Dental Anomalies in 1p36 Deletion Syndrome: A Case Report.

This report describes a nine-year, five-month-old Saudi girl with 1p36 deletion syndrome (1p36DS) referred for dental evaluation due to esthetic concerns. Clinical and radiographic assessment revealed multiple carious lesions, poor oral hygiene, dens invaginatus in maxillary incisors, infraocclusion of a primary molar, and, notably, agenesis of all primary and permanent canines except for one retained mandibular primary canine. Additionally, two supernumerary maxillary incisors (mesiodens) were identified, causing severe rotation of adjacent teeth, a combination of dental anomalies not previously documented in 1p36DS. A comprehensive treatment plan was executed, encompassing preventive measures, restorative care, surgical extraction of mesiodens, and interceptive orthodontic alignment using a 2×4 fixed appliance and transpalatal arch. These interventions led to improved esthetics, dental function, and psychosocial confidence. The management of 1p36DS patients requires a multidisciplinary approach due to the conjunction of craniofacial and systemic anomalies. Pediatric dentists play a crucial role in early detection, preventive care, and timely interventions, all of which contribute to enhanced function and quality of life. Routine panoramic imaging and vigilance for atypical dental patterns are critical for guiding genetic referrals and comprehensive care. This case broadens the known phenotypic spectrum of 1p36DS by documenting a novel dental presentation involving concurrent agenesis of both primary and permanent canines with supernumerary maxillary incisors. Early recognition of such anomalies enables timely diagnosis, personalized management, and multidisciplinary collaboration, emphasizing the vital role of pediatric dentists in the holistic care of patients with rare chromosomal disorders.