355 - Dental And Systemic Findings In Williams–Beuren Syndrome

Abstract: Dental And Systemic Findings In Williams–Beuren Syndrome
Elbrolossey H
Staten Island University Hospital, Staten Island, NY

Introduction: Williams–Beuren syndrome is a rare multisystem genetic disorder caused by a microdeletion of chromosome 7q11.23. The condition is characterized by cardiovascular anomalies, neurodevelopmental impairment, endocrine dysfunction, and distinctive orofacial features. Oral manifestations may include enamel hypoplasia, increased caries risk, and congenitally missing teeth, which complicate dental treatment planning.

Case Report: This presentation discusses a 7-year-old male with developmental delay, autism spectrum disorder, hypothyroidism, and a bicuspid aortic valve. Genetic test confirmed WBS. Dental examination revealed poor oral hygiene, generalized enamel hypoplasia, multiple severely carious and fractured primary teeth, and several congenitally missing permanent teeth. Due to significant behavioral challenges and intraoral pain, the patient underwent comprehensive oral rehabilitation under general anesthesia. Radiographs revealed multiple missing permanent teeth. Primary teeth #A, B, C, D, G, H, I, J, K, and L were extracted due to extensive caries, and occlusal composite restorations were placed on permanent teeth #3, 14, 19, and 30. Nutritional counseling and oral hygiene instructions were provided to the caregiver.

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