Clinical characteristics and treatment of esophageal atresia

Eun Young Chang,Hye Kyung Chang,Seok Joo Han,Seung Hoon Choi,Eui Ho Hwang,Jung-Tak Oh 대한외과학회 2012 Annals of Surgical Treatment and Research(ASRT) Vol.83 No.1

Purpose: Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. Subsequent to increased survival, postoperative morbidity has become an important issue in this disease. The aim of our study was to analyze our experience regarding the treatment of esophageal atresia. Methods: We reviewed and analyzed the clinical data of patients who underwent surgery for esophageal atresia at Severance Children’s Hospital from 1995 to 2010 regarding demographics, surgical procedures, and postoperative outcomes. Results: Seventy-two patients had surgery for esophageal atresia. The most common gross type was C (81.9%), followed by type A (15.3%). Primary repair was performed in 52 patients. Staged operation was performed in 17 patients. Postoperative esophageal strictures developed in 43.1% of patients. Anastomotic leakages occurred in 23.6% of patients, and recurrence of tracheoesophageal fistula was reported in 8.3% of patients. Esophageal stricture was significantly associated with long-gap (≥3 ㎝ or three vertebral bodies) atresia (P = 0.042). The overall mortality rate was 15.3%. The mortality in patients weighing less than 2.5 kg was higher than in patients weighing at least 2.5 kg (P = 0.001). During the later period of this study, anastomotic leakage and mortality both significantly decreased compared to the earlier study period (P = 0.009 and 0.023, respectively). Conclusion: The survival of patients with esophageal atresia has improved over the years and the rate of anastomotic leakage has been significantly reduced. However, overall morbidities related to surgical treatment of esophageal atresia still exists with high incidence.

Anorectal Malformations Associated with Esophageal Atresia in Neonates

변신연,임령경,박경희,조용훈,김해영 대한소아소화기영양학회 2013 Pediatric gastroenterology, hepatology & nutrition Vol.16 No.1

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. (Pediatr Gastroenterol Hepatol Nutr 2013; 16: 28∼33)

Anorectal Malformations Associated with Esophageal Atresia in Neonates

Byun, Shin Yun,Lim, Ryoung Kyoung,Park, Kyung Hee,Cho, Yong Hoon,Kim, Hae Young The Korean Society of Pediatric Gastroenterology 2013 Pediatric gastroenterology, hepatology & nutrition Vol.16 No.1

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.

기관 식도루를 동반한 선천성 식도 폐쇄증의 외과적 치료

김동원 대한흉부심장혈관외과학회 1994 Journal of Chest Surgery (J Chest Surg) Vol.27 No.7

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.

선천성 식도 폐쇄증의 외과적 치료

최필조,전희재,이용훈,조광조,성시찬,우종수 대한흉부심장혈관외과학회 1999 Journal of Chest Surgery (J Chest Surg) Vol.32 No.6

배경: 선천성 식도 폐쇄증의 수술적 교정은 많은 개선을 보였으나 아직도 사망률과 합병증률이 높은 상태이다. 방법: 1992년 1월부터 1997년 3월까지 본 동아대학교 병원 흉부외과학 교실에서는 27명의 선천성 식도 폐쇄증을 수술적 치료하였기에 이를 후향적으로 분석하고자 하였다. 결과: 남아가 21명 여아가 6명이었으며 평균 출생시 체중은 2.62$\pm$0.38 kg이었다. 원위부 기관식도루를 가진 식도폐쇄증이 24명이었고 나머지 3명은 순수 식도 폐쇄증이었다. Waterston의 위험군 분류상 A군이 4명, B군이 18명, C군이 5명이었다. 18명에서 동반 기형을 가졌으며 심혈관계 이상이 가장 흔한 동반 기형이었다. 식도 폐쇄증의 상하 식도 맹단 간의 거리는 1 cm이내의 short gap이 9명, 1내지 2 cm의 medium gap이 8명, 2 cm이상의 long gap이 7명, 3.5 cm 이상의 ultra-long gap이 3명이었다. 수술은 순수 식도 폐쇄증의 3례에서는 단계적으로 위루술 및 경부식도조루술을 시행하였고 나머지 24례는 모두 경흉강적 도달법으로 일시적 근치 교정술을 시행하였다. 추적기간 중 27명 중5명이 사망 하였는데 수술과 관련된 사망은 2명으로 그 중 1명은 급성 신부전으로 사망하였고, 1명은 문합부 누출로 인한 농흉 발생으로 사망하였다. 3명의 후기 사망이 있었는데 1명은 순수 식도 폐쇄증으로 식도-위-대장 문합술을 시행하였으나 인슐린 의존성 당뇨병으로 술후 29개월 째 사망하였고 1명은 동 괴사성장염(necrotizing enterocolitis)으로 사망, 나머지 1명은 \ulcorner선 확장술의 합병증으로 인해 술후 220일 째 십이지장 천공으로 인한 폐혈증으로 사망하였다 사망례의 분석을 통해 사망률에 영향을 미쳤던 요인들을 살펴 보았는데 gap length가 long gap이상일 경우가 사망률에 영향을 미치는 요소로서 통계적 유의성(p value<0.05)을 보였다. 결론: 수술적교정과 관련된 합병증률은 높은 편이나 이것이 수술사망률과 직결되는 것은 아니다. 전체적인 생존율은 효과적인 동반기형의 치료와 적극적인 수술후 관리가 병행될 때 더욱 향상시킬 수 있을 것이다. Background: Surgical correction of the full spectrum of esophageal atresia with tracheoesophageal fistula has improved over the years, but the mortality and morbidity assoiated with repair of these anomalies still remains high. Material and Method: We retrospectively analyzes 27 surgically treated patients with esophageal atresia and tracheoesophageal fistula at Dong-A University Hospital between January 1992 and March 1997. Result: There were 21 male and 6 female patients. Mean birth weight was 2.62$\pm$.385 kg(2.0~3.4 kg). Twenty- four(88.9%) had esophageal atresia with distal tracheoesophageal fistula, and 3(11.1%) had pure esophageal atresia. Four(14.8%) infants were allocated to Waterston risk group A, 18(66.7%) to group B, and 5(18.5%) to group C. In eighteen(66.7%) infants with associated anomalies, cardiovascular anomalies were the most common. Three had a gap length of 3.5 cm or greater(ultra-long gap) between esophageal segments, 7 had 2.0 to 3.5 cm(long gap), 8 had 1.0 to 2.0 cm(medium gap), and 9 had 1 cm or less(short gap) gap length. Among 27 neonates, 3 cases underwent staged operation, late colon interposition was done in 2, and all other 24 cases underwent primary esophageal anastomosis. Oerative mortality was 2/27(7.4%). Causes of death included acute renal failure(n=1), empyema from anastomotic leak(n=1), necrotizing enterocolitis(n=1), sepsis(n=1), insulin-dependent diabetus mellitus(n=1 . There were 4 anastomosis- related complications including stricture in 3, leakage in 1. Mortality was related to the gap length(p<.05). Conclusion: Although the complication rate associated with surgical repair of these anomalies is high, this does not always implicate the operative mortality. The overall survival can be improved by effective treatment for combined anomalies and intensive postoperatve care.

원간격결손 식도페쇄증에서 경열공적 위전위법을 이용한 식도재건술

한석주,김성도,김충배,오정탁,황의호,Han, Seok-Joo,Kim, Sung-Do,Kim, Choong-Sai,Oh, Jung-Tak,Hwang, Eui-Ho 대한소아외과학회 1997 소아외과 Vol.3 No.2

Transhiatal gastric transposition was 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. A portion of the proximal and the distal esophageal segment were excised by transcervical and transhiatal route, respectively. The mobilized stomach was pulled up to the neck through the esophageal hiatus and posterior mediastinum. 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.

식도 및 십이지장 병합폐쇄증 1례

서주영,이철,정우영,이순용,김연순 대한소아과학회 1985 小兒科 Vol.28 No.9

선천성 식도폐쇄 및 기관식도루 수술치험 2례

한동기 대한흉부심장혈관외과학회 1994 Journal of Chest Surgery (J Chest Surg) Vol.27 No.3

Early recognition, vigorous preoperative preparation, sophisticated supportive care, control of sepsis, and intensive care nursing have produced remarkably improved results in the management of esophageal atresia. Successful surgery for esophageal atresia and tracheoesophageal fistula was carried out recently. Two neonates with esophageal atresia and distal tracheoesophageal fistula were type C. Transpleural end-to-end repair was carried out after gastrostomy due to low birth weight in case I associated with ventricular septal defect. Case 2 underwent primary retropleural end-to-end repair. A simple one-layer anastomosis with the sutures passing through all layers of`the esophagus was performed in all cases.

Transhiatal gastric transposition of a long gap esophageal atresia

Han, Seok Joo,Kim, Choong Bai,Kim, Do Il,Hwang, Eui Ho Yonsei University, College of Medicine 1995 Yonsei medical journal Vol.36 No.1

선천성 식도폐쇄를 가진 식이장애 환아의 치아우식 치료 증례 보고

허선재(Seon-Jae Heo),이미연(Mi-Yeon Lee),신터전(Teo Jeon Shin),현홍근(Hong-Keun Hyun),김정욱(Jung-Wook Kim),장기택(Ki-Taeg Jang),이상훈(Sang-Hoon Lee),김종철(Chong-Chul Kim),김영재(Young-Jae Kim) Asia association of Disability and Oral health 2016 International Journal of Disability and Oral Healt Vol.12 No.1

저자는 선천적 식도폐쇄로 인한 섭식장애를 가진 5세 환아에 대한 임상적 및 방사선학적 관찰을 통해 다음과 같은 지견을 얻었다. 1. 환아는 식도-기관 누공을 동반하는 선천성 식도폐쇄로 인해 위절제술 및 식도 문합술을 시행받았다. 2. 환아는 잦은 음식 섭취와 구토로 치아부식과 함께 다발성 치아우식증 소견을 보이고 있다. 3. 환아의 구강 위생 관리 및 우식 예방을 위해 정기적인 치과 내원과 가정에서 체계적인 관리가 필요하며, 식이와 관련된 재활 치료가 필요하다. Esophageal atresia is the abnormal development of the esophagus that connects the mouth to the stomach. This birth defect results in the incomplete connection of the esophagus to the stomach causing an inability to swallow properly and breathing difficulties. Surgery is the only treatment for esophageal atresia. Patients undergone an esophageal reconstruction usually suffer from gastroesophageal reflex. A 5-year old boy with congenital esophageal atresia and Sciwitar syndrome visited Seoul National University Dental Hospital because generalized excessive loss of tooth structure. The patient had history of multiple operations for reconstruct esophagus. He had little food intake experience thought oral and had difficulty to swallowing. The patient was generalized severe erosive and decayed state. For patient, the dental procedure under general anesthesia was scheduled due to the multiple caries with erosion and poor cooperation. Under general anesthesia, pulpectomy and restoration as well as extraction was performed. Gastroesophageal reflex is potentially serious condition, with various extraesophageal adverse effects such as dental erosion. To prevent progression of dental caries, dietary counselling and oral hygiene instruction should be reinforced. Also, restorations to erosive teeth will maintain esthetics and function and preserve pulp vitality.