
- Article first published online: 11 JUN 2014
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.