• The Journal of Korean Academy of Prosthodontics
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• v.60 no.1
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• pp.91-99
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• 2022

Severely worn dentition causes various complications such as loss of tooth structure, discoloration, pulp complications and loss of function and aesthetics. In this case, the patient showed particularly severe attrition in the anterior teeth and lack of space for restoration. The amount of vertical dimension was determined based on the diagnostic wax up, and the patient's adaptation was evaluated by using a removable occlusal splint for 6 weeks. Thereafter, the coordination of the muscular nervous system, aesthetics, temporomandibular joint were re-evaluated for 3 months by restoring the fixed provisional restoration. Through the above treatment process, the final restoration was completed with full mouth fixed prosthesis using monolithic zirconia, and functionally and aesthetically stable results were obtained.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.40-44
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• 2019

Moyamoya disease (MMD) is a chronic, occlusive cerebrovascular disease of unknown etiology characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. The clinical presentations of MMD include transient ischemic attacks (TIA), ischemic stroke, hemorrhagic stroke, seizures, headache, and cognitive impairment. MMD is the most important cause of stroke or TIA in children in East Asian countries. A 5-year-3-month old boy with MMD experienced cerebral infarctions five times. Cerebrovascular anastomosis surgery was performed on him four years ago. He had dysphagia, developmental delay, hemiplegia, and strabismus. Besides, a number of dental caries in primary dentition were identified during clinical oral examination. Dental treatment under general anesthesia using sevoflurane was performed due to his lack of cooperation and underlying systemic disease. MMD is associated with various medical diseases requiring thoughtful consideration during dental treatment. Crying and hyperventilation in MMD patients may cause hypocapnia and have a cerebral vasoconstrictive effect. If dental treatment is required, control of pain and anxiety is very important. General anesthesia may be considered for dental treatment in uncooperative or very young patients with MMD.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.70-73
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• 2019

Congenital panhypopituitarism is an uncommon condition, present from birth, characterized by the decreased secretion of most of the hormones produced by the pituitary. The purpose of this case report is to present a case about caries treatment of a 26-month-old female patient with congenital panhypopituitarism under general anesthesia. A 26-month-old girl with congenital panhypopituitarism visited Seoul National University Dental Hospital for caries treatment of anterior primary teeth. Because of the child's age and underlying systemic disease, dental treatment under general anesthesia was considered. Prior to the dental procedure, 30mg of cortisol was administered intravenously in order to prevent possible adrenal crisis by stressful events. The dental procedure was successfully performed under general anesthesia. This case report suggests that general anesthesia may be useful for the dental treatment with congenital panhypopituitarism. Hormone deficiency should be assessed prior to dental procedure and, if necessary, stress hormone replacement therapy should be considered.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.45-49
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• 2019

Bartter syndrome is an inherited renal tubular disorder characterized by hypokalemia, hypochloremic metabolic alkalosis, hyperreninemia, hyperprostaglandinism, and normal blood pressure. Bartter syndrome is classified by neonatal and classic type. Clinical manifestation of Bartter syndrome considered in dental treatment include tendency to dehydration, vomiting and erosion, attrition and abrasion of the teeth. A 2-year-4 month old boy with Bartter syndrome type III was referred to the Seoul National University Dental Hospital for dental evaluation and treatment. He showed hypokalemic hypochloremic metabolic alkalosis and had been treated with indomethacin and potassium chloride. He had hypocalcified teeth with or without multisurface caries lesions in all dentition and the erosion of maxillary teeth was detected. Dental procedure under general anesthesia was scheduled due to multiple caries and his medical condition. The dental procedure was successfully performed. This case suggests that electrolyte imbalances need to be treated prior to dental treatment and complete coverage restoration is necessary to protect the eroded teeth. An appropriate management plan for the patients with Bartter syndrome should include considerations of the need for close interaction with the pediatrician for pre- and post-operative care. General anesthesia may be recommendable to manage the patients having multiple caries with Bartter syndrome.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.55-59
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• 2019

Pierre Robin sequence(PRS) is characterized by a triad of clinical signs: micrognathia, glossoptosis and cleft palate. These anatomical deformities of PRS predispose patients to respiratory problems and feeding difficulties at birth. Maintaining oral hygiene and enduring dental treatment are complicated by their general conditions of PRS patients. We present a case of dental treatment of PRS patient under general anesthesia. A 3-year-old boy with PRS visited Seoul National University Dental Hospital for caries treatment. Clinical and radiographic examinations revealed multiple carious lesions. Considering the patient's medical condition and compliance of treatment, dental treatment under general anesthesia was decided. Despite expected challenges of managing the airway of the patient, intubation was performed successfully. The patient was treated with pulp treatments and restorations using composite resin and stainless steel crowns. No complications were observed during and after the procedure.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.74-78
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• 2019

Atypical teratoid rhabdoidal tumor(AT/RT) is a rare and fast-growing tumor usually diagnosed in childhood. Routine magnetic resonance imaging(MRI) assessment of AT/RT patients is done to detect metastatic tumors and recurrent lesions. The purpose of this case report is to present a case about caries treatment of a 3-year-8-month old female patient with AT/RT under general anesthesia. A 3-year-8-month old patient with AT/RT visited Seoul National University Dental Hospital for caries treatment. At the first dental visit, she was receiving chemotherapy. Multiple caries were observed from clinical and radiographic examination. The dental procedure was successfully performed under general anesthesia. Patients with brain tumor may take routine MRI to detect metastatic tumors and recurrent lesions. Dentists should take into consideration of possible dislodgement of restoration by ferromagnetic properties and artifact formation on MRI image. Ready-made zirconia crowns can be a good restoration option for such patients.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.35-39
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• 2019

Oculo-cerebro-renal syndrome, known as Lowe syndrome, is an X-linked gene disorder characterized by congenital cataracts, brain abnormalities and renal dysfunction. Tooth crowding, taurodontism, delayed eruption in the permanent dentition and over-retained primary teeth with ectopic eruption of the permanent teeth have been reported as dental findings. Because of the high incidence of poor cooperation, patients with Lowe syndrome have difficulties in maintaining good oral hygiene, which may require dental treatment. We present a case of dental treatment for the uncooperative child with Lowe syndrome under general anesthesia. A 4-year-old, 11.2 kg boy with Lowe syndrome visited Seoul National University Dental Hospital for gingival swelling. The patient had multiple caries requiring dental treatment. Because of his past history of malignant hyperthermia during inhalation induction, anesthesia was induced and maintained with total intravenous anesthesia (TIVA) after medical consultation. Dental restorative treatments were successfully performed and no complications were observed during and after the procedure. Safe and effective dental management of the patients with Lowe syndrome could be performed with the help of general anesthesia and careful monitoring.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.50-54
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• 2019

Muscular dystrophy (MD) is a heterogeneous group of inherited neuromuscular disorders, characterized by progressive muscle weakness. Severity of the disease ranges from mild to severe, and the disease is mostly caused by mutations in a number of genes. These genetic mutations cause lack of proteins which are essential for muscle cell stability. Muscle fibers are gradually replaced by fat and fibrous tissue. The muscles of the head and neck are affected in several types of MD that manifest as altered craniofacial morphology and dental malocclusion. A 3-year-10-month old, 15.0 kg boy with MD presented to Seoul National University Dental Hospital, Seoul, South Korea because of extensive carious teeth. A number of dental caries in primary dentition were identified during clinical oral examination. Due to dental anxiety and underlying systemic disease, general anesthesia was considered. General anesthesia was induced and maintained with intravenous anesthetics, propofol and remifentanil. Caries treatments - resin restoration, pulpectomy, zirconia crown restoration, stainless steel crown restoration - were performed. Under general anesthesia, successful dental procedure was done. Total intravenous anesthesia (TIVA) was performed instead of inhalation anesthesia in order to avoid risk of complications such as malignant hyperthermia and life-threatening rhabdomyolysis. With decreasing muscle function, plaque control becomes more difficult and leads to gingivitis. Especially, the open-mouth posture worsens gingivitis and can leads to malocclusions and problems in swallowing. Regular and periodic dental care is essential for maintaining oral health for patients with MD.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.60-64
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• 2019

Swyer-James syndrome (SJS), also known as Swyer-James-MacLeod syndrome and unilateral hyperlucent lung syndrome, is rare acquired pulmonary disorder develops secondary to infectious etiologies in early childhood. Viral respiratory infection such as adenoviruses or Mycoplasma pneumoniae in infancy or early childhood rarely cause Swyer-James syndrome. It is generally characterized on radiographs by a unilateral small lung with hyperlucency and air trapping on expiration. In many cases unaffected lung tissue functions normally, compensating for affected lung portion. Preoperative assessment is needed to determinate individual's pulmonary function. A 4-year-old boy with Swyer-James syndrome visited Seoul National University Dental Hospital Department of pediatric dentistry for caries treatment. Clinical and radiographic examinations revealed multiple carious lesions on deciduous teeth. Considering patient's underling disease, age, and level of cooperation, dental treatment under general anesthesia was scheduled. Dental treatment was done with composite resin and stainless-steel crown. Since ventilation of Swyer-James syndrome patients was diminished because of airway obstruction, close monitoring of ventilation is necessary during dental treatment. Considering pulmonary pathology, general anesthesia rather than sedation is recommended when special behavior management is required for dental treatment. Swyer-James syndrome patients can tolerate general anesthesia and surgery well, according to several reports.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.79-83
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• 2019

Treacher Collins syndrome(TCS) is an autosomal craniofacial development disorder which results from mutations in the gene TCOF1. Major features include midface hypoplasia, micrognathia, microtia, conductive hearing loss. Oral manifestations are characterized by cleft palate, shortened soft palate, malocclusion, anterior open bite and enamel hypoplasia. The purpose of this presentation is to describe the interesting aspects of dental treatment of a patient with TCS. A 6-year-old boy with TCS visited Seoul National University Dental Hospital for dental caries. Multiple caries was observed from clinical and radiographic examination. Because of multiple caries and behavior management ploblem, dental treatment under general anesthesia was planned. Treatment of posterior teeth was performed and some primary teeth were extracted. General anesthesia was induced and maintained with sevoflurane, nitrous oxide and oxygen. Under general anesthesia, successful dental procedure was done. Considering behavior management problem and medical condition of patient with TCS, general anesthesia can be useful.