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Characteristics and dental considerations of patients with Sotos syndrome
Woojin Kwon,Ik-Hwan Kim,Chung-Min Kang,Jaeho Lee Asia Association for Disability and Oral Health 2020 International Journal of Disability and Oral Healt Vol.16 No.2
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.
Woojin Kwon,Ik-Hwan Kim,Chung-Min Kang,Je Seon Song Asia Association for Disability and Oral Health 2020 대한장애인치과학회지 Vol.16 No.2
In children with disabilities, malocclulsion prevalence is relatively high. Malocclusion negatively affects these patients and reduces quality of life. However, it is often difficult to perform ideal orthodontic treatment due to lack of cooperation. Therefore, simple and feasible plans should be established for patients with disabilities. This case report is on treatment of anterior crossbite using Frankel appliance in a disabled patient. A 6-year-old patient with intellectual disability visited the Department of Pdeiatric Dentistry, Yonsei University Dental Hospitalwith the chief complaint of anterior crossbite. Upon oral examination, The patient was diagnosed with pseudo class III malocclusion. Considering the patient’s medical history and degree of cooperation, it was decided to use the Frankel appliance III, with a focus on resolving the anterior crossbite. The patient was well adapted to the appliance, and the anterior crossbite resolved two months after the start of treatment. Since then, it has been maintained without relapse. The patient and their caregivers were very satisfied with the treatment results.