• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.12 no.1
• /
• pp.11-15
• /
• 2016

Angelman syndrome is characterized by a partial deficit of paired autosomal chromosome 15, which contains a subunit of the GABA (Gamma-Amino Butyric Acid) receptor. The main clinical characteristics of this syndrome are delayed neuropsychological development, intellectual disability, and speech impairment. The syndrome has oral manifestations such as diastemas, tongue thrusting, sucking swallowing disorder, and excessive chewing behavior. A 3-year-6-month old girl with Angelman syndrome was scheduled for dental treatment. She had multiple caries, but she was poorly cooperative for treatment due to developmental delay and movement disorder, so general anesthesia was considered. The patient with Angelman syndrome was successfully treated under general anesthesia. There were no postoperative complications related to anesthesia and dental treatment. When treating children with this syndrome, the dentist needs to manage their uncooperative behavior and medical problem.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.43 no.2
• /
• pp.207-212
• /
• 2016

Irritation fibroma is a common hyperplastic lesion of the oral mucosa that can occur in response to chronic irritation or trauma. This report presents an unusual case of irritation fibroma associated with ectopic eruption of the maxillary left central incisor in a patient with Angelman syndrome. Considering the patient's medical history and cooperative ability, excisional biopsy under intramuscular sedation using midazolam was performed. The patient exhibited successful healing without lesion recurrence. It is important to perform histopathological examination followed by excisional biopsy, because other benign or malignant tumors may mimic the clinical appearance of an irritation fibroma.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.13 no.2
• /
• pp.80-85
• /
• 2017

Angelman syndrome is a rare disorder caused by deletion or inactivation of genes on the maternally inherited chromosome 15. This neurodevelopmental disorder is characterized by developmental and intellectual delay, speech impairment, sleep disturbance, seizures, motor dysfunction, and frequent laughing or smiling. Orofacial characteristics include a prominent mandible, large mouth, prominent cheeks, a tendency to rest the tongue between the dental arches, excessive drooling, and excessive chewing behavior. Patients with this syndrome usually require general anesthesia even in a simple operation, because of risk of perioperative seizure during dental procedure. This is a case report about dental treatment of a 3-year-old female patient with Angelman syndrome under general anesthesia. This case suggests that the dental treatment under general anesthesia can be considered a safe component for the uncooperative, delayed developmental patients with underlying disease. Also, periodic dental exam appointment should be made to provide the patients with preventive treatments and to make them remain familiar with the dental environment.