• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.2
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• pp.99-104
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• 2010

Russell-Silver syndrome is a genetic disorder characterized by intrauterine and/or postnatal growth restriction and typical facies. The clinical feature is various due to heterogeneous genetic characters. Their common findings are short stature without catch-up growth, normal head size for age, a distinctive triangular face with prominent forehead and a pointed chin, low set ears and clinodactyly of the fifth fingers. Intraoral features of the syndrome are microdontia, delayed tooth eruption, hypodontia, and crowding. More than 400 case have been reported in the literature, and estimated incidence is from 1 in 3000 to 1 in 100,000. In this case we performed caries treatment under the general anesthesia for the patient with Russell-Silver syndrome. Dentist have to consider microstomia for the management of patients with Russell-Silver syndrome.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.10 no.1
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• pp.26-30
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• 2014

Prader-Willi syndrome (PWS) is a rare neurodevelopmental disease caused by abnormality of chromosome 15q11-13. The estimated prevalence of PWS is 1/10,000-30,000. Most common features of this disease are feeding problems characterized by poor sucking habit related with neonatal or infantile hypotonia and obesity due to early childhood hyperphagia involved with lack of satiety. In the orodental findings, enamel hypoplasia, rampant caries, delayed eruption, poor oral hygiene, hypodontia, supernumerary teeth, increased tooth wear, decreased salivary flow and change in saliva composition were reported. This case report describes the dental treatment of 3-year-9-months-old male patient with PWS. Periodic check-ups and conservative treatments were followed, however, rapid dental caries progression caused by estimating hyposalivation was observed. Because of lack of patient cooperation, dental procedures were performed under general anesthesia.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.1
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• pp.36-38
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• 2013

Takayasu's arteritis(TA) is commonly known as Aortitis Syndrome, Pulseless disease. Cardiac involvement due to vascular occlusion or stenosis is common in TA, expressed in forms of heart failure, aorta hypertension, alteration of artery or cardiac muscle. In this case, a 9 year old boy was referred to our dental clinic by his cardiology doctor for delayed eruption and gingival hyperplasia on upper incisor. The patient was diagnosed as TA with history of taking Amlodipine, a calcium channel blocker as hypertension medication. He was diagnosed as drug induced gingival hyperplasia. Under taking preventive antibiotic, gingivectomy was done. In case dental treatment of TA patient, dentist should be aware of two possible problems. First is the antibiotic prophylaxis due to the high risk of endocarditis. Second is the possibility of drug induced gingival hyperplasia.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.1
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• pp.108-112
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• 2000

Hypophosphatemic rickets is lack of reponse to physiologic doses of vitamin D, different from the vitamin D-dependent rickets. It is inherited in an X-linked dominant fashion. The prime features of this disorder are lowered serum phosphate levels, elevated serum alkaline phosphatase and normal serum calcium levels. The dental manifestation often include apical radiolucencies, abscess and fistulas of clinically sound teeth. Dental radiographs show ricketic bone trabeculations, abscent or abnormal lamina dura and abnormal cementum. This case which was diagnosed to hypophosphatemic rickets, showed multiple spontaneous periapical abscess and gingival fistula enlarged pulp chambers, extension of the pulp horns into the cusp tips and delayed eruption.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.1
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• pp.70-76
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• 2000

Congenital biliary atresia with progressive sclerosis of the intra- and extra-hepatic duct system occurs in 1 : 10,000 live births, and has a poor prognosis with an expected survival of less than 5 years. Etiology of biliary atresia is unclear, however, it is believed a genetic or developmental cause. The clinical characteristics include pronounced jaundice, hepatosplenomegaly, pruritus, steatorrhea, xanthomas, growth retardation, portal hypertension, bleedings, ascites and respiratory infections. Oral manifestations have seldom been reported in patients with biliary atresia, but there may be enamel hypoplasia, delayed tooth eruption, and green teeth. Early diagnosis and surgical intervention have decreased morbidity. returned growth and development to normal and improved the prognosis for survival. Authors report the clinical and radiologic characteristics, proper managements about two cases with biliary atresia.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.510-514
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• 2003

The Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by dental and ocular abnormalities. The essential ocular features include partial or complete bilateral hypoplasia of the iris stroma, abnormalities of the angle structures with congenital iris adhesions, and anterior displacement of Schwalbe's corpuscles. Common oral findings are hypodontia(especially in anterior maxillary segment), microdontia, misshaped teeth, delayed eruption of the teeth. Additionally, other systemic symptoms can be seen and early detection by the pedodontist through dental diagnosis should prevent visual impairment.

• The korean journal of orthodontics
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• v.50 no.6
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• pp.407-417
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• 2020

Objective: To investigate the dental phenotypes and treatment modalities (Tx-Mod) in Korean patients with Parry-Romberg syndrome (PRS) using longitudinal data. Methods: The samples consisted of 10 PRS patients, who were treated and/or followed-up at Seoul National University Dental Hospital between 1998 and 2019. Using a novel PRS severity index based on the numbers of the atrophy-involved area and asymmetry-involved item, we classified them into mild (n = 3), moderate (n = 2), and severe (n = 5). Dental phenotypes, including congenitally missing tooth (Con-Missing-Tooth), microdontia, tooth with short root (Short-Root), tooth with dilacerated root, and delayed eruption/impacted tooth, were investigated along with Tx-Mod. Results: The side of occurrence of all dental phenotypes showed 100% concordance with the side of PRS involvement. The most two common dental phenotypes were Con-Missing-Tooth and Short-Root (n = 29 and n = 17 in six patients). The sums of the average number of Con-Missing-Tooth and Short-Root increased from mild PRS to moderate PRS and severe PRS cases (1.0, 6.0, and 6.2). In terms of Tx-Mod, growth observation due to mild atrophy, fixed orthodontic treatment, and grafting were used for mild PRS cases. Tx-Mod for moderate PRS cases involved growth observation for surgery due to an early age at the initial visit. For severe PRS cases, diverse Tx-Mod combinations including unilateral functional appliance, fixed orthodontic treatment, growth observation, grafting, and orthognathic surgery were used. Conclusions: The novel PRS severity index may be useful to provide primary data for individualized diagnosis and treatment planning for PRS patients.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.1
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• pp.120-125
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• 2004

Cornelia de Lange syndrome is a disorder of unknown etiology resulting in an syndrome characterized by specific dysmorphic features. Therefore this syndrome is diagnosed only by clinical features and other examinations for diagnostic aim are not effective. There are general growth retardation, mental retardation, hypertrichosis, confluent eye brows, low hair line, broad nasal bridge, anteverted nose tip, malformed limbs, webbing of toes, heart defect, gastroesophageal reflux disease, ear and ocular problems. Features associated oral structures are micrognathia, delayed eruption of teeth, cleft lip, cleft plate, thin upper lip and downturned angles of mouth. These are cases about two children who visited Department of Pediatric Dentistry of Chonbuk National University because of dental caries with Cornelia do Lange syndrome.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.1
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• pp.64-71
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• 2014

Mesiodens is defined as a supernumerary tooth in the premaxillary region. It can cause several clinical manifestations in normal eruption and position of adjacent teeth, such as displacement and impaction. Although the mesiodens accompanied by a clinical complication is indicated for removal, the optimal time of mesiodens removal is still controversial. Some authors suggest immediate intervention defining the removal of mesiodens as soon as possible after the first detection. On the other hand, others recommend delayed intervention which denotes the removal of mesiodens after complete root development of adjacent teeth. This case report is presented with three cases of spontaneous correction and proper alignment of rotated maxillary central incisors by extraction of mesiodens when the crowns of rotated incisors were completely formed while the roots of them were at an early developmental stage.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.19 no.1
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• pp.89-101
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• 1989

The author observed and analyzed the age, sex, chief complaint and radiographic finding of sixty-one cases of benign odontogenic tumors seen in Yonsei Medical Center, for the period of Jan. 1979 to Aug. 1989. The results were as follows: 1. Benign odontogenic tumors of 61 cases included 52 cases (85.3%) of ameloblastoma and odontoma, and 9 cases of other lesions. Radiographically, the border of the lesions were well-defined. 2. Ameloblastoma constituting twenty-seven cases (44.3%) occurred the average age of 31.1 years and had a 3:1 male predominance. The most common complaint was swelling (20 cases, 74.0%) and followed by pain (13 cases, 48.2%). Radiographically, the most common site was mandibular body area (74.0%) and the lesions were mainly multiocular radiolucency; in 17 cases (63.0%) and unilocular radiolucent lesion were seen in 10 cases (37.0%). 16 cases (59.3%) showed the resorption of roots of adjacent teeth. 3. Odontoma constituting twenty-five cases (41.0%) discovered at the average age of 16.9 years and had a 3:2 male predominance. The most common complaint was delayed eruption of tooth (8 cases, 31.0%) and 7 cases (27.0%) detected on a routine radiograph of the area. Radiographically, 17 cases (68.0%) were of compound type and 8 were of complex variety and compound odontomas were common in the anterior maxilla, whereas complex odontomas occurred more frequently in the posterior mandible. 19 cases (76.0%) showed the impaction of adjacent teeth.