• The Journal of the Korean dental association
• /
• v.20 no.9 s.160
• /
• pp.753-758
• /
• 1982

선천이상 중에서도 순열과 구개열은 특상한 성질의 것으로서 안모의 변형, 발음장애, 신체타부와의 관계기형, 이비인후과질환을 병발하기 쉽고, 호흡기, 소화기 질환에 이환되기 쉬으며 환자는 정신적, 사회적으로도 매우 불리한 위치에 놓여 있는 신체장애자로, Fogh-Anderson은 순열과 구개열은 열성반성유전(recessive sexlinked heredity)을 한다고 보고했다. 그러나 구개열은 유전적추적이 불가능하기 때문에 그 발생원인으로 물리적원인을 우선적으로 들고있다. 발생기전은 태생 8~12조경의 제2차구개형성기에 어떤 이유로 양측의 구개돌기(palatine process)와 비중격이 분리되있는 채로 있든가, 접근해서도 유합되지 않고 있든가의 조해요인으로 태아의 모태내에 있어서의 자세, 특히 흉부에 의한 하악골압박에 의해 발생하는 소악증(micromandible), 설하수(glossoptosis)를 동반하는 Pierr Robin syndrome, 태생 8~10조경까지의 혀의 만기정유, 지, 제대의 원시구강내로의 미입등을 들수있다. 순열 및 구개열환자의 치료에 있어서 종래에는 전과정이 외과의사에게만 맡겨졌었으나, 구순, 비부의 추형등의 문제, 특징적으로 발생되는 하악의 열성장, 이별궁이 왜형, 발음장애등의 문제점해결을 위하여 필연적으로 이에 관계있는 명기다른 전문분야의 전문의가 시술에 임하게되는 multidiscipline approach로서 종합진단, 장기치료계획의 입안, 전문적 의견의 교환을 통해서 치료시기, 치료순서의 결정으로 성공적인 순열및 구개열의 치료목표를 달성하리라고 생각한다.

• Journal of Chest Surgery
• /
• v.32 no.7
• /
• pp.621-627
• /
• 1999

Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

• The korean journal of orthodontics
• /
• v.30 no.1 s.78
• /
• pp.43-52
• /
• 2000

Hemifacial microsomia ( HFM ) is the second most common craniofacial abnormalies. HFM represnted a spectrum of clinical findings such as hypoplasia of the mandibular ramus and condyle, confinement of maxilla growth, external and/or middle ear defects, involvement of some cranial suture, buccal soft tissue, facial nerve, and muscles in the affected side. HFM often showed progressive facial asymmetry and occlusal plane slanting to the affected side with growth. There were several reports about asymmetry of tooth maturation, hypodontia, delayed eruption, enamel hypoplasia in HFM. Since teeth develope in close association with size and morphology of the maxillary and the mandible, it is highly likely that dental changes will be present in HFM. So the Purpose of this study was to investigate the differences of the primary and the permanent teeth dimensions in the maxillary and the mandibular dentition between the affected and the non-affected side of HFM.. The sample of this study consisted of 34 unilateral HFM Patients (18 males and 16 females, average age : 5 year 11 months old). The authors examined the mesiodistal and the faciolingual dimensions of the primary and the permanent teeth and performed statistical study by using paired t-test. The results were as follows 1. The mesiodistal dimensions of the mandibular second primary molar and the mandibular first permanent molar in the affected side of HFM were significantly smaller than those of non-affected side. But there were no significant differences in the anterior teeth and the mandibular first primary molar. It means that a gradient of severity from anterior teeth to posterior teeth was found in the mandibular dentition. 2. Although there were no significant differences in the faciolingual dimensions of the primary and the permanent teeth in the maxillary and the mandibular dentition between the affected and non-affected side of HFM, there were general trend of compensatory increase in faciolingual dimension of the mandibular primary and the permanent teeth in the affected side Therefore these results showed that HFM might affect on the abnormality of tooth dimension, especially the most posterior teeth, in the affected side of the mandible.

• Journal of Chest Surgery
• /
• v.32 no.4
• /
• pp.341-346
• /
• 1999

Background: Thirty children ranging from 3 to 15 years of age underwent cardiac valve replacement at Dongsan Medical Center from 1982 to 1997. Material and Method: There were 16 boys and 14 girls. The mean age was 12.1. The underlying pathological cause for valve replacement was congenital heart disease in 17 children and acquired heart disease in 13. The valve replaced was mitral in 15 children, aortic in 11, tricuspid in 3, and combined aortic and mitral in 1. Twenty-one mechanical and 10 tissue valves were placed: primary mechanical valve have been utilized since 1985. Eight of ten patients with tissue valves have had successful second valve replacements 4 to 11 years after the initial operation. Result: The operative mortality was 6.7%, but mortality was higher among patients less than 5 years of age and patients who had previous cardiac operations. Of the 28 operative survivors, 4 patients were lost to follow-up: the remaining patients were observed for a total of 2091 patient/months(mean 74.7 months, maximum 187 months). There was one late death from dilated cardiomyopathy after mitral valve replacement in 7 year-old patient with atrioventricular septal defect. After the operation, all patients with mechanical valves were placed on a strict anticoagulant regimen with Coumadin. The actuarial survival rate was 96% at the end of the follow-up. No instance of thromboembolism or major bleeding were observed in the survivors. Conclusion: These results indicate that valve replacement can be performed with low mortality in children, and with satisfactory long-term survival.

• The korean journal of orthodontics
• /
• v.31 no.1 s.84
• /
• pp.63-69
• /
• 2001

Cleft lip and/or palate(CLP) is the most common congenital deformity in the craniofacial region. For a practical public health system for these patients it is necessary to have an understanding on the prevalence of CLP. However, it was difficult to estimate the exact number of CLP patients due to problems in sample selection, higher miscarriage and still birth rate, difficulty in classification, and adoptions to foreign countries. Therefore this study was to estimate the prevalence of CLP and the rate of orthodontic treatment, which is usually necessary in cleft lip and/or palate patients. The samples consisted of 218,322 Korean male adults from Seoul, Kwangju, Taegu and Pusan, all born in 1979. The screening method for recognizing the CLP patients was proceeded in steps and the results are as follows. 1. The prevalence of cleft lip and/or palate in Korean male adults born in 1979 was 0.65 out of the 1000 samples. 2. In the anteroposterior aspect of the 1000 samples, the prevalence of cleft lip, cleft lip and palate, and cleft palate was 0.26, 0.36 and 0.03 respectively. 3. In the transverse aspect of the 1000 samples, the prevalence of left, right and bilateral cleft was 0.35, 0.16, 0.12 respectively. The cleft in the left showed a much higher prevalence than in the rirht, while bilateral cleft showed a lower prevalence than unilateral cleft. 4. The orthodontic treatment rate of Korean male adults among cleft lip and/or palate Patients was $35\%$, and it was in the order of cleft lip and Palate, cleft lip, and cleft Palate, being $67\%,\;29\%\;and\;29\%$ respectively. The orthodontic treatment rate in patients with the more severe cleft lip and palate was higher than in patients solely having cleft lip or cleft palate.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.40 no.3
• /
• pp.209-215
• /
• 2013

Cleft lip and palate, the most common craniofacial anomalies, are severe congenital defects that have an incidence of 0.28 to 3.74 per 1000 live births. Although there has been great improvement in the field of cleft surgery, surgical approach cannot be the single solution to resolve the various problems encountered in patients with cleft lip and palate. The concept of presurgical infant orthopedics (PSIO) for gradual closure of the cleft gap and simplified surgical performance was first introduced by McNeil in 1950. Recently, there are many attempts not only to approximate the alveolar segments but also to reshape the nasal cartilage. Three infants with unilateral cleft lip and palate were referred from the department of Plastic Surgery for presurgical nasoalveolar molding (PNAM). Maxillary appliances using resin with orthodontic wire were fabricated. Then these appliance was applied until patients underwent lip surgery. In all cases, the patients could wear the appliance all day since they were able to eat even with the appliance on, This resulted in significant improvements in the nasal symmetry were found. Our appliance, namely K-NAM, extends the wearing time within the limited period and as a result it is expected to maximize the treatment effects. Used properly, this appliance would play a major role in enhancing nasal symmetry with satisfactory results.

• Journal of Chest Surgery
• /
• v.38 no.6 s.251
• /
• pp.403-409
• /
• 2005

Pulmonary atresia with intact ventricular septum is morphologically heterogeneous, and the surgical outcome remains suboptimal compared to other complex congenital heart defects. We evaluated the long-term results for repair of pulmonary atresia with intact ventricular septum, Material and Method: Between January 1992 and June 2004, 38 patients underwent repair of pulmonary atresia with intact ventricular septum. The average age was 18 days $(2\~382\;days)$. The average Z-value of the tricuspid annulus diameter was -3.1$(-5.6\~0.8)$. Thirteen $(36\%)$ patients had right ventricle-to-coronary artery fistulas, and $4(11\%)$ patients had right ventricle-dependent coronary circulation. Average follow-up was 55 months $(3\;months\~2.2\;years)$, Result: Twenty-four patients under-went initial right ventricle (RV) decompression and 14 patients underwent systemic-to-pulmonary arterial shunt only. The average size of the tricuspid annulus of the patients who underwent RV decompression was significantly larger than that of the patients who underwent systemic-to-pulmonary arierial shunt only (Z-value -2.2 vs. -4.8, p=0.000). There were $5(13\%)$ early and 1 late deaths. Early deaths occurred in 3 patients who had undergone RV decompression, and in 2 patients who had undergone systemic-to-pulmonary arterial shunt only (p=1.0). Biventricular repair was achieved in $12(32\%)$ patients, single ventricular repair in $8(21\%)$, and one and a half ventricular repair in $4(l1\%)$ patients. Nine $(24\%)$ patients are waiting for the definitive repair. Kaplan-Meier survival at 5 and 8 years was $83.2\%$, respectively. Conclusion: Most of the deaths occurred after the initial palliation. Overall long-term survival was satisfactory. Early mortality should be reduced with careful preoperative evaluation and proper surgical strategy.

• The korean journal of orthodontics
• /
• v.30 no.5 s.82
• /
• pp.543-552
• /
• 2000

The purposes of this study were to evaluate the reproducibility of posteroanterior(PA) cephalograms obtained by two methods, the Head Posture Aligner(HPA) method in natural head posture and the conventional method(operator-guided method), and to compare the vertical rotational differences of the head Posture between lateral and PA cephalograms according to the method. The sample was consisted of 30 adults. At first day, a PA cephalogram and a lateral cephalogram were obtained from each subject by two methods to investigate the difference of vertical rotational posture between lateral and PA cephalograms. Two weeks later, another PA cephalogram was obtained using each method to evaluate the reproducibility of head posture. Five height measurements and nine width measurements were used in the paired t-test to compare the reproducibility of the PA cephalometric measurements between two methods. The differences of vertical rotational posture between lateral and PA cephalograms were calculated from a computer program and compared according to the method used, and following results were obtained. 1. Height measurements obtained by operator-guided method showed significant differences according to the time interval and revealed low reproducibility. 2. Height measurements obtained by HPA method did not show significant differences according to the time interval and presented high reproducibility. 3. In the comparison of width measurement, two methods did not show distinct differences in reproducibility. 4. The difference of vertical rotational posture between lateral and PA cephalograms showed $0.8^{\circ}$ in the HPA method, more less than $2.5^{\circ}$ in the operator-guided method. The results of the present study suggest that the HPA may be helpful in the PA cephalometric radiography in terms of reproducibility.

• Clinical and Experimental Pediatrics
• /
• v.53 no.2
• /
• pp.248-252
• /
• 2010

Anomalous origins of coronary arteries are a rare type of disease among children. These anomalies can be categorized into 3 types according to the anatomical relationship of the aorta and pulmonary trunks. Among these types, the interarterial type, as observed in our case, needs early diagnosis and treatment, because it can increase the risk for the patient, causing sudden cardiac death in young individuals. Although there are controversies concerning the management of anomalous origins of the left coronary artery (LCA) in children, the result can be very beneficial, if treated accurately. Three well-known methods for correction of anomalous origins of LCA are re-implantation, coronary arterial bypass grafting (CABG), and unroofing. We report on the case of a 12-year-old girl who had chest discomfort and syncope with physical exercise and was later diagnosed with an anomalous origin of LCA by transthoracic echocardiography (TTE) and heart computed tomography (CT). She underwent a corrective operation by re-implantation, CABG, and unroofing.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.13 no.1
• /
• pp.44-52
• /
• 1991

Internal measurement technique has been commonly and classically used to guide down-fractured maxilla by Le Fort I osteotomy into its new position during intraoperative procedure for correlating preoperative model works with surgery. However, It has been challenged now by several authors due to some problems as its inaccuracy in three-dimensional changes at surgery, difficulty to measure during surgery and impossibility of rechecking at the end of surgery etc. The purpose of this study was to evaluate the accuracy of maxillary movement by external measuring technique and to determine its accuracy between the prediction tracing and a new maxillary position. The results indicate that the external measuring technique was predictable in the vertical, horizontal and transverse change of the maxilla as its prediction, however, it has a tendency to shift the maxilla more anterior and inferior in overall direction than prediction. Post-operative canting difference were mimic, however Ehange of the maxillary dental midline was large and had a right-shifting tendency.1 The precise methods to keep maxillary dental midline as same as prediction and the avoidance of uneven force applied to the mandible for autorotation should be necessary during surgery in use of external measurement technique.