IADR Abstract Archives
Oral and Maxillofacial Manifestations of DiGeorge Syndrome: A Case Report
Objectives: DiGeorge syndrome (DGS) is the unexplained micro-deletion of chromosome 22q11.2 during the 4th to 6th week of formation. This results in the abnormal development of the third (III) and fourth (IV) pharyngeal pouches. The oral cavity involvement has been highly overlooked. The purpose of this case report was to document the oral and maxillofacial manifestations of a DGS case and to raise awareness among both pediatric and general dental practitioners about the dental management of this genetic disorder.
Methods: A 10-year-old boy diagnosed with DGS was referred to Kuwait University Dental Clinics for dental treatment. He had history of severe hypocalcaemia, and surgical correction for Tetralogy of Fallot. The patient’s past dental history revealed multiple teeth extractions. The following investigations were conducted: general and dental examinations, facial and space analysis, full mouth series radiographs, lateral cephalometric radiograph, cone-beam CT study, and a saliva test.
Results: Craniofacial anomalies were present. Patient had severe generalized gingivitis, hypomineralized teeth, active carious lesions, hypodontia, root dilacerations, spacing (+15mm in the mandible and +8.4mm in the maxilla), bimaxillary prognathism and proclination (Max1 to SN =128, Man1 to MP = 127). Radiographically, there was evidence of generalized osteopenia with large marrows spaces and scanty trabeculation, loss of cortical definition in the craniofacial area, and both mid-face and gonial angle deficiency. The mandible appears hypoplastic in both vertical and horizontal dimensions with an increased in thickness of the soft palate. The skull appears slightly dolichocephalic. Saliva test revealed alkaline pH of unstimulated saliva with high viscosity. Dental management included professional fluoride application, extraction of unrestorable carious primary teeth, successful restoration of permanent teeth with stainless steel crowns and composite resin. Teeth sensitivity was present which was managed by supplemental intraligamentary injections and interim glass ionomer fillings.
Conclusions: Early prevention and intervention are crucial for this patient group to prevent tooth decay and early tooth loss. Education of the patient and their caregivers on oral hygiene measures and dental care is extremely important with DGS patients.
Methods: A 10-year-old boy diagnosed with DGS was referred to Kuwait University Dental Clinics for dental treatment. He had history of severe hypocalcaemia, and surgical correction for Tetralogy of Fallot. The patient’s past dental history revealed multiple teeth extractions. The following investigations were conducted: general and dental examinations, facial and space analysis, full mouth series radiographs, lateral cephalometric radiograph, cone-beam CT study, and a saliva test.
Results: Craniofacial anomalies were present. Patient had severe generalized gingivitis, hypomineralized teeth, active carious lesions, hypodontia, root dilacerations, spacing (+15mm in the mandible and +8.4mm in the maxilla), bimaxillary prognathism and proclination (Max1 to SN =128, Man1 to MP = 127). Radiographically, there was evidence of generalized osteopenia with large marrows spaces and scanty trabeculation, loss of cortical definition in the craniofacial area, and both mid-face and gonial angle deficiency. The mandible appears hypoplastic in both vertical and horizontal dimensions with an increased in thickness of the soft palate. The skull appears slightly dolichocephalic. Saliva test revealed alkaline pH of unstimulated saliva with high viscosity. Dental management included professional fluoride application, extraction of unrestorable carious primary teeth, successful restoration of permanent teeth with stainless steel crowns and composite resin. Teeth sensitivity was present which was managed by supplemental intraligamentary injections and interim glass ionomer fillings.
Conclusions: Early prevention and intervention are crucial for this patient group to prevent tooth decay and early tooth loss. Education of the patient and their caregivers on oral hygiene measures and dental care is extremely important with DGS patients.