• Journal of the Korean Society of Radiology
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• v.81 no.5
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• pp.1083-1095
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• 2020

Since 2019, ultrasound and magnetic resonance imaging for evaluation of urogenital disorders have been covered by the National Health Insurance (NHI) in Korea. Patients with urogenital malignancies were already insured by NHI for ultrasound and MRI. With the expansion of NHI coverage, patients with suspected prostate or gynecologic cancer, uterine fibroids before myomectomy and some other benign disease such as congenital anomaly can receive benefits of NHI. In consideration of these changes, radiologists and other clinicians should be aware of the indications and standard images of each examination and the required reporting forms. Clinical application based upon thorough understanding of the NHI guidelines will aid in improving the standard care of patients.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.280-287
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• 2007

Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.554-560
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• 2003

Purpose : To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. Methods : 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. Results : The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. Conclusion : Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.464-470
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• 2009

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

• Journal of Chest Surgery
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• v.38 no.10 s.255
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• pp.699-704
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• 2005

Background: Coronary artery fistula is rare congenital anomaly, which account for $0.27\~0.40\%$ of all congenital heart diseases. We report the clinical observations of 45 patients with coronary artery fistula. Material and Method: We reviewed all patients presented with or without symptoms of coronary artery fistula between 1987 and 2004. Age ranged from 1 to 83 years. Twenty-six patients were female. The patients were divided into 2 groups according to the presenting symptoms. Twelve patients were in group A (asymptomatic) and 33 patients in group B (symptomatic). The most common clinical presentation in group B was angina (18) followed by dyspnea (7), atypical chest pain (5), syncope (1), fatigue (1), and palpitation (1). Twenty-five patients were associated with other cardiac diseases, which were atrial septal defect (4), coronary artery occlusive disease (6), hypertension (12), and valvular heart disease (2). Result: Patients were followed-up for a mean period of $64.8\pm62.7$ months. There was no complication related to coronary artery fistula during the follow-up period in both group. There was no mortality related to coronary artery fistula. Conclusion: In symptomatic patients, early surgical treatment is recommended considering the low perioperative morbidity. In asymptomatic patients receiving medical treatment, close follow up may be necessary.

• Clinical and Experimental Pediatrics
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• v.49 no.10
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• pp.1073-1078
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• 2006

Purpose : This study was performed to assess how a fetal diagnosis of congenital heart disease affects parents, as regards pregnancy management and care of infants after birth. Methods : Database search to find out abnormal fetal echocardiography performed at Seoul National University Children's Hospital from July 1988 to June 2003 revealed 370 examinations. After excluding both arrhythmias without structural cardiac disease and multiple pregnancies, 299 pregnancies remained and this data formed the basis of this analysis. We retrospectively reviewed the medical records with special attention to pregnancy outcomes and also tried to find out factors influencing parental decisions on whether to continue or terminate pregnancy. Results : In this study, the mean gestation age at diagnosis was $28{\pm}6.0weeks$. The mean age of mothers was $30{\pm}3.9$ years old. Younger gestational ages at diagnosis(P=0.000), more severe grades of fetal heart disease(P=0.002) and younger mothers(P=0.014) correlated with terminations of pregnanies. But the grades of fetal status, the grades of associated anomaly, whether in-vitro-fertilization was carried out or not and numbers of previous children were not significant. Conclusion : This study found that the earlier gestational ages at diagnosis, younger maternal age and higher grades of fetal heart disease tended to lead parent to select abortions. Fetal echocardiographies were performed too late. Moreover Koreans have a biased view that malformation is a something incurable and a tragedy not only to oneself, but also to a family. So parents select terminations of pregnancy, even in curable cases. This is very unethical.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.870-874
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• 2006

Purpose : Popular use of fetal ultrasonography has increased to detect congenital hydronephrosis(CH) which is the most common anomaly prenatally detected. We'd like to determine the frequency and clinical characteristics of prenatally diagnosed CH and outcome of ureteropelvic junction stenosis(UPJS). Methods : The records of births between January 1994 and June 2003 in Chonnam National University Hospital(CNUH), and the records of children who were diagnosed with CH in the Department of Pediatrics of CNUH during the above period, were retrospectively analyzed. In the patients with UPJS, the initial anterior posterior diameters of renal pelvis(APD) were compared between the spontaneous regression (SR) and operation group(OP). In the SR group, sequential regression rates of APD were estimated. Results : Among a total 9,076 births, 231(2.54 percent) patients with 293 renal units were diagnosed as CH and 19(6.78 percent) renal units spontaneously regressed 3 days after birth. In 228 children(56 bilateral; 172 unilateral; total 284 renal units) diagnosed with CH in the department of pediatrics of CNUH, male(71.9 percent) and left kidney(69.2 percent) predilection were found and 78.1 percent of CH were caused by UPJS. The initial APD of the SR group(121 units) in UPJS was $7.8{\pm}6.28mm$, which was significantly smaller than the APD($26.8{\pm}12.14mm$) of the OP group(25 unit)(P<0.05). In the SR group, 81 percent spontaneously regressed within one year. Conclusions : In CH, male and left kidney predilection were found. UPJS was the most common cause of CH and initial APD in UPJS at 3 days of age was a good prognostic indicator. Close monitoring should be done for at least one year because most SR in UPJS regressed spontaneously within one year.

• Journal of Chest Surgery
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• v.37 no.10
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• pp.833-838
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• 2004

Background: Despite the excellent early results after the repair of congenital bicuspid aortic valve (BAV) disease, the mid-term durability of the repaired valve has still controversies. Material and Method: To evaluate the mid-term results of BAV repair, retrospective review of medical records and echocardiographic data were done. Between 1994 and 2003, twenty-two patients underwent reparative procedure for either regurgitant or stenotic congenital bicuspid aortic valve (BAV). Result: Mean age was $41\pm14$ years with male predominance (Male=17, Female=5). The pathophysiologies of the BAV were regurgitation-dominant in 20 (91%) and stenosis-dominant in 2 (9%) cases. Various repair techniques were used for raphe, prolapsed leaflet, thickened leaflet, and commissures; 1) release of raphe in 19 (86%), 2) wedge resection and primary repair in 11 (50%), pericardial patch reinforcement after plication of the leaflet in 6 (27%), and plication of the leaflet in 3 (14%), 3) slicing of thickened leaflet was used in 12 (55%) cases, 4) commissuroplasty in 8 (36%), and commissurotomy in 6 (27%) cases. There was no in-hospital mortality. During the mean follow-up of $38\pm17$ months, one patient underwent aortic valve replacement after developing acute severe regurgitation from dehiscence of the suture on postoperative 2 months. New York Heart Association functional class was improved from $1.9\pm0.6$ to $1.2\pm0.5$ (p<0.01). Left ventricular end-systolic and diastolic dimension (LVESD/LVEDD) were also improved from $45\pm9$ and $67\pm10$ to $37\pm10$ and $56\pm10,$ respectively (p<0.01). The grade of aortic regurgitation (AR) was improved from preoperative $(3.1\pm1.2)$ to post-bypass $(0.9\pm0.7).$ However, the grade at last follow-up $(1.7\pm1.1)$ was deteriorated during the follow-up period (p<0.01). Freedom from grade III and more AR at one, three, and four year were 89.7%, 89.7%, and 39.9% respectively. Conclusion: Midterm clinical result of the BAV repair was favorable. But, the durability of the repaired valve was not satisfactory.

• Advances in pediatric surgery
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• v.17 no.2
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• pp.154-161
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• 2011

Tracheoesophageal fistula without esophageal atresia (H-type TEF) is a congenital anomaly that is characterized by a fistula between the posterior wall of the trachea and the anterior wall of the esophagus, not accompanied by esophageal atresia. The purpose of this study is to investigate the clinical characteristics, diagnostic time, the side of cervical approach and short term result after surgery by searching medical records of patients treated for H-type TEF. The search was done at University of Ulsan, Department of Pediatric Surgery of Asan Medical Center, and the total number of patients from May 1989 to December 2010 was 9 with M:F ratio of 1:2. The median gestational age was $39^{+6}$ ($32^{+6}{\sim}41^{+0}$) wks. Seven out of nine patients were born at term and the other two were born premature. The clinical presentation was aspiration pneumonia, difficulty in feeding, chronic cough, vomiting, abdominal distension and growth retardation. The symptoms presented right after birth. The diagnosis was made with esophagography and the median time of diagnosis was 52 days of life. The majority of surgical corrections were performed within two weeks of diagnosis (median; 15d, range; 1d - 6m). Six patients had associated anomalies, and cardiac anomalies were most common. The cervical approach was utilized in all cases (right 2, left 7). Transient vocal cord palsy and minor esophageal leakage complicated two cases. Although the diagnosis of H-type TEF was difficult and often delayed, we had a good short term result. The left cervical approach was preferred.

• Korean Journal of Cleft Lip And Palate
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• v.2 no.1_2
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• pp.5-14
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• 1999

The aim of treatment of cleft lip and palate is to correct the cleft and associated problems surgically and thus hide the anomaly so that patients can lead normal lives. This correction involves surgically producing a face that does not attract attention, a vocal apparatus that permits intelligible speech, and a dentition that allows optimal function and esthetics. In neonatal periods, gross distortion of tissues surrounding the cleft requires considerable effort and time due to post operative functional defect and scarring and induces milk feeding problem, malocclusion of deciduous or permanent dentition, congenital missing teeth, skeletal dysplasia. The occurrence of a cleft deformity is a source of considerable shock to the parents of an afflicted baby, and the most appropriate approach is very important things. Thus we tried to analysis of dental arch, shape and size of deformity in cleft patients. The results were obtained as follows. 1. When the cast measurements of UCLP subjects at first visit it was found that the mean length was 9.29mm at the alveolar cleft width, also that was 11.7mm at the anterior width and 14mm at the posterior cleft width. 2. Comparison of UCLP group at first visit and just lip surgery, it was found that the older group showed a insignificant reduction in the width of the cleft in the alveolar, canine, and tuberosity regions. 3. The maxillary casts of the UCLP group at 6 months differ Significantly from those of the at 3 months in both length and width. but there was no statistical difference except anterior ridge length of nonclefted site. 4. Comparison at 6 months and 18 months, there was a greater change in length of the alveolar cleft width, intercanine width, and anterior cleft width. Maxillary arch became wider at both the canine region and intertuberosity region. also posterior anteroposterior length was increased but anterior AP length was decreased from 8.1mm to 7.7mm. There was meaningful increase at intertuberosity length; however, a significant reduction in width t-t'