• Journal of the korean academy of Pediatric Dentistry
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• v.47 no.3
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• pp.344-351
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• 2020

Amelogenesis imperfecta (AI) occurs either in isolation or in association with other dental abnormalities and systemic disorder. A rare syndrome associating AI with nephrocalcinosis was named as Enamel Renal Syndrome (ERS; OMIM #204690). This syndrome is characterized by severe enamel hypoplasia, failed tooth eruption, intra pulpal calcifications, enlarged gingiva, and nephrocalcinosis. Nephrocalcinosis is a condition where calcium salts are deposited in renal tissue, and this may lead to critical kidney complications. This rare syndrome shows pathognomonic oral characteristics that are easily detectable at an early age, which proceeds the onset of renal involvement. Pediatric dentists are the first oral health practitioners whom ERS patients will meet at early age. The role of pediatric dentists is critically important for early diagnosis and referral of patients to both nephrologists for renal assessment and geneticists for identification of causative mutation and diagnosis. Early detection of renal involvement may provide chances to prevent further undesired renal complications.

• 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 Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.36-40
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• 2018

CATCH22 syndrome or DiGeorge syndrome is a medical acronym of cardiac defects, abnormal facial appearances, thymic hypoplasia, cleft palate, and hypocalcemia. Patients with CATCH22 syndrome are susceptible to infection due to an absent or hypoplastic thymus and often have difficulties in maintaining good oral hygiene, which may require dental treatment. We present a case of dental treatment for the uncooperative child with CATCH22 syndrome under general anesthesia. A 4-year-old, 14.8 kg boy with CATCH22 syndrome visited Seoul National University Dental Hospital for dental check up. The patient had multiple caries requiring dental treatment. He experienced the corrective heart surgery due to Tetralogy of Fallot a few years ago. General anesthesia was planned because his heart rate and vital sign had shown unstable during the previous conscious sedation procedure. 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 CATCH22 syndrome could be performed with the help of general anesthesia and careful monitoring.

• Journal of the korean academy of Pediatric Dentistry
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• v.39 no.4
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• pp.412-417
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• 2012

Mucopolysaccharidosis (MPS) is a disorder which is caused by the defect of the lysosomal enzyme that is essentially needed for resolution of glycosaminoglycans (GAGs). Metabolite of GAGs will accumulate in the lysosome of cells and will result in the dysfunction of cells, tissues, and organs. Eventually, patients will manifest both mental retardation and physical disorders. In worst cases, mucopolysaccharidosis can cause premature death. The current clinical types have been classified as MPS from type I to type IX according to the defect of certain enzyme. The dental complications have been reported as delay of eruption, enamel hypoplasia, microdontia, malocclusion, condylar defects, gingival hyperplasia and dentigerous cystlike follicle. This clinical report presents the case of a boy with MPS type II, Hunter Syndrome which has various dental complications.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.607-612
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• 2009

The malignant tumor in childhood is one of the main causes of children s death due to disease. The traditional treatment for the malignancy is known for the radiation therapy and the chemical therapy or both. However, the treatments tend to induce intraoral complications. Different from adults, almost all children on cancer therapy are expected to have dental complications, because their permanent teeth are on the developmental stage. The degree of dental complication depends on the patient's age, type of chemical and other factors-radiation dose and frequency. In this report, 3 children who had experienced the anti-cancer therapy on their age between 1 and 4 years were selected and dental complications were examined. The children have chance for the various oral complications including the developmental problems such as agenesis, microdontia and hypoplasia of the teeth. Therefore, it's important to understand the side-effects of anticancer therapy during the permanent teeth had been developmental stage in young patients. Also, oral health care specialists, including pediatric and hospital dentist can support the oncology team by providing basic oral care, implementing oral care protocols, delivering emergency dental treatment undergoing anticancer treatment.

• Journal of Veterinary Clinics
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• v.26 no.5
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• pp.490-494
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• 2009

A 2-year-old Maltese and a 5-month-old Yorkshire terrier were presented with ataxia. Tetraparesis was observed in a 9-year -old Yorkshire terrier. The localizations of the lesions suggested brain or cervical spinal cord by the neurological examination, and the following images was achieved: radiography, axial images of computed tomography (CT), reconstruction image of CT such as multi-planar reformation(MPR) and 3-dimensional(3D) reconstruction and magnetic resonance imaging (MRI). On radiography, the misalignment between atlas (C1) and axis (C2), absent dens of axis, and increased space between the dorsal arch of C1 and spinous process of C2 were found. The discontinuation between dens and body of C2 was identified through axial CT images, and the fragmentation of dens separated from axis was observed through MPR and 3D image in all case. The hyperintense lesions and the spinal cord compression on T2-weighted MR images were represented in a dog with tetraparesis, the others represented only spinal cord compression. Three dogs were diagnosed as atlantoaxial instability (AAI) by dens fracture of C2. The dog with tetraparesis was euthanized due to guarded prognosis. The others were recovered completely. It is difficult to differentiate dens fracture of C2 from abnormal dens such as agenesis and hypoplasia. We thought that CT is very useful to evaluate the dens of C2 and differentiate the causes of AAI, and the reconstruction images of CT such as MPR and 3D make the translation of the fragmented dens or axis of AAI more precisely evaluate.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.4
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• pp.669-676
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• 1999

The dentigerous cysts originate through alteration of the reduced enamel epithelium after amelogenesis is completed, with accumulation of fluid between the layers of the enamel epithelium, or between this epithelium and the tooth crown. Next to the radicular cyst, they are the second most common type of odontogenic cyst. They occur over a wide age range with a peak frequency in the 2nd to 3rd decade. A substantial majority involve the mandibular third molars, followed in order of frequency by the maxillary permanent canines, mandibular second premolars, and maxillary third molars. With regard to the treatment of these cysts, the marsupialization procedure with obturator is recommended during the age when the eruptive force of the teeth is still strong. It can be effective when preservation of the displaced teeth is desirable. We treated the dentigerous cyst by marsupialization with obturator and guided the eruption of involved teeth to normal position. And we got the results as follows : 1. Severely dislocated teeth associated with dentigerous cyst erupted into proper position. 2. The enamel hypoplasia and the root deformity were observed some cases. 3. The bone expansion and defect were healed without infection and recurrence.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.703-708
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• 2005

Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.3
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• pp.352-358
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• 2010

Chemotherapy or radiotherapy used for the treatment of pediatric cancer may have many adverse effects on the oral cavity. Oral mucositis, reduced salivary flow, oral infection, hypodontia, microdontia, arrested root development, and enamel hypoplasia are common oral complications. The aim of this study is to evaluate the effects of cancer therapy on dental caries activities. The children who had been treated for neuroblastoma in the department of pediatrics, Samsung Medical Center, were included and healthy children served as controls. The salivary flow rate, salivary buffering capacity, and Streptococcus mutans counts of both groups were evaluated using Dentocult$^{(R)}$ SM and Dentobuff$^{(R)}$ Strip. The dental caries activity related to the age at the start of treatment and the time elapsed since treatment completion were also evaluated. As a result, neuroblastoma patients had significantly lower salivary flow rate than the controls, while there were no significant differences between two groups as for salivary buffering capacity and Streptococcus mutans counts. The dental caries activities related to the age at the start of treatment and the time elapsed since treatment completion were not significantly different.

• The korean journal of orthodontics
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• v.50 no.1
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• pp.33-41
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• 2020

Objective: To investigate the distribution and phenotypes of hemifacial microsomia (HFM) and its association with other anomalies. Methods: This study included 249 Korean patients with HFM, whose charts, photographs, radiographs, and/or computed tomography scans acquired during 1998-2018 were available from Seoul National University Hospital and Dental Hospital. Prevalence according to sex, side involvement, degree of mandibular deformity, compensatory growth of the mandibular body, and Angle's classification, and its association with other anomalies were statistically analyzed. Results: Prevalence was not different between male and female patients (55.0% vs. 45.0%, p > 0.05). Unilateral HFM (UHFM) was more prevalent than bilateral HFM (BHFM) (86.3% vs. 13.7%, p < 0.001). Although distribution of the Pruzansky-Kaban types differed significantly in patients with UHFM (I, 53.0%; IIa, 18.6%; IIb, 24.7%; III, 3.7%; p < 0.001), no difference was observed in occurrence between the right and left sides (52.6% vs. 47.4%, p > 0.05). Among patients with BHFM, prevalence of different Pruzansky-Kaban types on the right and left sides was greater than that of the same type on both sides (67.6% vs. 32.4%, p < 0.05). Despite hypoplasia of the condyle/ramus complex, compensatory growth of the mandibular body on the ipsilateral side occurred in 35 patients (14.1%). Class I and II molar relationships were more prevalent than Class III molar relationships (93.2% vs. 6.8%, p < 0.001). Forty-eight patients (19.3%) had other anomalies, with 50.0% and 14.4% in the BHFM and UHFM groups (p < 0.001). Conclusions: Patients with HFM require individualized diagnosis and treatment planning because of diverse phenotypes and associations with other anomalies.