Comprehensive Oral Diagnosis and Management for Women with Turner Syndrome.

Turner Syndrome (TS) is a rare genetic disorder that affects females when one of the X chromosomes is partially or completely missing. Due to high genetic and phenotypic variability, TS diagnosis is challenging and is often delayed until adolescence, resulting in poor clinical management. Numerous oral, dental and craniofacial anomalies have been associated with TS, yet a comprehensive description is still lacking. This study addresses this gap through a detailed analysis of oral health and craniofacial characteristics in a cohort of 15 females with TS and their first-degree relatives. Subjects with TS ranged from 3 to 48 years old, none showed evidence of periodontal disease and only the youngest was in mixed dentition. Using the Multifunction System, we identified an aggregation of multiple signs and symptoms in each TS subject, including tooth anomalies (supernumerary molars, agenesis, microdontia, enamel defects, alterations in eruption patterns -advanced and delayed for chronological age-, crowding, rotations and transpositions), malocclusion (class II/1 and II/2) and Class II facial profile, while relatives exhibited fewer manifestations. The early detection of these signs and symptoms is crucial for appropriate referral and the optimal clinical management of TS, especially during the critical period of 9 to 10 years when congenital dental anomalies appear. The use of an established taxonomy to describe these phenotypic features is essential for early detection. Multidisciplinary teams are required to ensure holistic care management in rare diseases like TS.

Cornelia de Lange syndrome: a case report.

Cornelia de Lange is a genetic syndrome which affects between 1/10.000 and 1/60.000 neonates, but its genetic bases are still not clear. Its principal clinical characteristics are the delay in growth and development, hirsute, structural anomalies in the limbs and distinctive facial characteristic. Dental problems are frequent and include: ogival palate, micrognathia, dental malalignment, delayed teething, microdontic teeth, periodontal disease and dental erosion produced by gastric reflux. Discussed is the case of a 29 year old patient affected by the syndrome in question, which presents the principal clinical characteristics. The patient's general state of health is acceptable, without cardiac or respiratory alterations. The intraoral exploration shows policaries, periodontal disease, persistence of the temporal teeth and ectopic molars. After completing the necessary pre-operatory preparations, the entire odontological treatment was carried out under general aesthesia, due to the patient's total lack of collaboration.

Cornelia de Lange syndrome: A case report

Cornelia de Lange is a genetic syndrome which affects between 1/10.000 and 1/60.000 neonates, but its genetic bases are still not clear. Its principal clinical characteristics are the delay in growth and development, hirsute, structural anomalies in the limbs and distinctive facial characteristic. Dental problems are frequent and include: ogival palate, micrognathia, dental malalignment, delayed teething, microdontic teeth, periodontal disease and dental erosion produced by gastric reflux. Discussed is the case of a 29 year old patient affected by the syndrome in question, which presents the principal clinical characteristics. The patient’s general state of health is acceptable, without cardiac or respiratory alterations. The intraoral exploration shows policaries, periodontal disease, persistence of the temporal teeth and ectopic molars. After completing the necessary pre-operatory preparations, the entire odontological treatment was carried out under general aesthesia, due to the patient’s total lack of collaboration

El síndrome de Cornelia de Lange es un síndrome genético que afecta entre 1/10.000 y 1/60.000 neonatos, y cuyas bases genéticas todavía no están claras. Sus características clínicas principales son el retraso en el crecimiento y en el desarrollo, hirsutismo, anomalías estructurales en miembros y unas características faciales distintivas. Los problemas dentales son frecuentes e incluyen: Paladar ojival, micrognátia, malposición dental, retraso en la erupción, microdoncia, enfermedad periodontal y erosión dental producida por el reflujo gástrico. Se describe el caso de una paciente de 29 años de edad afecta por el síndrome en cuestión, que presenta las principales características clínicas. El estado general de la paciente es aceptable, sin alteraciones cardíacas ni respiratorias. La exploración intraoral muestra policaries, enfermedad peridontal,pesistencia de dientes temporales y cordales ectópicos. Después de realizar las pruebas preoperatorios necesarias, se realiza el tratamiento odontológico completo bajo anestesia general, debido a la nula colaboración de la paciente

Odontoma mandibulart con inclusión de incisivo y canino permanentes / Mandibular odontoma with inclusion of permanent incisor and canine. Clinical case

Los odontomas son anomalías del desarrollo resultado del crecimiento y diferenciación epitelial de células mesenquimales. Estas células forman cantidades variables de esmalte, dentida y tejido pulpar. Algunos autores afirman que actualmente los dontomas son los tumores más frecuentes de los maxilares y representan entre un 22 por ciento y un 67 por ciento de todos los tumores odontogénicos de los maxilares. Sin embargo, la presencia de un dontoma en el sector anterior de la mandíbula es poco frecuente. En este artículo presentamos un caso de impactación de un incisivo con un quiste folicular y la inclusión del canino del lado afectado por la presencia de un odotoma mandibular

Odontoma mandibulart con inclusión de incisivo y canino permanentes / Mandibular odontoma with inclusion of permanent incisor and canine. Clinical case

Los odontomas son anomalías del desarrollo resultado del crecimiento y diferenciación epitelial de células mesenquimales. Estas células forman cantidades variables de esmalte, dentida y tejido pulpar. Algunos autores afirman que actualmente los dontomas son los tumores más frecuentes de los maxilares y representan entre un 22 por ciento y un 67 por ciento de todos los tumores odontogénicos de los maxilares. Sin embargo, la presencia de un dontoma en el sector anterior de la mandíbula es poco frecuente. En este artículo presentamos un caso de impactación de un incisivo con un quiste folicular y la inclusión del canino del lado afectado por la presencia de un odotoma mandibular(AU)