• Journal of Chest Surgery
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• v.25 no.1
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• pp.23-31
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• 1992

The ideal approach in the staged management of patients with pulmonary atresia has been a challenging problem and the result has not been always satisfactory. We reviewed our early result of initial palliative surgeries in fifteen cases of pulmonary atresia with interventricular communication Included are eight cases of simple pulmonary atresia with ventricular septal defect and seven cases of pulmonary atresia associated with other complex cardiac anomalies. The ages of the patients were less than one year except one. The morphology of pulmonary vasculature was highly variable and showed unfavorable conditions in most cases. Pulmonary artery was nonconfluent in two. Two-thirds of all cases showed significant problems such as juxtaductal stenosis or diffuse hypoplasia. The ductus arteriosus usually narrowed at its pulmonic end. Initial palliation was done by modified Blalock-Taussig shunt in six, central shunt with or without pulmonary angioplasty in five, right ventricular outflow tract [RVOT] reconstruction in three and direct connection of nonconfluent pulmonary arteries with bilateral cav-opulmonary shunt in one patient. There were 3 hospital deaths. Two of them underwent simultaneous repair of the associated anomaly of TAPVR. Among the six patients who received modified Blalock-Taussig shunt, three needed early second palliative procedure by central shunt, RVOT patch reconstruction and pulmonary angioplasty in each case, All patients who received central shunt showed marked clinical improvement. Among the twelve patients who survived the palliative procedures, two patients underwent total correction 13 months and 18 months after initial palliation respectively. We think that the choice of palliative procedure must be individualized according to the morphology of the pulmonary arteries. More experience and long term follow-up data are necessary to meet this challenging problem.

• Advances in pediatric surgery
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• v.14 no.2
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• pp.125-133
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• 2008

To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.19-26
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• 2000

This paper reports our 9-year experience treating 34 infants with biliary atresia utilizing a new non-invasive diagnostic method, ultrasonographic "triangular cord"(TC) sign. The TC sign is present when there is visualization of a triangular or a band-like echogenicity just cranial to the portal vein. The ultrasonographic TC sign appears to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia. Sensitivity is 84 %. Active bile excretion was restored in 90 % of the patients who were treated between 31-60days, 78 % of those between 61-90 days, and 33 % of those being 91days or older. The incidence of postoperative cholangitis was 36 %. Construction of an antireflux valve in the Roux-en-Y loop did not affect the incidence of postoperative cholangitis(p=0.18). Among 34 infants with biliary atresia, 23(68 %) are alive for 2-102 months after operation, and 12 are alive for more than 5 years. Five-year estimate survival by Kaplan-Meier method was 66 %.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.979-990
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• 2022

Biliary atresia is a progressive, idiopathic, obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period. It is the most common indication for liver transplantation in children. If untreated, progressive liver cirrhosis leads to death by two years of age. Nowadays, more than 90% of biliary atresia patients survive into adulthood with the development of Kasai portoenterostomy and liver transplantation technology. Early diagnosis is critical since the success rate of the Kasai portoenterostomy decreases with time. This study comprehensively reviews the recent advances in the etiology, classification, prevalence, clinical manifestations, treatment, and prognosis of biliary atresia.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.358-362
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• 1986

Esophageal atresia and tracheoesophageal fistula may occur as separate entities but usually occur in combination. Recently we were experienced a case of esophageal atresia with distal tracheoesophageal fistula in infant patient who presented the symptoms of projectile vomiting and dyspnea. The diagnosis was made by the esophagography and the Haight`s operation was performed transpleurally through 4th intercostal space after gastrostomy. Operative patient tolerated all the operative procedures well in spite of postoperative respiratory complication and recovered uneventfully, permitted feeding on 10th postoperative day. On follow up study after 5th months, Patient reveals good health without other problem.

• 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.23 no.5
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• pp.936-943
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• 1990

The Fontan operation for tricuspid atresia was first performed in 1968[Fontan and Baudet 1971] and several technical modifications of procedure were developed. We have experienced a case of modified Fontan operation for tricuspid atresia type Ic who was 5 years old female. She had previous palliative procedure \ulcornerpulmonary artery banding due to excessively increased pulmonary blood flow. The modified Fontan operation was right atrium to right ventricle connection with valve-bearing conduit. The result was good and the patient was discharged 20 days after operation.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.381-385
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• 1983

Congenital bronchoesophageal fistula without esophageal atresia is very rare and often has an insidious clinical course that occasionally persists into adult life. Here are presented five cases of congenital bronchoesophageal fistula without esophageal atresia in adults treated successfully in the Seoul National University Hospital. The patients included two women and three men in the range of 16 and 45 years old. [mean age: 32 years old] Three of five cases could be diagnosed preoperatively by esophagogram and bronchogram but two of them could only be found in operative field. Cineesophagogram is recommended, on review of the literature, to be the most rewarding diagnostic procedure.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.549-556
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• 1985

Since the first report on a successful method of correcting tricuspid atresia by Fontan and Baudet in 1971, several alternative surgical techniques have been described. Bjrk`s modification was performed three patients, 3, 8, and 9 years of age, in our Hanyang University Hospital. The diagnoses were tricuspid atresia type lb, tricuspid atresia type lib and d-TGA with huge VSD and valvular PS. None had previous palliative procedures. The patients were operated on with good results. The CVP after operation was 10-14 mmHg and early extubation was done. Postoperatively, none had peripheral edema or ascites. Postoperatively, all three were acyanotic and had improved physical capacity compared with their preoperative status.

• Journal of Chest Surgery
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• v.52 no.3
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• pp.170-173
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• 2019

A rare case of esophageal atresia/tracheo-esophageal fistula (EA-TEF) with an associated tracheobronchial remnant (TBR) is reported and discussed herein. A 13-month-old patient was seen with a complaint of vomiting of solid food 1 year after EA-TEF repair. An esophagogram showed a tapered narrowing in the lower segment of the esophagus. A re-operation was carried out and a pathologic examination of the resected stenotic segment revealed the presence of a TBR.