PHACES syndrome and multi-regional odontodysplasia: a case report.
J Clin Pediatr Dent
; 48(3): 166-170, 2024 May.
Article
em En
| MEDLINE
| ID: mdl-38755995
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.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Coartação Aórtica
/
Anormalidades do Olho
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Odontodisplasia
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Síndromes Neurocutâneas
Limite:
Child, preschool
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Humans
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Male
Idioma:
En
Revista:
J Clin Pediatr Dent
Ano de publicação:
2024
Tipo de documento:
Article