• Journal of Chest Surgery
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• v.34 no.9
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• pp.720-723
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• 2001

A case of Marfan’s syndrome with atresia of right coronary artery is reported. A 45-year-old woman, who was diagnosed as Marfan’s syndrome 1 year ago, came to the hospital complaining of acute chest pain. The patient showed arachynodactyly, pectus carinatum, and long and slender extremities. In echocardiography there were severe aortic regurgitation measured grade IV and aortic dilatation of ascending aorta maximally 5.9 cm in diameter. Mitral regurgitation was mild, but there were also moderate left ventricular dilation and moderately decreased ejection fraction of left ventricle. At operation, atresia of right coronary artery was found. We performed Bentall type operation with SJM 27mm valved conduit for left coronary artery, and Piehler’s modification for right coronary artery bypass using 6mm PTFE graft. The atretic portion of right coronary artery from the suspected right coronary ostium to distal coronary flow was about 4 cm in length. The combination of right coronary artery atresia and Marfan’s syndrome is very rare. The author describes the rare case, which is treated with combined technique of Bentall and Piehler modification for reconstruction of coronary circulation.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.105-111
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• 2007

We reviewed the records of 25 patients who were re-operated upon after primary repair of esophageal atresia with or without fistula at the Department of Pediatric Surgery, Seoul National University Children's Hospital, from January 1997 to March 2007. Types of the esophageal atresia anomalies were Gross type A in 5 patients, C in 18, and E in 2. The indications for re-operation were anastomosis stricture (n = 14), tracheo-bronchial remnant (n = 4), persistent anastomosis leakage (n = 3), recurrent tracheo-esophageal fistula (n = 2) and esophageal web (n = 2). The interval between primary and secondary surgery was from 48 days to 26 years 5 months (mean: 2 years and 4 months). Four patients required a third operation. The interval between the second and third operation was between 1 year 1 month and 3 year 10 month (mean: 2 years 5 months). Mean follow up period after last operation was 35 months (1 years-8 years 6 months). The secondary surgery was end-to-end esophageal anastomosis in 15, esophagoplasty in 5, gastric tube replacement in 5. After secondary operation, 6 patients had anastomosis stricture (4 patients were relieved of the symptoms by balloon dilatation, 2 patients underwent tertiary operation). Five patients had leakage (sealed on conservative management in all). Two patients had recurrent tracheo-esophagel fistula (1 patient received chemical cauterization and 1 patient underwent tertiary operation). Currently, only one patient has feeding problems. There were no mortalities. Secondary esophageal surgery after primary surgery for esophageal atresia was effective and safe, should be positively considered when complications do not respond to nonoperative therapy.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1983.05a
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• pp.18.3-19
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• 1983

Choanal atresia, described first by Johann Roedere (1755) is an obstruction between the nasal cavity and nasopharyngeal vault, and the diagnosis and treatment were developed because of severity of the disease. Embryologically, incomplete development of olfactory pit, or failure of nasobuccal membrane to rupture, or persistent remaining of buccopharyngeal membrane, etc, all forms the congenital choanal atresia. And the acquired type was the result from syphilis or diphtheria with a resultant stenosing cicatrix and after the inexpert surgery and the trauma. Multiple abnormalities may be present particularly affecting the head, the heart and the alimentary system in the congenital type. The operative technique employed would depend upon the type of obstruction(whether membranous or bony), the age of the patient, and the presence or absence of any associated pathologic condition. Since Emmert (1853) first tried blind puncture of the atretic plate with the trocar, other surgical techniques have been introduced over the years for the correction of choanal atresia, which were the transnasal, transpalatal, transantral and the transseptal approach. Among them, transpalatal approach was proved to be a popular technique, that it provides a direct route, thus permitting an exact reconstruction and low restenosis rate. Recently, we have experienced two cases of choanal atresia and treated successfully with transpalatal approach, so authors report these cases with a review of the literature.

• Advances in pediatric surgery
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• v.4 no.2
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• pp.163-165
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• 1998

The association of congenital colonic atresia with imperforate anus is extremely rare. There are only three cases reported in the English literatures. The authors have recently managed one case in female infant.

• Advances in pediatric surgery
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• v.15 no.2
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• pp.157-160
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• 2009

Although the incidence of esophageal atresia (EA) is higher in twins than in singletons by two to three times, EA usually affects only one member of twins. We report one pair of twins concordant for EA. A 31-year-old healthy woman bore monozygotic female twins at 36 weeks of gestation. They weighed 2,216 and 2,480 g, respectively. They had EA with distal tracheoesophageal fistula and underwent primary esophageal anastomosis on the birth day and the $2^{nd}$ day of life, respectively. Twin A also had suspicious antral obstruction and pyloroplasty was done simultaneously with esophageal repair. She needed antral web excision for continued gastric stasis one month after $1^{st}$ operation and three balloon dilatations of the esophagus. Twin B recovered uneventfully.

• Journal of Chest Surgery
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• v.30 no.5
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• pp.524-527
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• 1997

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a rare and complex lesion in which great morphologic variability exists regarding the sources of pulmonary blood flow. We report a case of this disease with no true central pulmonary arteries in a 9-month-old-boy successfully treated by one-stage complete unifocalization and repair from a midline stemotomy approach.

• Journal of Chest Surgery
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• v.20 no.3
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• pp.565-569
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• 1987

The first description of the pathologic anatomy of esophageal atresia was presented by Duration in 1670, it was not successfully treated until 1939 when the first two survivors of staged correction were described by Ladd and Levin. In 1941 Haight and Towsley performed the first successful primary repair. Recently we were experienced a case of esophageal atresia with tracheoesophageal fistula an infant patient who presented the symptoms of vomiting and dyspnea. The diagnosis was made by the esophagography with Diagnosis. The operation was performed extrapleurally through 4th intercostal space after gastrostomy. The fistula was closed by triple ligation and the upper pouch was then brought into apposition with the presenting surface of the lower esophageal segment and an end to side anastomosis was fashioned with a single layer of sutures. Operative patient tolerated all the operative procedure well in spite of postoperative respiratory complication and recovered uneventfully, permitted feeding on 9th postoperative day after esophagography.

• Journal of Chest Surgery
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• v.46 no.1
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• pp.56-59
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• 2013

Cardiac surgery in neonates with congenital heart disease has progressed dramatically in the past three decades. However, low-birth-weight neonates with congenital heart disease continue to challenge the intellectual and technical skills of healthcare professionals. We present a case of a low-birth-weight neonate with pulmonary atresia and a ventricular septal defect, in whom palliation was achieved with a right ventricular outflow tract stent using a hybrid procedure.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.126-129
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• 1999

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst ($4.5{\times}4.0$ cm in size) in the left hepatic lobe and another tubular dilatation ($2.0{\times}0.8$ cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangiodrainage(PTCD) and cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring $6.6{\times}5.0$ cm in size. Following drainage and administration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks. The patient successfully underwent intrahepatic cystojejunostomy with intraoperative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.

• Advances in pediatric surgery
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• v.7 no.2
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• pp.166-169
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• 2001

A 37-week gestation female neonatal infant presented with lower abdominal distension. Ultrasonography showed a hydrocolpos, measuring $8.3cm{\times}6.9cm{\times}6.1cm$ in size and on perineal examination, vaginal atresia was noticed. On a follow-up ultrasonography performed 41 days after aspiration, the hydrocolpos was enlarged to $10cm{\times}8cm{\times}7cm$ in size, and compressed adjacent small bowel significantly with concomitant bilateral hydronephrosis. Temporary tubed vaginostomy was carried out with the provision of excellent drainage and easy access for contrast studies to outline the pathologic anatomy. We are planning to perform vaginal reconstructive surgery on her age around 2 years, when her vaginal structure might grow sufficiently for reconstructive surgery.