• Korean Journal of Cleft Lip And Palate
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• v.5 no.1
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• pp.21-25
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• 2002

Cleft lip and palate are most common congenital deformity to affect the orofacial region. Cleft lip and palate are caused by abnormal development of primary and secondary palate. It's causative mechanism is not completely understood, but genetic and environmental factors play an important role. Many epidemiologic surveys have been done extensively about incidence, racial influence, sex ratio, parent age, associated syndrome, and genetic factors. These researches are useful to dissolve many problems in prevention and treatment of cleft lip and palate. We performed epidemiologic survey of cleft lip and palate who visited the department of Oral & Maxillofacial surgery, Guro Hospital of Korea University from 1995 to 2001.

• Journal of Chest Surgery
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• v.29 no.4
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• pp.466-471
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• 1996

Esophageal atresia with tracheoesophageal fistula is a common form of congenital malformation of the esophagus, which was usually overlooked during the physical examination after delivery. The first report of esophageal atresia with tracheoesophageal fistula was done by Thomas-Gibson in 1696. We have performed primary repair by extrapleural approach through the right 4th intercostal space in 4 cases of congenital esophageal atresia with distal racheoesophageal fistula. End to end anastomosis was performed by Haight method in all cases. Postoperatively, two patients showed severe stenosis and one patient showed mild stenosis at the anastomotic site. Ballots dilatation was performed two cases with severe anastomotic stenosis.

• Journal of Chest Surgery
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• v.20 no.2
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• pp.300-308
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• 1987

Clinical and pathologic data were reviewed in 20 patients who had have surgery for isolated aortic valvular heart disease between April 1978 and April, 1987. Hospital mortality was 10%, with no additional late mortality during a mean follow-up period of 24.1 months. Prosthetic valve failure developed in 3 patients and two had reoperation. Niety four percent of the survivors who were in NYHA Funtional class III or IV before operation are now in class I or II. Ninety percent of all patients are still alive at a maximum follow up of 9 years. The clinical histories, gross and histologic examination of valves estabilished the causes for isolated aortic valve disease: 3 rheumatic, 2 congenital bicuspid, 2 hypertention, 2 aortitis and each one case of floppy valve, medial cystic necrosis of aorta, bacterial endocarditis. But etiology was unknown in 8 cases. Sixteen patietns had myxoid degeneration, defined as significant disruption of the valve fibrosa and its replacement by acid mucosaccharides and cystic changes. Myxoid degeneration was also the primary pathologic abnormality in the patients with 2 hypertention, 2 rheumatic, 1 aortitis, 1 bacterial endocarditis, 1 floppy valve, 1 congenital bicuspid. The patients with myxoid degeneration of uncertain origin were 8. Histologic finding of all of them revealed nonspecific patients with myxoid degeneration of uncertain orgin were 8. Histologic finding of all of them revealed nonspecific chronic valvulitis with myxoid degeneration. This finding may indicate that the etiology w uld be infectious.

• Journal of Chest Surgery
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• v.27 no.7
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• pp.603-609
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• 1994

Esophageal atresia is a rare congenital anomaly and it usually associated with tracheo-esophgeal fistula and other congenital anomalies. The first report of esophageal atresia with tracheo-esophageal fistula was done by Thomas Gibson in 1696. In 1941, Haight performed the first successful primary anastomosis for esophageal atresia. These accomplishments opened the gateway for clinical studies that have resulted in reinforcements and improvement in the care of infants born with this anomaly. From January 1986 to April 1994, 14 cases of esophgeal atresia with tracheo-esophgeal fistula were diagnosed in Kyung Hee Uinv. Hospital. There were 9 male and 5 female infants. 12 infants were Gross classification type C and 2 infants were type A. The average body weight was 2.7$\pm$0.4kg and Waterson Category A contained 4 infants, B contained 3 infants and C contained 7 infants. Among these infants, 9 infants were underwent anastomosis procedures. We performed retropleural approach in 6 infants, transpleural approach in 2 infants and 1 infant was performed colon interposition through substernal space.By the method of anastomosis, end-to-side anastomosis was performed in 5 infants, end-to-end anastomosis in 3 infants and esophagocologastrostomy in 1 infant.The former 8 infants were Gross classification type C and the latter was type A. Among the type C infants, 6 infants were anastomosed with one layer interrupted suture and 2 infants with 2 layer interrupted suture. Post- operative death was in 1 infant and 8 infants were discharged with good result and have been in good condition.

• Clinical and Experimental Pediatrics
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• v.58 no.9
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• pp.317-324
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• 2015

Kabuki syndrome (KS) is a rare syndrome characterized by multiple congenital anomalies and mental retardation. Other characteristics include a peculiar facial gestalt, short stature, skeletal and visceral abnormalities, cardiac anomalies, and immunological defects. Whole exome sequencing has uncovered the genetic basis of KS. Prior to 2013, there was no molecular genetic information about KS in Korean patients. More recently, direct Sanger sequencing and exome sequencing revealed KMT2D variants in 11 Korean patients and a KDM6A variant in one Korean patient. The high detection rate of KMT2D and KDM6A mutations (92.3%) is expected owing to the strict criteria used to establish a clinical diagnosis. Increased awareness and understanding of KS among clinicians is important for diagnosis and management of KS and for primary care of KS patients. Because mutation detection rates rely on the accuracy of the clinical diagnosis and the inclusion or exclusion of atypical cases, recognition of KS will facilitate the identification of novel mutations. A brief review of KS is provided, highlighting the clinical and genetic characteristics of patients with KS.

• Investigative Magnetic Resonance Imaging
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• v.20 no.2
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• pp.114-119
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• 2016

We report a case of vegetation in a 4-year-old female with infective endocarditis, diagnosed by late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) imaging. The patient had a history of primary closure for ventricular septal defect and presented with mild febrile sensation. No remarkable clinical symptoms or laboratory findings were noted; however, transthoracic echocardiography demonstrated a 14 mm highly mobile homogeneous mass in the right ventricle. On LGE CMR imaging, the mass showed marginal rim enhancement, which suggested the diagnosis of vegetation rather than thrombus. The extracellular volume fraction (${\geq}42%$) of the lesion was higher than that of normal myocardium. Based on the patient's clinical history of congenital heart disease and pathologic confirmation of the lesion, a diagnosis of infective endocarditis with vegetation was made.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.289-294
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• 1983

The report is concerned to our experience of 12 cases of open heart surgery under the extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery, Chosun University Hospital during the period between Nov, 1979 and April, 1983. 1. There were 4 cases of congenital anomaly and 8 cases of acquired heart disease. 2. There were 6 male and 6 female patients with a mean age of 20 years. [range 9 to 33 years]. 3. The cases induced 2 ventricular septal defect, 2 atrial septal defects and 8 acquired valvular heart diseases. 4. The surgical managements were 2 primary repair for atrial septal defect and 2 patch closure for ventricular septal defect, 1 triple valve replacement [AVR MVR TVR], 1 aortic valve replacement, 4 double valve replacement [AVR MVR] and 2 open mitral commissurotomy for pure mitral stenosis. 5. The average cardiopulmonary bypass time was 61.5 minutes for congenital heart disease and 201.4 minutes for acquired valvular heart disease and the average aortic cross clamping time was 36.75 minutes for the former and 165.6 minutes for the latter. 6. Postoperatively, there were 1 Alopecia, 1 Electric burn and 1 wound infection as complication. 7. Overall operative mortality was 8.3%. 7. All patients received valve replacement were recommended anticoagulation with persantin.

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.35-41
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• 1994

Newborns with ileal atresia freqently present with abdominal distension, bilious vomiting. and failure to pass meconium. Diagnosis is usually established on plain x-ray of the abdomen by the findings of distended small bowel loops and air-fluid levels. In the period of October 1988 to February 1994, 8 patients with congenital ileal atresia were operated and the following results were obtained. 1. Eight patients were comprised of 4 males and 4 females, the ratio of male and female was 1 : 1. 2. Six patients(75%) had been admittted to our hospital during three days of life. 3. Congenital ileal atresia was in 8 cases : Type I in two(25%), Type II in two(25%), Type IIIa in three(37.5%), Type IIIb in one(12.5%). 4. There was one premature patient who was small for gestational age. 5. Overall, abdominal distension and bilious vomiting occurring in seven patients, were frequent presenting complaints. 6. Diagnosis was possible with clinical symptom and simple abdomen. 7. Operative treatment was undertaken as soon as the diagnosis was made. In seven cases a primary end-to-end anastomosis was performed after resection of dilated proximal loop. 8. A total of four associated congenital anomalies were found in one patient. 9. Postoperative complications occurred in three cases(37.5%).

• Journal of Chest Surgery
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• v.39 no.9 s.266
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• pp.725-728
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• 2006

A six-month old boy and a thirty-month old girl who suffered from dyspnea were admitted to our hospital. Their primary disease was congenital myopathy, and both of them had a history of recurrent pneumonia. Chest X-ray scan showed unilateral diaphragmatic eventration. To minimize the injury of weakened respiratory muscle in children with myopathy, VATS plication was performed under double lung ventilation. Each of the two patients were discharged on the 17th and 24th postoperative day. We report two cases of successful VATS plication in children with diaphragmatic eventration associated with congenital myopathy.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1383-1390
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• 1992

This is the result of the annual statistic analysis of thoracic and cardiovascular surgical cases in 1991, Korea. 14,715 cases of surgery[thoracic 8,995/cardiovascular 5,720] were done by 53 institutes replied. The order of frequency of cell type in primary lung cancer was squamous [62.3%] / adeno [23.9%] / small [6.4%] / adenosquamous [3.0%], and in mediastinal tumor, neurogenic[27.l%] / thymoma [27.1%] / teratoma[26.4%] / congenital cystic[12.0%]. Surgery for tuberculosis was decreased to 15.8% of overall infectious disease from the recent 6 year`s average 35.7%. In general thoracic surgery, the single most frequent operation was closed thoracostomy[4,047 cases] for pleural pathology. The ratio of congenital to acquired heart disease was 2:1, and acyanotic to cyanotic was 3:1. The order of frequency of congenital acyanotic heart disease was VSD [45.6%] / ASD [25.6%] / PDA [20.4%] / PS [2.9%], and that of cyanotic heart disease was TOF [42.6%] / PA [12.9%] / TGA [9.9%] / DORV [8.8%]. In 1,364 cases of valvular surgery, single mitral pathology was the most frequent candidate[729 cases, 53.4%]. In 243 cases of coronary surgery, bypassing graft materials were great saphenous vein[41.6%], internal mammary [39.5%], and artificial vessel[18.9%]. There were no specific differences in aortic surgery, assisted device implantation, and antiarrhythmic surgery as compared to previous study. This nation-wide inquiry will be continued and reported annually by KTCS Society.