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PHACES syndrome and multi-regional odontodysplasia: a case report

  • Jean Marie Star1,*,
  • Richard C. Jordan2
  • Ray E. Stewart1

1Department of Orofacial Sciences, University of California San Francisco, San Francisco, CA 94143, USA

2UCSF Dermatopathology & Oral Pathology Service, University of California San Francisco, San Francisco, CA 94143, USA

DOI: 10.22514/jocpd.2024.070 Vol.48,Issue 3,May 2024 pp.166-170

Submitted: 24 May 2023 Accepted: 13 July 2023

Published: 03 May 2024

*Corresponding Author(s): Jean Marie Star E-mail: jean.star@ucsf.edu

Abstract

PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional odontodysplasia, commonly referred to as “ghost teeth”, is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child’s dentition as it develops.


Keywords

Odontodysplasia; PHACES syndrome; Dental anomaly


Cite and Share

Jean Marie Star,Richard C. Jordan,Ray E. Stewart. PHACES syndrome and multi-regional odontodysplasia: a case report. Journal of Clinical Pediatric Dentistry. 2024. 48(3);166-170.

References

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