IADR Abstract Archives
Clinical Characteristics and Dental Treatment of Cornelia De Lange Syndrome : a Rare Case Report and Review of the Litterature
Objectives: The Cornelia de Lange syndrome is a rare, multiple congenital defect that affects newborns and whose diagnosis is established based on medical history, clinical examination and genetic testing.
The aim of this work is to present a rare clinical report of The Cornelia de Lange syndrome in a newborn and to adress its frequent orofacial manifestations in the dental litterature.
Methods: A case of a 2 months old boy who came to our attention at the pediatric dentistry department in la rabta Hospital for incomplete cleft palate. We managed this case in collaboration with the paediatric department.
We also conducted a research in the following electronic databases: PubMed, The Cochrane Library and science direct, using the following keywords : Cornelia de Lange syndrome AND ((dental OR oral) AND (manifestations OR findings)) with a year of publication limit set to 2011, to compare dental findings in our patient with litterature
Results: The most representative dental features found in the literature are delayed eruption, presence of diastemas and malocclusions.
The clinical investigations of our patient showed that he met the diagnosis criteria for this disease which are : prenatal onset growth retardation, bushy eyebrows and synophrys, long culy eyelashes, short nose, anteverted nostrils, prominent area between upper lip and nose, small or square chin and thin lips with downturned corners.
The oral manifestations of this patient were a cleft palate with a high arched palate. Accordingly, our treatment plan consisted in a feeding obturator appliance , a palatoplasty programmed in the pediatric department and routine six months check ups. We overcame the feeding problem with improvement of the infant's weight gain after 3 weeks follow up.
Conclusions: The assessment of orofacial manifestations in Children with Cornelia de Lange syndrome indicates that they are not exclusive of the syndrome, since they can also be present in other diseases. It is very important to know CdLS and its facial and oral characteristics in order to obtain a correct diagnosis and apply an appropriate approach treatment based on a multidsciplinary strategy.
The aim of this work is to present a rare clinical report of The Cornelia de Lange syndrome in a newborn and to adress its frequent orofacial manifestations in the dental litterature.
Methods: A case of a 2 months old boy who came to our attention at the pediatric dentistry department in la rabta Hospital for incomplete cleft palate. We managed this case in collaboration with the paediatric department.
We also conducted a research in the following electronic databases: PubMed, The Cochrane Library and science direct, using the following keywords : Cornelia de Lange syndrome AND ((dental OR oral) AND (manifestations OR findings)) with a year of publication limit set to 2011, to compare dental findings in our patient with litterature
Results: The most representative dental features found in the literature are delayed eruption, presence of diastemas and malocclusions.
The clinical investigations of our patient showed that he met the diagnosis criteria for this disease which are : prenatal onset growth retardation, bushy eyebrows and synophrys, long culy eyelashes, short nose, anteverted nostrils, prominent area between upper lip and nose, small or square chin and thin lips with downturned corners.
The oral manifestations of this patient were a cleft palate with a high arched palate. Accordingly, our treatment plan consisted in a feeding obturator appliance , a palatoplasty programmed in the pediatric department and routine six months check ups. We overcame the feeding problem with improvement of the infant's weight gain after 3 weeks follow up.
Conclusions: The assessment of orofacial manifestations in Children with Cornelia de Lange syndrome indicates that they are not exclusive of the syndrome, since they can also be present in other diseases. It is very important to know CdLS and its facial and oral characteristics in order to obtain a correct diagnosis and apply an appropriate approach treatment based on a multidsciplinary strategy.