• Journal of Chest Surgery
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• v.5 no.2
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• pp.153-158
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• 1972

The esophageal atresia with tracheoesophageal fistula occurs approximately once in 3, 000 live births. In Korea, about 20 cases were reported with five successful surgical corrections. The atresia characteristically occurs at the level of, or just cephalad to, the carina and is associated with a tracheoesophageal fistula. In about 90% of the cases, the upper esophagus ends in a blind pouch, and the lower esophageal segment communicates with the trachea through the membranous posterior wall just above the carina. Many of the Infants with esophageal atresia have other congenital anomalies. The most common of these are congenital cardiac anomalies, imperforate anus, genitourinary malformations, and intestinal atresia. Recently we experienced four cases of esophageal atresia, of which three were Gross type C and one was type A. Two of them were treated by primary repair, and one [type A] was taken cervical esophagostomy and gastrostomy. The another was refused surgery.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.81-87
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• 2011

This study was aimed to evaluate associated congenital anomalies in the patients with esophageal atresia with tracheoesophageal fistula (EA/TEF). Forty-two neonates with the diagnosis of EA/TEF treated over a 10 year period in a single institution were included in this study. The demography of EA/TEF was analyzed. Major associated anomalies including vertebral, anal, cardiac, renal, limb, neurologic and chromosome were reviewed and categorized. Males were slightly more dominant than females (1.47:1) and all patients had Gross type C EA/TEF. Only 19 % of the patients had solitary EA/TEF without associated anomalies. Cardiac anomalies were the most common associated congenital anomaly in patients with EA/TEF (73.8 %). But 47.6 % were cured spontaneously or did not affect patients' life. Atrial septal defect (ASD) was the most common cardiac anomaly followed by patent ductus arterious (PDA) and ventricular septal defect (VSD). Among gastrointestinal anomalies (23.8 %), anorectal malformations were the most frequent, 70 % Vertebral and limb abnormalities accounted for 11.9 % and urogenital malformations 9.5 % of the anomalies in patients with EA/TEF. VACTERL associated anomalies were 23.8 % and 1.8% had full VACTERL. Almost 12 % of EA/TEF had neurologic anomalies. Patients with EA/TEF require preoperative evaluation including neurologic evaluation to detect anomalies not related to VACTERL. Though associated cardiac anomaly occurred in 73.8 % of patients in our study, only 21.42 % needed surgical correction. The authors suggesrs further studies with large numbers of patients with EA/TEF.

• 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.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.40-44
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• 2000

Congenital esophageal stenosis (CES) is a narrowing of the esophageal lumen from birth. Three types of CES have been described; tracheobronchial remnants (TBR), membranous web (MW), and fibromuscular stenosis (FMS). We reviewed the clinical features and the surgical outcome of 14 patients, pathologically confirmed as CES. Nine patients had TBR, 3 FMS, and 2 MVV. The mean age at operation was 3.8 years. Five patients were boys and 9 girls. Four patients had other congenital anomalies. Segmental resection of the lesion and end to end anastomosis was utilized in all cases except one who underwent myotomy. The stenotic segment was located at the distal esophagus in all patients. There were 8 complications in 6 patients, but no mortality. The mean follow-up period was 68 months. There were no feeding problems but 3 patients had minor gastroesophageal reflux. Our result indicates that segmental resection and anastomosis is a satisfactory surgical procedure in the management of CES.

• Journal of Chest Surgery
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• v.48 no.6
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• pp.439-442
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• 2015

Tracheal agenesis is an extremely rare and typically lethal congenital disorder. Approximately 150 cases have been described since 1900, and very few cases of survival have been reported. We describe tracheal reconstruction with external esophageal stenting in a patient with Floyd's type II tracheal agenesis. Neither long-term survival nor survival without mechanical ventilation for even a single day has previously been reported in patients with Floyd's type II tracheal agenesis. The infant in the present case survived for almost a year and breathed without a ventilator for approximately 50 days after airway reconstruction using external supportive stents.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.2
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• pp.79-86
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• 2017

Purpose: Congenital esophageal atresia (CES) is a rare congenital disease. The severity of symptoms is variable; thus, diagnosis is difficult and tends to be delayed. CES is frequently accompanied by esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). We investigated the characteristics of CES by reviewing our experience with CES patients and researched the differences between CES with EA-TEF and isolated CES. Methods: A total of 31 patients underwent operations for CES were reviewed retrospectively. The patients were divided into two groups according to the association with EA-TEF, and compared the differences. Results: Sixteen boys and 15 girls were included. The mean age at symptom onset was 8 months old, and the mean age at diagnosis was 21 months old. Nine patients with EA-TEF were included group A, whereas the other 22 patients were assigned to group B. There were no differences in sex, gestational age, associated anomalies and pathologic results between the groups. In group A, the age at diagnosis and age at surgery were younger than in group B despite the age at symptom occurrence being similar. Postoperative complications occurred only in group A. Conclusion: In this study, symptoms occurred during the weaning period, and vomiting was the most frequent symptom. CES patients with EA-TEF tended to be diagnosed and treated earlier despite the age at symptom occurrence being similar. CES patients with EA-TEF had more postoperative complications; therefore, greater attention should be paid during the postoperative period.

• Journal of Chest Surgery
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• v.28 no.9
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• pp.869-871
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• 1995

Esophageal duplication cysts are uncommon, benign lesions of the esophagus. They are rare congenital foregut anomalies. To be considered an esophageal duplication cyst, a lesion must meet the following criteria:1 the cyst in the esophageal wall 2 the cyst is covered with a muscularis propria,generally of two layers and 3 the cyst has an epithelial lining consistent with that of the 4 week embryo, which may be columnar or pseudostratified columnar, and may be ciliated. Herein we report a case of an esophageal cyst located within the thoracic cavity, which is, to the best of our knowledge, the first case reported in Korea. Surgery is generally the treatment of choice for esophageal cyst, and was indicated in this case for the diagnosis and management of symptoms attributable to the cyst.

• Advances in pediatric surgery
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• v.1 no.2
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• pp.149-161
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• 1995

The first national survey on esophageal atresia and tracheoesophageal fistula was conducted to access the current status of its incidence, clinical manifestation, preoperative diagnosis and management, type of its anomaly, associated anomalies, and surgical results and course. The 43 members of the Korea Association of Pediatric Surgeons received questionnaires and registration forms to be filled out on each patient who were born during the three years from January 1, 1992 through December 31, 1994. Questionnaires composed of six broad areas which include 1) preoperative diagnosis and management, 2) surgical technic, 3) long gap, 4) postoperative management, and 5) complications and courses. A total of 148 cases was returned by 28 members working at 23 institutions and 27 members returned questionnaires. We obtained the following results by analysis of the 148 cases of tracheoesophageal anomalies. The incidence of tracheoesophageal anomaly was about 1/10,000-11,000 in 1994, which is one third of that of anorectal malformations in Korea and the distribution of the patients was almost proportionate to the size of each province. Both sexes are about equally affected. Majority of the members make diagnosis of tracheoesophageal anomaly by taking a simple infantogram with a radiopaque tube in upper pouch and a little under half(46%) prefers to perform echocardiography as a part of preoperative management to identify congenital heart disease and lateralize the aorta. Esophageal atresia with distal TEF(87.5%) was by far the most common and there were pure esophageal atresia(5.6%), H-type TEF(2.1%), and so on. About half(49%) of the patients had one or more associated anomalies in addition to tracheoesophageal anomalies. Congenital heart disease was associated in 46 cases(31%), anorectral malfomations in 19 cases(13%), musculoskeletal anomalies in 15 cases(10%), genitourinary anomalies in 10 cases(7%) and gastrointestinal anomalies in 7 cases(5%). Postoperatively, parenteral nutrition and assisted ventilation were given in 66% and 52% of patients respectively. Ninety three(74%) of 126 cases who underwent sugical procedure, experienced one or more complications such as respiratory complication(65%), leak(22%), stricture(21%) and so on. The survival rates related to the Waterston risk categories were 90.2% in group A, 71.4-75% in $B_1$, $B_2$, and $C_1$, groups, and 28% in group $C_2$, and the overall survival rate was 71.4%. Thirty six(28.6%) of 126 cases died of pneumonia/sepsis(12 cases), respiratory failure(12 cases), and congenital heart disease(4 cases). With short term follow-up, 69% of patients have been excellent whereas remainders of the cases have suffered from some sort of morbidity related to gastroesophageal reflux, recurrent respiratory infection, and esophageal stricture.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.137-142
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• 2005

Purpose: To evaluate the safety, efficacy and technical problems of the endoscopic balloon dilatation of esophageal anomalies in children. Methods: The medical records of 8 children treated by endoscopic balloon dilatation for esophageal anomalies over a 10-year period at Pusan National University Hospital were reviewed retrospectively. The balloon catheter (Maxforce TTS or CRE, Boston Scientific Co., USA) was positioned across the area of narrowing by direct visualization. The balloon was slowly inflated with normal saline to specified pressures for each balloon and maintained for 60 seconds and then deflated. After 60 seconds pause, the procedure was repeated with a larger sized balloon (increments of 1 mm for each subsequent dilation) till effective dilatation was confirmed by direct visualization without complications. Results: Three male and five female were included and their mean age was 4.2 years. A total of 27 (average of 3.2 per patient) dilatation were performed. Underlying diseases of patients are postoperative stricture of esophageal atresia in 3 cases, esophageal ring in 2 cases, achalasia, corrosive esophagitis and hypertensive LES in one case respectively. The size of initial dilating balloon was chosen on the basis of the diameter of the narrowing determined by endoscopy. The first dilation in patients with severe esophageal stricture was made with a 6 mm sized balloon. Complications observed were esophageal perforation and respiratory holding during the procedure in one case respectively. Successful outcome was seen in 6 patients (75%). Conclusion: Endoscopic balloon dilatation can provide a safe and effective mean of treating esophageal anomalies in children and should be considered the treatment of choice in the initial management of those cases.

• Advances in pediatric surgery
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• v.3 no.1
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• pp.15-23
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• 1997

During a 6-year period, from January 1990 to December 1995, 101 neonates with congenital anomalies were admitted to the division of Pediatric Surgery of Youngdong Severance Hospital. All of them had prenatal screening more than once with ultrasound. Fifty eight of them had prenatally detectable anomalies by ultrasonography. However abnormalities were prenatally detected in 24 neonates(41%). The detection rate was 70% in patientws who had the prenatal screening at our hospital, whereas, the rate was 24% when it was performed at other medical facilities. Duodenal and jejuno-ileal atresia showed the highest detection rate(86%) followed by abdominal mass. Esophageal atresia was suggested by maternal polyhydramnios in 3 patients (25%). Only one patient with diaphragmatic hernia(1.75%) was prenatally detected and none with gastroschisis. The mean interval from birth to operation was 32 hours in the prenatally detected patients and 50 hours in the non detected. The complication rate and the mortality after emergency operation were 20% and 7% in the detected group, and 58% and 23% in the nondetected, respectively. The average period of the hospitalization was 20 days in the detected group and 39 days in the nondetected. We conclude that the prenatal detection of anomalies is necessary to ensure adequate care for the mothers and the babies with congenital anomalies. This includes early transfer, timing of optimal delivery and operation.