Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association

Abstract Background A communicating bronchopulmonary foregut malformation (CBPFM) group IB is very rare congenital malformation. Group IB is associated with tracheoesophageal fistula and esophageal atresia (TEF-EA) and a portion of one lung arisen from the esophagus (Gerle et al. in N Engl J Med. 27...

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Main Authors: Toshio Harumatsu, Tatsuru Kaji, Ayaka Nagano, Mayu Matsui, Masakazu Murakami, Koshiro Sugita, Makoto Matsukubo, Satoshi Ieiri
Format: Article
Language:English
Published: Japan Surgical Society 2021-01-01
Series:Surgical Case Reports
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Online Access:https://doi.org/10.1186/s40792-020-01099-y
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author Toshio Harumatsu
Tatsuru Kaji
Ayaka Nagano
Mayu Matsui
Masakazu Murakami
Koshiro Sugita
Makoto Matsukubo
Satoshi Ieiri
author_facet Toshio Harumatsu
Tatsuru Kaji
Ayaka Nagano
Mayu Matsui
Masakazu Murakami
Koshiro Sugita
Makoto Matsukubo
Satoshi Ieiri
author_sort Toshio Harumatsu
collection DOAJ
description Abstract Background A communicating bronchopulmonary foregut malformation (CBPFM) group IB is very rare congenital malformation. Group IB is associated with tracheoesophageal fistula and esophageal atresia (TEF-EA) and a portion of one lung arisen from the esophagus (Gerle et al. in N Engl J Med. 278:1413–1419, 1968). The coexistence of TEF-EA and dextrocardia is also a rare and challenging setting for repair of TEF-EA. Therefore, the thoracoscopic surgery for TEF-EA require the technical devise because of the small operative space. We herein report a rare case of CBPFM group IB with intralobar sequestration of lung and a successful performing of thoracoscopic surgery for EA with dextrocardia in VACTERL association. Case presentation A 2.2-kg term male neonate was born with an anal atresia, coarctation of the aorta, TEF-EA, renal anomalies, radial hemimelia, limb abnormalities (VACTERL association) and hypoplasia of the right lung with dextrocardia. The patient developed respiratory distress after admission. A two-stage operation for the TEF-EA was planned because of multiple anomalies and cardiac condition. In the neonatal period, esophageal banding at the gastroesophageal junction and gastrostomy were performed to establish enteral nutrition. After gaining body weight and achieving a stable cardiac condition, thoracoscopic surgery for TEF-EA was performed. The thoracoscopic findings revealed a small working space due to dextrocardia. To obtain a sufficient working space and to perform secure esophageal anastomosis, an additional 3-mm assistant port was inserted. To close the upper and lower esophagus, anchoring sutures of the esophagus were placed and were pulled to suspend the anastomotic site. Esophageal anastomosis was successfully performed. An esophagogram after TEF-EA surgery showed the connection between the lower esophagus and right lower lung. The definitive diagnosis was CBPFM group IB with intralobar sequestration. The thoracoscopic surgery was performed again for establishing oral intake. After transection of the bronchoesophageal fistula, the patient could perform oral feeding without pneumonia or respiratory distress. Conclusions CBPFM type IB with intralobar sequestration is a rare condition. CBPFM type IB should be considered for a patients with respiratory symptom after radical operation for TEF-EA. In the present case, suspending the anastomotic site was effective and useful in thoracoscopic surgery for a TEF-EA patient with dextrocardia.
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spelling doaj-art-8ce2252880d347bbac33dda5e7572ae12025-08-20T03:56:55ZengJapan Surgical SocietySurgical Case Reports2198-77932021-01-01711510.1186/s40792-020-01099-ySuccessful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL associationToshio Harumatsu0Tatsuru Kaji1Ayaka Nagano2Mayu Matsui3Masakazu Murakami4Koshiro Sugita5Makoto Matsukubo6Satoshi Ieiri7Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityDepartment of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityDepartment of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityDepartment of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityDepartment of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityDepartment of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityDepartment of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityDepartment of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education Assembly, Kagoshima UniversityAbstract Background A communicating bronchopulmonary foregut malformation (CBPFM) group IB is very rare congenital malformation. Group IB is associated with tracheoesophageal fistula and esophageal atresia (TEF-EA) and a portion of one lung arisen from the esophagus (Gerle et al. in N Engl J Med. 278:1413–1419, 1968). The coexistence of TEF-EA and dextrocardia is also a rare and challenging setting for repair of TEF-EA. Therefore, the thoracoscopic surgery for TEF-EA require the technical devise because of the small operative space. We herein report a rare case of CBPFM group IB with intralobar sequestration of lung and a successful performing of thoracoscopic surgery for EA with dextrocardia in VACTERL association. Case presentation A 2.2-kg term male neonate was born with an anal atresia, coarctation of the aorta, TEF-EA, renal anomalies, radial hemimelia, limb abnormalities (VACTERL association) and hypoplasia of the right lung with dextrocardia. The patient developed respiratory distress after admission. A two-stage operation for the TEF-EA was planned because of multiple anomalies and cardiac condition. In the neonatal period, esophageal banding at the gastroesophageal junction and gastrostomy were performed to establish enteral nutrition. After gaining body weight and achieving a stable cardiac condition, thoracoscopic surgery for TEF-EA was performed. The thoracoscopic findings revealed a small working space due to dextrocardia. To obtain a sufficient working space and to perform secure esophageal anastomosis, an additional 3-mm assistant port was inserted. To close the upper and lower esophagus, anchoring sutures of the esophagus were placed and were pulled to suspend the anastomotic site. Esophageal anastomosis was successfully performed. An esophagogram after TEF-EA surgery showed the connection between the lower esophagus and right lower lung. The definitive diagnosis was CBPFM group IB with intralobar sequestration. The thoracoscopic surgery was performed again for establishing oral intake. After transection of the bronchoesophageal fistula, the patient could perform oral feeding without pneumonia or respiratory distress. Conclusions CBPFM type IB with intralobar sequestration is a rare condition. CBPFM type IB should be considered for a patients with respiratory symptom after radical operation for TEF-EA. In the present case, suspending the anastomotic site was effective and useful in thoracoscopic surgery for a TEF-EA patient with dextrocardia.https://doi.org/10.1186/s40792-020-01099-yCommunicating bronchopulmonary foregut malformationsTracheoesophageal fistulaEsophageal atresiaDextrocardiaThoracoscopic repairSuspending technique of anastomotic site
spellingShingle Toshio Harumatsu
Tatsuru Kaji
Ayaka Nagano
Mayu Matsui
Masakazu Murakami
Koshiro Sugita
Makoto Matsukubo
Satoshi Ieiri
Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association
Surgical Case Reports
Communicating bronchopulmonary foregut malformations
Tracheoesophageal fistula
Esophageal atresia
Dextrocardia
Thoracoscopic repair
Suspending technique of anastomotic site
title Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association
title_full Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association
title_fullStr Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association
title_full_unstemmed Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association
title_short Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association
title_sort successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group ib with dextrocardia a case report of vacterl association
topic Communicating bronchopulmonary foregut malformations
Tracheoesophageal fistula
Esophageal atresia
Dextrocardia
Thoracoscopic repair
Suspending technique of anastomotic site
url https://doi.org/10.1186/s40792-020-01099-y
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