311 - STING-Associated Vasculopathy with Onset in Infancy

STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disease. It is a rare disorder involving abnormally increased inflammation throughout the body especially in the skin, blood vessels, and lungs. This presentation discusses the dental management performed for a 7-year-old boy with SAVI. The patient also presented with an acquired saddle nose deformity, failure to thrive (child), midfacial hypoplasia, velopharyngeal insufficiency, sleep-related breathing disorder, and oligoarticular juvenile idiopathic arthritis. Dental findings included multiple congenitally missing teeth, crowding, restricted maxillary arch and alveolar bone loss, and caries. The patient was seen in the operating room with his ENT physician for full-mouth rehabilitation. Unrestorable carious primary teeth were extracted, and a stainless-steel crown was placed on restorable carious molars. The patient is seen every three months for recare appointments, where extra-oral, intra-oral exam, prophy, caries risk assessment, radiographs (6-12 months), topical fluoride application, and anticipatory guidance are performed.

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