• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.10-13
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• 2016

Chromosome 11q13 deletion syndrome has been previously reported as either otodental syndrome or oculo-oto-dental syndrome. The otodental syndrome is characterized by dental abnormalities and high-frequency sensorineural hearing loss, and by ocular coloboma in some cases. The underlying genetic defect causing otodental syndrome is a hemizygous microdeletion involving the FGF3 gene on chromosome 11q13.3. Recently, a new form of severe deafness, microtia (small ear) and small teeth, without the appearance of eye abnormalities, was also reported. In this report, we describe a 1-year-old girl presenting with ptosis of the left upper eyelid, right auricular deformity, high-arched palate, delayed dentition, simian line on the right hand, microcephaly, and developmental delay. In this patient, we identified a deletion in the chromosome 11q13.2-q13.3 (2.75 Mb) region by using an array-comparative genomic hybridization analysis. The deletion in chromosome 11q13 results in a syndrome characterized by variable clinical manifestations. Some of these manifestations involve craniofacial dysmorphology and require a functional workup for hearing, ophthalmic examinations, and long-term dental care.

• Journal of Korean Academy of Oral Health
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• v.42 no.4
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• pp.238-241
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• 2018

Williams syndrome is a rare congenital disorder with various physical abnormalities and characterized by facial, oral, and dental features. Individuals with Williams syndrome typically have eating disorders in the early childhood, which lead to prolonged night feeding. Prolonged night feeding is a risk factor for rampant dental caries. Williams syndrome is caused by the microdeletion of chromosome 7, resulting in elastin deficiency. Elastin is integral to cardiovascular health. Many patients with Williams syndrome have complex cardiovascular abnormalities that must be considered a part of dental management. Complications related to cardiovascular diseases may induce adverse effects such as dangerously elevated blood pressure. This may occur in patients during stressful dental treatment. In addition, characteristics of auditory hyperalgesia and anxiety disorders among patients with William syndrome, complicate receiving routine dental management. Therefore, dental treatment under sedation or general anesthesia may be preferable for patients with Williams syndrome; in particular, patients who are very uncooperative and/or needs extensive dental treatment. A thorough evaluation of each patient's physical condition is required before making decisions regarding dental treatment. Careful monitoring and preparation for emergencies are very important during and shortly after dental treatment under general anesthesia or sedation. Monitoring is critical until vital signs have stabilized and return to normal. A 28-month-old man diagnosed as having Williams syndrome, visited the Dental Hospital of ${\bigcirc}{\bigcirc}$ University for the management of rampant dental caries. We reported on the management of this patient who had peripheral pulmonic stenosis, and received dental treatment under general anesthesia. We also reviewed the characteristics of Williams syndrome and discussed considerations for dental treatment under general anesthesia.

• Journal of Dental Anesthesia and Pain Medicine
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• v.20 no.1
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• pp.49-53
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• 2020

Cri-du-chat syndrome (CdCS) is caused by the deletion of the short arm of chromosome 5. Most patients with CdCS develop intellectual disabilities. Therefore, they have poor oral hygiene and a high caries index. However, treating such patients is not an easy task, because of the difficulty in communication. General anesthesia may be a useful option in adult patients with CdCS and intellectual disability. General anesthesia should be administered very carefully, owing to the presence of comorbid diseases, which may need airway management. Infants with CdCS need general anesthesia if they have a concomitant cardiac anomaly. Intubation is reportedly difficult for such patients was, owing to the structural and functional abnormalities in the larynx and vocal cords. The purpose of this study was to report a case of difficult intubation while inducing general anesthesia in a patient with CdCS during dental treatment, due to a narrow larynx and trachea.

• Imaging Science in Dentistry
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• v.49 no.3
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• pp.191-199
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• 2019

Purpose: To compare the diagnostic ability of undergraduate dental students to detect maxillary sinus abnormalities in panoramic radiographs(PR) and cone-beam computed tomography (CBCT). Materials and Methods: This was a retrospective study based on the evaluation of PR and CBCT images. A pilot study was conducted to determine the number of students eligible to participate in the study. The images were evaluated by 2 students, and 280 maxillary sinuses were assessed using the following categories: normal, mucosal thickening, sinus polyp, antral pseudocyst, nonspecific opacification, periostitis, antrolith, and antrolith associated with mucosal thickening. The reference standard was established by the consensus of 2 oral radiologists based on the CBCT images. The kappa test, receiver operating characteristic curves, and 1-way analysis of variance with the Tukey-Kramer post-hoc test were employed. Results: Intraobserver and interobserver reliability showed agreement ranging from substantial (0.809) to almost perfect (0.922). The agreement between the students' evaluations and the reference standard was reasonable (0.258) for PR and substantial(0.692) for CBCT. Comparisons of values of sensitivity, specificity, and accuracy showed that CBCT was significantly better(P<0.05). Conclusion: CBCT was better than PR for the detection of maxillary sinus abnormalities by dental students. However, CBCT should only be requested after a careful analysis of PR by students and more experienced professionals.

• Imaging Science in Dentistry
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• v.40 no.1
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• pp.25-32
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• 2010

Purpose : This study was designed to evaluate the efficacy and utility of panoramic radiograph for the improvement of the periodic oral examinations. Materials and Methods : Clinical examinations and panoramic examinations were done for the 242 subjects of oral examinations. The results of panoramic radiograph interpretation were compared with the clinical findings. Two questionnaires were created. One was carried out before the panoramic examination and the other done afterwards, to find out the subjects' cognition and satisfaction for the clinical and panoramic examinations. Results : 1. Panoramic findings showed a higher detection rate of 31.9% for periodontal diseases, and 23.1% for dental caries than clinical findings. 2. The additional abnormalities detected through panoramic examinations were impacted tooth in 81 subjects (33.6%), maxillary sinus abnormalities in 28 subjects (11.6%), condylar abnormalities in 5 subjects (2.1%), congenital and acquired dental anormalies in 59 subjects (24.5%), and other miscellaneous abnormalities in 34 subjects (14.1%). 3. 164 subjects (67.8%) were satisfied with the current periodic oral examination, and 75 subjects (31.1%) hoped for better accuracy. 4. In the first and second questionnaire, 154 subjects (67.0%) and 163 subjects (70.6%) responded respectively that panoramic examination was necessary, and 193 subjects (83.2%) responded that it actually helped. Conclusion : The panoramic examination was revealed to improve the effectiveness of the periodic oral examination and to increase the satisfaction of the subjects of examination.

• Imaging Science in Dentistry
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• v.41 no.4
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• pp.177-181
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• 2011

Pyknodysostosis is a rare autosomal recessive disorder characterized by the post natal onset of short limbs, short stature, and generalized hyperostosis along with acro-osteolysis with sclerosis of the terminal phalanges, a feature that is considered essentially pathognomonic. Other features include persistence of fontanelles, delayed closure of sutures, wormian bones, absence of frontal sinuses, and obtuse mandibular gonial angle with relative mandibular prognathism. We report a case of 17-year-old girl who presented with a chief complaint of retention of deciduous teeth. General physical examination demonstrated short stature, frontal and parietal bossing, depressed nasal bridge, beaked nose, hypoplastic midface, wrinkled skin over the finger tips, and nail abnormalities. Radiographs showed multiple impacted permanent and supernumerary teeth, hypoplastic paranasal sinuses with acro-osteolysis of terminal phalanges, and open fontanelles, and sutures along with wormian bones in the lambdoidal region.

• Journal of Oral Medicine and Pain
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• v.47 no.3
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• pp.126-134
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• 2022

Purpose: This study aimed to propose an efficient treatment approach for infection with different candida species. Methods: Fifty-three patients who presented with a chief complaint of oral mucosal pain and exhibited positive candida culture findings were divided into two groups (Candida albicans and non-albicans). Pain, mucosal manifestations, salivary flow rates, durations of disease and treatment, and responses to treatment (nystatin and clonazepam) were investigated in both groups. Results: Patients in the C. albicans group exhibited more prominent clinical characteristics (erythematous lesions, tongue coatings, and hyperalgesia) than those in the non-albicans group. In total, 70% of patients in the non-albicans group showed no abnormalities in the oral mucosa. Patients in the C. albicans group showed increased resistance to nystatin treatment compared to those in the non-albicans group, especially with longer disease durations. The patients resistant to nystatin treatment showed positive responses to clonazepam. Conclusions: Patients with oral mucosal pain should be tested for the presence of Candida, even in the absence of mucosal abnormalities, especially those infected with non-albicans species. If no response to antifungal therapy is observed, treatment with clonazepam should be initiated, especially in patients infected with C. albicans.

• Journal of Dental Rehabilitation and Applied Science
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• v.25 no.3
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• pp.211-224
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• 2009

The objective of this study is to examine the condylar surfaces in order to find out the types, the incidence and common occurrence area of the osseous abnormalities of the condyles according to the age, genders and the purpose of CT taking, and to compare those between the groups for TMD diagnosis and the other groups. 3D CT images of 199 patients which were scanned with the $i-CAT^{TM}$ Cone Beam Computed Tomography were collected from Sanbon Dental Hospital of Wonkwang university and the MPR images were transfered to the TMJ mode to be showed serial sagittal images and coronal images. The images were macroscopically examined by three independent observers for the types and incidences of the osseous abnormalities, their common occurrence area and general shapes of the condyles. As a result, type F is most common ever than type N. The common occurrence area in sagittal images is antero-superior and superior area except for type D-C which were showed on postero-superior area commonly. In coronal images, latero-superior and superior area is most common except for type E which were present on mesio-superior and superior area most frequently. The osseous abnormalities of the condyles are more common in TMD diagnosis group except for type D-C, that is type N and type D-C are more common in the other groups. In this study, abnormalities of the condyles are classified into 6 types and it has a common occurrence area each. And TMD diagnosis group shows a tendency to have higher rate for osseous abnormalities except for type D-C.

• Korean Journal of Cleft Lip And Palate
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• v.10 no.2
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• pp.81-88
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• 2007

Cleft lip and palate (CLP) is one of the most prevalent congenital craniofacial anomalies. It has a significantly greater incidence of dental abnormalities in number, size, shape, and eruption of the teeth. Knout-out mouse model can identify several genes which play an important role in tooth agenesis. Since disruption of these genes has been confirmed to result in tooth agenesis in humans, CLP associated with hypodontia may be the best models for isolated tooth agenesis. According to the studies of dental abnormalities in CLP, the severity of dental defect is known to be influenced by the CLP phenotype. The cumulative data obtained from mouse and human genetic studies indicated that MSX1, PAX9 and AXIN2 are considered as candidate genes in non-syndromic hypodontia, while Shh, Pitx2, Irf6, p63 and EDA pathway genes are involved in syndromic one. We expect that genetic approach of CLP can offer the basis for tooth regeneration and be a new target in hypodontia therapy.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.15 no.1
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• pp.67-74
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• 1985

The incidence and associated dental abnormalities of taurodontism were studied radiographically (panoramic view) in 1895 patients of deciduous dentition and 2167 patients of permanent dentition who had visited to the Department of Oral Radiology, Kyung Hee University in Korea. The obtained results were as follows: 1. The prevalence of taurodontism was 3.59% in the deciduous dentition group, and 1.8% in the permanent dentition group. 2. There was no definite sex difference in the deciduous dentition, and the permanent dentition group. The type of mesotaurodontism was predominent in the deciduous dentition group, and hypotaurodontism in the permanent dentition group. 4. The vast majority of this abnormalities had occurred in the mandibular 1st deciduous molar in the deciduous dentition group, and mandibular 2nd permanent molar in the permanent dentition group. 5. Of the cases with taurodontism, the deciduous dentition group revealed 95.6% bilaterally, and 82% bilaterally in the permanent dentition group. 6. Multiple teeth occurance, not single tooth only, were involved in 98.5% of the cases in the deciduous dentition group, and 82% in the permanent dentition group.