500 - Langer-Giedion Syndrome and Associated Manifestations in the Oral Cavity

This presentation is based on the findings of a 4-year-old male who presented to our dental clinic for a comprehensive exam. According to the patient's mother, the patient occasionally shows signs of discomfort in his mouth. Mother confirms a diagnosis of Langer-Giedion Syndrome (LGS) at birth but denies any current medications. Patient has a history of skeletal malformations in the form of congenital exostoses which necessitated surgical intervention. He was evaluated and then categorized into the high-risk category for dental caries as he presented with extensive carious lesions throughout all four quadrants of the mouth. His plan of care included treatment in the operating room (Complete Oral Rehabilitation under General Anesthesia). This case report will evaluate the dental implications often found in LGS patients; previously conducted research suggests that LGS patients face various dental implications including poorer quality of mineralized dental tissue resulting in less acid-resistant enamel and dentin.