Oral Manifestations in the Cornelia De Lange Syndrome: A Systematic Review of the Dental Literature.
Journal Title: IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) - Year 2017, Vol 16, Issue 5
Abstract
The Cornelia de Lange syndrome (CdLS) is a genetic disorder that affects newborns between 1/10.000 and 1:100.000, and whose diagnosis is established based on medical history and clinical examination, characterized by growth retardation and development, hirsutism, structural abnormalities in the limbs and distinctive facial features. The aim of this paper is to provide a systematic review of the literature on oral manifestations of this rare disease, describing 7 published cases of CdLS in the indexed literature (PubMed/MEDLINE) - the first corresponds to the year 1980 and the last in 2009. Dental problems among these patients are common and include: delayed eruption, malocclusion, micrognathia, dental malpositions, extensive caries or periodontal disease. In any case, the delayed eruption, which may be the cause of diastemas and / or malocclusion is the most common oral finding associated with CdLS - which early diagnosis is key.
Authors and Affiliations
José González- Serrano, Víctor Manuel Paredes- Rodríguez, Carlos González- Serrano, Gema Torrijos Gómez, Jorge A. Cano- Durán, Juan Francisco Peña- Cardelles, Daniel Ortega- Concepción
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