Characteristics and dental considerations of patients with Sotos syndrome

Abstract

"Sotos syndrome, also called cerebral gigantism, is a rare genetic disease characterized by large height, increased bone age, large head size, characteristic facial morphology which is a long and inverted triangle face, and learning disabilities. Common oral findings include premature eruption of teeth, high and arched palate, and rarely prognathism. Also, congenital missing of the second premolar is often observed. A 7-year-old patient with Sotos syndrome visited the Department of Pediatric Dentistry, Yonsei University Dental Hospital with ectopic-eruption of the maxillary right first molar. In periapical radiograph, tooth germ of maxillary right second premolar was not observed, so maxillary right primary second molar was early extracted and the mesial shift of the maxillary right first molar was induced considering the possibility of congenital missing of the second premolar, a characteristic finding of Sotos syndrome. The second patient was a 10-year-old boy, and visited for dental examination. The patient cooperation was good, and in the panoramic radiography, early loss of the deciduous teeth, congenital missing of mandibular left second premolar and delayed root development of mandibular right canine teeth were found. Patients with Sotos syndrome have dental characteristics and dentists should understand these characteristics and strive to provide better care for the patients. ["