• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.11 no.1
• /
• pp.5-8
• /
• 2015

Neuroblastoma is a common malignant tumor of the sympathetic nervous system in childhood, arises from embryonic neural crest cells. The period of tooth development is matched with peak times of diagnosis and treatment of neuroblastoma. The intensive multimodality treatment including radiotherapy and chemotherapy is used in patients with neuroblastoma has been shown to have late adverse effects and disturbances in dental development like tooth agenesis, microdontia, enamel hypoplasia and short roots. A 8-year old girl had been on medication and radiotherapy for neuroblastoma since she was 15 months old at Department Pediatrics, Chonnam National University Hospital. Oligodontia, microdontia, and short root formation was notable in clinical and radiological examination. Mobility of lower permanent incisor was detected and measured at about degree 2. Resin wire splint using mini-screw implantation on buccal alveolar bone was conducted for maintenance of mandibular incisors and alveolar bone. Excessive mobility has been eliminated and maintained well so far. Further treatment is planned for re-evaluation of mobility, preventing dental caries and regular oral hygiene management. Although we need further evaluation, this treatment could be one of alternative therapy for those who have similar dental anomalies.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.6 no.2
• /
• pp.112-115
• /
• 2010

Chemotherapy and radiotherapy proved conservative and effective in treating tumors. However, both the cancer therapies will also have aberrant effects on developing maxillofacial and dental organs of children. The purpose of this report is to describe the clinical cases of Disturbances of maxillofacial and dental development after Cancer therapy. The first case reported a 7-year-old female patient diagnosed at age 2 years with bilateral retinoblastoma, receiving chemotherapy and radiotherapy. She had agenesis of premolar, microdontia, short tapered teeth in lower anterior area and generalized root stunting. The second case presented a 12-year-old female patient treated with chemotherapy and radiotherapy for neuroblastoma in her early childhood. She presented with a class III malocclusion on a skeletal III base due to maxillary retrognathism. Contemporay oncology had improved survival of children with malignant disease. It will be needed prevention of these side effect after cancer therapy to improve the quality of life.

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

• Imaging Science in Dentistry
• /
• v.54 no.1
• /
• pp.13-24
• /
• 2024

Purpose: Pycnodysostosis (PYCD), an autosomal recessive syndrome, is characterized by an imbalance in bone remodeling that produces various clinical and radiographic craniofacial manifestations. This review represents a systematic examination of these manifestations, as well as oral features associated with PYCD. Materials and Methods: A systematic review was conducted across 8 databases from February to March 2023. The search strategy focused on studies reporting cases of PYCD that examined the clinical and radiographic craniofacial and oral characteristics associated with this syndrome. Results: The review included 84 studies, encompassing a total of 179 cases of PYCD. More than half of the patients were female (55.3%), and the mean age was 14.7 years. Parental consanguinity was reported in 51.4% of the cases. The most common craniofacial clinical manifestation was a prominent nose, observed in 57.5% of cases. Radiographically, the most frequently reported craniofacial characteristics included the presence of an obtuse mandibular angle (84.3%) and frontal cranial bosses(82.1%). Clinical and radiographic examinations revealed oral alterations, with micrognathia present in 62.6% of patients and malocclusion in 59.2%. Among dental anomalies, tooth agenesis was the most commonly reported, affecting 15.6% of patients. Conclusion: Understanding the clinical and radiographic craniofacial features of PYCD is crucial for dental professionals. This knowledge enables these clinicians to devise effective treatment plans and improve patient quality of life.

• The korean journal of orthodontics
• /
• v.41 no.5
• /
• pp.346-353
• /
• 2011

Objective: The purpose of this study was to investigate the prevalence of dental anomalies in outpatient clinics. Methods: The subjects of this study were 3,133 patients who visited the clinic between January 2009 and June 2011. The dental records and panoramic films of the patients and detection of supernumerary, missing, and impacted teeth, transposition, and peg lateralis were reviewed. The results were analyzed according to gender and types and locations of dental anomalies. Results: Among the patients, 362 had dental anomalies, with a prevalence rate of 11.55%. Congenital missing teeth (5.71%) ranked first in the categories, and impacted teeth (3.09%) ranked second. The percentage of patients having supernumerary teeth, peg lateralis, and dislocated teeth were 1.79%, 1.66%, and 0.45%, respectively. Conclusions: Congenital missing teeth and impacted teeth are, respectively, the first and second most common dental anomalies in Korean dental patients.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.50 no.4
• /
• pp.373-384
• /
• 2023

This study aims to confirm the average chronologic age according to the developmental stages of the mandibular canine (L3), first and second premolars (L4, L5), and second and third molars (L7, L8) in children and adolescents, and to confirm the developmental stage of L3, L4, L5, and L7, which can estimate the development of L8. A total of 1,956 digital panoramic radiographs of healthy individuals aged between 6 and 15 years who visited Seoul National University Dental Hospital from January 2019 to December 2020 were selected. The developmental stages of L3, L4, L5, L7, and L8 on both sides were evaluated using the dental maturity scoring system proposed by Demirjian and Goldstein. The average age at which the follicle of L8 was first observed was around 9.34 ± 1.35 years and varied from 6 to 12 years. The possibility of agenesis of L8 was high when no traces of L8 were observed after the following stages: L3, L4, and L5 at the developmental stage F and L7 at the developmental stage E; the age was about 10 years. In estimating the development of L8, when only one tooth was considered, estimation accuracy with L5 was the highest, and there was no significant difference when all four teeth were included. This study showed the age distribution according to the developmental stages of L3, L4, L5, L7, and L8 in children and adolescents and confirmed the developmental stages of L3, L4, L5, and L7, which can be used to estimate the development of L8.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.26 no.3
• /
• pp.486-491
• /
• 1999

Hypoparathyroidism has abnormally decreased secretion of parathyroid hormon which responds to the blood calcium level. Wherease, Pseudohypoparathyroidism has normal activity of thyroid hormon, but end-organs, such as urinary tract and osteoclast, do not respond to parathyroid hormon. The cause of this disease is due to the mutation of Guanine stimulating(Gs) protein regulating Gs gene, which is the receptor to this hormon. Pseudohypoparathyroidism is usually noted before 20 years old on average of 8-9 years old. The clinical features of this disease includes delayed growth and development, round face, obesity, soft tissue calcification, ectopic ossification, shortening of metacarpals and metatarsals by epiphyseal closure in advance of age. The mutation of Gs gene which are found in brain, endocrine organs, and chondrocytes is the cause of those features. Reaction to Glucagon, gonadal hormon, and thyroid stimulating hormon is not expected in both cases. The common dental manifestations include enamel hypoplasia, delayed eruption, agenesis of tooth, hypodontia, dysplastic short roots, widened pulpal space, microdontia, intrapulpal calcification, and malocclusion are also often reported. This case which is diagnosed to Pseudohypoparathyroidism showed short and under-developed root of permanent troth, delayed eruption, and non-eruption of premolars and molars. And morphogenesis imperfecta of first and second premolars were also found.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.43 no.3
• /
• pp.313-319
• /
• 2016

Wolf-Hirschhorn syndrome (WHS), associated with the deletion of the short arm of chromosome 4, causes multiple congenital malformations. Patients suffer from various deformities, including mental and growth disorders, epilepsy, hypotonia, congenital heart defects, and atypical craniofacial features. The "Greek warrior helmet appearance" is the most characteristic feature, with a prominent glabella, high arched eyebrow, broad nasal bridge, and hypertelorism. Cleft lip with or without cleft palate is observed in 30% of patients. Dental structure anomalies also exist including multiple tooth agenesis and over-retained primary molars caused by MSX1 gene impairment, and cone-shaped and taurodontic teeth. This case, a 9-year-old girl with WHS, showed intellectual disability, delayed growth development, previous occurrence of seizures, otitis media, and the typical facial features of WHS. Dental findings included multiple congenital missing teeth, over-retained primary teeth, and severe caries on the primary molars. Dental treatments were performed under general anesthesia. This report documents the characteristics of WHS, including general and oral features, and discusses the importance of oral hygiene and preventive dental management.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.15 no.1
• /
• pp.65-69
• /
• 2019

Wolf-Hirschhorn syndrome(WHS) is a congenital disorder caused by deletions of the short arm of chromosome 4. The most common characteristics are mental and growth retardation, dietary disorder and craniofacial features with a characteristic 'Greek warrior helmet' appearance. The dental characteristic of WHS includes delayed development, tooth agenesis, clefts, microdontia, taurodontism, and severely worn dentition. The purpose of this case report is to describe the dental treatment of a patient with WHS. 3-year-old boy with WHS visited the Seoul National University Dental Hospital for dental treatment. He had difficulty with nasotracheal intubation because of craniofacial anomalies and also had poor oral hygiene due to a limitation of mouth opening and dietary disorder. Due to his airway problem, behavior management and severity of dental conditions, dental treatment was performed under general anesthesia. This case suggests general anesthesia can be chosen with WolfHirschhorn syndrome patients to safely care for their dental problems.