• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.1
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• pp.51-56
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• 2002

Russell-Silver syndrome is a type of intrauterine growth retardation, characterized by short stature noted at birth, hemiatrophy or asymmetry, variation in sexual development and other abnormalities, including cafe-aulait pigmentation and clinodactyly. Facial features commonly associated with this syndrome are a small triangular face, decreased facial height, down-turned corners of the mouth(shark's mouth), a small mandible, and occasionally asymmetry. The major intra-oral features of the syndrome that have been reported are a high-arched palate, delayed tooth eruption, microdontia, hypodontia, and crowding. These cases were diagnosed at birth as Russell-Silver syndrome by clinical features such as prenatal growth retardation, short stature, low body weight, et al., and have been treated with growth hormone. The purpose of this paper is to report the dental findings of two patients and review the pertinent literature through the two cases.

• Archives of Plastic Surgery
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• v.46 no.6
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• pp.511-517
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• 2019

Background Cleft treatment is frequently performed in Indonesia, mostly in charity missions, but without a postoperative protocol it is difficult to establish the risks and complications of cleft treatment. The present study was designed to give an overview of current cleft lip and palate treatment strategies in Indonesia and to assess the complication rates during and after surgery. Methods This prospective study evaluated anesthetic, intraoperative surgical, and short-term postoperative complications in patients undergoing primary, secondary, or corrective surgery for cleft lip and palate deformities. The population consisted of 98 non-syndromic cleft patients. The main anesthetic complication that occurred during general anesthesia was high blood pressure, whereas the main intraoperative surgical complication was excessive bleeding and the main early postoperative complication was extremely poor wound hygiene. Results In this study, there were no cases of perioperative or postoperative mortality. However, in 23 (23.4%) of the 98 operations performed, at least one perioperative complication related to anesthesia occurred. The intraoperative and early postoperative complications following cleft lip and/or palate were assessed. There was a significant difference in the complication rate between procedure types (χ²=0.02; P<0.05). However, no relationship was found between perioperative complications related to anesthesia and the occurrence of postoperative complications (χ²=1.00; P>0.05). Nonetheless, a significant difference was found between procedure types regarding perioperative complications and the occurrence of postoperative complications (χ²=0.031; P<0.05). Conclusions Further evaluation of these outcomes would help direct patient management toward decreasing the complication rate.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.291-294
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• 2003

Ring chromosome 21 causes a multitude of phenotypes, ranging from severe abnormalities to normal. The proposed mechanism of ring formation, breakage of both short and long arms of a chromosome with subsequent end to end fusion, remains unproven. We encountered a 4-year-old boy who presented developmental delay, microcephaly, micrognathia, hypertelorism, low-set ears, mild optic nerve hypoplasia, cleft lip and palate, scoliosis and left foot valgus, but normal brain MRI. Chromosome study from peripheral blood showed 46,XY, r(21)(p11.2q22.1) karyotype. The authors report the first case of ring chromosome 21 in Korea with a review of the literature.

• Journal of Interdisciplinary Genomics
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• v.3 no.2
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• pp.30-34
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• 2021

Campomelic dysplasia (CD) is a rare genetic disorder characterized by multiple skeletal anomalies and the abnormal development of male reproductive organs. To date, the SOX9 gene is the only known causal gene for CD, and approximately 90 causative mutations in SOX9 have been identified worldwide. CD is diagnosed based on clinical characteristics of skeletal dysplasia (e.g., short bowed long bones, kyphoscoliosis, bell-shaped thoracic cage with 11 pairs of ribs, and hypoplastic scapulars), typical facial features of Pierre Robin sequence with cleft palate, and gonadal dysgenesis in 46,XY individuals. Most patients with CD exhibit life-threatening respiratory failure owing to laryngotracheomalacia and hypoplastic thorax during the neonatal period. Although fatal complications decrease after infancy, several medical conditions continue to require proper management. A better understanding of this rare but lethal condition may lead to more appropriate treatments for patients.

• Archives of Craniofacial Surgery
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• v.23 no.1
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• pp.34-38
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• 2022

Severe temporal hollowing deformities can occur in patients who undergo craniectomy after intracranial hemorrhage. Reconstruction surgery for cosmetic purposes using silicone implants in patients with temporal hollowing deformities after craniectomy is advantageous because the procedure is simple and rapid, with a short recovery time, resulting in lower overall treatment costs. Of paramount importance, this option yields highly satisfactory results for patients. The authors present three cases of simple and fast surgery using silicone implants where highly satisfactory outcomes were obtained.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.7 no.2
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• pp.123-126
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• 2011

Noonan syndrome (NS) is a developmental disorder characterized by dysmorphic facial features in association with short stature, mental retardation and congenital heart disease. NS may be sporadic or inherited as an autosomal dominant or recessive trait. The children with NS usually have ocular hypertelorism, downslanting palpebral fissures, low-set ears and a webbed neck, chest deformity. In addition, oral features include micrognathia, high arched palate, dental malocclusion, dental anomalies and rarely, cleft palate. The phenotype of NS bears similarities to that of Turner syndrome. However, NS occurs in both males and females with a normal sex chromosome 46, XX and 46, XY constitution. This case presents the intermittent treatment of an 8-year-old girl who was referred from a local clinic for the extraction of supernumerary teeth and treatment of dental caries. The focus of this case report is the oral aspects on NS and particularities of the dental treatment in subjects affected by this genetic disease.

• The korean journal of orthodontics
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• v.53 no.2
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• pp.106-115
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• 2023

Objective: To evaluate the null hypothesis that there is no difference in a set of clinical predictors of potentially impacted canines between low-risk patients with and without displaced canines. Methods: The normal canine position group consisted of 30 patients with 60 normally erupting canines ranked in sector I (age, 9.30 ± 0.94 years). The displaced canine group comprised 30 patients with 41 potentially impacted canines ranked in sectors II to IV (age, 9.46 ± 0.78 years). Maxillary lateral incisor crown angulation, inclination, rotation, width, height, and shape, as well as palatal depth, arch length, width, and perimeter composed a set of clinical predictors, which were evaluated on digital dental casts. Statistical analyses consisted of group comparisons and variable correlations (p < 0.05). Results: There was a significant association between sex and mesially displaced canines. Unilateral canine displacement was more prevalent than bilateral displacement. The crown of the maxillary lateral incisors was significantly angulated more mesially and rotated mesiolabially in low-risk patients with displaced canines, who also had a shallower palate and shorter anterior dental arch length. Lateral incisor crown angulation and rotation, as well as palatal depth and arch length, were significantly correlated with the canine displacement severity. Conclusions: The null hypothesis was rejected. Maxillary lateral incisor angulation inconsistent with the "ugly duckling" stage as well as a shallow palate and short arch length are clinical predictors that can significantly contribute to the early screening of ectopic canines in low-risk patients.

• Journal of Veterinary Clinics
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• v.39 no.1
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• pp.1-8
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• 2022

The current, retrospective study aimed to assess the short-term prognosis and postoperative complications associated with the surgical correction of elongated soft palate using harmonic scalpel and to compare the postoperative complications associated with the application of harmonic scalpel and traditional surgery using Metzenbaum scissors. Harmonic scalpel was used to perform staphylectomy in 21 dogs. A total of ten dogs underwent sacculectomy; six dogs with harmonic scalpel and four dogs using Metzenbaum scissors. Stenotic nares were corrected by wedge resection. Postoperative complications were recorded through monitoring and radiographic examinations. Telephone interviews were conducted on the first, third, and seventh day after discharge and continued until the resolution of postoperative complications. Postoperative edema at the surgical site was identified and mitigated within a day or two. Snoring and dyspnea improved dramatically in the group that underwent staphylectomy alone. Moreover, three dogs presented with postoperative gastrointestinal complications, especially retching. The symptoms persisted for seven days and ten days in two dogs that underwent sacculectomy with harmonic scalpel and for two days in one dog that underwent sacculectomy with Metzenbaum scissors. The clinical signs and symptoms of brachycephalic syndrome disappeared without recurrence. Harmonic scalpel provides a hemostatic effect during staphylectomy, is convenient, and does not cause postoperative complications. Conversely, the use of harmonic scalpel during sacculectomy necessitated a longer period for the resolution of complications without any significant hemostatic efficacy, compared to traditional surgery.

• Archives of Craniofacial Surgery
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• v.17 no.1
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• pp.1-4
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• 2016

Absorbable plates are used widely for fixation of facial bone fractures. Compared to conventional titanium plating systems, absorbable plates have many favorable traits. They are not palpable after plate absorption, which obviates the need for plate removal. Absorbable plate-related infections are relatively uncommon at less than 5% of patients undergoing fixation of facial bone fractures. The plates are made from a mixture of poly-L-lactic acid and poly-DL-lactic acid or poly-DL-lactic acid and polyglycolic acid, and the ratio of these biodegradable polymers is used to control the longevity of the plates. Degradation rate of absorbable plate is closely related to the chance of infection. Low degradation is associated with increased accumulation of plate debris, which in turn can increase the chance of infection. Predisposing factors for absorbable plate-related infection include the presence of maxillary sinusitis, plate proximity to incision site, and use of tobacco and significant amount of alcohol. Using short screws in fixating maxillary fracture accompanied maxillary sinusitis will increase the rate of infection. Avoiding fixating plates near the incision site will also minimize infection. Close observation until complete absorption of the plate is crucial, especially those who are smokers or heavy alcoholics. The management of plate infection is varied depending on the clinical situation. Severe infections require plate removal. Wound culture and radiologic exam are essential in treatment planning.

• Journal of Genetic Medicine
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• v.9 no.2
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• pp.93-97
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• 2012

Kniest syndrome (OMIM #156550) is a rare autosomal dominant disorder caused by a dysfunction of type II collagen, which is encoded by the COL2A1 gene (OMIM +120140) mapped to chromosome 12q13.11. Type II collagen, a molecule found mostly in the cartilage and vitreous tissues, is essential for the normal development of bones and other connective tissues. Kniest syndrome is a type II collagenopathy that presents as skeletal abnormality associated with disproportionate dwarfism, kyphoscoliosis, enlarged joints, visual loss, hearing loss, and cleft palate. This report describes a Korean patient with Kniest syndrome who was diagnosed with typical clinical features and radiologic findings. The patient presented with disproportionately short stature and kyphoscoliosis from birth. A skeletal survey revealed fused lamina in the thoracic spine, hemivertebrae, flexion deformities in multiple joints, and plagiocephaly.