Dental Treatment of A Child with Digeorge Syndrome: A Case Report

Abstract

DiGeorge syndrome (DGS) is a rare disorder resulting from the chromosome deletion. It is discovered that deletion in chromosome 22q11.2 are present in most patients with DGS. This disorder is also called ＂chromosome 22q11.2 deletion syndrome.＂The incidence of DGS is around one per 4000 to 5000 newborns. It accompanies variable clinical phenotypes and different abnormalities such as congenital heart defects, craniofacial abnormalities. Embryologic defect involving the third and fourth bronchial pouches and arches leads to thymic aplasia (hypoplasia) and parathyroid gland disorder. These problems result in immunodeficiency. The common oral findings in DGS are velopharyngeal incompetence with or without cleft palate, small mouth, hypotonic musculature of orofacial areas, impaired saliva secretion, enamel hypoplasia and hypomineralization and dental caries, etc. This case report presents a 3-year-old girl with DiGeorge syndrome. She had severe dental caries and without ability to cooperate to receive dental treatment at outpatient department. She received full mouth dental treatment under general anesthesia. Her younger brother was also be diagnosed of DiGeorge syndrome. This report will discuss of the treatment need of dental aspect and compare the differences between this patient and other patients with DGS.