• Clinical and Experimental Pediatrics
• /
• v.51 no.8
• /
• pp.892-895
• /
• 2008

H-type tracheoesophageal fistula (TEF) is extremely rare in infants and children, and clinical manifestations of this condition are diverse based on its severity. Some cases of congenital TEF diagnosed in adulthood have been reported, which indicate the difficulty of early diagnosis of this disease. Gastroesophageal reflux (GER) may induce chronic aspiration, pulmonary aspiration, apparent life-threatening events, and failure to thrive. We report a 5-month-old boy whose recurrent pneumonia and wheezing did not improve under usual treatment and led to acute respiratory distress syndrome. He was found to have severe GER on the second-trial of the esophagogram and was eventually revealed to have congenital H-type TEF upon repeated evaluation.

• Advances in pediatric surgery
• /
• v.17 no.2
• /
• pp.154-161
• /
• 2011

Tracheoesophageal fistula without esophageal atresia (H-type TEF) is a congenital anomaly that is characterized by a fistula between the posterior wall of the trachea and the anterior wall of the esophagus, not accompanied by esophageal atresia. The purpose of this study is to investigate the clinical characteristics, diagnostic time, the side of cervical approach and short term result after surgery by searching medical records of patients treated for H-type TEF. The search was done at University of Ulsan, Department of Pediatric Surgery of Asan Medical Center, and the total number of patients from May 1989 to December 2010 was 9 with M:F ratio of 1:2. The median gestational age was $39^{+6}$ ($32^{+6}{\sim}41^{+0}$) wks. Seven out of nine patients were born at term and the other two were born premature. The clinical presentation was aspiration pneumonia, difficulty in feeding, chronic cough, vomiting, abdominal distension and growth retardation. The symptoms presented right after birth. The diagnosis was made with esophagography and the median time of diagnosis was 52 days of life. The majority of surgical corrections were performed within two weeks of diagnosis (median; 15d, range; 1d - 6m). Six patients had associated anomalies, and cardiac anomalies were most common. The cervical approach was utilized in all cases (right 2, left 7). Transient vocal cord palsy and minor esophageal leakage complicated two cases. Although the diagnosis of H-type TEF was difficult and often delayed, we had a good short term result. The left cervical approach was preferred.

• Clinical and Experimental Pediatrics
• /
• v.53 no.6
• /
• pp.705-710
• /
• 2010

Purpose: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. Methods: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. Results: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were $37^{+2}$ weeks and $2.5{\pm}0.7kg$, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. Conclusion: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.

• Journal of Chest Surgery
• /
• v.43 no.6
• /
• pp.820-823
• /
• 2010

There are still some controversies in treatment strategy for the very low-birth-weight baby with esophageal atresia even though the result of primary repair has been improving. We report a successful end to end anastomosis with staged approach in one of twin weighing 1,270 g at birth.

• Advances in pediatric surgery
• /
• v.1 no.2
• /
• pp.162-169
• /
• 1995

Since 1988, we have performed esophageal replacement with the reversed gastric tube on four esophageal atresia patients. Three patients had long-gap esophageal atresias and one patient had recurrent tracheoesophageal fistula that was previously operated on three times. One combined imperforate anus. The youngest patient was 6-month-old and the oldest, 34-month-old at the time of procedure. The technique of gastric tube construction is described. There have been both major and minor complications. Although two patients had shown distal tube strictures as late complications. those were solved with tuboplasties on 29 months and 48 months, postoperatively. Growth and development have been acceptable in all four patients, although most remain in the lower percentiles for growth and height, a condition that usually predates the esophageal substitution. Conclusively, reversed gastric tube interposition has proved very satisfactory for long-gap esophageal atresia that cannot be anastomosed primarily even by spiral myotomy and esophageal atresia with recurrent tracheoesophageal fistula having destroyed esophagus due to previous operation.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
• /
• v.12 no.2
• /
• pp.152-157
• /
• 2001

Background : Electrolarynx, Esophageal voice, and Silicone voice prosthesis with tracheoesophageal(T-E) fistula have been used as vocal rehabilitating methods for the post-laryngectomized patients. Prosthetic rehabilitation of voice after total laryngectomy has gained wide acceptance and has become a common practice in many clinics since the pioneering works of Singer and Blom In 1979. Since the introduction of tracheo-esophageal puncture and application of Blom Singer$\circledR$ voice prosthesis in 1980, several reliable voice prostheses have been developed and are successfully being used. Objectives : Even though quality of voice produced by Silicone voice prosthesis with T-E fistula is superior to other modalities, it still has some disadvantages. We devised a new cannulatyped silicone voice prosthesis. Methods : 1) Devising a new prototype of cannula-typed silicone voice prosthesis. 2) Application of the prototype using canine animal model(laryngectormized dog) and fitting trial on human patient whose previously inserted Silicone voice prosthesis is not functioning due to presumed fungal infection. Discussion : Final form of prototype was made after several times of major and minor modifications. Insertion of the newly developed Cannula-typed Silicone voice prosthesis on canine animal model and human trial were done without any difficulty. There were no serious leakage of saliva or food during swallowing. Conclusion : The newly developed Cannula-typed Silicone voice prosthesis(So-Mang$\circledR$) and the modified replacement method will further improve the results of post-laryngectomized prosthetic voice rehabilitation. Long-term animal study and human trial are planned in the near future.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.16 no.1
• /
• pp.28-33
• /
• 2013

Purpose: Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia. Methods: Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted. Results: The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems. Conclusion: This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.

• Neonatal Medicine
• /
• v.25 no.4
• /
• pp.186-190
• /
• 2018

Esophageal atresia (EA) with proximal tracheoesophageal fistula (TEF; gross type B) is a rare defect. Although most patients have long-gap EA, there are still no established surgical guidelines. A premature male infant with symmetric intrauterine growth retardation (birth weight, 1,616 g) was born at 35 weeks and 5 days of gestation. The initial diagnosis was pure EA (gross type A) based on failure to pass an orogastric tube and the absence of stomach gas. A "feed and grow" approach was implemented, with gastrostomy performed on postnatal day 2. A fistula was detected during bronchoscopy for recurrent pneumonia; thus, we confirmed type B EA and performed TEF excision and cervical end esophagostomy. As the infant's stomach volume was insufficient for bolus feeding after reaching a body weight of 2.5 kg, continuous tube feeding was provided through a gastrojejunal tube. On the basis of these findings, esophageal reconstruction with gastric pull-up was performed on postnatal day 141 (infant weight, 4.7 kg), and he was discharged 21 days postoperatively. At 12 months after birth, there was no catch-up growth; however, he is currently receiving a baby food diet without any complications. In patients with EA, bronchoscopy is useful for confirming TEF, whereas for those with long-gap EA with a small stomach volume, esophageal reconstruction with gastric pull-up after continuous feeding through a gastrojejunal tube is worth considering.

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

• Advances in pediatric surgery
• /
• v.11 no.2
• /
• pp.157-164
• /
• 2005

Esophageal atresia without tracheoesophageal fistula accounts for 7-11 % of all types of esophageal atresia and is very difficult to treat. In our hospital from 1990 to 2005, we operated upon 40 patients with esophageal atresia, and 6 had pure atresia. The preoperative characteristics, operative findings and post operative course of the six patients with pure atresia were analysed. Immediate gastrostomy was performed in all 6 patients. One patient had simultaneous cervical esophagostomy. Esophageal reconstruction procedures were transhiatal gastric pull up in 3 patients, esophagocologastrostomy utilizing left colon in 1, and transthoracic esophagoseophagostomy with esophageal bougination in 2. Postoperative complications were pneumonia, anastomosis leakage, and gastroesophageal reflux symptom. Conservative management was effective in all patients. A larger series of cases would be required to demonstrate the most effective treatment for this particular anomalous condition.