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Three-Stage Reconstruction of the Airway and Alimentary Tract in a Case of Tracheal Agenesis

2010; Elsevier BV; Volume: 89; Issue: 6 Linguagem: Inglês

10.1016/j.athoracsur.2009.11.021

1552-6259

Noriaki Usui, Masafumi Kamiyama, Gakuto Tani, Yuichi Takama, Hideki Soh, Shuichiro Uehara, Masahiro Fukuzawa,

Head and Neck Anomalies

In the few surviving cases of tracheal agenesis, infants have not been capable of oral intake because the esophagus was used as a substitute for the trachea. We performed a three-stage reconstruction of the airway and alimentary tract in an infant with tracheal agenesis. This procedure involved a double cervical esophagostomy followed by an anastomosis of the upper mid-esophagus and carinal trachea. Finally, the esophagus was reconstructed by an anastomosis of the cervical esophagus to the lower esophagus. This novel procedure may become a highly effective surgical treatment for some infants in critical condition due to tracheal agenesis. In the few surviving cases of tracheal agenesis, infants have not been capable of oral intake because the esophagus was used as a substitute for the trachea. We performed a three-stage reconstruction of the airway and alimentary tract in an infant with tracheal agenesis. This procedure involved a double cervical esophagostomy followed by an anastomosis of the upper mid-esophagus and carinal trachea. Finally, the esophagus was reconstructed by an anastomosis of the cervical esophagus to the lower esophagus. This novel procedure may become a highly effective surgical treatment for some infants in critical condition due to tracheal agenesis. Tracheal agenesis is a rare, congenital abnormality in which the tracheal segment between the cricoid cartilage and the carina is absent or severely stunted. This fetal malformation was first described by Payne [1Payne W.A. Congenital absence of the trachea.Brooklyn Med J. 1900; 14: 568-570Google Scholar] and classified by Floyd and colleagues [2Floyd J. Campbell D.C. Dominy D.E. Agenesis of the trachea.Am Rev Respir Dis. 1962; 86: 557-560PubMed Google Scholar]. This classification is based on the degree of distal tracheobronchial development and its fistulous connection to the esophagus. Several surgical approaches have been attempted, although a definitive treatment strategy has not been established. Fonkalsrud and colleagues [3Fonkalsrud E.W. Martelle R.R. Maloney Jr, J.V. Surgical treatment of tracheal agenesis.J Thorac Cardiovasc Surg. 1963; 45: 520-525PubMed Google Scholar] initially reported a case in which the patient survived for 6 weeks after reconstructive surgery that involved trachealizing the esophagus and dividing the distal esophagus. However, in most cases, the patients are unable to be weaned off mechanical ventilation. Recently, a few cases of spontaneous respiration and long-term survival have been reported [4Soh H. Kawahara H. Imura K. et al.Tracheal agenesis in a child who survived for 6 years.J Pediatr Surg. 1999; 34: 1541-1543Abstract Full Text PDF PubMed Scopus (42) Google Scholar, 5Watanabe T. Okuyama H. Kubota A. et al.A case of tracheal agenesis surviving without mechanical ventilation after external esophageal stenting.J Pediatr Surg. 2008; 43: 1906-1908Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar]. Even in these cases, however, the infants were incapable of oral intake because the esophagus was used as a substitute for the trachea, with the exception of a case in which the esophagus was reconstructed by colonic interposition [6Hiyama E. Yokoyama T. Ichikawa T. Matsuura Y. Surgical management of tracheal agenesis.J Thorac Cardiovasc Surg. 1994; 108: 830-833Abstract Full Text PDF PubMed Scopus (54) Google Scholar]. We planned a three-stage reconstruction of the airway and the alimentary tract during the neonatal period. A female infant with a history of hydramnios was born at 35 weeks gestation (weight, 2,240 g). She had respiratory distress develop immediately after birth, but this condition improved after esophageal intubation. Endotracheal intubation was unsuccessful, which suggested the absence of the trachea and the existence of a tracheoesophageal fistula. She was suspected to have tracheal agenesis and was transferred to our hospital. The diagnosis of tracheal agenesis was confirmed by flexible fiberscopic examination. Distal esophageal banding with gastrostomy was performed 6 hours after birth. The diagnosis of Floyd's type I tracheal agenesis was made as the tracheal bifurcation with an opening to the right tracheal bronchus that was visualized at the anterior wall of the esophagus through a tracheoesophageal fistula. Ultrasonography revealed an association with tetralogy of Fallot. A plan for the three-stage reconstruction of the airway and the alimentary tract was developed, and the first step of the operation was performed the next day. A horizontal skin incision was made above the sternal notch. The larynx was disrupted at the end of the cricoid cartilage and no trachea was detected in front of the esophagus (Fig 1A). A proximal cervical esophagostomy was created on the right anterior chest wall to divert salivary secretions. A distal cervical esophagostomy was created as the entrance of the newly established airway. A tracheostomy cannula was positioned in the esophagus connecting the infant to the ventilator (Fig 2). A three-dimensional reconstructed computed tomographic scan demonstrated the presence of a stenotic tracheoesophageal fistula requiring repeated balloon dilation (Fig 1B). The patient was momentarily free from mechanical ventilation. However, the tracheoesophageal fistula was not rigid and easily collapsed, which resulted in unstable respiration. Consequently, airway reconstruction was attempted at 8 months of age.Fig 2Diagram of the airway and alimentary tract after a double cervical esophagostomy and esophageal banding.View Large Image Figure ViewerDownload (PPT) After a right thoracotomy through the fourth intercostal space, the mid-esophagus, tracheoesophageal fistula, and carinal trachea were isolated. The lower mid-esophagus was divided below the tracheoesophageal fistula, which was resected using cardiopulmonary bypass. Anastomosis of the upper mid-esophagus to the carinal trachea was performed, and the end of the lower mid-esophagus was closed and left in the thoracic cavity. A ringed, expanded polytetrafluoroethylene graft (diameter, 20 mm; length, 20 mm) was placed with radial traction sutures around the esophagus as an external supportive stent [6Hiyama E. Yokoyama T. Ichikawa T. Matsuura Y. Surgical management of tracheal agenesis.J Thorac Cardiovasc Surg. 1994; 108: 830-833Abstract Full Text PDF PubMed Scopus (54) Google Scholar] to prevent esophageal collapse (Fig 3). The distal esophageal banding was released to avoid strangulation and the cervical esophagus was elongated 1 month after the operation. Granulation developed at the anastomotic site and required potassium titanyl phosphate (KTP) laser ablation multiple times. In this process, a portion of the posterior "trachealized" esophageal wall was peeled off and the expanded polytetrafluoroethylene graft was exposed. The respiratory state subsequently stabilized at 1 year of age. After additional esophageal elongation, esophageal reconstruction was performed at 1 year and 3 months of age. The entire cervical esophagus was dissected and a space was created between the trachealized esophagus and the right carotid artery. After a right thoracotomy, a tunnel between the neck and chest was created and the cervical esophagus was introduced into the thoracic cavity. The lower mid-esophagus was isolated and anastomosed to the cervical esophagus. Postoperative right diaphragmatic eventration required diaphragm plication 3 months after the surgery, and gastroesophageal reflux accompanied by hemorrhagic esophagitis required fundoplication 4 months after the operation (Fig 4). The patient, now at 3 years of age, has displayed normal neurologic and physiologic development for her age. Half of her dietary intake is taken orally through the reconstructed esophagus and the rest is received through a gastrostomy. Two major problems are encountered by the limited number of surviving cases of tracheal agenesis. First, spontaneous respiration is difficult due to a nonrigid airway composed of the esophagus and tracheoesophageal fistula. Second, oral intake is inhibited due to the cervical esophagostomy for salivary drainage. We reconstructed the airway by trachealizing the interstitial segment of the esophagus. Most previously reported cases were unable to be weaned off mechanical ventilation, because the tracheoesophageal fistula was easily collapsed. We resected the fistula and anastomosed the end of the esophagus directly to the carinal trachea with an external supportive stent [6Hiyama E. Yokoyama T. Ichikawa T. Matsuura Y. Surgical management of tracheal agenesis.J Thorac Cardiovasc Surg. 1994; 108: 830-833Abstract Full Text PDF PubMed Scopus (54) Google Scholar] to prevent esophageal collapse. Although we used a ringed, expanded polytetrafluoroethylene graft, a semicircle graft for the anterior wall may be sufficient, because the posterior wall is supported by vertebrae. Although we had concern for the blood supply of the isolated interstitial esophageal segment, collateral circulation to the esophagus from the peripheral tissue was already established at the time of airway reconstruction. Even in the few, long-term survival cases with spontaneous respiration, no infant has been capable of oral intake [4Soh H. Kawahara H. Imura K. et al.Tracheal agenesis in a child who survived for 6 years.J Pediatr Surg. 1999; 34: 1541-1543Abstract Full Text PDF PubMed Scopus (42) Google Scholar, 5Watanabe T. Okuyama H. Kubota A. et al.A case of tracheal agenesis surviving without mechanical ventilation after external esophageal stenting.J Pediatr Surg. 2008; 43: 1906-1908Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar] due to the esophageal reconstruction. We used the extrathoracic esophageal elongation technique, which has been used in the repair of long gap esophageal atresia. This technique enables the use of the native esophagus for reconstruction and prevents the development of a food aversion by oral sham feeding during the staged repair. However, in preparation for the reconstruction, the cervical esophagus should be preserved as long as possible, and the lower esophagus should be preserved as long as possible at the time of airway reconstruction. In conclusion, we developed a surgical procedure for three-stage reconstruction of the airway and the alimentary tract that was planned during the neonatal period. This procedure may become an effective surgical treatment for a subgroup of infants with tracheal agenesis.