• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.32-36
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• 2016

Joubert syndrome (JS) is characterized by the "molar tooth sign" (MTS) with cerebellar vermis agenesis, episodic hyperpnea, abnormal eye movements, and hypotonia. Ocular and oculomotor abnormalities have been observed; however, Horner syndrome (HS) has not been documented in children with JS. We present the case of a 2-month-old boy having ocular abnormalities with bilateral nystagmus, left-dominant bilateral ptosis, and unilateral miosis and enophthalmos of the left eye, which were compatible with HS. Brain magnetic resonance imaging (MRI) revealed the presence of the MTS. Neck MRI showed no definite lesion or mass around the cervical sympathetic chain. His global development was delayed. He underwent ophthalmologic surgery, and showed some improvement in his ptosis. To the best of our knowledge, the association of HS with JS has not yet been described. We suggest that early neuroimaging should be considered for neonates or young infants with diverse eye abnormalities to evaluate the underlying etiology.

• 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 Dental Anesthesia and Pain Medicine
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• v.17 no.3
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• pp.215-217
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• 2017

Branchio-oto-renal syndrome (BOR) is a rare autosomal dominant disorder. The features include branchial cysts, hearing loss, ear malformation, preauricular pits, retrognathia, congenital heart disease, and renal abnormalities. However, anesthetic management of these patients has seldom been reported. We report a case in which general anesthesia was performed for dental treatment in a patient with BOR. Airway management, renal function, and hemodynamic changes can be of critical concern during anesthetic management. A 13-year-old girl diagnosed with BOR had severe right hearing loss, right external ear malformation, renal abnormalities, and postoperative patent ductus arteriosus (PDA). Dental extraction under general anesthesia was scheduled for a supernumerary tooth. The procedure was completed with sufficient urine volume, adequate airway management, and stable hemodynamics.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.1
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• pp.142-145
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• 2001

Supernumerary tooth is one of the abnormalities in tooth number. Supernumerary tooth result from excessive proliferation of dental lamina and incidence reports identify a range of $0.3\sim0.8%$ in primary dentition, $1.0\sim3.5%$ in permanent dentition with males being affected twice as frequently as females, maxilla nine times as frequently as mandible The incidence is more in anterior tooth region than in posterior region. The most common supernumerary tooth is the mesiodens, which located between maxillary central incisors. The occurrence is very rare in the incisor region of mandible and the reports on incidence is 2%. In this case, there were two supernumerary teeth in the mandibular region and we could acquire normal alignment of mandibular incisors by extraction and orthodontic treatment.

• Korean Journal of Environmental Biology
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• v.25 no.1
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• pp.66-71
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• 2007

The fourth instar larvae of C. riparius were treated with potential endocrine disruption chemicals (EDCs) such as bisphenol A (BPA) and 4-nonylphenol and the effects of morphological abnormalities were observed. The deformities of the mentum following exposure to EDCs showed the smooth tooth, the loss of tooth and deformed tooth. The incidence rates of the mentum deformity were associated with chemicals: BPA $31{\sim}90%$, and 4-nonlyphenol was $40{\sim}80%$. As the concentration of BPA increased, the incidence of deformed mentum was dose dependent. While the incidences of deformed mentum following exposure to 4-nonlyphenol was not associated with their concentrations. The deformed MLT observed smooth or round tooth and the deformity of LT showed loss of one or more than tooth. Also, the MIX type was usually smooth or loss tooth. The abundance of deformity type for the mentum showed MIX (MLT+LT) > LT (lateral teeth) > MLT (median lateral teeth).

• The Journal of the Korean dental association
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• v.37 no.8 s.363
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• pp.608-613
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• 1999

During diagnostic process of the orthodontic patients, it is not unusual to find palatally erupted canines. Palatally erupted canines are related with the positional abnormalities rather than the tooth size/arch-length discrepancies. It is very important to conserve the original arch shape during traction of palatally erupted canines to their proper position. On the following case, the patient was diagnosed as malocclusion with palatally erupted canines, and were treated by 0.9mm auxiliary arch wire during traction of ectopic canines for maintenance of the original arch shape.

• Journal of Korean Dental Science
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• v.15 no.2
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• pp.181-189
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• 2022

Molar incisor malformation (MIM) has been introduced as a new type of dental anomaly. Currently, the morphological and histological characteristics of MIM are known; however, its etiology has not been clearly identified. To date, the long-term prognosis of first permanent molars (FPM) affected by MIM has rarely been reported, and few treatment guidelines have been established. The purpose of this case report was to present guidelines for the extraction of FPM affected by MIM, depending on the presence of the third molar. In patients with a third molar, spontaneous mesial shift of the posterior molars might be induced by extracting the FPM at an appropriate time, that is, when the second permanent molar is at an early furcation stage of the tooth. However, it is recommended that FPM be preserved for as long as possible if a third molar does not exist. When an FPM needs to be extracted, it is suggested to consider space maintenance.

• Journal of Oral Medicine and Pain
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• v.40 no.3
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• pp.110-114
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• 2015

Purpose: Growth and differentiation factor (GDF)-11 is a transforming growth factor-${\beta}$ family member that plays important regulatory roles in development of multiple tissues which include axial skeletal patterning, palatal closure, and tooth formation. Proteins that have been identified as GDF-11 inhibitors include GDF-associated serum protein (GASP)-1 and GASP-2. Recently, we found that mice genetically engineered to lack both Gasp1 and Gdf11 have an increased frequency of cleft palate. The goal of this study was to investigate the roles of GDF-11 and its inhibitors, GASP-1 and GASP-2, during dental and craniofacial development and growth. Methods: Mouse genetic studies were used in this study. Homozygous knockout mice for Gasp1 ($Gasp1^{-/-}$) and Gasp2 ($Gasp2^{-/-}$) were viable and fertile, but Gdf11 homozygous knockout ($Gdf11^{-/-}$) mice died within 24 hours after birth. The effect of either Gasp1 or Gasp2 deletion in $Gdf11^{-/-}$ mice during embryogenesis was evaluated in $Gasp1^{-/-}$;$Gdf11^{-/-}$ and $Gasp2^{-/-}$;$Gdf11^{-/-}$ mouse embryos at 18.5 days post-coitum (E18.5). For the analysis of adult tissues, we used $Gasp1^{-/-}$;$Gdf11^{+/-}$ and $Gasp2^{-/-}$;$Gdf11^{+/-}$ mice to evaluate the potential haploinsufficiency of Gdf11 in $Gasp1^{-/-}$ and $Gasp2^{-/-}$ mice. Results: Although Gasp2 expression decreased after E10.5, Gasp1 expression was readily detected in various ectodermal tissues at E17.5, including hair follicles, epithelium in nasal cavity, retina, and developing tooth buds. Interestingly, $Gasp1^{-/-}$;$Gdf11^{-/-}$ mice had abnormal formation of lower incisors: tooth buds for lower incisors were under-developed or missing. Although $Gdf11^{+/-}$ mice were viable and had mild transformations of the axial skeleton, no specific defects in the craniofacial development have been observed in $Gdf11^{+/-}$ mice. However, loss of Gasp1 in $Gdf11^{+/-}$ mice occasionally resulted in small and abnormally shaped auricles. Conclusions: These findings suggest that both GASP-1 and GDF-11 play important roles in dental and craniofacial development both during embryogenesis and in adult tissues.

• Imaging Science in Dentistry
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• v.52 no.1
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• pp.67-74
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• 2022

Purpose: To focus on the effects of the presence of mesiodens on adjacent teeth and to investigate the timing of its safe removal. Materials and Methods: Cone-beam computed tomography examinations, obtained at Okayama University Hospital over a three-year period, were inspected. Data were recorded including the number of mesiodens; associated abnormalities; and the relationship with neighboring structures. Depending on multiple factors, the risk of developing complications due to early extraction of a mesiodens was divided into three categories: high, medium, and low risk. Results: A total of 5,958 cone-beam computed tomography exams were obtained, 460 patients aged 3-85 years were diagnosed with a total of 568 mesiodens, 382 (67.3%) of which were discovered in young patients (age <10 years), and 333 (87.2%) of these were associated with abnormalities. Regarding the risk categories, 11 (1.9%) were considered to be in the high-risk, five (0.9%) in the medium-risk and 552 (97.2%) in the low-risk categories. Moreover, eight out of 11 high-risk mesiodens were extracted and no post-operative complications have been seen. Conclusion: As the results showed that no postoperative complications were seen in all the extracted cases of high-risk mesiodens, this indicates the possibility of safe extraction at an early age which could reduce related future complications.

• The Journal of Korean Academy of Prosthodontics
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• v.56 no.1
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• pp.31-39
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• 2018

The collapse of the posterior occlusion destroys the normal occlusal plane and causes excessive wear reducing the vertical dimension. Reduced vertical dimension of occlusion causes not only aesthetic and functional problems but also overloading on the temporomandibular joints and abnormalities of muscle nerve system. In order to improve the collapsed occlusal relationship, it is necessary to consider the change of the vertical dimension. It is necessary to make a precise diagnosis and analysis before the treatment and to evaluate the adaption of patient to the new vertical dimension of occlusion. A patient with excessive overbite often has occlusal problems of tooth wear and tooth eruption. Considering these considerations, overall prosthodontic restoration is required to solve the problem. A patient of 68 year old man in this case who suffered major tooth wear and maxillary posterior teeth loss was treated with elevation of vertical dimension of occlusion by maxillary removable dental prosthesis and mandibular fixed prosthesis.