249 - Dental and Medical Considerations for Patient with Neurofibromatosis Type 1

Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder caused by pathogenic
variants in the NF1 gene with an estimated incidence of 1 in 3,000 births. The condition is
characterized by café-au-lait macules, neurofibromas, skeletal dysplasia, and craniofacial
abnormalities that may significantly impact oral health, growth, and dental management. This case
report describes a 7-year-old male with NF1 presenting with severe facial dysmorphia secondary to
an extensive plexiform neurofibroma, necessitating comprehensive dental treatment in the operating
room that includes dental extractions, restorative procedures, and non-operative management delivered
under general anesthesia. Given the progressive craniofacial involvement of NF1, lifelong
multidisciplinary coordination among neurosurgery, plastic surgery, otolaryngology, and orthodontics is
essential to guide dental and orthodontic care, optimize function and esthetics, and improve overall
quality of life.