• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Korean Journal of Clinical Laboratory Science
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• v.51 no.2
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• pp.265-269
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• 2019

Ebstein's anomaly is attached to the right ventricle with the tricuspid leaf attached to the lower right ventricle without any attachment to the tricuspid annulus. Most patients in their 20s are alive. Patients older than 25 years of age are similar in the incidence of heart failure and sudden death, and survive approximately 70% from 2 years, and 50% from 3 years, from 5% to over 50 years of age. Through this example, Ebstein's anomaly exists in various forms. A 22 year old case with WPW syndrome and those who survived to 77 years of age are reported. The normal heart structure in the apical four-chamber view should be observed on similar lines, even though the tricuspid annulus is slightly lower than the mitral annulus. If not, there will be some doubt regarding this anomaly. Furthermore, echocardiography plays an important role in the diagnosis and prognosis of congenital heart disease.

• 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.

• Journal of Chest Surgery
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• v.20 no.2
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• pp.432-437
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• 1987

Pulmonary atresia with intact ventricular septum is a extremely rare congenital cardiac anomaly. With the history of cyanosis and failure to thrive, this anomaly should not be excluded and emergency management is necessary. Our patient was 69 day-old male with pulmonary atresia which was confirmed by cardiac angiography. Prostaglandin E1 was used for maintenance of pulmonary blood flow preoperatively. Right ventricular outflow reconstruction with pericardial patch and concomitant pulmonary valvotomy were done on beating heart for palliation. With this method, growing of right ventricle and tricuspid annulus are highly expected.

• Journal of Chest Surgery
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• v.54 no.1
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• pp.65-67
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• 2021

A fistula between the right pulmonary artery and the left atrium is a very rare congenital anomaly, for which there is no definitive embryogenetic explanation. Patients present with cyanosis or clubbing, and the treatment strategy is to close the fistula, which can be done by an open surgical technique or by percutaneous intervention. Although rare, this condition should be considered in the differential diagnosis when evaluating a patient with central cyanosis.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.509-512
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• 2009

Ebstein's anomaly is a complex congenital defect of the tricuspid valve and right ventricle. Various surgical methods to repair the regurgitant tricuspid valve have been reported, and most of them depend on monocuspidalization with using the anterior leaflet. We report here on our first experience with Ebstein's anomaly in a 31-year-old female patient who underwent cone reconstruction of the tricuspid valve with using three leaflets.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1455-1460
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• 1992

Anomalous drainage of the right superior vena cava into the left atrium is a very rare congenital cardiac anomaly. Recently a patient with this venous anomaly was surgically corrected and forms the basis of this report. Patient findings were as follows: The patient has no other symptom but cyanosis which prompted cardiac evaluation Chest PA and electrocariogram were usual. Cross-sectional echocardiogram showed normally connected heart without intracardiac defect, Inferior vena cava drained normally into right atrium and coronary sinus was not dilated. Contrast, given into the right atrium, appeared in the left atrium This rare venous anomaly was confirmed by surgery. Surgical correction consisted of division of superior vena cava above the junction of left atrium and reanastomosis into right atrial appendage. Postoperative digital subtracion angiography confirmed the successful repair. She has doing well for 6months since operation. Systemic venous anomalies without intracardiac defect are very rare. However this anomalies should be considered in the differential diagnosis of cyanosis. The successfully corrected case is reported and literature is reviewed.

• Journal of Chest Surgery
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• v.20 no.3
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• pp.630-634
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• 1987

Anomalies of the diaphragm, particularly eventration, are not encountered frequently in clinical practices. Diaphragmatic eventration is generally accepted as an anomaly high position of part or all of the diaphragm, usually associated with a marked decrease in muscle fibers and a membranous appearance of the abnormal area. We experienced 3 cases of the congenital diaphragmatic eventration at the department of the thoracic and cardiovascular surgery, Maryknoll Hospital, which were treated successfully.

• Journal of Chest Surgery
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• v.23 no.5
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• pp.1009-1013
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• 1990

Congenital bronchoesophageal fistula is a rare anomaly that can appear in adult life uncommonly. There was a largest review that include 100 cases in the literature. In our case, he was 48 years old male patient who admitted for chronic coughing and recurrent lobar pneumonia on the right lower lobe since 10 years old. We could confirmed the fistular preoperatively by barium swallow examination and performed division of the fistula. The patient’s postoperative course was unremarkable. According Braimbridge’s classification, it was belonged to the type II.

• Journal of Chest Surgery
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• v.27 no.7
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• pp.631-633
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• 1994

Congenital bronchoesophageal fistula is a rare anomaly that can appear in adult uncommonly. Especially sick sinus syndrome with bronchoesophageal fistula is very uncommon.The patient was 53 years old male who admitted for chronic coughing recurrent lobar pneumonia on RLL since few years ago. And he had familial history of sick sinus syndrome.We confirmed the fistula by barium swallow examination and performed ligation of the fistula and pacemaker implantation.