Dental Management of a Patient with Congenital heart defects, dysmorphic facial features and intellectual developmental disorders (CHDFIDD) under General Anesthesia: A Case Report
Chun Se-Jin, 김현태, 송지수, 신터전, 현홍근, 김정욱, 장기택, 김영재,
소속 상세정보
( Chun Se-Jin ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김현태 ( Kim Hyun-Tae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
송지수 ( Song Ji-Soo ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
신터전 ( Shin Teo-Jeon ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
현홍근 ( Hyun Hong-Keun ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김정욱 ( Kim Jung-Wook ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
장기택 ( Jang Ki-Taeg ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김영재 ( Kim Young-Jae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
KMID : 1144020220180010025
Abstract
Congenital heart defects, dysmorphic facial features and intellectual developmental disorders (CHDFIDD) is an autosomal dominant hereditary disease caused by mutation in cyclin-dependent kinase 13 (CDK13) gene. It was first discovered in 2016 by exome sequencing of patients with congenital heart defects and now, more than 50 patients have been diagnosed for CHDFIDD since the initial discovery of the disease and the number is increasing every year. Dental manifestation of wide-spaced or peg-shaped teeth are reported in patients with CHDFIDD. This case report describes the dental treatment of a 5-year-girl with CHDFIDD under general anesthesia. Through clinical and radiographic examination, interproximal caries lesion on primary molars and severe attrition on primary incisors with deep bite were detected. Fibrous gingival hyperplasia on buccal interproximal area between lower right lateral incisor and canine was also observed. Considering the extensive treatment need and patient’s uncooperative behavior, we planned to do dental treatment under general anesthesia. Conservative resin restoration, stainless steel crown restoration and excisional biopsy were done. Dental treatment of patients with CHDFIDD is challenging due to poor cooperation. Preventive dental care including oral hygiene instruction, plaque control, dietary consultation and fluoride application are necessary for maintaining oral health and preventing the development and progression of dental caries.
키워드
Congenital heart defects; dysmorphic facial features and intellectual developmental disorders; Dental caries; Attrition; Dental treatment; General anesthesia
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