Resumen

UMEREZ, Cristina; SOSA, Rafael Darío  y  SIMOSA, Venancio. Síndrome de Ectrodactilia-Displasia Ectodérmica-Hendidura ( Eec. ): Revisión de la Literatura. Reporte de un caso.. Acta odontol. venez [online]. 2002, vol.40, n.2, pp.172-176. ISSN 0001-6365.

Ectrodactyly-Ectodermal dysplasia-Fissure (EEC) is a condition characterized for the presence of this triad. Many times this association is confused with other type of entities being the patient misdiagnosed, reason why are varied differential diagnoses established.The present work is a clinical case of a 5 year old male patient with the presumtive diagnosis of Chondroectodermal Dysplasia which courses with complete bilateral lip palatal fissure associated with hair dyschromia, feet and hands ectrodactyly,post-axial polydactyly, dental alterations and obstruction of both lacrimal ducts. A genetic study, clinical exam, panoramic and torax X rays, and ophtalmological and neurological exams were performed. From these exams we may conclude based on genetic and clinical features that this represents a case of EEC. These results suggest that the diagnoses of this type of clinical-pathologic entities must be done together with the involved specialists in order to establish an adequate treatment plan for the patient. Finally with detail the diagnoses and the dental treatment plan established on the child is described.

Palabras clave : Syndrome; Dysplasia; Fissure.