- Email: [email protected]
Three-Stage Reconstruction of the Airway and Alimentary Tract in a Case of Tracheal Agenesis
CASE REPORT USUI ET AL AIRWAY RECONSTRUCTION IN TRACHEAL AGENESIS
2019
Comment
References
Sequestration is thought to occur when an accessory lung bud migrates abnormally during differentiation from the caudal foregut together with its blood supply, which becomes the aberrant supplying artery [1]. Extralobar sequestration is associated with one or more congenital anomalies in about 50% of patients, commonly including diaphragmatic hernia, communication with foregut structures, or cardiopulmonary malformations. Only about 14% of intralobar sequestrations are associated with additional anomalies [3]. Despite the presence of both extralobar and intralobar sequestrations in this patient, no such anomalies were found. Sequestration is identified most frequently among the infant and pediatric populations, either during workup for respiratory distress or recurrent infection, or as an incidental finding on imaging studies performed for unrelated reasons. As with this patient, adults most frequently present with recurrent pneumonia or chest pain, or both. Symptomatic sequestrations are predominately intralobar [4]. The case presented represents an extremely rare variation of pulmonary sequestration. Subphrenic extralobar sequestration is an unusual finding, comprising 1.5% to 2.5% of reported cases. The initial presentation of these lesions is most commonly an incidentally imaged abdominal mass, and when the radiographic evaluation cannot exclude malignancy, the mass is resected [5, 6]. Duplicated intralobar and extralobar sequestration is an even rarer variant, with 9 reported patients with coexisting ipsilateral intralobar and extralobar sequestrations and 3 with an intralobar and contralateral extralobar sequestration [3, 7, 8]. Our patient presented with sequestrations on both sides of the diaphragm. Derivation of the arterial supply from the celiac trunk is reported in 1% of sequestrations [3]. Principles of management for pulmonary sequestration in the adult patient were highlighted in this case and include careful preoperative evaluation of functional pulmonary reserve and precise characterization of aberrant arterial supply to prevent uncontrolled intraoperative hemorrhage (conventional angiography is not necessary if computed tomography or magnetic resonance angiogram adequately define the anatomy) [2, 4]. Symptomatic sequestrations should be resected, and many experts believe that all intralobar sequestrations should be surgically treated due to the high rate of infection associated with these lesions. Subphrenic sequestrations have been reported primarily in the pediatric population, with resection undertaken to definitively exclude pediatric renal neoplasm in nearly all cases. Given that the typical clinical course of an asymptomatic extralobar sequestration is benign, conservative management is a reasonable option for management of an asymptomatic lesion in the adult patient if a definitive diagnosis of extralobar sequestration can be reliably established radiographically [1, 3].
1. Kays DW. Congenital diaphragmatic hernia and neonatal lung lesions. Surg Clin North Am 2006;86:329 –52. 2. Gezer S, Tastepe I, Sirmali M, Findik G, et al. Pulmonary sequestration: a single institutional series composed of 27 cases. J Thorac Cardiovasc Surg 2007;133:955–9. 3. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979;34:96 –101. 4. Raemdonck DV, De Boek K, Devlieger H, et al. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg 2001;19:388 –95. 5. Franko J, Bell K, Pezzi C. Intraabdominal pulmonary sequestration. Curr Surg 2006;63:35– 8. 6. Bak DM, Vest T, Hodges S. Subdiaphragmatic extralobar pulmonary sequestration. JDMS 2001;17:41–5. 7. Jeanfaivre T, Afi M, L’hoste P, Tuchais E. Simultaneous discovery of bilateral intralobar and extralobar pulmonary sequestrations. Ann Thorac Surg 1997;63:1171–3. 8. Kim HJ, Kim JH, Chung SK, RHi S, Chung SH. Coexistent intralobar and extralobar pulmonary sequestration: imaging findings. AJR Am J Roentgenol 1993;160:1199 –200.
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
Three-Stage Reconstruction of the Airway and Alimentary Tract in a Case of Tracheal Agenesis Noriaki Usui, MD, PhD, Masafumi Kamiyama, MD, PhD, Gakuto Tani, MD, Yuichi Takama, MD, Hideki Soh, MD, PhD, Shuichiro Uehara, MD, PhD, and Masahiro Fukuzawa, MD, PhD Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
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. (Ann Thorac Surg 2010;89:2019 –22) © 2010 by The Society of Thoracic Surgeons
T
racheal 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 [1] and classified by Floyd and colleagues [2]. This classification is based on the degree of distal tracheobronchial development and its fistulous connection to the esophaAccepted for publication Nov 4, 2009. Address correspondence to Dr Usui, Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan; e-mail: [email protected].
0003-4975/$36.00 doi:10.1016/j.athoracsur.2009.11.021
FEATURE ARTICLES
Ann Thorac Surg 2010;89:2019 –22
2020
CASE REPORT USUI ET AL AIRWAY RECONSTRUCTION IN TRACHEAL AGENESIS
Ann Thorac Surg 2010;89:2019 –22
Fig 1. (A) The airway was disrupted at the end of the cricoid cartilage (arrowhead) and there was no trachea in front of the esophagus (arrow). (B) Three-dimensional reconstructed computed tomographic scan at 5 months of age demonstrated a narrow tracheoesophageal fistula (arrow) between the cannula and carinal trachea.
FEATURE ARTICLES
gus. Several surgical approaches have been attempted, although a definitive treatment strategy has not been established. Fonkalsrud and colleagues [3] 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 [4, 5]. 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 [6]. 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. After a right thoracotomy through the fourth intercostal space, the mid-esophagus, tracheoesophageal fistula, and carinal trachea were isolated. The lower midesophagus 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 [6] to prevent esophageal collapse (Fig 3). The distal esophageal banding was released to avoid strangulation and the cervical esophagus was elongated 1
Fig 2. Diagram of the airway and alimentary tract after a double cervical esophagostomy and esophageal banding.
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.
Comment 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
CASE REPORT USUI ET AL AIRWAY RECONSTRUCTION IN TRACHEAL AGENESIS
2021
Fig 4. Diagram of the airway and alimentary tract after the reconstruction. (EPTFE ⫽ expanded polytetrafluoroethylene.)
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 [6] 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 [4, 5] 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.
References Fig 3. Schematic drawing of the airway reconstruction. (EPTFE ⫽ expanded polytetrafluoroethylene.)
1. Payne WA. Congenital absence of the trachea. Brooklyn Med J 1900;14:568 –70.
FEATURE ARTICLES
Ann Thorac Surg 2010;89:2019 –22
2022
CASE REPORT ADACHI ET AL ATRIAL HISTOLOGY AFTER APC FONTAN
2. Floyd J, Campbell DC, Dominy DE. Agenesis of the trachea. Am Rev Respir Dis 1962;86:557– 60. 3. Fonkalsrud EW, Martelle RR, Maloney JV Jr. Surgical treatment of tracheal agenesis. J Thorac Cardiovasc Surg 1963;45: 520 –5. 4. Soh H, Kawahara H, Imura K, et al. Tracheal agenesis in a child who survived for 6 years. J Pediatr Surg 1999;34:1541–3. 5. Watanabe 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 – 8. 6. Hiyama E, Yokoyama T, Ichikawa T, Matsuura Y. Surgical management of tracheal agenesis. J Thorac Cardiovasc Surg 1994;108:830 –3.
Histologic Findings in a Dilated Atrium Serving as a Fontan Pathway for 23 Years Iki Adachi, MD, Hajime Ichikawa, MD, Takayoshi Ueno, MD, Yumiko Hori, MD, Toru Kuratani, MD, Goro Matsumiya, MD, and Yoshiki Sawa, MD Division of Cardiovascular Surgery, Department of Surgery, and Department of Pathology, Osaka University Graduate School of Medicine, Osaka, Japan
Ann Thorac Surg 2010;89:2022– 4
we report herein our microscopic findings on atrial tissues obtained 23 years after the atriopulmonary connection Fontan operation. A 35-year-old woman with a dominant right ventricle in the setting of mirror-imaged arrangement of atrial appendages (ie, situs inversus) presented to our clinic with complaints suggestive of heart failure. At the age of 12, she was palliated with a classical Fontan operation by means of a direct connection between the main pulmonary artery and the appendage of a right-sided but morphologically left atrium, into which the superior caval and hepatic veins drained. Flow from the left-sided inferior caval vein was redirected into the systemic venous channel by placing a Dacron patch (DuPont, Wilmington, DE). Although the patient had been doing well without major limitation in her daily activity for many years, she experienced exercise intolerance and leg edema in recent years. The patient was catheterized in view of possible indication for TCPC conversion. She was also found to have a marked decrease in cardiac index of 1.5 L/min/m2, which was presumably due to flow stag-
FEATURE ARTICLES
We microscopically examined atrial tissues surgically obtained from a 35-year-old woman who had been palliated with the atriopulmonary connection in a Fontan operation 23 years earlier. Apart from mild hypertrophy of myocytes and trivial interstitial fibrosis, no gross abnormalities were identified. Although this finding is somehow surprising, considering the duration of atrial pressure-loading, it seems to be in accordance with the result of her Holter recordings, which demonstrates the absence of any rhythm disturbances other than sporadic premature contractions. Because of the shortage of histologic investigations in this group of patients, we present this case with a review of the literature. (Ann Thorac Surg 2010;89:2022– 4) © 2010 by The Society of Thoracic Surgeons
I
t is known that an atrial chamber used in the atriopulmonary connection (APC) in a Fontan operation eventually becomes dilated, leading to hemodynamic insufficiency and thus requiring a conversion to total cavopulmonary connection (TCPC) at some stage after the conventional procedure [1]. An overstretched atrial wall is prone to structural remodeling, particularly aggregation of interstitial fibrotic materials, which is a substrate for development of atrial arrhythmias [2]. In an effort to establish the optimal timing for TCPC conversion [3], it is certainly of clinical importance to accumulate knowledge of the histologic alterations in this unique pumpless circulation. Nevertheless, the number of reports conveying such data is surprisingly small [4, 5]. To augment this lacking, but clinically relevant information,
Accepted for publication Oct 29, 2009. Address correspondence to Dr Sawa, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan; e-mail: [email protected].
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Internal view of the right-sided atrium to which the inferior caval vein (ICV) and hepatic vein (HV) individually drained. There was no terminal crest inside the atrium, with the pectinate muscles being confined within the appendage, a finding consistent with a diagnosis of a morphologically left atrium. An upper part of the Dacron patch previously placed was removed to allow adequate interatrial mixing. The atrial wall was thickened but still pliable without any patchy fibrosis. 0003-4975/$36.00 doi:10.1016/j.athoracsur.2009.10.062
2019
Comment
References
Sequestration is thought to occur when an accessory lung bud migrates abnormally during differentiation from the caudal foregut together with its blood supply, which becomes the aberrant supplying artery [1]. Extralobar sequestration is associated with one or more congenital anomalies in about 50% of patients, commonly including diaphragmatic hernia, communication with foregut structures, or cardiopulmonary malformations. Only about 14% of intralobar sequestrations are associated with additional anomalies [3]. Despite the presence of both extralobar and intralobar sequestrations in this patient, no such anomalies were found. Sequestration is identified most frequently among the infant and pediatric populations, either during workup for respiratory distress or recurrent infection, or as an incidental finding on imaging studies performed for unrelated reasons. As with this patient, adults most frequently present with recurrent pneumonia or chest pain, or both. Symptomatic sequestrations are predominately intralobar [4]. The case presented represents an extremely rare variation of pulmonary sequestration. Subphrenic extralobar sequestration is an unusual finding, comprising 1.5% to 2.5% of reported cases. The initial presentation of these lesions is most commonly an incidentally imaged abdominal mass, and when the radiographic evaluation cannot exclude malignancy, the mass is resected [5, 6]. Duplicated intralobar and extralobar sequestration is an even rarer variant, with 9 reported patients with coexisting ipsilateral intralobar and extralobar sequestrations and 3 with an intralobar and contralateral extralobar sequestration [3, 7, 8]. Our patient presented with sequestrations on both sides of the diaphragm. Derivation of the arterial supply from the celiac trunk is reported in 1% of sequestrations [3]. Principles of management for pulmonary sequestration in the adult patient were highlighted in this case and include careful preoperative evaluation of functional pulmonary reserve and precise characterization of aberrant arterial supply to prevent uncontrolled intraoperative hemorrhage (conventional angiography is not necessary if computed tomography or magnetic resonance angiogram adequately define the anatomy) [2, 4]. Symptomatic sequestrations should be resected, and many experts believe that all intralobar sequestrations should be surgically treated due to the high rate of infection associated with these lesions. Subphrenic sequestrations have been reported primarily in the pediatric population, with resection undertaken to definitively exclude pediatric renal neoplasm in nearly all cases. Given that the typical clinical course of an asymptomatic extralobar sequestration is benign, conservative management is a reasonable option for management of an asymptomatic lesion in the adult patient if a definitive diagnosis of extralobar sequestration can be reliably established radiographically [1, 3].
1. Kays DW. Congenital diaphragmatic hernia and neonatal lung lesions. Surg Clin North Am 2006;86:329 –52. 2. Gezer S, Tastepe I, Sirmali M, Findik G, et al. Pulmonary sequestration: a single institutional series composed of 27 cases. J Thorac Cardiovasc Surg 2007;133:955–9. 3. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979;34:96 –101. 4. Raemdonck DV, De Boek K, Devlieger H, et al. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg 2001;19:388 –95. 5. Franko J, Bell K, Pezzi C. Intraabdominal pulmonary sequestration. Curr Surg 2006;63:35– 8. 6. Bak DM, Vest T, Hodges S. Subdiaphragmatic extralobar pulmonary sequestration. JDMS 2001;17:41–5. 7. Jeanfaivre T, Afi M, L’hoste P, Tuchais E. Simultaneous discovery of bilateral intralobar and extralobar pulmonary sequestrations. Ann Thorac Surg 1997;63:1171–3. 8. Kim HJ, Kim JH, Chung SK, RHi S, Chung SH. Coexistent intralobar and extralobar pulmonary sequestration: imaging findings. AJR Am J Roentgenol 1993;160:1199 –200.
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
Three-Stage Reconstruction of the Airway and Alimentary Tract in a Case of Tracheal Agenesis Noriaki Usui, MD, PhD, Masafumi Kamiyama, MD, PhD, Gakuto Tani, MD, Yuichi Takama, MD, Hideki Soh, MD, PhD, Shuichiro Uehara, MD, PhD, and Masahiro Fukuzawa, MD, PhD Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
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. (Ann Thorac Surg 2010;89:2019 –22) © 2010 by The Society of Thoracic Surgeons
T
racheal 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 [1] and classified by Floyd and colleagues [2]. This classification is based on the degree of distal tracheobronchial development and its fistulous connection to the esophaAccepted for publication Nov 4, 2009. Address correspondence to Dr Usui, Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan; e-mail: [email protected].
0003-4975/$36.00 doi:10.1016/j.athoracsur.2009.11.021
FEATURE ARTICLES
Ann Thorac Surg 2010;89:2019 –22
2020
CASE REPORT USUI ET AL AIRWAY RECONSTRUCTION IN TRACHEAL AGENESIS
Ann Thorac Surg 2010;89:2019 –22
Fig 1. (A) The airway was disrupted at the end of the cricoid cartilage (arrowhead) and there was no trachea in front of the esophagus (arrow). (B) Three-dimensional reconstructed computed tomographic scan at 5 months of age demonstrated a narrow tracheoesophageal fistula (arrow) between the cannula and carinal trachea.
FEATURE ARTICLES
gus. Several surgical approaches have been attempted, although a definitive treatment strategy has not been established. Fonkalsrud and colleagues [3] 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 [4, 5]. 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 [6]. 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. After a right thoracotomy through the fourth intercostal space, the mid-esophagus, tracheoesophageal fistula, and carinal trachea were isolated. The lower midesophagus 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 [6] to prevent esophageal collapse (Fig 3). The distal esophageal banding was released to avoid strangulation and the cervical esophagus was elongated 1
Fig 2. Diagram of the airway and alimentary tract after a double cervical esophagostomy and esophageal banding.
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.
Comment 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
CASE REPORT USUI ET AL AIRWAY RECONSTRUCTION IN TRACHEAL AGENESIS
2021
Fig 4. Diagram of the airway and alimentary tract after the reconstruction. (EPTFE ⫽ expanded polytetrafluoroethylene.)
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 [6] 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 [4, 5] 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.
References Fig 3. Schematic drawing of the airway reconstruction. (EPTFE ⫽ expanded polytetrafluoroethylene.)
1. Payne WA. Congenital absence of the trachea. Brooklyn Med J 1900;14:568 –70.
FEATURE ARTICLES
Ann Thorac Surg 2010;89:2019 –22
2022
CASE REPORT ADACHI ET AL ATRIAL HISTOLOGY AFTER APC FONTAN
2. Floyd J, Campbell DC, Dominy DE. Agenesis of the trachea. Am Rev Respir Dis 1962;86:557– 60. 3. Fonkalsrud EW, Martelle RR, Maloney JV Jr. Surgical treatment of tracheal agenesis. J Thorac Cardiovasc Surg 1963;45: 520 –5. 4. Soh H, Kawahara H, Imura K, et al. Tracheal agenesis in a child who survived for 6 years. J Pediatr Surg 1999;34:1541–3. 5. Watanabe 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 – 8. 6. Hiyama E, Yokoyama T, Ichikawa T, Matsuura Y. Surgical management of tracheal agenesis. J Thorac Cardiovasc Surg 1994;108:830 –3.
Histologic Findings in a Dilated Atrium Serving as a Fontan Pathway for 23 Years Iki Adachi, MD, Hajime Ichikawa, MD, Takayoshi Ueno, MD, Yumiko Hori, MD, Toru Kuratani, MD, Goro Matsumiya, MD, and Yoshiki Sawa, MD Division of Cardiovascular Surgery, Department of Surgery, and Department of Pathology, Osaka University Graduate School of Medicine, Osaka, Japan
Ann Thorac Surg 2010;89:2022– 4
we report herein our microscopic findings on atrial tissues obtained 23 years after the atriopulmonary connection Fontan operation. A 35-year-old woman with a dominant right ventricle in the setting of mirror-imaged arrangement of atrial appendages (ie, situs inversus) presented to our clinic with complaints suggestive of heart failure. At the age of 12, she was palliated with a classical Fontan operation by means of a direct connection between the main pulmonary artery and the appendage of a right-sided but morphologically left atrium, into which the superior caval and hepatic veins drained. Flow from the left-sided inferior caval vein was redirected into the systemic venous channel by placing a Dacron patch (DuPont, Wilmington, DE). Although the patient had been doing well without major limitation in her daily activity for many years, she experienced exercise intolerance and leg edema in recent years. The patient was catheterized in view of possible indication for TCPC conversion. She was also found to have a marked decrease in cardiac index of 1.5 L/min/m2, which was presumably due to flow stag-
FEATURE ARTICLES
We microscopically examined atrial tissues surgically obtained from a 35-year-old woman who had been palliated with the atriopulmonary connection in a Fontan operation 23 years earlier. Apart from mild hypertrophy of myocytes and trivial interstitial fibrosis, no gross abnormalities were identified. Although this finding is somehow surprising, considering the duration of atrial pressure-loading, it seems to be in accordance with the result of her Holter recordings, which demonstrates the absence of any rhythm disturbances other than sporadic premature contractions. Because of the shortage of histologic investigations in this group of patients, we present this case with a review of the literature. (Ann Thorac Surg 2010;89:2022– 4) © 2010 by The Society of Thoracic Surgeons
I
t is known that an atrial chamber used in the atriopulmonary connection (APC) in a Fontan operation eventually becomes dilated, leading to hemodynamic insufficiency and thus requiring a conversion to total cavopulmonary connection (TCPC) at some stage after the conventional procedure [1]. An overstretched atrial wall is prone to structural remodeling, particularly aggregation of interstitial fibrotic materials, which is a substrate for development of atrial arrhythmias [2]. In an effort to establish the optimal timing for TCPC conversion [3], it is certainly of clinical importance to accumulate knowledge of the histologic alterations in this unique pumpless circulation. Nevertheless, the number of reports conveying such data is surprisingly small [4, 5]. To augment this lacking, but clinically relevant information,
Accepted for publication Oct 29, 2009. Address correspondence to Dr Sawa, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan; e-mail: [email protected].
© 2010 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Internal view of the right-sided atrium to which the inferior caval vein (ICV) and hepatic vein (HV) individually drained. There was no terminal crest inside the atrium, with the pectinate muscles being confined within the appendage, a finding consistent with a diagnosis of a morphologically left atrium. An upper part of the Dacron patch previously placed was removed to allow adequate interatrial mixing. The atrial wall was thickened but still pliable without any patchy fibrosis. 0003-4975/$36.00 doi:10.1016/j.athoracsur.2009.10.062