Simultaneous correction of esophageal lung and ...

J Ped Surg Case Reports 1 (2013) 288e292

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Simultaneous correction of esophageal lung and congenital tracheal stenosis: Lessons learnedq Michael Berger a, b, José Antonio Matute de Cárdenas a, *, Reza Hosseinpour c, Juan Carlos de Agustín Asensio a a b c

Thoracic and Airway Surgery Unit, Department of Pediatric Surgery, Hospital Universitario Virgen del Rocio, Avenida Manuel Siurot S/N, 41013 Seville Spain Department of Pediatric Surgery, Dr. von Hauner Children’s Hospital, Ludwig Maximilians University Munich, Lindwurmstrasse 4, 80337 Munich Germany Department of Cardiac Surgery, Hospital Universitario Virgen del Rocio, Avenida Manuel Siurot S/N, 41013 Seville Spain

a r t i c l e i n f o

a b s t r a c t

Article history: Received 9 May 2013 Received in revised form 7 July 2013 Accepted 8 July 2013

Esophageal lung is a rare variant of communicating bronchopulmonary foregut malformation (CBPFM) that can be associated with congenital tracheal stenosis (CTS). Ten cases have been reported so far. Treatment options include complete pulmonary resection versus detachment and reinsertion into the trachea. If CTS is severe, additional tracheal reconstruction via slide tracheoplasty may become necessary. Here we present jet another child with such a rare variant of CBPFM. The right main bronchus originated from an otherwise healthy esophagus and was associated with long segment congenital tracheal stenosis. The child underwent one-stage reinsertion of the bronchus at the level of the carina and simultaneous slide tracheoplasty at 3 months of age. Unfortunately, the child died from a bronchopleural fistula with massive air leak during the postoperative course. To our best knowledge, this was the first time a one-stage approach was intended for this particular malformation. Because recently the successful correction of a similar malformation was described using a multiple-step approach, by reflecting our own unsuccessful case we critically discuss the management possibilities for this extremely rare variant of foregut malformations. Ó 2013 The Authors. Published by Elsevier Inc. All rights reserved.

Key words: Esophageal lung Bronchopulmonary foregut malformations (BPFMs) Congenital tracheal stenosis Cardiopulmonary bypass (CPB)

Bronchopulmonary foregut malformations (BPFMs) are rare congenital anomalies arising from faulty separation of tracheal and esophageal tissue during embryological development [1,2]. These malformations include a wide variety of anomalies such as intralobular or extralobular pulmonary sequestration, foregut duplication cysts and diverticula of the upper gastrointestinal tract or pulmonary tree [1,3]. If a tract is preserved between the respiratory and alimentary system, these malformations are termed communicating bronchopulmonary foregut malformations (CBPFMs) [2,3]. Esophageal lung is a rare variant of CBPFM defined by an anomalous origin of the main stem bronchus from the esophagus [3,4]. By definition, and in contrast to the systemic blood supply of pulmonary sequestration, in esophageal lung blood supply derives exclusively from the pulmonary artery. Less than 20 cases of true

q This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-No Derivative Works License, which permits non-commercial use, distribution, and reproduction in any medium, provided the original author and source are credited. * Corresponding author. E-mail address: [email protected] (J.A. Matute de Cárdenas).

esophageal lung have been described [2e18]. Approximately half of all cases are associated with esophageal atresia (EA), and 10 cases have been found to be associated with additional congenital tracheal stenosis (CTS). Recently, for the first time, the successful correction of both malformations present in one child has been reported using a multiple-step approach including bronchotracheal reconstruction and slide tracheoplasty [19]. Here, we critically discuss our experience with a one-step approach for such a variant, which was unsuccessful. 1. Case report A 2-month old former 33 weeks premature infant boy had been suffering from lower respiratory tract infections and severe respiratory insufficiency since birth. There was no gross developmental delay or chest wall deformities. On auscultation, there was no air entry on the right side. Initial chest radiography showed a cloudy right hemithorax compatible with lung atelectasis (Fig. 1A). Computed-tomography (CT) scan of the chest confirmed right lung atelectasis and additionally revealed a derivation of the right main

2213-5766/$ e see front matter Ó 2013 The Authors. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2013.07.017

M. Berger et al. / J Ped Surg Case Reports 1 (2013) 288e292

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Fig. 2. Graphic imprint of the malformation encountered.

Fig. 1. Diagnostic workup. Chest radiography showed complete whitening of the right lung (A). In (B), barium swallow is shown. Appreciate the contrast material in the right lung. The Computed tomography (CT) scan confirmed complete right lung atelectasis and demonstrated the derivation of the right main bronchus from the esophagus (C).

supine. The chest was re-opened through a median sternotomy for cardiopulmonary bypass (CPB). The bronchus was mobilized anteriorly and reinserted into the trachea with primary suture at the level of the carina without difficulty. This part was carried out under induced total circulatory arrest due to dextrocardia. Then, slide tracheoplasty was performed, again without notable technical difficulty. The initial postoperative course was complicated by a lifethreatening right pneumothorax, which was managed successfully with additional chest tubes. There had been a minor air leak onand-off, but the patient remained stable over the subsequent weeks. However, the patient could not be extubated due to continued respiratory insufficiency. On postoperative day 51, the child suddenly developed a severe air leak and clinically deteriorated rapidly. Upon re-exploration on CPB, a significant bronchopleural fistula was identified representing a complete breakdown of the broncho-tracheal anastomosis. Even though there was no kinking of the pulmonary vessels, the right lung was completely necrosed. The suture lines of the slide tracheoplasty remained intact. Attempts to repair the fistula were futile due to the large area involved and the compromised tissue surrounding the defect. Support was withdrawn shortly thereafter when the child could not be weaned off bypass. 2. Discussion

bronchus from the lower esophagus (Fig. 1C). Barium swallow confirmed the diagnosis (Fig. 1B). Fiberoptic bronchoscopy and esophagoscopy showed the absence of a right main bronchus without any residual pouch in the trachea and demonstrated its origin from the lower esophagus (Fig. 2). Additionally, a type III congenital tracheal stenosis (classified by Grillo [20]) in the lower third of the trachea was identified. An aortogram showed no systemic blood supply to the lungs (Fig. 3). At 3 months of age, the child underwent operative exploration through a posterolateral thoracotomy and, as expected, the right main bronchus was found to originate entirely from the distal esophagus (Fig. 4A). There was no additional communication to the pulmonary tree. The bronchus was detached and air was blown into it via the tubing of an oxygen mask connected to a ventilation-bag (Fig. 4BeD). The lung inflated easily and gave rise to a healthy looking lung parenchyma (Fig. 4D). Surfactant was administered. The thoracotomy was closed and the child was repositioned into

Esophageal lung is an extremely rare type of CBPFM compromised by an abnormal origin of a main bronchus from the esophagus. Typically, these malformations are associated with EA [2e18]. In the cases of associated EA, it is usually the right main bronchus that is positioned anomalously and connects to the distal esophagus in addition to the presence of a tracheoesophageal fistula (TEF) [2e5,7,8]. A similar origin of the bronchus can be seen in other rare malformations of the trachea. For example, in type III tracheal agenesis, both main bronchi originate from the esophagus while the trachea itself is completely absent [21]. The association between CBPFM and CTS is a malformation of similar rarity, with only eleven cases (including the case presented here) described in the English literature [19]. In our case, respiratory insufficiency and recurrent infections of the lower respiratory tract were the central problems that lead to diagnostic work-up. As for all congenital tracheal stenosis, ultra

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Fig. 3. The aortogram shows perfusion for lungs to arise from the pulmonary artery. The white arrow in the right panel depicts the right pulmonary artery.

thin-slicing CT scan and fiberoptic bronchoscopy are essential. If additional CBPFM is suspected, or to simply rule out an H-type TEF, standard barium swallows can help and in our case lead to the diagnosis. Alternatively to a barium swallow, in the presence of a gastrostomy, air can be inflated from below and expansion of the lung can be observed either clinically or radiologically [3]. Naturally, this technique is inferior to barium swallow, especially in the setting of lung hypoplasia. Both the detachment of the main bronchus from the esophagus and re-implantation into the trachea as well as the detachment followed by pulmonary resection are reasonable treatment options [6,19]. Pneumonectomy seems to be an acceptable approach especially in the setting of severe lung hypoplasia. It can be

performed through a thoracotomy without CPB and is therefore technically much simpler, faster and consists of less risk compared to bronchial reconstruction. However, due to the shortage of described cases, no clear-cut criteria exist on whether or not a lung is too hypoplastic to be successfully inserted into the trachea. Also, CPB is not obligatory for either bronchial re-implantation or slide tracheoplasty, but this depends on the individual variant. The successful reinsertion of the bronchus into the trachea in esophageal lung has been described and seems to be feasible especially if the diagnosis is made in the newborn period [4,6,19,22]. Slide tracheoplasty is generally accepted for congenital tracheal stenosis and has high success rates when performed in specialized centers [23,24].

Fig. 4. Intraoperative findings. The right main bronchus is exposed through a right thoracotomy (A). Note the intact esophagus especially proximal to the insertion of the bronchus. (B) shows the esophageal defect after detachment of the right main bronchus. The deflated lung can be appreciated. In (C), the caliber of the detached bronchus is shown. After insufflation of the bronchus, the lung expanded nicely (D).


In our case, we chose to perform a thoracotomy first in order to detach the bronchus from the esophagus and then performed the reinsertion into the trachea through a sternotomy on CPB with circulatory arrest. This approach was motivated by the fact that not only did we find a large caliber main bronchus amendable to suturing but also considered the lung parenchyma to be well developed. We felt that preservation of potentially functional lung tissue ought to be attempted, especially in a child. Also, independently from lung resection, the child required both sternotomy and CPB support for the repair of tracheal stenosis. However, for slide tracheoplasty alone, it would have not required induced circulatory arrest, which can add to the overall risk. In hindsight, we reconsider our decision. A simple pneumonectomy combined with slide tracheoplasty would have still been a major but potentially more straightforward operation with less risk. However, this would have come at the expense of losing the complete right lung at a very young age. Another possibility would have been the successful multiple-step approach recently described by Takamizawa and colleagues [19]. They initially dilated the stenosed trachea and intubated the healthy main bronchus by force. Then they reinserted the affected bronchus into the trachea, protecting the fresh anastomosis by remaining the endotracheal tube in the healthy bronchus. A tracheostomy and subsequently a slide tracheoplasty were performed. These steps were carried our over the course of approximately 20 months. The child was described to strive well 2 years after completion of treatment. We did not consider this elegant option, because the publication was not available at the time we intended our repair. Due to their rarity, it is exceedingly difficult to extract which approach is superior over another for children with esophageal lung and combined tracheal stenosis. Given the data presented here, it seems convenient to suggest that either simple pneumonectomy or a multiple-step approach is superior to a single-stage correction. Certainly, with respect to our own case, if imaginatively ever given again the privilege to care for a child with this exact malformation, most likely we would opt for a correction based on a multi-step attempt. However, our own interpretation is certainly biased, and no two malformations are identical. Therefore, thorough risk and benefit evaluation must supervene on an individual basis in order to select the best treatment options. In our opinion, the long postoperative intubation time of 51 days until death must be considered as a major additional culprit. Manning and colleagues found preoperative mechanical ventilator support and duration of CPB to be significant predictors of the need for prolonged postoperative intubation after slide tracheoplasty [25]. Our patient had both risk factors plus the additional risk from bronchotracheal anastomosis. Pulmonary toilet was optimized daily and ventilator settings were weaned to minimal settings, and several attempt were made to extubate the child, all without success. We did consider tracheostomy, but decided against it due to the recent slide tracheoplasty. Extracorporeal membrane oxygenation (ECMO) followed by extubation and lungrecruitment maneuvers has been described as a successful rescue therapy in a child with tracheal dehiscence following slide tracheoplasty [26]. At the time of clinical deterioration caused by the bronchopleural fistula, we considered ECMO, for which we have immediate capacity at our institution, but felt that early intervention would give us the best chance to surgically repair the defect. Unfortunately, we are unable to tell if the reinserted lung parenchyma was ever functional. We did considered performing a nuclear lung scan during the postoperative course in order to evaluate the functionality of the affected lung, as this could have pushed us towards secondarily performing pneumonectomy. However, repeated chest X-rays and echocardiograms prior to

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clinical deterioration were encouraging, and we felt that performing nuclear lung scan in an intensive care setting would have added additional risks to the patient. Upon re-exploration, we found the affected side of the lung to be completely necrotic. We did not find kinking of the pulmonary vessels as a cause of necrosis, which in turn surely affected the development of a bronchopleural fistula. Likely, local inflammation paired with the above mentioned small air leak prohibited adequate healing and ultimately lead to the unfortunate outcome described here. 3. Conclusion In conclusion, esophageal lung associated with tracheal stenosis represents an extremely rare variant of CBPFMs. The clinical presentation and diagnostic workup is similar to that of other CBPFMs, with the addition of special attention to the tracheal stenosis. Treatment options compromise in pneumonectomy versus bronchial reconstruction with simultaneous slide tracheoplasty, potentially as a multi-step approach. Thorough risk versus benefit evaluation must guide the choice of one approach over another on an individual basis, and a special focus should be laid on an optimal postoperative care. Conflict of interest There are no conflicts of interest to declare. Sources of funding There have been no sources of funding for this work. References [1] Girosi D, Bellodi S, Sabatini F, Rossi GA. The lung and the gut: common origins, close links. Paediatr Respir Rev 2006;7(Suppl. 1):S235e9. [2] Leithiser RE, Capitanio MA, Macpherson RI, Wood BP. “Communicating” bronchopulmonary foregut malformations. Am J Roentgenol 1986;146: 227e31. [3] Saydam TC, Mychaliska GB, Harrison MR. Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management. J Pediatr Surg 1999;34:615e8. [4] Sugandhi N, Sharma P, Agarwala S, Kabra SK, Gupta AK, Gupta DK. Esophageal lung: presentation, management, and review of literature. J Pediatr Surg 2011; 46:1634e7. [5] Keeley JL, Schairer AE. The anomalous origin of the right main bronchus from the esophagus. Ann Surg 1960;152:871e4. [6] Lallemand D, Quignodon JF, Courtel JV. The anomalous origin of bronchus from the esophagus: report of three cases. Pediatr Radiol 1996;26:179e82. [7] Matsumoto Y, Ohi R, Hayashi Y, Chiba T. Right pneumonectomy syndrome: report of two cases. Surg Today 1995;25:278e80. [8] Linke F, Kraemer W, Ansorge M, Brzezinska R, Berger S. Right esophageal lung in a preterm child with VACTERL association and Mayer-Rokitansky-KusterHauser syndrome. Pediatr Surg Int 2005;21:285e8. [9] Horigome H, Hirano T, Umesato Y, Kemmotsu H, Joe K. Oesophageal lung with systemic arterial blood supply. Eur J Pediatr 1989;149:72e3. [10] Warner FS, McGraw CT, Peterson HG, Cleland RS, Meyer BW. Lung ectopia and agenesis with heart dextrorotation. Am J Dis Child 1961;101:514e8. [11] Thomson NB, Aquino T. Anomalous origin of the right main-stem bronchus. Surgery 1962;51:668e76. [12] Nikaido H, Swenson O. The ectopic origin of the right main bronchus from the esophagus. A case of pneumonectomy in a neonate. J Thorac Cardiovasc Surg 1971;62:151e60. [13] Toyama WM. Esophageal atresia and tracheoesophageal fistula in association with bronchial and pulmonary anomalies. J Pediatr Surg 1972;7:302e7. [14] Lacina S, Townley R, Radecki L, Stockinger F, Wyngaarden M. Esophageal lung with cardiac abnormalities. Chest 1981;79:468e70. [15] Schwartz DL, So HB, Anban T, Creedon JJ. Total lung sequestration in association with oesophageal atresia and multiple tracheo-oesophageal fistulae. Z Kinderchir 1983;38:410e1. [16] Chiba T, Ohi R, Hayashi Y, Uchida T. Bronchopulmonary foregut malformation in 3 infantsewith special references to cases in childhood. Z Kinderchir 1989; 44:105e8. [17] Mukai S, Kikuchi H, Akiyama H, Morio M. Management of anaesthesia in an infant with an anomalous lung arising from the oesophagus. Br J Anaesth 1977;49:379e82.

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