Dental and Dentofacial Findings in a Child With CHOPS Syndrome Managed Under General Anesthesia.

BACKGROUND

CHOPS syndrome is an extremely rare genetic disorder characterized by cognitive impairment, coarse facial features, heart defects, obesity, pulmonary involvement, and skeletal dysplasia. Although systemic features have been described, dental and dentofacial manifestations have not been previously reported.

CASE PRESENTATION: An 11-year-old male diagnosed with CHOPS syndrome was referred with multiple dental caries. Extraoral examination revealed midface hypoplasia, thick eyebrows, broad forehead, large ears, and coarse facial appearance. Intraoral findings included a narrow and high palatal vault, enamel hypoplasia, and multiple carious lesions. Panoramic radiography demonstrated reduced bone mineralization and agenesis of all second premolars and Tooth 47. Due to limited cooperation associated with cognitive impairment, comprehensive dental treatment was performed under general anesthesia.

CONCLUSIONS

This report highlights previously unrecognized oral and craniofacial features of CHOPS syndrome and emphasizes the importance of multidisciplinary planning and safe anesthetic management in affected children.