RESUMEN
Diprosopus is a congenital anomaly in which partial or complete duplication of craniofacial structures occurs. Because it is rare, the mortality rate is high, and information concerning this anomaly is scarce. This study describes a case of human diprosopus in a 9-year-old male individual, who has severe complications associated with the central nervous, cardiovascular, respiratory, and digestive systems. Since birth, he has been monitored in a specialized hospital environment, where he has undergone several surgeries and multidisciplinary treatments. Regarding the craniofacial aspects, he had agenesis of the corpus callosum, floor of the nasal cavity, and floor of the anterior cranial fossa, in addition to the presence of bone dysplasia, ocular hypertelorism and cleft palate with nasal and oral teratoma. Regarding dental characteristics, the patient has duplication of the maxilla, mandible, tongue, and some teeth. After complementary imaging exams, several supernumerary teeth were found, with some being impacted and in complex regions, with an indication for extraction due to the risks of impaction, irruptive deviation, root resorption, and associated cystic or tumoral lesions. Because of the numerous complications, knowledge, and preparation of the entire team is necessary for the correct management of the case.
Asunto(s)
Anomalías Craneofaciales , Humanos , Masculino , Niño , Agenesia del Cuerpo Calloso , Fisura del Paladar , Diente Supernumerario/diagnóstico por imagen , Diente Supernumerario/cirugía , Teratoma/congénito , Teratoma/cirugía , Teratoma/diagnóstico por imagen , Hipertelorismo , Anomalías Múltiples , Cavidad Nasal/anomalías , Cavidad Nasal/diagnóstico por imagenRESUMEN
The Alagille syndrome (AGLS) is a rare condition, with few studies reported in the literature, especially in the field of dentistry. It consists of a disease involving many systemic problems and specific facial features. The liver and heart are the most intensely affected organs, and depending on the severity, it may be necessary to perform transplants. It is an autosomal dominant disease with a variable expressivity, and its prevalence is 1/100,000 live births. Dental findings are conflicting. Some authors claim that dental anomalies occur only in deciduous dentitions; however, there is evidence that permanent teeth can also be affected, as will be described in this paper, through a case report of a patient diagnosed with AGLS, who sought out the Dentistry service at Pontifical Catholic University of Minas Gerais, complaining of a strong stain in her teeth, severe dental crowding, and a facial appearance of prognathism.