PHACES syndrome and multi-regional odontodysplasia: a case report.
Autor: | Star JM; Department of Orofacial Sciences, University of California San Francisco, San Francisco, CA 94143, USA., Jordan RC; UCSF Dermatopathology & Oral Pathology Service, University of California San Francisco, San Francisco, CA 94143, USA., Stewart RE; Department of Orofacial Sciences, University of California San Francisco, San Francisco, CA 94143, USA. |
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Jazyk: | angličtina |
Zdroj: | The Journal of clinical pediatric dentistry [J Clin Pediatr Dent] 2024 May; Vol. 48 (3), pp. 166-170. Date of Electronic Publication: 2024 May 03. |
DOI: | 10.22514/jocpd.2024.070 |
Abstrakt: | 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. Competing Interests: The authors declare no conflict of interest. (©2024 The Author(s). Published by MRE Press.) |
Databáze: | MEDLINE |
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