Abstract
Modern Research in Dentistry Cornelia De Lange Syndrome and Dental Treatment: A Case Report Chaza Kouchaji* and Maram Mohamad Samir al Saitary Pediatric dental department, Faculty of dental medicine, Damascus university, Syria *Corresponding author:Chaza Kouchaji, Pediatric dental department, Faculty of dental medicine, Damascus university, Syria Submission: August 08, 2020;Published: March 16, 2021 DOI: 10.31031/MRD.2021.06.000631 ISSN:2637-7764Volume6 Issue2
Highlights
HistoryPreviously, in 1849, the anatomist Willem Vrolik (1801-1863) reported a case as an extreme example of oligodactyly, and the German doctor Brachmann published, in 1916, a case of symmetric monodactyly, antecubital webbing, dwarfism, cervical ribs, and hirsutism
It is a genetic disorder that affects many organs, leading to various clinical presentations. In this case presentation we will report a case of Cornelia de Lange Syndrome (CdLS) phenotype, along with extra and intra oral finding and radiographic, dental management and its follow up
This syndrome consists of multiple skeletal deformities with moderate to severe mental retardation associated with facial dysmorphism
Summary
In 1849, the anatomist Willem Vrolik (1801-1863) reported a case as an extreme example of oligodactyly, and the German doctor Brachmann published, in 1916, a case of symmetric monodactyly, antecubital webbing, dwarfism, cervical ribs, and hirsutism. A Dutch pediatrician after which this syndrome is occasionally named (Brachmann-De Lange Syndrome). Cornelia De Lange (1871-1950) who reported her first two cases in 1933 (Figure 1) and presented an account of this syndrome in 1941 in the meeting of the Neurology Society of Amsterdam (2). Frequency This syndrome affects 1 in 10000 to 30000 newborn [2]. Cornelia de Lange Syndrome (CdLS) has five distinctive genotypes numbered from 1 to 5 with CdLS 1 is the most common which contributes 50-60% of cases. The affected genes (NIPBL, SMC1A, SMC3, RAD21, and HDAC8) all code proteins which are part of the cohesion pathways
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