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Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction
Journal of Pediatric Surgery 50 (2015) 2009–2011
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
PAPS Papers
Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction Yuko Tazuke a,⁎, Hiroomi Okuyama a, Shuichiro Uehara a, Takehisa Ueno a, Keigo Nara a, Hiroaki Yamanaka a, Hisayoshi Kawahara b, Akio Kubota b, Noriaki Usui b, Hideki Soh b, Motonari Nomura b, Takaharu Oue c, Takashi Sasaki c, Satoko Nose c, Ryuta Saka c a b c
Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Osaka, Japan Department of Pediatric Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
a r t i c l e
i n f o
Article history: Received 29 July 2015 Accepted 24 August 2015 Key words: Tracheal agenesis Reconstruction Long-term outcomes Prognosis
a b s t r a c t Purpose: The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal/alimentary reconstruction. Materials and Methods: We reviewed the medical records of four long-term survivors of tracheal agenesis and collected the following data: age, sex, type of tracheal agenesis, method of reconstruction, nutritional management, and physical and neurological development. Results: The patients consisted of three boys and one girl, who ranged in age from 77 to 109 months. The severity of their condition was classified as Floyd's type I (n = 2), II (n = 1), or III (n = 1). Mechanical respiratory support was not necessary in any of the cases. Esophageal/alimentary reconstruction was performed using the small intestine (n = 2), a gastric tube (n = 1), and the esophagus (n = 1). The age at esophageal reconstruction ranged from 41 to 55 months. All of the cases required enteral nutrition via gastrostomy. Three of the patients were able to swallow a small amount of liquid and one was able to take pureed food orally. The physical development of the subjects was moderately delayed–borderline in childhood. Neurological development was normal in two cases and slightly delayed in two cases. Conclusions: None of the long-term survivors of tracheal agenesis required the use of an artificial respirator, and their development was close to normal. Future studies should aim to elucidate the optimal method for performing esophageal reconstruction to allow tracheal agenesis patients to achieve their full oral intake. © 2015 Elsevier Inc. All rights reserved.
Tracheal agenesis (TA) is a rare congenital lethal malformation first reported in 1900 by Payne [1]. The incidence of TA has been reported to be less than 1:50,000 with a male to female ratio of 2:1. In general, 52% of all cases are associated with premature delivery and approximately half of these cases are associated with polyhydramnios [2,3]. It normally presents immediately at birth with cyanosis and respiratory distress. The infant makes no audible sounds. Floyd et al. [4] classified tracheal agenesis into three categories according to the type of fistula and the presence or absence of the trachea (Fig. 1). Although tracheal agenesis is often associated with esophageal communication with the trachea or main bronchi, most neonates with tracheal agenesis die within hours of birth owing to difficulties in diagnosis and treatment [5]. Most previous patients, even those successfully rescued and stabilized, could not be weaned off mechanical ventilation and/or suffered from neurological impairment because of the difficulty in achieving long-term airway maintenance or owing to the lack of an established ⁎ Corresponding author at: Department of Pediatric Surgery, Osaka University Graduate School of Medicine 2-2 Yamadaoka, Suita, Osaka, 594-1101, Japan. Tel.: +81 6 6879 3753; fax: +81 6 6879 3759. E-mail address: [email protected] (Y. Tazuke). http://dx.doi.org/10.1016/j.jpedsurg.2015.08.014 0022-3468/© 2015 Elsevier Inc. All rights reserved.
effective treatment method [6,7]. Recently, however, there have been a few case reports concerning patients with tracheal agenesis treated with esophageal reconstruction and did not require mechanical ventilation [8–10]. The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal or alimentary reconstruction. 1. Materials and methods We retrospectively reviewed our experience with four long-term survivors of tracheal agenesis who underwent reconstructive surgery at our institutions during an 11-year period from January 2004 to January 2015. The following patient data were collected: age, sex, Floyd's type, major congenital deformities, method used to secure the respiratory tract, method of alimentary reconstruction, nutritional management and physical and neurological development. The current study involved the collection of demographic variables for each patient and scoring of their performance status using the Eastern Cooperative Oncology Group (ECOG) Performance Status scale.
2010
Y. Tazuke et al. / Journal of Pediatric Surgery 50 (2015) 2009–2011 Table 2 Respiratory status after airway reconstruction. Case
Secure of respiratory tract (age)
Inside stent
External stent
O2 requirement
Mechanical ventilation
1
Cervical esophagostomy (0 day) Cervical esophagostomy (0 day) Resection and anastomosis of TEF (9 m) Cervical esophagostomy (0 day) Cervical esophagostomy (0 day) Resection and anastomosis of TEF (2 m)
Yes
No
Sometime
Not require
Yes
Yes
Not require
Not require
Yes
No
Not require
Yes
Yes
Not require during daytime Require
2
3 4 Fig. 1. Diagrams showing Floyd's classification.
Not require
2. Results 2.2. Nutritional management The demographic data are shown in Table 1. The patients in this series consisted of three boys and one girl who ranged in age from 77 to 109 months. The condition of tracheal agenesis was classified as Floyd's type I in two cases, type II in one case and type III in one case. None of the subjects demonstrated severe complications after treatment for associated major anomalies, including anorectal malformation and Fallot's tetralogy. However, the patient in case 4 became bedridden owing to postoperative complications of tracheal reconstruction. In all patients, the respiratory tract was secured via cervical esophagostomy using long tracheostomy tubes in the neonatal period (Table 2). Additionally, two Floyd type I patients (cases 2 and 4) required resection of a tracheoesophageal fistula and reanastomosis using cardiopulmonary bypass with external supportive stent at 9 months and 2 months of age, respectively [10,11]. Mechanical respiratory support was not needed in any of these cases. 2.1. Esophageal/alimentary reconstruction After achieving stabilization of the respiratory status, esophageal/ alimentary reconstruction was performed in all patients (Fig. 2) (Table 3). These details regarding surgical reconstruction are described simply, as follows: Case 1: Esophageal reconstruction was performed at 55 months of age with a gastric tube via retro-sternum after division of the stomach. Anastomosis was carried out between the upper esophagus and the proximal end of the gastric tube. Case 2: Esophageal reconstruction was performed at 43 months of age with a jejunum via ante-sternum. Anastomosis was performed between the upper esophagus and the jejunum, using the Roux-en Y method. Case 3: Esophageal reconstruction was performed at 41 months of age with a jejunal interposition (30 cm in length) via the right intrathoracic route. After performing anastomosis between the upper esophagus and the proximal end of the jejunal pedicle graft, anastomosis was carried out between the distal end of the jejunal pedicle graft and the stomach. Case 4: Esophageal reconstruction was performed at 16 months of age with the patient's own esophagus via the right intrathoracic route. Anastomosis was performed between the upper esophagus and the lower esophagus. Table 1 Demographic data of the long-term survivors of tracheal agenesis. Case The age of the month Sex
Type of Floyd Associated major anomalies
1 2 3 4
II I III I
77 109 84 98
Male Male Male female
None Anorectal malformation None Fallot's tetralogy
Enteral nutrition via gastrostomy was necessary in all patients. Regardless of whether they could ingest food after reconstruction, none of the patients wished to eat on their own. Recently, as a result of ingestion rehabilitation, three patients have become able to take a small amount of liquid orally and one has become able to take pureed food. All of the patients were considered to be slightly light in body weight and slightly short in body height. The data in cases 1 and 3 were within the normal range, while those in cases 2 and 4 were less than −2 SD (Table 4). 2.3. Communication skills and performance status All patients were able vocalize syllables to communicate with the medical staff and their family members. The performance status score was 1 in two cases, 2 in one case and 4 in one case. Three of the four patients were able to walk freely and attend kindergarten or standard elementary school. Neurological development was normal in one of these cases and slightly delayed in two cases. The patient in case 4 became bedridden owing to postoperative complications of tracheal reconstruction and is currently undergoing rehabilitation and learning at a school for the handicapped (Table 5). 3. Discussion Few reports have thus far evaluated the long-term outcomes of patients with tracheal agenesis, as many neonates with this condition die within a few hours after birth. Once an airway is established after successful resuscitation, however, long-term survival is possible. In two patients in our series, the respiratory tract was secured by emergent cervical esophagostomy and the insertion of an inner stent tube. The remaining two patients required cervical esophagostomy and resection of a tracheoesophageal fistula in order to stabilize the respiratory status. The establishment of a stable airway is indispensable for achieving a long term survival in such patients. In the cases assessed in the present series, it was possible to wean all patients off mechanical ventilation. There are some recent case reports of patients with tracheal agenesis treated with reconstruction of both the airway and esophagus and associated alimentary canal [8–10]. Esophageal/alimentary reconstruction is necessary to improve the quality of life of long-term survivors with tracheal agenesis. Several reconstruction routes were selected in our case series. Although there is no established method or route of reconstruction, in order to enable ingestion, it is necessary that the method and timing be appropriate for the individual patient. At present, no previous reports have compared the benefits of different reconstruction routes, since many tracheal agenesis patients die early [2,3,7,12]. The intrathoracic route was chosen in two of our cases in which some ingestion was possible. Anastomosis, which, in the intrathoracic route, is in the direction
Y. Tazuke et al. / Journal of Pediatric Surgery 50 (2015) 2009–2011
2011
Fig. 2. Schematic appearance after surgical reconstructions.
of the neck to the abdomen, seems to be relatively safe and involves few complications. Esophageal/alimentary reconstruction is not the only method that should be employed to improve food intake in tracheal agenesis patients. The long period of time during which these patients are unable to ingest food can lead to the development of eating disorders. Deglutition training, at the appropriate time, may help to improve food intake. Without vocal cords, the patients in the current series made voluntary utterances using musical instruments or by producing sounds with the lips and tongue using air collected inside the larynx. One of these patients has gradually gained another speech technique
Table 3 Summary of esophageal/alimentary reconstruction. Case
1 2 3 4
The age of reconstruction (months)
Substitute of esophagus
Reconstruction method
Reconstructive route
55 43 41 16
Stomach Jejunum Jejunum Lower esophagus
Gastric tube Roux-en Y Interposition Self-esophagus
Retro-sternum Ante-sternum Right intra-thoracic Right intra-thoracic
Table 4 Summary of nutritional management strategies. Case
Nutrition management
Oral intake
Body height (cm/SD)
Body weight (kg/SD)
1 2 3 4
Via. gastrostomy Via. gastrostomy Via. gastrostomy Via. gastrostomy
Only water Only limited liquids Pureed food None
109 (−1.3) 116 (−2.5) 119 (0) 108 (−3.0)
21.2 (−0.2) 19.7 (−1.5) 19.7 (−0.8) 15.5 (−2.0)
Table 5 Communication skills and performance status. Case
Communication skills
Performance status score
Walking
Learning at
1 2
Syllabic conversation Syllabic conversation, completely understood Syllabic conversation Mutual understanding
2 1
Possible Possible
Hospital school Elementary school
1 4
Possible Not possible
Kindergarten School for the handicapped
3 4
wherein sound is produced by trapping air in the esophagus and forcing it out again. Regarding the long-term prognosis of tracheal agenesis, the patient's predicted survival may be reduced by cerebral hypoxia after resuscitation or postoperative complications not related to the type of reconstructive surgery. The long-term survivors in the present case series, in whom these risks were avoided, were able to attend school. While it might be difficult to save neonates with tracheal agenesis, our experience reveals that good long-term convalescence is possible after successful airway/alimentary tract reconstruction. Improving unsatisfactory communication skills and poor oral ingestion in long-term survivors are challenges for the future. The method of esophageal reconstruction should therefore be re-evaluated in future research in order to identify a technique that allows long-term survivors to achieve full oral intake. References [1] Payne WA. Congenital absence of the trachea. Brooklyn Med J 1900;14:568. [2] Das BB, Nagaraj A, Rao AH, et al. Tracheal agenesis: report of three cases and review of the literature. Am J Perinatol 2002;19:395–400. [3] de Groot-van der Mooren MD, Haak MC, Lakeman P, et al. Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature. Eur J Pediatr 2012;171:425–31. [4] Floyd J, Campbell DC, Dominy DE. Agenesis of trachea. Rev Respir Dis 1962;86: 557–60. [5] Felix JF, van Looij MA, Pruijsten RV, et al. Agenesis of the trachea: phenotypic expression of a rare cause of fatal neonatal respiratory insufficiency in six patients. Int J Pediatr Otorhinolaryngol 2006;70:365–70. [6] Soh H, Kawahawa H, Imura K, et al. Tracheal agenesis in a child who survived for 6 years. J Pediatr Surg 1999;34:1541–3. [7] Sankaran K, Bhagirath CP, Bingham WT, et al. Tracheal atresia, proximal esophageal atresia, and distal tracheoesophageal fistula: Report of two cases and review of literature. Pediatrics 1983;71:821–3. [8] Hiyama E, Yokoyama T, Ichikawa T, et al. Surgical management of tracheal agenesis. J Thorac Cardiovasc Surg 1994;108:830–3. [9] Okuyama H, Sasaki T, Nose S, et al. Jejunal pedicle graft reconstruction of the esophagus in a child with tracheal agenesis. Eur J Pediatr Surg 2012;22:485–7. [10] Usui N, Kamiyama M, Tani G, et al. Three-stage reconstruction of the airway and alimentary tract in a case of tracheal agenesis. Ann Thorac Surg 2010;89:2019–22. [11] 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. [12] Akl BF, Yabek SM, Berman Jr W. Total tracheal reconstruction in three-month-old infant. J Thorac Cardiovasc Surg 1984;87:543–6.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
PAPS Papers
Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction Yuko Tazuke a,⁎, Hiroomi Okuyama a, Shuichiro Uehara a, Takehisa Ueno a, Keigo Nara a, Hiroaki Yamanaka a, Hisayoshi Kawahara b, Akio Kubota b, Noriaki Usui b, Hideki Soh b, Motonari Nomura b, Takaharu Oue c, Takashi Sasaki c, Satoko Nose c, Ryuta Saka c a b c
Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi, Osaka, Japan Department of Pediatric Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
a r t i c l e
i n f o
Article history: Received 29 July 2015 Accepted 24 August 2015 Key words: Tracheal agenesis Reconstruction Long-term outcomes Prognosis
a b s t r a c t Purpose: The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal/alimentary reconstruction. Materials and Methods: We reviewed the medical records of four long-term survivors of tracheal agenesis and collected the following data: age, sex, type of tracheal agenesis, method of reconstruction, nutritional management, and physical and neurological development. Results: The patients consisted of three boys and one girl, who ranged in age from 77 to 109 months. The severity of their condition was classified as Floyd's type I (n = 2), II (n = 1), or III (n = 1). Mechanical respiratory support was not necessary in any of the cases. Esophageal/alimentary reconstruction was performed using the small intestine (n = 2), a gastric tube (n = 1), and the esophagus (n = 1). The age at esophageal reconstruction ranged from 41 to 55 months. All of the cases required enteral nutrition via gastrostomy. Three of the patients were able to swallow a small amount of liquid and one was able to take pureed food orally. The physical development of the subjects was moderately delayed–borderline in childhood. Neurological development was normal in two cases and slightly delayed in two cases. Conclusions: None of the long-term survivors of tracheal agenesis required the use of an artificial respirator, and their development was close to normal. Future studies should aim to elucidate the optimal method for performing esophageal reconstruction to allow tracheal agenesis patients to achieve their full oral intake. © 2015 Elsevier Inc. All rights reserved.
Tracheal agenesis (TA) is a rare congenital lethal malformation first reported in 1900 by Payne [1]. The incidence of TA has been reported to be less than 1:50,000 with a male to female ratio of 2:1. In general, 52% of all cases are associated with premature delivery and approximately half of these cases are associated with polyhydramnios [2,3]. It normally presents immediately at birth with cyanosis and respiratory distress. The infant makes no audible sounds. Floyd et al. [4] classified tracheal agenesis into three categories according to the type of fistula and the presence or absence of the trachea (Fig. 1). Although tracheal agenesis is often associated with esophageal communication with the trachea or main bronchi, most neonates with tracheal agenesis die within hours of birth owing to difficulties in diagnosis and treatment [5]. Most previous patients, even those successfully rescued and stabilized, could not be weaned off mechanical ventilation and/or suffered from neurological impairment because of the difficulty in achieving long-term airway maintenance or owing to the lack of an established ⁎ Corresponding author at: Department of Pediatric Surgery, Osaka University Graduate School of Medicine 2-2 Yamadaoka, Suita, Osaka, 594-1101, Japan. Tel.: +81 6 6879 3753; fax: +81 6 6879 3759. E-mail address: [email protected] (Y. Tazuke). http://dx.doi.org/10.1016/j.jpedsurg.2015.08.014 0022-3468/© 2015 Elsevier Inc. All rights reserved.
effective treatment method [6,7]. Recently, however, there have been a few case reports concerning patients with tracheal agenesis treated with esophageal reconstruction and did not require mechanical ventilation [8–10]. The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal or alimentary reconstruction. 1. Materials and methods We retrospectively reviewed our experience with four long-term survivors of tracheal agenesis who underwent reconstructive surgery at our institutions during an 11-year period from January 2004 to January 2015. The following patient data were collected: age, sex, Floyd's type, major congenital deformities, method used to secure the respiratory tract, method of alimentary reconstruction, nutritional management and physical and neurological development. The current study involved the collection of demographic variables for each patient and scoring of their performance status using the Eastern Cooperative Oncology Group (ECOG) Performance Status scale.
2010
Y. Tazuke et al. / Journal of Pediatric Surgery 50 (2015) 2009–2011 Table 2 Respiratory status after airway reconstruction. Case
Secure of respiratory tract (age)
Inside stent
External stent
O2 requirement
Mechanical ventilation
1
Cervical esophagostomy (0 day) Cervical esophagostomy (0 day) Resection and anastomosis of TEF (9 m) Cervical esophagostomy (0 day) Cervical esophagostomy (0 day) Resection and anastomosis of TEF (2 m)
Yes
No
Sometime
Not require
Yes
Yes
Not require
Not require
Yes
No
Not require
Yes
Yes
Not require during daytime Require
2
3 4 Fig. 1. Diagrams showing Floyd's classification.
Not require
2. Results 2.2. Nutritional management The demographic data are shown in Table 1. The patients in this series consisted of three boys and one girl who ranged in age from 77 to 109 months. The condition of tracheal agenesis was classified as Floyd's type I in two cases, type II in one case and type III in one case. None of the subjects demonstrated severe complications after treatment for associated major anomalies, including anorectal malformation and Fallot's tetralogy. However, the patient in case 4 became bedridden owing to postoperative complications of tracheal reconstruction. In all patients, the respiratory tract was secured via cervical esophagostomy using long tracheostomy tubes in the neonatal period (Table 2). Additionally, two Floyd type I patients (cases 2 and 4) required resection of a tracheoesophageal fistula and reanastomosis using cardiopulmonary bypass with external supportive stent at 9 months and 2 months of age, respectively [10,11]. Mechanical respiratory support was not needed in any of these cases. 2.1. Esophageal/alimentary reconstruction After achieving stabilization of the respiratory status, esophageal/ alimentary reconstruction was performed in all patients (Fig. 2) (Table 3). These details regarding surgical reconstruction are described simply, as follows: Case 1: Esophageal reconstruction was performed at 55 months of age with a gastric tube via retro-sternum after division of the stomach. Anastomosis was carried out between the upper esophagus and the proximal end of the gastric tube. Case 2: Esophageal reconstruction was performed at 43 months of age with a jejunum via ante-sternum. Anastomosis was performed between the upper esophagus and the jejunum, using the Roux-en Y method. Case 3: Esophageal reconstruction was performed at 41 months of age with a jejunal interposition (30 cm in length) via the right intrathoracic route. After performing anastomosis between the upper esophagus and the proximal end of the jejunal pedicle graft, anastomosis was carried out between the distal end of the jejunal pedicle graft and the stomach. Case 4: Esophageal reconstruction was performed at 16 months of age with the patient's own esophagus via the right intrathoracic route. Anastomosis was performed between the upper esophagus and the lower esophagus. Table 1 Demographic data of the long-term survivors of tracheal agenesis. Case The age of the month Sex
Type of Floyd Associated major anomalies
1 2 3 4
II I III I
77 109 84 98
Male Male Male female
None Anorectal malformation None Fallot's tetralogy
Enteral nutrition via gastrostomy was necessary in all patients. Regardless of whether they could ingest food after reconstruction, none of the patients wished to eat on their own. Recently, as a result of ingestion rehabilitation, three patients have become able to take a small amount of liquid orally and one has become able to take pureed food. All of the patients were considered to be slightly light in body weight and slightly short in body height. The data in cases 1 and 3 were within the normal range, while those in cases 2 and 4 were less than −2 SD (Table 4). 2.3. Communication skills and performance status All patients were able vocalize syllables to communicate with the medical staff and their family members. The performance status score was 1 in two cases, 2 in one case and 4 in one case. Three of the four patients were able to walk freely and attend kindergarten or standard elementary school. Neurological development was normal in one of these cases and slightly delayed in two cases. The patient in case 4 became bedridden owing to postoperative complications of tracheal reconstruction and is currently undergoing rehabilitation and learning at a school for the handicapped (Table 5). 3. Discussion Few reports have thus far evaluated the long-term outcomes of patients with tracheal agenesis, as many neonates with this condition die within a few hours after birth. Once an airway is established after successful resuscitation, however, long-term survival is possible. In two patients in our series, the respiratory tract was secured by emergent cervical esophagostomy and the insertion of an inner stent tube. The remaining two patients required cervical esophagostomy and resection of a tracheoesophageal fistula in order to stabilize the respiratory status. The establishment of a stable airway is indispensable for achieving a long term survival in such patients. In the cases assessed in the present series, it was possible to wean all patients off mechanical ventilation. There are some recent case reports of patients with tracheal agenesis treated with reconstruction of both the airway and esophagus and associated alimentary canal [8–10]. Esophageal/alimentary reconstruction is necessary to improve the quality of life of long-term survivors with tracheal agenesis. Several reconstruction routes were selected in our case series. Although there is no established method or route of reconstruction, in order to enable ingestion, it is necessary that the method and timing be appropriate for the individual patient. At present, no previous reports have compared the benefits of different reconstruction routes, since many tracheal agenesis patients die early [2,3,7,12]. The intrathoracic route was chosen in two of our cases in which some ingestion was possible. Anastomosis, which, in the intrathoracic route, is in the direction
Y. Tazuke et al. / Journal of Pediatric Surgery 50 (2015) 2009–2011
2011
Fig. 2. Schematic appearance after surgical reconstructions.
of the neck to the abdomen, seems to be relatively safe and involves few complications. Esophageal/alimentary reconstruction is not the only method that should be employed to improve food intake in tracheal agenesis patients. The long period of time during which these patients are unable to ingest food can lead to the development of eating disorders. Deglutition training, at the appropriate time, may help to improve food intake. Without vocal cords, the patients in the current series made voluntary utterances using musical instruments or by producing sounds with the lips and tongue using air collected inside the larynx. One of these patients has gradually gained another speech technique
Table 3 Summary of esophageal/alimentary reconstruction. Case
1 2 3 4
The age of reconstruction (months)
Substitute of esophagus
Reconstruction method
Reconstructive route
55 43 41 16
Stomach Jejunum Jejunum Lower esophagus
Gastric tube Roux-en Y Interposition Self-esophagus
Retro-sternum Ante-sternum Right intra-thoracic Right intra-thoracic
Table 4 Summary of nutritional management strategies. Case
Nutrition management
Oral intake
Body height (cm/SD)
Body weight (kg/SD)
1 2 3 4
Via. gastrostomy Via. gastrostomy Via. gastrostomy Via. gastrostomy
Only water Only limited liquids Pureed food None
109 (−1.3) 116 (−2.5) 119 (0) 108 (−3.0)
21.2 (−0.2) 19.7 (−1.5) 19.7 (−0.8) 15.5 (−2.0)
Table 5 Communication skills and performance status. Case
Communication skills
Performance status score
Walking
Learning at
1 2
Syllabic conversation Syllabic conversation, completely understood Syllabic conversation Mutual understanding
2 1
Possible Possible
Hospital school Elementary school
1 4
Possible Not possible
Kindergarten School for the handicapped
3 4
wherein sound is produced by trapping air in the esophagus and forcing it out again. Regarding the long-term prognosis of tracheal agenesis, the patient's predicted survival may be reduced by cerebral hypoxia after resuscitation or postoperative complications not related to the type of reconstructive surgery. The long-term survivors in the present case series, in whom these risks were avoided, were able to attend school. While it might be difficult to save neonates with tracheal agenesis, our experience reveals that good long-term convalescence is possible after successful airway/alimentary tract reconstruction. Improving unsatisfactory communication skills and poor oral ingestion in long-term survivors are challenges for the future. The method of esophageal reconstruction should therefore be re-evaluated in future research in order to identify a technique that allows long-term survivors to achieve full oral intake. References [1] Payne WA. Congenital absence of the trachea. Brooklyn Med J 1900;14:568. [2] Das BB, Nagaraj A, Rao AH, et al. Tracheal agenesis: report of three cases and review of the literature. Am J Perinatol 2002;19:395–400. [3] de Groot-van der Mooren MD, Haak MC, Lakeman P, et al. Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature. Eur J Pediatr 2012;171:425–31. [4] Floyd J, Campbell DC, Dominy DE. Agenesis of trachea. Rev Respir Dis 1962;86: 557–60. [5] Felix JF, van Looij MA, Pruijsten RV, et al. Agenesis of the trachea: phenotypic expression of a rare cause of fatal neonatal respiratory insufficiency in six patients. Int J Pediatr Otorhinolaryngol 2006;70:365–70. [6] Soh H, Kawahawa H, Imura K, et al. Tracheal agenesis in a child who survived for 6 years. J Pediatr Surg 1999;34:1541–3. [7] Sankaran K, Bhagirath CP, Bingham WT, et al. Tracheal atresia, proximal esophageal atresia, and distal tracheoesophageal fistula: Report of two cases and review of literature. Pediatrics 1983;71:821–3. [8] Hiyama E, Yokoyama T, Ichikawa T, et al. Surgical management of tracheal agenesis. J Thorac Cardiovasc Surg 1994;108:830–3. [9] Okuyama H, Sasaki T, Nose S, et al. Jejunal pedicle graft reconstruction of the esophagus in a child with tracheal agenesis. Eur J Pediatr Surg 2012;22:485–7. [10] Usui N, Kamiyama M, Tani G, et al. Three-stage reconstruction of the airway and alimentary tract in a case of tracheal agenesis. Ann Thorac Surg 2010;89:2019–22. [11] 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. [12] Akl BF, Yabek SM, Berman Jr W. Total tracheal reconstruction in three-month-old infant. J Thorac Cardiovasc Surg 1984;87:543–6.