Dental Treatment Approach in an Adolescent Patient With Coffin-Siris Syndrome

Abstract

Coffin-Siris Syndrome (CSS) is a rare genetic disease with delayed growth, congenital anomalies, abnormal facial features, and mental retardation. Hypoplasia of the 5th fingers or nail agenesis, hirsutism, coarse facial appearance, thick eyebrows, wide lips and nose, dentofacial anomalies, and delayed eruption of teeth are clinically distinctive features. Specific findings and multidisciplinary approac-hes are important for dental treatments. This case report includes the clinical, oral findings, and dental treatments of the patient with CSS. A 15.5-year-old female patient consulted our clinic with the complaint of tooth sensitivity and missing teeth. The patient's family was informed that the patient has CSS. In clinical examination, coarse face, mental retardation, hypoplastic 5th fingers, wide mouth, and lips, sparse hair, and hirsutism, in the oral examination, poor oral hygiene, dental caries, delayed dentition, lack of upper perma-nent first incisor, gingival hyperplasia, gingivitis, and hypoplasia were seen. Panoramic radiography and tomography revealed that the upper permanent incisor and supernumerary canine teeth were impacted. Under general anesthesia, dental scaling, fillings were made, impacted permanent incisors, canine, and lower left third molars were extracted. After general anesthesia, fluoride application and a fiber-supported Maryland bridge designed from the patient's teeth were applied to the anterior region. Regular controls of the patient continue. After the growth and development, the oral surgery department will be consulted for implant application. In den-tistry, preventive applications, oral hygiene motivation, pharmacological and non-pharmacological treatments are important for CSS patients. After consultation with relevant departments, multidisciplinary approaches and appropriate treatments increase the quality of life.