• Advances in pediatric surgery
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• v.1 no.2
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• pp.140-148
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• 1995

To study the clinical profiles and outcome of surgery in infants with esophageal atresia, we reviewed 96 esophageal atresia cases who were treated from April, 1978 to June, 1995. There were 51 male and 45 female infants, a ratio of 1.1:1. The low birth weight(<2500g) patients were 32%. Clinical findings at the time of diagnosis included drooling in 57%, choking in 50%, cyanosis in 38%, respiratory distress in 27% and swallowing difficulty in 20%. Gross classification included 6 cases of type A esophageal atresia(6%), 79 cases of type C(82%), 3 cases of type E (3%) and 8 cases of type F(8%). Associated anomalies occurred in 34 infants(35%). Among them, cardiac anomalies were most common(60%). A primary repair of the defect was carried out in 76 patients with type A or C. A staged operation comprising a repair or gastric tube interposition after gastrostomy was performed in 8 patients. In all 3 infants with H-type, a division of fistula was performed. Esophageal resection and anastomosis was done in 8 infants with esophageal stenosis. In one infant, a gastrostomy was performed and he expired before staged operation. Anastomotic complications included leakage in 16 cases(17%), stricture in 37 cases(39%) and recurrent tracheoesopohageal fistula in 3 cases(3%). The mortality rate was 14% and the leading cause of death was pneumonia. The overall survival rate was 86%, and according to Waterston criteria, the survival rates were 93%, 85% and 58% in class A, Band C, respectively. 75 patients were followed up with median follow up 6.4 years. Among them, 93% were uneventful and 7% had frequent pneumonia.

• Tuberculosis and Respiratory Diseases
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• v.65 no.5
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• pp.430-434
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• 2008

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

• Journal of Chest Surgery
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• v.38 no.2 s.247
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• pp.164-167
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• 2005

Quadricuspid aortic valve is a rare congenital abnormality but it is well recognized as the cause of significant aortic regurgitation. We describe 5 patients who underwent surgery for severe aortic regurgitation associated with quadricuspid valve. In all patients, this abnormality had been incidentally detected during surgery. Two of the patients had infective endocarditis. In accordance with the Hurwitz and Roberts classification, two valves were type d, two were type a, and one was type c.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1503-1511
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• 2002

Purpose : Although the short- and long-term outcomes of low birth weight(LBW) neonatal intensive care unit(NICU) survivors have been extensively studied, much less information is available for normal birth weight(NBW) infants(greater than 2,500 gm) who require NICU care. Methods : We retrospectively examined the neonatal hospitalizations and one year health status of 302 NBW and 131 LBW admissions to our NICU. Information on the neonatal hospitalization was obtained from a review of medical records. Postdischarge health status was collected by using telephone surveys and medical records. Results : After initial discharge, 21.2% of the NBW infants and 23% of the LBW infants required rehospitalization during the first year of life and there was no significant difference between the two groups. The reasons for rehospitalization of the NBW infants included respiratory disorders (32.1%), G-I problems(26.2%), genitourinary problems(11.9%), surgery(10.7%), cardiac problems(7.1%), and congenital/developmental problems(1.2%). For the LBW infants, the order of frequency was the same, with the percentages slightly different. Neonatal risk factors related to the rehospitalization of the NBW infants included mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. But no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. Conclusion : Low and normal birthweight NICU survivors were rehospitalized at similar rates. The most common cause of rehospitalization was respiratory problems. Neonatal risk factors related to rehospitalization of NBW infants were mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. However, no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. The data suggests that NBW infant survivors, as well as LBW infant NICU survivors, require close follow up.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.766-771
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• 2003

It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. Truncus arteriosus is a rare congenital anomaly that has an unfavorable natural course. We report a successful surgical correction of truncus arteriosus in an 13-day-old premature infant with body weight of 1.5 kg and gestational age of 32 weeks. We used autologous untreated pericardial conduit without valve in right ventricular outflow reconstruction. The patients remains in good condition with normal body weight (50 percentile) and wide right ventricular outflow tract 20 months after the operation.

• Journal of Preventive Medicine and Public Health
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• v.17 no.1
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• pp.193-202
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• 1984

Clinical and statistical observations were performed on 1,930 cases of pregnant women who were admitted for delivery in the Department of Obstetrics, Kyung Hee University Hospital during 1 year (1982) and on 1,961 cases of neonates who were born to the former. The results were obtained as follows: 1. Concerning maternal age distribution, the commonest age group was that of $25{\sim}29$ and the proportion of the age group $20{\sim}29$ was 82.4% of all. 2. Concerning obstetrical history, the proportion of the women who had no prior experience of delivery nor abortion was the highest, 45.5%. 3. Concerning abortion history, 36.1% of the women had experienced it and the mean number was 1.8. 4. Type of delivery was as follows: Spontaneous delivery; 58.1%, Vacuum extracted delivery; 22.4%, Cesarean section; 18:8%, Breech delivery; 0.7%. 5. Gestational period distribution of the neonates was as follows: Under 37 weeks (Preterm); 7.1%, Between 38 and 42 weeks (Term); 87.2%, More than 43 weeks (Postterm); 5.7%. 6. Sex ratio of male to female of the neonates was 1.03:1. 7. Birth weight distribution was as follows: Under 2,500gm.; 9.0%, Between 2,501 and 4,000 gm.; 85.5%, More than 4,001gm.; 5.5%. 8. The measured growth data of neonates were as follows: Body weight; 3.28kg. for male, 3.18kg. for female, Body height; 50.40cm for male, 49.77cm for female, Chest circumference; 32.54cm for male. 32.17cm for female, Head circumference; 33.49cm for male, 33.11cm for female. 9. The mean values of Apgar score per 1 minute were 7.70 for male and 7.63 for female. 10. The incidence rate of neonatal jaundice was 50.0% and no difference in sex respectively, but more prevalent in preform baby. 11. The incidence rate of neonatal diseases was 8.9% and the commonest disease was neonatal infection (35.6%). 12. Concerning multiple pregnancy, ratio to single births was 1 : 64.3 and the sex ratio of male to female was 1 : 1.03. 13. The incidence rate of congenital anomaly was 2.4% and the commonest anomaly was digestive system anomaly (30.9%). 14. The neonatal mortality rate was 11.73 per 1,000 neonates, and the majority of neonatal deaths were in low birth weight and preform neonates (78.3%). 15. The causes of neonatal deaths in decreasing order of frequency were abnormal ventilation (39.1%), prematurity (30.4%), congenital anomaly (13.0%) and etc.

• Journal of Chest Surgery
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• v.42 no.2
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• pp.244-247
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• 2009

A left atrial appendage aneurysm is a very rare medical condition which can develop by an inflammatory reaction or a degenerative change. If there is no accompanying anomaly, a left atrial appendage is considered a congenital disease. The majority of left atrial appendage aneurysms are detected incidentally because they usually do not cause any symptoms. Surgery is indicated, even for asymptomatic patients, because of the risk of life-threatening complications, such as atrial fibrillation, supraventricular tachycardia, systemic embolization, and cardiac arrest. Left atrial appendage aneurysms are usually treated by a median sternotomy with extracorporeal circulation, especially if the aneurysm has a broad base or contains a thrombus, but can treated by thoracotomy without extracorporeal circulation. We report a case of a successfully treated left atrial appendage aneurysm that was misdiagnosed as a partial pericardial defect without extracorporeal circulation in a 13-year old child.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.3
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• pp.374-380
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• 2010

Treacher Collins syndrome(TCS) is a rare, incurable condition occurring in approximately 1 of 25,000 to 50,000 births. It may occur as a spontaneous mutation out of genetically normal parents or it may be manifested as an autosomal dominant pattern. TCS is characterized by facial deformities such as, underdevelopment of the maxilla, mandible and zygoma, malocclusion, bilateral deformities of auricles, antimongoloid slant of the palpebral fissures. The syndrome is often associated with cleft lip and palate, ear malformations and hearing loss, short stature, and anomalies of the heart and skeleton. Respiratory difficulty associated with air way obstruction may also be observed, and there is considerable difficulty in airway management during general anesthesia. It is necessary that dentists provide safe dental treatments and guidelines to TCS patients by providing adequate understanding about the characteristics of the syndrome and proper ways of managements. The purpose of this study is to report the dental and medical characteristics of the patient who visited the Department of Pediatric Dentistry, Yonsei University for multiple dental caries treatment and to review the literatures of TCS.

• Journal of Chest Surgery
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• v.32 no.1
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• pp.5-9
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• 1999

Background: Ebstein anomaly is a rare congenital disease distinguished by its unique deformity in tricuspid valve and right ventricle & atrium. In its surgical treatment , tricuspid valve reconstruction and valve replacement are well known method, but various surgical methods were suggested. Material and Method : From January 1984 to December 1995, 8 patients with Ebstein anomaly underwent surgical correction. Age and sex distribution, clinical symtoms, radiologic findings, preoperative studies, operative findings, operative methods and its results were analyzed. Result: The sex ratio was 5 to 3(male : female). Patients' ages were averaged 17.6(2-28) years. In all cases, it showed typical deformities of the tricuspid valve. Associated anomalies were permenant foramen ovale, atrial septum defect, pulmonary stenosis. Surgical procedures included tricuspid valve replacement(n=4) and tricuspid valve reconstuction(n=4). Two cases of sinus tachycardia and complete AV block occured postoperatively. There were two hospital death and no late death. All survivors are in NYHA class I or II with median follow up of 64.8 months. 2-D echocardiogram disclosed improvement tricuspid regurgitation during the follow up period. Conclusion: Even though operative method of Ebstein anomaly should be decided according to each anatomical characteristics, we recommended that tricuspid valvuloplasty and plication can be one of the good methods method in the selective cases.

• Journal of Chest Surgery
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• v.26 no.4
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• pp.320-324
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• 1993

Congenital cystic adenomatoid malformation [CCAM] of the lung is extremely rare. The patient was 10 year old female and had no specipic signs and symptoms except right lower chest pain for 5 days ago before admission.On simple chest X-ray and thoracic CT scan, about 9x8x8cm sized, heterogenous marginal enhanced multiseparated hypodence lesion with air fluid level and gas bubbles in posterior pleural space in right lower chest .The culture result of needle aspiration of cavity was apergillus flavus. Right lower lobectomy was carried out and the result of biopsy was congenital cystic adenomatoid malformation.