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"Esophageal Atresia"

Original Article

[English]
Comparative Analysis of Two National Surveys on Esophageal Atresia With or Without Tracheoesophageal Fistula: A Retrospective Study by the Korean Association of Pediatric Surgeons
Jinyoung Park, Dae Yeon Kim, Seong Chul Kim, Hyun-Young Kim, So Hyun Nam, Jeong-Meen Seo, Jung-Tak Oh, Myung-Duk Lee, Suk-Koo Lee, Soo Min Ahn, Hye Kyung Chang, Sung Eun Jung, Yeon Jun Jeong, Eunyoung Jung, Jae Hee Chung, Yong Hoon Cho, Soon Ok Choi, Seung Hoon Choi, Yun Mee Choe, Seok Joo Han, Jeong Hong, Nam-Hyuk Lee
Adv Pediatr Surg 2024;30(1):1-8.   Published online May 31, 2024
DOI: https://doi.org/10.13029/aps.2024.30.1.1
Purpose
The Korean Association of Pediatric Surgeons (KAPS) conducts annual nationwide surveys on various aspects of pediatric surgical diseases, with the results being discussed during KAPS’s annual spring meetings.
Methods
KAPS conducted two national surveys, in 1995 and 2016, to investigate esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). The authors analyzed data from these surveys to identify differences or changes in the annual occurrence, demographic characteristics, clinical presentation, preoperative diagnostic methods, anatomical type, associated anomalies, surgical treatment, and postoperative outcomes among patients with EA/TEF treated by KAPS members.
Results
The first and second national surveys included 148 and 211 patients with EA/TEF, respectively. Excessive salivation was the most prevalent clinical symptom in both surveys. Type C was the most common form of EA/TEF in both surveys. The first survey included 126 patients, all of whom underwent open surgery. In the second survey, 152 (78.4%) of 194 patients underwent open surgery, while 34 (17.5%) underwent thoracoscopic surgery. Primary esophageal repair was performed on 96 (76.2%) of 126 patients in the first survey and on 160 (82.5%) of 194 patients in the second survey. Anastomotic strictures developed in 21.4% and 32.5% of patients, anastomotic leakage in 22.2% and 10.3%, recurrent fistula in 2.4% and 4.2% during the first and second surveys, respectively. The respective survival rates for group A were 90.2% and 98.3% in the first and second surveys. For group B, the rates were 73.9% and 98.1%, and for group C, they were 34.5% and 68.1%, respectively, according to the Waterston classifications.
Conclusion
These nationwide surveys provide comprehensive information on the status, detailed treatment, and outcomes for Korean pediatric patients with EA/TEF. They are anticipated to be an invaluable resource and guide for pediatric surgeons seeking to expand their knowledge on EA/TEF and its treatment options.
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Case Reports

[English]
Successful Correction of Long Gap Esophageal Atresia with Gastric Tube through Totally Laparoscopic and Thoracoscopic Procedure
Hee-Beom Yang, Hyun-Young Kim, Sung Eun Jung
Adv Pediatr Surg 2019;25(1):24-28.   Published online June 28, 2019
DOI: https://doi.org/10.13029/aps.2019.25.1.24

Esophageal atresia (EA) is a diverse disease entity. We present a case of long gap EA without fistula corrected through totally laparoscopic and thoracoscopic esophageal replacement using gastric tube. A male baby weighing 3,000 g, with suspicion of EA, was born at gestational age of 37+6 weeks. Gastrostomy was made at an age of two days; seven months later, definite operation was planned. We determined to perform the gastric tube replacement due to long gap revealed by fluoroscopy. Gastric mobilization, gastric tube formation, and pyloroplasty were performed laparoscopically. An isoperistaltic 9 cm gastric tube was made using 2 Endo GIA 45, and interrupted end-to-end esophago-esophagostomy was performed thoracoscopically. With laparoscopy, gastropexy to the diaphragm was performed through the interrupted suture. Operation time was 370 minutes; there was no intraoperative event. Postoperative course was uneventful. He underwent esophageal balloon dilatation due to anastomosis stenosis in the months after surgery.

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[English]
Esophageal Atresia with Bronchogenic Cyst
Youngmin Kim, Chaeyoun Oh, Joong-Kee Youn, Ji-Won Han, Hyun-Young Kim, Sung-Eun Jung
J Korean Assoc Pediatr Surg 2017;23(1):5-8.   Published online June 26, 2017
DOI: https://doi.org/10.13029/jkaps.2017.23.1.5

A baby was diagnosed with esophageal atresia (EA) with tracheoesophageal fistula (TEF) on the next day after birth, and end-to-end anastomosis of esophagus with TEF ligation was performed. The distance between proximal and distal esophageal pouch was checked as 3 vertebral body lengths and a 1 cm-sized bronchogenic cyst (BC) was identified near carina on the right side, just below the proximal esophageal pouch. This case report described the baby who have a BC was located between the both esophageal pouch and a longer esophageal gap than usual EA with distal TEF.

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Original Articles

[English]
High Incidence of Hiatal Hernia in Esophageal Atresia and Its Etiologic Factors
Hai Young Son, Eun Young Chang, Hye Kyung Chang, Jung tak Oh, Seok Joo Han
J Korean Assoc Pediatr Surg 2011;17(2):170-178.   Published online December 31, 2011
DOI: https://doi.org/10.13029/jkaps.2011.17.2.170

Hiatal hernia is a very rare disease in the pediatric population. However information from our esophageal atresia postoperative follow-up program has hypotheses; “Hiatal hernia may more frequently occur in postoperative esophageal atresia patients (EA group) than in the general pediatric population (GP group)” and “The tension on the esophagus after esophageal anastomosis may be an important etiologic factor of hiatal hernia in EA group”. To prove the first hypotheses, we compared the incidence of hiatal hernia in the GP group with the incidence in the EA group. The Incidence in the GP group was obtained from national statistic data from Statistics Korea and Health Insurance Review and Assessment Service of Korea. The incidence in the EA group was obtained from the medical record and the imaging studies of our esophageal atresia postoperative follow-up program. To prove the second hypothesis, the presumptive risk factors for the development of hiatal hernia in EA group, such as the type of esophageal atresia, degree of esophageal gap, the stage operation and the redo-operation with resection and re-anastomosis of esophagus were analyzed statistically. The total number of patients in the EA group was ninety-nine and there were 5 hiatus hernias. The incidence of EA group (5 %) is significantly higher than incidence of GP group (0.024 %). (p=0.0001) The statistical analysis of the presumptive risk factors for hiatal hernia development in EA group failed to show any evidence of correlation between postoperative esophageal tension and the hiatal hernia. This study shows that the postoperative patients with esophageal atresia have high occurrence of hiatal hernia and should be followed up carefully to detect hiatal hernia.

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[English]
Clinical Experience of H-type Tracheoesophageal Fistula
Hwui dong Cho, So Hyun Nam, Min Jeng Cho, Tae Hoon Kim, Dae Yeon Kim, Seong Chul Kim, In Koo Kim
J Korean Assoc Pediatr Surg 2011;17(2):154-161.   Published online December 31, 2011
DOI: https://doi.org/10.13029/jkaps.2011.17.2.154

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)~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.

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Case Report

[English]
Hypertrophic Pyloric Stenosis Occurring in Fasting State with Gastrostomy After Surgery for Esophageal Atresia with Tracheoesophageal Fistula
Eunyoung Jung, Soon Ok Choi, Woo Hyun Park
J Korean Assoc Pediatr Surg 2011;17(1):88-92.   Published online June 30, 2011
DOI: https://doi.org/10.13029/jkaps.2011.17.1.88

The onset of hypertrophic pyloric stenosis in the postoperative course of esophageal atresia with tracheoesophageal fistula is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed since birth. The infant presented with an increased amount of orogastric tube drainage and consistently distended gastric air on simple abdominal X-ray. Abdominal ultrasonography showed hypertrophic thick pyloric muscle. The diagnosis of pyloric stenosis was confirmed d is rarely reported. The diagnosis could be delayed due to its mimicking symptoms of other postoperative complications including gastroesophageal reflux or anastomotic stricture. We present an infant who had surgery for esophageal atresia with tracheoesophageal fistula. He had never fed. The infant presented with uring surgery. After pyloromyotomy, the patient's condition improved.

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Original Article

[English]
Associated Anomaly of Esophageal Atresia
Jino Baek, Eunyoung Jung, Woo Hyun Park, Soon Ok Choi
J Korean Assoc Pediatr Surg 2011;17(1):81-87.   Published online June 30, 2011
DOI: https://doi.org/10.13029/jkaps.2011.17.1.81

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 arteriosus (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 4.8% 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.

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Case Reports

[English]

Tracheal injury is a rare complication of endo-tracheal intubation. However in neonates, the rates of morbidity and mortality are high. Recommendations for treatment are based on the several reports of this injury and are individualized. Conservative management can be effective in some cases. We describe the case of a neonate who presented with subcutaneous emphysema after intubation in a neonatal intensive care unit. This patient suffered full VACTERL syndrome and had 1.7mm diameter subglottic stenosis. Conservative management resulted in no further increase in subcutaneous emphysema and after 10 days the patient was stable.

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[English]
Esophageal Atresia with Distal Tracheoesophageal Fistula in Both Members of Monozygotic Twins
Seong Chul Kim, So Hyun Nam, Dae Yeon Kim, In Koo Kim
J Korean Assoc Pediatr Surg 2009;15(2):157-160.   Published online December 31, 2009
DOI: https://doi.org/10.13029/jkaps.2009.15.2.157

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 2,480g, respectively. They had EA with distal tracheoesophageal fistula and underwent primary esophageal anastomosis on the birth day and the 2nd 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 1st operation and three balloon dilatations of the esophagus. Twin B recovered uneventfully.

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Original Article

[English]
The Long-term Outcome of Esophageal Atresia
Jae Hong Jeong, Hye Eun Kim, Suk Koo Lee, Jeong Meen Seo
J Korean Assoc Pediatr Surg 2008;14(2):117-124.   Published online December 31, 2008
DOI: https://doi.org/10.13029/jkaps.2008.14.2.117

Children who underwent reparative operations for esophageal atresia (EA) with or without tracheoesophageal fistula (TEF), are confronted with many gastrointestinal or respiratory problems, especially during the early years of life. We reviewed the medical records of 50 patients who underwent repairs of EA with or without TEF at the Division of Pediatric Surgery, Samsung Medical Center, from December 1994 to December 2005. Current status of children was accessed by telephone-interview, but only 27 of them were accessible. Of 50 patients, 3 patients (6%) were type A, 45 patients (90%) were type C, and 2 patients (4%) were type E. The mean interval between primary operation and interview was 5.5 years. The incidences of growth retardation (<10 percentile of height/weight) were 39% and 21 % during the first 5 years after repairs, respectively. The incidences of dysphagia or gastroesophageal reflux and recurrent respiratory infections were 33% and 39 %, respectively. However, these problems were likely to improve as the children grew. The incidences of growth retardations (<10 percentile of height/weight) were 11% and 11% for the children more than five years postoperative. The incidences of dysphagia or gastroesophageal reflux and recurrent respiratory infections were 22% and 22%, respectively. Children with EA with or without TEF are faced with many obstacles. Close observation and adequate treatment for delayed postoperative complications are necessary to improve the quality of life for these children.

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Case Report

[English]
Esophageal Atresia with Double Tracheoesophageal Fistula: A Case Report
So Hyun Nam, Dae Yeon Kim, Seong Chul Kim, In Koo Kim
J Korean Assoc Pediatr Surg 2008;14(1):88-93.   Published online June 30, 2008
DOI: https://doi.org/10.13029/jkaps.2008.14.1.88

Esophageal atresia with double tracheoesophageal fistula is a very rare anomaly and is difficulty to diagnose preoperatively. We treated a full term baby with esophageal atresia with double tracheoesophageal fistula. At the first operation, only the distal tracheoesophageal fistula was identified and ligated. When the upper esophageal pouch was opened, intermittent air leakages in sequence with positive bagging were noticed. However, intraoperative bronchoscopy did not identify a fistula in the proximal pouch, and the operation was completed with end to end anastomosis of the esophagus. On the 7th postoperative day, esophagography showed another tracheoesophageal fistula proximal to the esophageal anastomosis. A wire was placed in the fistula preoperatively under bronchoscopy. At the 2nd operation through the same thoracotomy incision the proximal fistula was identified and ligated. On the 12th postoperative day, esophagography showed neither stricture nor leakage.

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Original Articles

[English]
Clinical Experience with Esophageal Atresia Combined with Duodenal Atresia
Yu Mi Lee, So Hyun Nam, Dae Yeon Kim, Seong Chul Kim, In Koo Kim
J Korean Assoc Pediatr Surg 2008;14(1):21-26.   Published online June 30, 2008
DOI: https://doi.org/10.13029/jkaps.2008.14.1.21

There is significant morbidity and mortality associated with the combination of esophageal atresia (EA) and duodenal atresia (DA). Nevertheless, the management protocol for the combined anomalies is not well defined. The aim of this study is to review our experience with the combined anomalies of EA and DA. From May 1989 to August 2006, seven neonates were diagnosed as EA with DA at Asan Medical Center. In all cases, the type of EA was proximal EA and distal tracheoesophageal fistula (TEF). The diagnosis of DA was made in theprenatal period in 1, at birth in 4, 4 days after birth in 1 (2 days after EA repair) and at postmortem autopsy in 1. Except the one case where DA was missed initially, primary simultaneous repair was attempted. DA repair with gastrostomy followed by EA repair in 2, EA repair followed by DA repair without gastrostomy in 2, and TEF ligation followed by DA repair with gastrostomy in 1. There were two deaths. One baby had a large posterolateral diaphragmatic hernia, and operative repair was not attempted. The other infant who had a TEF ligation and DA repair with gastrostomy expired from cardiac failure due to a large patent ductus arteriosus. Simultaneous repair of EA and DA appears to be an acceptable management approach for the combined anomalies, but more experience would be required for the selection of the primary repair of both anomalies.

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[English]
A Clinical Review of Esophageal Atresia : One Surgeon's Experience
Sung Sik Kim, Si Youn Rhim, Poong Man Jung
J Korean Assoc Pediatr Surg 2008;14(1):12-20.   Published online June 30, 2008
DOI: https://doi.org/10.13029/jkaps.2008.14.1.12

From 1979 to 2006, fifty eight patients with esophageal atresia were treated by one pediatric surgeon at Hanyang University Hospital. We analyzed the clinical findings and outcome of these 58 patients. There were 30 males and 28 females. Their mean birth weight was 2,960 ± 400 g (1,170~4,020 g). The most common type of anomaly was Gross type C (49 patients; 84.5 %). There was no type B. Fifty-two patients underwent definitive surgery. Postoperative complications were as follows: anastomotic leakage in 17 patients (32.7 %), anastomotic site stricture in 15 (28.8 %), gastroesophageal reflux in 10 (19.2 %) and recurrent TEF in 1 (1.9 %). A total of 152 associated anomalies were detected from 48 patients (82.2 %). The cardiovascular system was the most commonly affected (30 patients with 46 anomalies). The VACTERL association was present in 14 patients (24.1 %). Operative mortality was 17.3 % including self-discharge patients after operation.

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[English]
Esophageal Atresia with Tracheoesophageal Fistula: A 20 Year Experience of 51 Cases
Jae Hyun Han, Jae Hee Chung, Young Tack Song
J Korean Assoc Pediatr Surg 2008;14(1):1-11.   Published online June 30, 2008
DOI: https://doi.org/10.13029/jkaps.2008.14.1.1

The aim of this study is to analyze the outcomes of the esophageal atresia with tracheoesophageal fistula over the last 2 decades. The records of 51 patients born between 1987 and 2006 were reviewed. Twenty-seven patients were male. Mean values of the age, gestational age and birth weight were 2.9 days, 296 days and 2.7kg, respectively. All patients had Gross type C anomalies. Thirty-one patients (60.7 %) had one or more associated congenital anomalies and the most common anomaly was cardiac malformation. In 48 cases, primary anastomosis was done and staged operation was done in one case. Circular myotomies in the proximal esophagus were performed in 9 cases. Postoperative complication developed in 26 cases (54 %): pulmonary complication in 12 cases, anastomotic leakage in 10 and anastomotic stricture in 10, recurrent trachoesophageal fistula in one and tracheomalasia in 2 cases. Reoperation was carried out in 2 patients with anastomotic leaks, the remaining leaks were managed non operatively. Three of the strictures were reoperated upon and the others were successfully managed by balloon dilatations. Overall mortality rate was 15.6 %. Mortality rate of the second 10 years (8 %) period decreased significantly compared to that of the first 10 years (23 %) period.

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[English]
Result of Secondary Surgery after Primary Surgery for Esophageal Atresia Anomalies
Soo Chan Im, Suk Bae Moon, Sung Eun Jung, Seong Cheol Lee, Kwi Won Park
J Korean Assoc Pediatr Surg 2007;13(2):105-111.   Published online December 31, 2007
DOI: https://doi.org/10.13029/jkaps.2007.13.2.105

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.

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[English]
The Experience of the VATER Association in One Hospital
So Hyun Nam, Seong Chul Kim, In Koo Kim, Dae Yeon Kim
J Korean Assoc Pediatr Surg 2006;12(2):175-182.   Published online December 31, 2006
DOI: https://doi.org/10.13029/jkaps.2006.12.2.175

VATER association is defined as a combination of 3 or more anomalies-vertebra(V), imperforate anus (A), esophageal atresia with or without tracheoesophageal fistula (TE), renal and radial anomaly(R). We reviewed our experiences in one center to determine etiology, prevalence, clinical manifestation, other associated anomaly and prognosis. Two hundred and twenty-three cases that underwent operations for imperforate anus or esophageal atresia were analyzed retrospectively through medical records at Department of Pediatric Surgery, Asan Medical Center from June, 1989 to July, 2005. The total number of neonates who had been admitted during period of study were 46,773 and VATER association was 9 (0.019 %, 1.92 persons per 10,000 neonates). Median gestational age and birth weight were 37+4wk (35+1 – 41+4) and 2,594 g (1,671–3,660), respectively and median age of mother was 32 years (23–38). There was no family history. Three patients were twins but their counterparts had no anomalies. Patients who have 3 anomalies were 6, 4 anomalies in two and 5 anomalies in one patient. Vertebra anomalies were detected in 7(77.7 %), imperforate anus in 8(88.9 %), esophageal atresia in 5 patients (55.6 %), renal anomaly in 6(66.7 %), and radial anomaly in 5(55.6 %), respectively. Four patients are alive, 2 patients were lost during follow up period. Three patients died due to neonatal sepsis, respiratory dysfunction and cardiac failure. VATER association did not appear to be a definite risk factor, but merely a randomized combination of 5 anomalies. The prognosis was dependent on the other associated anomalies, appropriateness of management and operation. Careful follow-up and aggressive treatmentare required for improving survival and quality of life.

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[English]
Long Gap Esophageal Atresia Successfully Treated by Esophageal Lengthening Using External Traction Sutures
Doo Sun Lee, Hwan Namgung, Jung Suk Yoon
J Korean Assoc Pediatr Surg 2005;11(2):165-170.   Published online December 31, 2005
DOI: https://doi.org/10.13029/jkaps.2005.11.2.165

The repair of esophageal atresia with a long gap still continues to pose difficulties for the surgeon. There is general agreement that the child's own esophagus is best, but it is also believed that a primary repair is not always possible. Foker JE et al. (1997) developed a technique of esophageal lengthening using external traction sutures. We experienced one case of esophageal atresia with a 4.5cm gap (4 vertebral spaces) which was repaired using the external traction suture technique.

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[English]
Gastric Tube Replacement in Esophageal Atresia
Chang Sup Lim, Hyun Young Kim, Kwi Won Park, Sung Eun Jung, Seong Cheol Lee, Woo Ki Kim
J Korean Assoc Pediatr Surg 2004;10(2):92-98.   Published online December 31, 2004
DOI: https://doi.org/10.13029/jkaps.2004.10.2.92

The history of esophageal replacement in infants or children is the history of development of various kinds of alternative conduits such as stomach, colon, and small bowel. The gastric tube has been the most widely used conduit. From January 1988 to May 2003, 23 esophageal replacements with gastric tube were performed at the Department of Pediatric Surgery, Seoul National University Childrens Hospital. Statistical analysis was performed using Windows SPSS11.0 Pearson exact test. There were Gross type A(n=10), type B(n=1), type C(n=11), type D(n=1). Ten patients who had long gap esophageal atresia (type A-8, type B-1, type C-1) and 13 patients (type A 2, type C-10, type D-1) who had stenosis, leakage, recurred tracheoesophageal fistula, and esophagocutaneous fistula after previous corrective operations, had esophageal replacement with gastric tube. Mean follow-up periods were 4 year 2 months (7 months-15 year 1 month). There were postoperative complications including GERD in 16 (69.6 %), leakages in 7 (30.4 %), diverticulum at anastomosis in 2 (8.7 %), anastomosis site stenosis in 4 (17.3 %), and distal stenosis of the gastric tube in 1 (4.3 %). There was no statistical significance between operation types and postoperative leakage and gastroesophageal reflux. In conclusion, esophageal replacement with gastric tube may be a useful surgical option in esophageal atresia with long gap and esophageal atresia complicated by previous corrective operation.

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Reviews

[English]
Postoperative Complications and Their Management
Jung Hong
J Korean Assoc Pediatr Surg 2003;9(2):145-147.   Published online December 31, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.2.145

No abstract available.

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[English]
Postoperative Management
Jeong Meen Seo
J Korean Assoc Pediatr Surg 2003;9(2):143-144.   Published online December 31, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.2.143

No abstract available.

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[English]
Operative Management of the Long Gap Esophageal Atresia and Rare Forms
Kwi won Park
J Korean Assoc Pediatr Surg 2003;9(2):140-142.   Published online December 31, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.2.140

No abstract available.

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[English]
Preoperative Preparation and Operative Management
Soon Ok Choi
J Korean Assoc Pediatr Surg 2003;9(2):134-139.   Published online December 31, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.2.134

No abstract available.

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[English]
Associated Anomalies and Prognostic Factors
Doo Sun Lee
J Korean Assoc Pediatr Surg 2003;9(2):131-133.   Published online December 31, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.2.131

No abstract available.

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[English]
Diagnosis
Sang Young Chung
J Korean Assoc Pediatr Surg 2003;9(2):128-130.   Published online December 31, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.2.128

No abstract available.

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[English]
Introduction Etiolology and Classification
In Koo Kim
J Korean Assoc Pediatr Surg 2003;9(2):126-127.   Published online December 31, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.2.126

No abstract available.

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Original Article

[English]
Clinical Experience of Esophageal Atresia
Seong Chul Kim, Dae Yeon Kim, Ellen Ai Rhan Kim, Ki Soo Kim, Soo Young Pi, In Koo Kim
J Korean Assoc Pediatr Surg 2003;9(1):6-11.   Published online June 30, 2003
DOI: https://doi.org/10.13029/jkaps.2003.9.1.6

This study reviews 14 years' experience of esophageal atresia with special emphasis on the clinical profile and the outcome. From May 1989 to February 2003, 65 cases of esophageal atresia (EA) were treated at Asan Medical Center. Boys outnumbered girls 2.4 to 1. Prematutity and low birth weight were 27.7% and 38.5%. Esophageal atresia with distal tracheoesophageal fistula (TEF) was the most common type (87.7%), followed by pure EA and H type fistula. Forty-six patients (70.8%) had one or more associated anomalies, cardiac malformations were the most common. Duodenal atresia was found in 7 cases. There were 6 patients (9.2%) with VATER cluster. VACTERL cluster was present in 18 patients (27.7%), one of who fulfilled the complete syndrome. Waterston group A, B and C made up 21.5%, 40.0% and 38.5% of the total group. Surgical treatment was attempted in 63 patients and deferred in 2 who had severe associated malformations. For EA with distal TEF, primary esophago-esophagostomy was carried out in 51 cases, and division of TEF and gastrostomy in 4 cases and no operation in 2 cases. For pure EA, colonic graft was done in 2 after gastrostomy and esophagostomy, and esophago-esophagostomy was performed in 2 after gastrostomy. Two TEF was carried out in 2 cases with H type TEF. The overall survival rate was 76.9%, and survival by Waterstuon classification was 100% in group A, 80.8% in B and 60.0% in C. Thorough workup for associated anomalies, interdepartmental approach and more careful surgical decision and technique are required to improve the outcome of EA.

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Case Report

[English]
Congenital Tracheomalacia Associated with Esophageal Atresia
Seok Joo Han, Eun Joo Jung, Se Heon Kim, Choon Sik Yoon, Kyu Dae Shim, Yong Taek Nam, Jai Eok Kim, Eui Ho Hwang
J Korean Assoc Pediatr Surg 2002;8(2):161-165.   Published online December 31, 2002
DOI: https://doi.org/10.13029/jkaps.2002.8.2.161

This is a case of tracheomalacia associated with esophageal atresia. An 11-month-old- male boy presented with a life-threatening apneic spell after correction of esophageal atresia (Gross type C). After complete exclusion of the other possible causes of the apneic spell, the presumptive diagnosis of tracheomalacia was made with fluoroscopy and 3-dimensional chest CT. The final diagnosis was made with rigid bronchoscopy under spontaneous respiration. The aortopexy was performed with intraoperative bronchoscopic examination. The postoperative period was unremarkably uneventful. The patient was discharged 9 days after the aortopexy and has remained well to date (5 months after the aortopexy).

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Original Article

[English]
Total Parenteral Nutrition (TPN) via Peripheral Veins in Neonatal Surgical Patients
Jong In Lee, Poong Man Jung
J Korean Assoc Pediatr Surg 1998;4(1):16-26.   Published online June 30, 1998
DOI: https://doi.org/10.13029/jkaps.1998.4.1.16

Parenteral nutntlon has been an essential part of postoperative care of neonates requiring major surgery who are unable to tolerate enteral feeding for long periods during the postoperative period. However, TPN via central venous catheters(central TPN), used in increasing trend, still presents significant morbidity. To find out whether TPN via peripheral veins(peripheral TPN) could be used as a viable alternative for postoperative parenteral nutrition in neonates, a clinical study was carried out by a retrospective analysis of 53 neonates subjected to peripheral TPN for more than 7 days after surgery. Operations consisted of procedures for esophageal atresia with tracheoesophageal fistula, gastroschisis and omphalocele. Surgery was performed at the Division of Pediatric Surgery, Department of Surgery, Hanyang University Hospitall, from 1983 to 1994. The mean total duration of TPN was 13.3 days (range; 7-58 days), the average daily total fluid intake was 117.6 ml/kg during TPN and 158.6 ml/kg during subsequent oral feeding. The average daily total calorie intake was 57.7 kcal/kg during full strength TPN and 101.3 kcal/kg during subsequent oral feeding. The mean urine output was maintained at 3.5 ml/kg/hour during TPN and at 3.6 ml/kg/hour during subsequent oral feeding. The increment of body weight observed during TPN was 132g in TEF, 53g in gastroschisis and 3g in omphalocele patients, while loss of body weight was not observed. The mortality rate was 5.7%(3/53) and was related to the underlying congenital anomalies, not the TPN. The most common complication of peripheral TPN observed was laboratory findings suggestive of liver dysfunction in 23 cases( 43.4%) with no significant clinical symptom or signs in any case, transient pulmonary edema in one case, and generalized edema in one case. None of the major complications usually expected associated with central TPN were observed. The result of this study suggest that peripheral TPN can be used for adeguate postoperative nutritional support in neonates requiring 2 to 3 weeks of TPN.

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Case Reports

[English]
Esophageal Replacement with Transhiatal Gastric Transposition in the Long Gap Esophageal Atresia: Report of Two Cases
Seok Joo Han, Sung Do Kim, Choong Bai Kim, Jung Tak Oh, Eui Ho Hwang
J Korean Assoc Pediatr Surg 1997;3(2):152-159.   Published online December 31, 1997
DOI: https://doi.org/10.13029/jkaps.1997.3.2.152

Transhiatal gastric transpositions were performed in two case of long gap esophageal atresia without tracheoesophageal fistula. The patients were a 12 months old female and an 18 months old male. Stamm type gastrostomies were performed at other hospitals in both cases. The stomach was mobilized preserving the right gastric artery, the right gastroepiploic artery and spleen. The proximal and distal esophageal pouches were excised by transcervical and transhiatal route, respectively. The mobilized stomach was pulled up to the neck through esophageal hiatus and posterior mediastinal route. The esophagogastrostomy, the only one anastomosis of this procedure, was performed in the neck. There was no clinical evidence of anastomotic leakage, stricture, regurgitation, difficulty of gastric emptying, hoarseness or respiratory problem. Transhiatal gastric transposition seems to be a safe and easy alternative surgical procedure for esophageal replacement in long gap esophageal atresia.

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[English]
Double Tracheoesophageal Fistula
Young Soo Huh, Hee Sub Lee, Geun Woo Kim
J Korean Assoc Pediatr Surg 1996;2(1):68-71.   Published online June 30, 1996
DOI: https://doi.org/10.13029/jkaps.1996.2.1.68

Esophageal atresia(EA) with a double tracheoesophageal fistula(TEF) is rare. It accounts for only 0.7% of all cases of EA and TEF. A male newborn weighing 2860g was born by normal vaginal delivery at 41weeks' gestation to a 27-year-old mother who had a normal pregnancy. But immediately developed recurrent choking and respiratory distress. Feeding tube was inserted and chest X ray showed the feeding tube coiled in the proximal pouch(T-2 level). With a preoperative diagnosis of EA and distal TEF, the thoracotomy was performed on the third day of life. At thoracotomy, TEF was proved to be a double fistula. Both fistulas were divided and an esophageal anastomosis was performed. The postoperative course was uncomplicated until the eighth postoperative day when a minor anastomotic leak. The patient was discharged at 103 days of age.

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Original Articles

[English]
Reversed Gastric Tube Interposition in Esophageal Atresia
Seung Huh, Yang-Soon Chun, Kwi-Won Park, Woo-Ki Kim
J Korean Assoc Pediatr Surg 1995;1(2):162-169.   Published online December 31, 1995
DOI: https://doi.org/10.13029/jkaps.1995.1.2.162

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.

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[English]
Esophageal Atresia and Tracheoesophageal Fistula in Korea: A National Survery of Its Members by the Korean Association of Pediatric Surgeons
WH Park, SI Kwon, SC Kim, SK Kim, WK Kim, IK Kim, JE Kim, HH Kim, KW Park, YS Park, YT Song, JW Yang, SM Oh, SY Yoo, DS Lee Lee, SC Lee, SK Lee, TS Lee, SI Chang, SY Chun, ES Chung, SY Chung, SE Chung, PM Chung, MH Cho, JS Joo, SO Choi, SH Choi, YS Huh, C Hong
J Korean Assoc Pediatr Surg 1995;1(2):149-161.   Published online December 31, 1995
DOI: https://doi.org/10.13029/jkaps.1995.1.2.149

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 I, 1992 through Decestricurember 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 returped 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(87.50/0) was by far the most common and threre were pure esopahgeal atresia(5.6%), H-type TEF(2.l%), 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 surgical 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 grpup A, 71.4-75% in B1, B2, and C groups, and 28% in group C2 and the overall survival rate was 71.4%. Thirty six(28.6%) of 126 cases died of pneumonia/sepsis(12 cases), respiratory failure(l2 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 stricure.

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[English]
Seventeen Years' Experience with Ninety-six Esophageal Atresias
Yang-Soon Chun, Sung-Eun Jung, Soong-Cheal Lee, Kwi-Won Park, Woo-Ki Kim
J Korean Assoc Pediatr Surg 1995;1(2):140-148.   Published online December 31, 1995
DOI: https://doi.org/10.13029/jkaps.1995.1.2.140

To study the clinical profiles and outcome of surgery in infants with esophageal atresia, we reviewed 96 esophageal atresia cases who were treated from April, 1978 to June, 1995. There were 51 male and 45 female infants, a ratio of 1.1:1. The low birth weight(<2500g) patients were 32%. Clinical findings at the time of diagnosis inculded drooling in 57%, choking in 50%, cyanosis in 38%, respiratory distress in 27% and swallowing difficulty in 20%. Gross classification included 6 cases of type A esophageal atresia(6%), 79 cases of type C(82%). 3 cases of type E (3%) and 8 cases of type F(8%). Associated anomalies occurred in 34 infants(35%). Among them, cardiac anomalies were most common(60%). A primary repair of the defect was carried out in 76 patients with type A or C. A staged operation comprising a repair or gastric tube interposition after gastrostomy was performed in 8 patients. In all 3 infants with H-type, a division of fistula was performed. Esophageal resection and anastomosis was done in 8 infants with esophageal stenosis. In one infant, a gastrostomy was performed and he expired before staged operation. Anastomotic complications incuded leakage in 16 cases(l7%), stricture in 37 cases(39%) and recurrent tracheoesopohageal fistula in 3 cases(3%). The mortality rate was 14% and the leading cause of death was peumonia. The overall survival rate was 86%, and according to Waterston cirteria, the survival rates were 93%, 85% and 58% in class A, Band C, respectively. 75 patients were followed up with median follow up 6.4 years. Among them, 93% were uneventful and 7% had frequent pneumonia.

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Case Report

[English]
Primary Esophageal Repair of Long-gap Esophageal Atresia: Report of One Case
Myung Duk Lee
J Korean Assoc Pediatr Surg 1995;1(1):53-58.   Published online June 30, 1995
DOI: https://doi.org/10.13029/jkaps.1995.1.1.53

A delayed primary esophago-esophagostomy of a case of long-gap esophageal atresia without tracheoesophageal fistula was performed in success with three months' intermittent periodic bougienage of the upper pouch via mouth as well as the lower esophagus through Janeway gastrostomy. Meanwhile, an effective continuous sump suction from the upper pouch seemed to be a critical part of the patient management. The extra length of esophagus for primary anastomosis could be achieved by a circular myotomy. Stricture at the myotomy site, found 4 months later, was treated with periodic pneumatic baloon dilations only with temporary symptomatic reliefs. After 4 months' trials, operative esophagoplasty was performed successfuly. A careful follow-up schedule for the myotomy site would be required for early detection of stricture. The previous neonatal patient is currently 8 years old, healthy schoolboy, and has a normal barium swallow without stricture or gastroesophageal reflux.

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Original Articles

[English]
Evaluation of Prognosis in Esophageal Atresia
Kim, Hyun Hahk , Jeong, Hyeon A , Kim, Kyung Hun , Kim, Jung Han , Lee, Suk Koo
J Korean Assoc Pediatr Surg 2001;7(1):1-6.
DOI: https://doi.org/10.13029/jkaps.2001.7.1.1
The aims of this study were to evaluate the prognostic predictabilities of the risk classifications (Waterston, Montreal, and Spitz), preoperative treatment of pneumonia and the length of esophageal gap in esophageal atresia. Twenty-five cases of esophageal atresia were reviewed retrospectively. The data was analyzed with Likelihood ratio test for trend and Fisher's exact test. Survival rates were 100.0% in group A and B, and 60.0% in group C according to Waterston classification (p=0.027). By Montreal classification, 95.7% in group I and 50.0% in group II (p=0.086). By Spitz classification, 95.5% in group I and 66.7% in group II (p=0.159). The survival rate was 85.7% in the patients with pneumonia preoperatively treated and 94.4% in the patients without pneumonia. The difference was not significant (p=0.490). Survival rate in the patients with long gap (> or = 3cm) was 50.0% and 100.0% in patients with short gap (< 3cm) with significant difference (p=0.020). The Waterston classification could be modified into two groups because the prognosis of both group A and B were the same. Preoperative pneumonia does not seem to be a risk factor. The length of the esophageal gap showed prognostic value.
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[English]
Nissen Fundoplication for the Treatment of Gastroesophageal Reflux After Repair of Esophageal Atresia
Ho, In Geol , Ihn, Kyong , Jeon, Ho Jong , Lee, Dongeun , Oh, Jung-Tak , Han, Seok Joo
Adv Pediatr Surg 2022;28(1):9-16.
DOI: https://doi.org/10.13029/aps.2022.28.1.9
Purpose
Gastroesophageal reflux disease (GERD) frequently affects patients with esophageal atresia (EA). GERD is often resistant to medical therapy, and anti-reflux surgery in the form of fundoplication is required. However, there are no generally accepted indications for fundoplication in EA patients with significant gastroesophageal reflux (GER). This study aimed to report the outcomes and review the clinical characteristics and treatment of GERD with Nissen fundoplication among EA patients.
Methods
The medical records of patients with EA repair who underwent Nissen fundoplication for GERD treatment from January 2005 to December 2018 were retrospectively reviewed. Twenty patients were included in this study.
Results
In total, 166 patients with EA underwent treatment during the study period. Among these, 20 (12%) patients underwent Nissen fundoplication. The median age at the time of fundoplication was 5.3 (range, 1.6–49.4) months, and the mean body weight was 6.3±2.4 kg. The indications for Nissen fundoplication were hiatal hernia in 18 (90%) patients, feeding intolerance in 17 (85%), recurrent aspiration pneumonia in 13 (65%), GER-related refractory anastomosis stenosis in seven (35%), and reflux-related acute life-threatening events in five (25%). The median follow-up duration after Nissen fundoplication was 5.7 (range, 2.4–10.7) years; two (10%) patients experienced surgery-related complications, 11 (55%) had recurrent hiatal hernia, and three (15%) required re-operation. Feeding intolerance improved (p<0.001), and the number of esophageal dilatations decreased following Nissen fundoplication (p=0.038). There was no mortality during the follow-up period.
Conclusion
Nissen fundoplication was required in patients with more complicated EA and was performed at a young age. Nissen fundoplication was relatively effective in controlling EA-associated GERD. However, Nissen fundoplication in patients with EA may be associated with a high rate of recurrent hiatal hernia. A careful follow-up is necessary because poor long-term outcomes are common for such patients.
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[English]
Do Children with Esophageal Atresia Show Worse Growth Outcomes?
Ko, Dayoung , Lee, Changhoon , Youn, Joong Kee , Yang, Hee-Beom , Kim, Hyun-Young
Adv Pediatr Surg 2020;26(2):54-60.
DOI: https://doi.org/10.13029/aps.2020.26.2.54
Purpose
Patients with esophageal atresia (EA) often have feeding problems due to esophageal dysmotility, gastroesophageal reflux, and dysphagia, which can delay growth in children. The purpose of this study was to investigate whether proper growth is achieved during the early childhood of patients with EA and to predict associated factors.
Methods
Clinical data of patients with EA who underwent corrective surgery between 2014 and 2017 were collected retrospectively. We analyzed a total of 17 patients who were followed up for at least two years after surgery, except for patients with long-gap and type E EA. The median follow-up period was 979.0 months. We analyzed the weight, height, and weight-forheight (WFH) as z-scores. Linear regression analysis was performed to determine the factors affecting WFH at two years of age.
Results
Of the 17 patients, 11 underwent open surgery and six underwent thoracoscopic surgery. The median time to full feeding was approximately 14 (range, 12.0–53.0) days. In patients with anastomotic stenosis, esophageal balloon dilatation was performed 1–6 times. There was no mortality in our study, and the median follow-up period was 979 days. The mean height was 49.0 cm, the mean weight at birth was 2.69 kg, and the z-scores were −0.55 and −1.44, respectively. The WFH z-score decreased from −1.66 at birth to −1.82 one week postoperatively; however, it improved to −0.2 after six months. In multivariate linear regression analysis, only WFH at birth was a significant variable for WFH at two years of age.
Conclusion
Patients with EA tend to have lower weight and WFH at birth, which worsened after surgery; however, six months after surgery, both weight and WFH recovered to the 50th percentile. The factors that influence WFH at two years of age were significantly related to WFH at birth.
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