• Archives of Craniofacial Surgery
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• v.12 no.2
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• pp.102-106
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• 2011

Purpose: It is accepted universally that correction of the cleft lip nasal deformity requires multiple stages of surgery. Following primary lip repair in infancy or early childhood, secondary surgery to improve the deformity of the lip and nose is frequently necessary. A suitable surgical procedure to correct the accompanying deformity, such as cleft palate and alveolus, must be carried out at an appropriate age. In developing countries, it is common for patients with cleft lip nasal deformity to present severe secondary deformities in adolescence, because of poor follow-up and inappropriate surgery. Methods: The first patient was a 12 year old Mongolian boy. He presented prominent lip scar, short lip, wide columella, asymmetric nostril, palatal fistula, cleft alveolus, and velopharyngeal incompetence. He underwent cheilorhinoplasty, transpositional flap, alveoloplasty by iliac bone graft, and sphincter pharyngoplasty. On follow-up, a bilateral maxillary hypoplasia and a class III malocclusion developed. He underwent LeFort I osteotomy and maxillary advancement at the age of 16 years. The second patient was an 18 year old Eastern Russian girl. She presented with a deviated nose, right alar base depression, short lip, protrusion on vermilion, large palatal fistula, and severe VPI due to short palate. She underwent the combined procedure of cheilorhinoplasty, corrective rhinoplasty, tongue flap for palatal fistula, and superiorly based pharyngeal flap. And the tongue flap was detached at postoperative 3 weeks. Results: The overall results have been extremely pleasing and satisfactory to patients. There were no postoperative complications. Conclusion: We discovered the one stage operation for radical correction was sufficient procedure to provide excellent clinical outcomes in patients with severe cleft lip nose deformity.

• Imaging Science in Dentistry
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• v.49 no.4
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• pp.307-315
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• 2019

This report describes 3 cases of cleidocranial dysplasia (CCD) and presents relevant findings on long-term follow-up radiographic images of impacted permanent teeth with delayed eruption. Radiographic images of 3 CCD patients were reviewed retrospectively. These images were mainly composed of panoramic and skull radiographs, and the follow-up periods were 3, 13, and 13 years, respectively. The distinct features revealed by the images were described, and the eruption state of impacted permanent teeth was evaluated. The features common to the 3 cases were multiple supernumerary teeth, the presence of Wormian bone, underdevelopment of the maxilla and the maxillary sinus, and clavicular hypoplasia. The eruption of impacted permanent teeth was not observed without proper dental treatment in adult CCD cases, even after long time periods had elapsed. When proper orthodontic force was applied, tooth movement was observed in a manner not significantly different from the general population.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.7 no.2
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• pp.119-122
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• 2011

Achondroplasia is one of the most common types of dwarfism and is inherited as an autosomal dominant trait. Clinical features of achondroplasia include disproportionate short stature with normal trunk length, shortening of the extremities, bowing of the lower extremities, short stubby trident hands, spinal stenosis and lumbar lordosis. Characteristic craniofacial features include macrocephaly, prominent forehead, depressed nasal bridge, maxillary hypoplasia, otolaryngeal system dysfunction, and foramen magnum stenosis. These characteristics may lead to number of complications including hydrocephalus, apnea, upper-airway obstruction, otitis media, sinusitis and dental malocclusion. Apart from these features, the affected children have good general health and normal intelligence. Dentists should be aware of the clinical characteristics of achondroplasia and the complications that may arise as a result of this disorder. This case report is to present dental treatment of a patient with achondroplasia under general anesthesia and discuss special considerations.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.48 no.4
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• pp.232-236
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• 2022

A ranula is a pseudocyst that originates from the sublingual gland after trauma. Acute cases of ranulas that progress rapidly and cause respiratory distress are rare. Holoprosencephaly is a complex brain malformation caused by incomplete cleavage of the prosencephalon. Children with holoprosencephaly may experience upper airway obstruction due to the associated dentoalveolar malformations and oromotor dysfunctions. We present the case of an eight-year-old female patient with holoprosencephaly and a plunging ranula that manifested as an acute course due to difficult airway management. She required gastrostomy for oromotor dysfunctions related to feeding and swallowing and difficulty managing oral secretions. The sublingual gland and ranula were removed under general anesthesia. Postoperatively, urgent reintubation and close monitoring in the intensive care unit were required due to upper airway obstruction. We successfully managed the patient with close cooperation of a pediatrician and an anesthetist, and no recurrence was observed at the one-year follow-up. A ranula can be caused by trauma to the floor of the mouth in association with lingually inclined mandibular teeth, a type of dentoalveolar compensation seen in maxillary hypoplasia associated with holoprosencephaly. Careful consideration is needed in such cases since airway management can be difficult due to postoperative swelling and oromotor dysfunctions.

• Journal of the korean academy of Pediatric Dentistry
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• v.51 no.1
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• pp.66-79
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• 2024

This study aimed to assess the prevalence and distribution pattern of dental anomalies in the permanent teeth of patients with non-syndromic cleft lip with or without cleft palate. Additionally, it aimed to compare differences in dental anomalies between cleft and non-cleft areas, considering gender and cleft phenotype. Panoramic radiographs of 164 patients diagnosed with non-syndromic orofacial clefts were retrospectively analyzed by a single examiner to confirm dental anomalies. The dental anomalies investigated included tooth agenesis, supernumerary teeth, microdontia, rotation, ectopic eruption, and enamel hypoplasia. Cleft phenotypes were categorized into 7 types based on medical and dental records. A significantly higher prevalence of supernumerary teeth was observed in males than females within non-cleft areas (p = 0.017), with no significant differences in other dental anomalies. In non-cleft area, patients with cleft palate exhibited a high prevalence of tooth agenesis (p < 0.0001) and microdontia (p = 0.012) compared to other cleft phenotypes. Maxillary incisor rotation was closely associated with adjacent tooth agenesis in unilateral cleft lip and palate cases (p = 0.034). This study suggests that the additional subphenotype based on dental anomalies in patients with orofacial cleft may serve as applicable clinical markers.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.2
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• pp.113-117
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• 2012

Axenfeld-Rieger syndrome (ARS) is a rare disorder with variable morphology characterized by malformations of the anterior segment of the eye such as iris hypoplasia, iridocorneal adhesions, corectopia and polycoria. Craniofacial and dental anomalies are also regularly reported in connection with ARS. Dental features include hypodontia in primary and permanent dentition, microdontia, short roots and abnormally shaped teeth. The patient in this case is a 14-year-old girl who has visited Seoul National Dental Hospital in 2002 for the first time. A panorama was taken on the day of her first visit, and total absence of maxillary anterior dentition and mandibular premolars were observed. The patient was on a treatment schedule by the department of ophthalmology at Seoul National Hospital for a morphological dyplasia of the pupil and she was diagnosed with ARS. Periodic panoramic observations were performed followingly, and she was finally diagnosed of congenital missing of maxillary anteriors and mandibular second premolars. In 2009, composite resin build-ups of maxillary primary central incisors were performed, and she has been on her regular follow-ups until now.

• The korean journal of orthodontics
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• v.30 no.1 s.78
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• pp.43-52
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• 2000

Hemifacial microsomia ( HFM ) is the second most common craniofacial abnormalies. HFM represnted a spectrum of clinical findings such as hypoplasia of the mandibular ramus and condyle, confinement of maxilla growth, external and/or middle ear defects, involvement of some cranial suture, buccal soft tissue, facial nerve, and muscles in the affected side. HFM often showed progressive facial asymmetry and occlusal plane slanting to the affected side with growth. There were several reports about asymmetry of tooth maturation, hypodontia, delayed eruption, enamel hypoplasia in HFM. Since teeth develope in close association with size and morphology of the maxillary and the mandible, it is highly likely that dental changes will be present in HFM. So the Purpose of this study was to investigate the differences of the primary and the permanent teeth dimensions in the maxillary and the mandibular dentition between the affected and the non-affected side of HFM.. The sample of this study consisted of 34 unilateral HFM Patients (18 males and 16 females, average age : 5 year 11 months old). The authors examined the mesiodistal and the faciolingual dimensions of the primary and the permanent teeth and performed statistical study by using paired t-test. The results were as follows 1. The mesiodistal dimensions of the mandibular second primary molar and the mandibular first permanent molar in the affected side of HFM were significantly smaller than those of non-affected side. But there were no significant differences in the anterior teeth and the mandibular first primary molar. It means that a gradient of severity from anterior teeth to posterior teeth was found in the mandibular dentition. 2. Although there were no significant differences in the faciolingual dimensions of the primary and the permanent teeth in the maxillary and the mandibular dentition between the affected and non-affected side of HFM, there were general trend of compensatory increase in faciolingual dimension of the mandibular primary and the permanent teeth in the affected side Therefore these results showed that HFM might affect on the abnormality of tooth dimension, especially the most posterior teeth, in the affected side of the mandible.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.1
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• pp.144-150
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• 2008

Multimodal cancer therapy including surgery, chemotherapy, and radiotherapy could not only improve the prognosis of malignancy but also reduce the dosage and toxicity of cancer drug for treatment of malignant tumor. The effects of radiotherapy are generally localized, additive, and accumulative, and depend on dosage, site and cell sensitivity. However, in growing individuals, the dental and skeletal sequelae to radiotherapy result in dental or facial abnormalities that are irreversible : arrested root development, disturbances in enamel formation, microdontia, anodontia, altered tooth eruption and mandibular or maxillary hypoplasia. Especially, the teeth which are developing is affected according to the stage. We report three cases of developmental disturbance of permanent teeth after radiotherapy. These children had received radiotherapy for malignant tumor at the age of 3 to 4 years, in which root hypoplasia, short tapered root and early apex closure were observed. For the management of radiation caries and radiotherapy-related teeth, periodic recall check and oral hygiene instruction are required.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.3
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• pp.539-547
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• 2008

Apert syndrome is an autosomal dominant condition characterized by craniosynostosis, midface hypoplasia, and syndactyly of the hands and feet. It occurs in about 1 of every 65,000 to 160,000 births and is caused by a mutation in the fibroblast growth factor receptor 2(FGFR2) gene. Apert syndrome typically produces acrobrachycephaly(tower skull). The occiput is flattened, and there is a tall appearance to the fore head. Ocular proptosis is a characteristic finding, along with hypertelorism and downward slanting lateral palpebral fissures. The middle third of the face is markedly retruded and hypoplastic, resulting in a relative mandibular prognathism. The reduced size of the nasopharynx and narrowing of the posterior choana can lead to mouth breathing, contributing to an open-mouth apprance. Three fourths of all patients exhibit either a cleft of the soft palate or a bifid uvula. The maxillary hypoplasia leads to a V-shaped arch and crowding of the teeth. A 6-year-old male patient visited to the Department of Pediatric dentistry, Kangnung National University of Dental Hospital. He visited the hospital to get treatment of carious teeth. The purpose of this report is to present a specific dental manifestations about the apert syndrome.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.33 no.5
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• pp.559-566
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• 2007

Distraction osteogenesis(DO) is a surgical method of bone formation that involves an osteotomy and sequential stretching of the healing callus by gradual movement and subsequent remodeling. DO is used to correct facial asymmetry, such as in patients with hemifacial microsomia, maxillary or mandibular retrusion, cleft lip and palate, alveolar defects, and craniofacial deficiency. It is accomplished with the aid of a distraction device, which is secured with screws placed directly into bone, for a predetermined length of time. Hemifacial microsomia is characterized by unilateral facial hypoplasia, often with unilateral shortening of the mandible and subsequent malocclusion. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, tilted occlusal plane, and short mandible. Early treatment is necessary to avoid subsequent impaired midfacial growth. The standard treatment of these malformations consists of the application of bone grafts, which can lead to unpredictable growth. The new bone-lengthening procedure represents a limited surgical intervention and opens up a new perspective for treatment, especially in younger children with severe deformities. This report describes a case of hemifacial microsomia(Type-II left-sided hemifacial microsomia). The patient, a 10-year-old child, visited our clinic for facial asymmetry correction. He had a hypoplastic mandible, displaced ear lobe, 10 mm canting on the right side, and malocclusion. We planned DO to lengthen the left mandible in conjunction with a Le Fort I osteotomy for decanting and then perform a right intraoral vertical ramus osteotomy(IVRO). Progressive distraction at a rate of 0.5 mm/12 hours was initiated 7 days postoperatively. The duration of DO was 17 days. The consolidation period was 3 months. Satisfactory results were obtained in our case, indicating that DO can be used successfully for functional, aesthetic reconstruction of the mandible. We report a case involving DO in conjunction with orthognathic surgery for correcting mandibular hypoplasia with a review of the literature.