• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.190-194
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• 2010

Purpose : To investigate the nature of deviant voice physiology in preoperative children with congenital heart disease. Methods : Ninety-four children with congenital heart disease were enrolled. Their cries and related acoustic variables (fundamental frequency, duration, noise to harmonic ratio, jitter and shimmer) were analyzed using a multi-dimensional voice program. Results : The average fundamental frequency showed a significant decrease in patent ductus arteriosus, ventricular septal defect, and tetralogy of Fallot, except in atrial septal defect and pulmonary stenosis. The length of the analyzed sample (duration) did not show a significant difference when compared with the control group. There was a significant increase in jitter percent in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. There was an increase in shimmer in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. The noise-to-harmonic ratio increased in ventricular septal defect, patent ductus arteriosus, and atrial septal defect but there was no significant difference in pulmonary stenosis and tetralogy of Fallot. While analyzing acoustic variables, the voice change was significantly higher, especially in patent ductus arteriosus followed by ventricular septal defect and atrial septal defect. Most of these acoustic variables were deviant in left-to-right shunt lesions in congenital heart disease, especially in patent ductus artriosus. Conclusion : The results of the voice change analysis of preoperative children with congenital heart disease revealed that the acoustic variables differed by each congenital heart disease. Moreover, the acoustic variables were prominently deviant in congenital heart disease with left-to-right shunts.

• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.1
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• pp.122-130
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• 2006

Congenital Missing Teeth(hypodontia, oligodontia) is the developmental absence of one or more teeth. It has been reported as being the most common anomaly of dental development in human, relatively common in the permanent dentition. In a recent review, Vastardis has quoted incidence ranges of $1.6%{\sim}9.6%$ in the permanent dentition. Brook has quoted a prevalence of $3.5%{\sim}6.5%$ in most populations, with severe hypodontia, defined as the absence of six or more teeth, having a prevalence of $0.3{\sim}0.4%$. The most commonly affected teeth are third molars, followed by maxillary lateral incisor, and second premolars. The etiology is unknown, several hypotheses include trauma, nutritional deficiency, infection, metabolic abnormalities, systemic disease and genetic influence. The multiple congenital missing is commonly associated with specific syndrome or severe systemic abnormalities such as cleft lip & palate and Down's syndrome. These cases present that children have multiple congenital missing teeth in the permanent dentition, without any systemic disease. Management of this condition must be considered orthodontic and prosthodontic treatment comprehensively. In these cases, children were treated by space maintainer or orthodontic appliance and follow-up checked.

• Journal of the korean academy of Pediatric Dentistry
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• v.44 no.1
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• pp.56-63
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• 2017

Delayed eruption of the first molar, without a generalized or localized cause, is usually associated with delayed development of the affected tooth. The aim of this study was to investigate the clinical features of the first permanent molar showing delayed development and eruption, and its association with developmental anomalies of other teeth. Panoramic radiographs of 40 healthy children showing delayed development and eruption of first permanent molars were analyzed. The clinical features of affected first molars and developmental anomalies of other teeth (except third molars) were evaluated. Delayed first molars were more frequent in the maxilla. The incidence of bilateral delayed development of first molars was greater than that of unilateral cases in female patients. In contrast, male patients showed unilateral delayed development of the first molar more frequently. A higher incidence of congenitally missing teeth was observed in patients with delayed first molar. In each case, delayed development or congenital absence was observed in the second molar adjacent to the delayed first molar. Overall, delayed first molar seems to be associated with congenital absence of additional teeth. Understanding the developmental mechanisms of this phenomenon requires further studies.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.1
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• pp.91-96
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• 2010

Many studies have shown that there is a strong relationship between the congenitally missing primary teeth and their succedaneous permanent teeth. However, especially in case of lower anterior region, we can observe the existence of permanent teeth even though their precedent primary teeth were missed at times. The purpose of this study was to reveal the relationship between the congenitally missing primary lower anterior teeth and their succedaneous permanent teeth in Korean children. Total of 14,307 children, under 6 years of age, who attended the department of pediatric dentistry, Seoul National University Dental Hospital, Seoul, Korea from January 1st 2005 to September 5th 2008 were radiologically examined using the panoramic x-ray in order to analyze the relationship between the congenitally missing primary lower anterior teeth and their succedaneous permanent teeth. The results were as follows : 1. The prevalence of congenitally missing lower anterior teeth in primary dentition was 0.24%. The odds ratio of both the primary and permanent anterior teeth being present was 7163.5 and only the permanent anterior teeth being present was 0.79. 2. The percentage of all succedaneous permanent teeth being present was 44.12% in cases of missing lower primary anteriors. The odds ratio of at least one succedaneous permanent teeth being present in cases of bilateral primary anterior teeth missing was 1.57 times more common than in cases of unilateral primary teeth missing. 3. The odds ratio of primary missing teeth being unilateral was 2.2 times higher in females. Moreover, the odds ratio of succedaneous permanent teeth being present in cases of primary teeth missing was 2.22 times higher in males.

• Journal of the Korean Society of Radiology
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• v.82 no.3
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• pp.749-755
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• 2021

Congenital defects of the pericardium, which are generally asymptomatic, are rare disorders characterized by complete or partial absence of the pericardium. Here, we report a rare case of a 19-year-old male who was incidentally diagnosed with congenital absence of the left pericardium during examination for symptoms of pneumothorax. Chest radiography and CT revealed a collapsed left lung without any evidence of trauma, no unusual findings of free air spaces along the right side of the ascending aorta, heart shifted toward the left side of the thorax, and a shallow chest. Subsequent thoracoscopy confirmed the absence of the left pericardium and displacement of the heart toward the left thoracic cavity. We further discuss the correlation between radiologic images and surgical findings of a congenital pericardial defect associated with spontaneous pneumothorax.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.584-587
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• 1999

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.2
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• pp.245-252
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• 2009

The congenital missing of teeth is common, which takes place since the proliferation and differentiation are not allowed in that tooth bud fail to start development. The purpose of this study is to research incidence rate, number, and missing part of congenital missing teeth, and to study whether a person who has missing teeth has other abnormality of teeth or not. For this study, 1,520 subjects(aged 2.9$\sim$17) who had visited pediatric dentist department of Chonbuk national university dental hospital within 2 years were examined with an panoramic radiograph; exempting third molar missing state. The obtained results are as follows. 1. 8.88% among total subjects show missing teeth; male 9.05%, female 8.64% 2. The most frequently missing permanent teeth were the mandibular second premolars(22.3%). The most frequently missing primary teeth are mandibular lateral incisors(50%). 3. 43.3% patients have one permanent missing tooth, 34.3% have two, and 10.4% have more than six, respectively. In primary teeth, 86.7% patients have one missing tooth, and 13.3% have two missing teeth. 4. 18 patients(13.3%) have missing teeth as well as hyperdontia, while some patients have microdont, ectopic eruption, and fusion teeth.

• Neonatal Medicine
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• v.18 no.2
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• pp.359-364
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• 2011

Purpose: Aplasia cutis congenita (ACC) is a rare condition characterized by a localized absence of skin, and it can be associated with other congenital anomalies. This study was done to evaluate clinical course and outcome of ACC in neonates. Methods: Based on the medical records, we retrospectively reviewed 8 neonates diagnosed with ACC at Cheil General Hospital and Women's Health Care Center, Kwandong university College of Medicine from January 2004 to December 2010. We classified ACC by Frieden's classification and analyzed the patient's demographic data, clinical course and outcome. Results: Among 8 patients with ACC, 5 patients were classified to group 1; scalp ACC without anomalies and 3 patients to group 7; ACC localized to extremities without blistering. Defect size was from 0.3 cm to 1.5 cm, limited in the superficial skin or subcutaneous tissue without associated anomalies. Defects were healed under conservative treatment with mild scar formation within four months. Conclusion: ACC could be diagnosed easily through physical examination in neonates. All cases showed good clinical outcome without surgical treatment. However because of small numbers and small sized defects of cases, further study including lesions of large size is needed.

• Journal of Chest Surgery
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• v.31 no.8
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• pp.770-775
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• 1998

Background: Atrial septal defect (ASD) is the most common congenital cardiac anomaly, accounting for 30 percent of congenital heart disease detected in the adult. Many patients with ASD are well tolerated and reach adult without significant symptoms. The patients with ASD die 4th and 5th decades, but prolonged survival is not uncommon. In general, the survival depends on whether pulmonary hypertension develops during adulthood or not. The most common cause of death in the patients with ASD is right ventricular failure or arrhythmias. Materials and methods: From January 1988 to June 1997, 33 cases of ASD underwent open heart surgeries in our hospital. Among them, 31 cases were adult ASD, and 2 tricuspid regurgitation, 1 pulmonic stenosis, 1 mitral regurgitation, 1 tricuspid regurgitation, and 1 coronary artery disease were combinded. All of the patients underwent surgical repair using autologus pericardial patch or direct closure. Results: The postoperative course was smooth and uneventful. Most of the patients showed significant improvement in ECG finding, hemodynamic profile, radiologic finding, and echocardiography, after surgery. Conclusions: Conclusively, most of the ASD should be closed even in patients over the age of 60 years, and early surgical repair must be done to prevent pulmonary hypertension, right ventricular failure, and arrythmias.

• Archives of Craniofacial Surgery
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• v.9 no.2
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• pp.90-92
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• 2008

Purpose: Congenital upper eyelid defect is rare anomaly whether it is compared with syndromic anomaly or not. It has many clinical manifestation in the extent, location. Many operation procedures such as simple closure, semicircular rotation flap, Cutler Beard procedure, lower lid rotation flap, etc can be used to reconstruct eyelid defects. We intend to introduce a simple, congenital eyelid defect which was not compared with syndromical anomaly, ophthalmic complication. Methods: Our experience is a case of 19 years old female who had a upper eyelid coloboma without any other anomalies. we could not find any skeletal deformity in orbital CT scan. she had no ophthalmic problem. we reconstructed the defect with bilateral marginal flap after deepithelization of supramarginal area and tarsal reposition. Results: There were no visible deformity of lid lining. postoperative scar was favorable. satisfactory results were obtained in cosmetic and functional aspects. Conclusion: Upper lid coloboma without other anomalies is rare. we obtained satisfactory outcome as treated this rare case with marginal flap advancement.