RESUMO
Saethre-Chotzen syndrome is characterized primarily by facial and digital anomalies. This case report describes multiple dental anomalies in a patient with Saethre-Chotzen, not reported in previous descriptions of the syndrome. These features are teeth with broad, bulbous crowns, thin, narrow tapering roots, and diffuse pulp stones in the pulp chambers of all posterior teeth. These dental findings, along with being associated with the syndrome, may influence dental care for these patients.
Assuntos
Acrocefalossindactilia/complicações , Anormalidades Dentárias/etiologia , Adolescente , Coroas , Humanos , Masculino , Radiografia , Tratamento do Canal Radicular , Anormalidades Dentárias/diagnóstico por imagem , Anormalidades Dentárias/reabilitaçãoRESUMO
Oral-facial-digital syndrome (OFDS) type VI (Váradi syndrome) is an autosomal recessive trait of orofacial anomalies, cerebellar dysgenesis, and polysyndactyly. Developmental anomalies of the posterior fossa, including cerebellar hypoplasia and variants of the Dandy-Walker complex, are the most common central nervous system malformations reported in patients with this syndrome. We report hypothalamic hamartoma, supernumerary maxillary incisor, and precocious puberty in a boy with OFDS type VI. We propose that hypothalamic hamartoma is an occasional manifestation of OFDS type VI.
Assuntos
Hamartoma/genética , Doenças Hipotalâmicas/genética , Síndromes Orofaciodigitais/genética , Puberdade Precoce/etiologia , Genes Recessivos , Hamartoma/complicações , Humanos , Doenças Hipotalâmicas/complicações , Recém-Nascido , MasculinoRESUMO
Chemoradiation therapy used on pediatric oncology patients often causes dental developmental anomalies that affect future dental care. Defects noted include tooth and root agenesis, root thinning and shortening, and localized enamel defects. Histologically, these defects appear as osteoid-like niches in the developing dentin which alter the overlying enamel. Odontogenic cell sensitivity is dependent upon the position on the cell cycle and the mitotic activity at the time of chemoradiation therapy. Knowledge of the stage of dental development at the time of oncology treatment and the type of therapy allows the clinician to predict dental effects of the chemoradiation. Representative cases illustrate the clinical manifestations of chemoradiation on the developing dentition.