Manejo Odontoestomatológico del Síndrome de Costello:a propósito de un caso clínico

Abstract

Costello Syndrome (CS) is a congenital disease of genetic origin caused by heterozygous germline HRAS mutation in the chromosome 11p15.5 in the 85% of the cases, which is characterized by slow developmental growth (both physical and mental), coarse face, stomatognathic system alterations, and cardiac problems. Furthermore, this disease also tends to result in the development of benign and malignant tumors, much of these on the oral and perioral area. Cases that are negative for HRAS mutations may be explained by the presence of locus heterogeneity, like the presence of other genes in different chromosomal regions, which is cause of the alterations. This article describes a CS clinical case, looking to increase the number of reported cases in the literature of this complex syndrome. The article will focus on the special considerations needed to be taken before starting the oral treatment.