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A Novel Treatment Approach to H-Type Esophageal Lung in Presence of Pulmonary Artery Sling with Pneumonectomy and Intrathoracic Tissue-Expander PlacementEsophageal Lung and Pulmonary Arterial Sling FREE TO VIEW

Michael R. Phillips, MD; Jeff J. Dehmer, MD; Timothy M. Weiner, MD; Sang Lee, MD
Author and Funding Information

From the Division of Pediatric Surgery, North Carolina Children’s Hospital, The University of North Carolina at Chapel Hill, Chapel Hill, NC.

Correspondence: Sang Lee, MD, Division of Pediatric Surgery, North Carolina Children’s Hospital, The University of North Carolina at Chapel Hill, G141 Physician’s Office Bldg, 170 Manning Dr, Campus Box 7223, Chapel Hill, NC, 27599-7223; e-mail: sang_lee@med.unc.edu


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;145(6):1402-1406. doi:10.1378/chest.13-2343
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Congenital tracheal anomalies occur in 1:10,000 births and can be associated with congenital cardiac disease. This patient presented with right mainstem atresia, right bronchoesophageal fistula without esophageal atresia (H-type esophageal lung), and left pulmonary arterial (PA) sling. Taking this into consideration, surgical management included right pneumonectomy and placement of expandable prosthesis into the thoracic cavity to buttress the mediastinum and prevent tracheal deviation-induced kinking of the PA sling, which provided pulmonary blood flow to the remaining functional lung. To our knowledge, this is the first reported case of esophageal lung in conjunction with a PA sling, as well as the first documented use of an expandable prosthesis to prevent acute postpneumonectomy syndrome. This case can provide clinicians with increased clinical knowledge for treatment of this rare and potentially lethal combination of congenital anomalies.

Figures in this Article

Tracheoesophageal anomalies occur in 1:5,000 to 1:10,000 births.1,2 A rare subtype is when the anomalous connection occurs between a lung bronchus and the esophagus, also known as an esophageal lung.3,4 Tracheoesophageal anomalies are part of the VACTERL (vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and limb defects) association.2 Included within the spectrum of cardiac anomalies is pulmonary arterial (PA) sling, an anomalous origin of the left pulmonary artery from the right pulmonary artery, encircling the right mainstem bronchus and trachea before proceeding to the left lung.5

PA sling in conjunction with esophageal lung has never been reported. At our institution, an infant presented with this rare combination of anomalies. This unique case presented several therapeutic challenges, including the possibility of acute occlusion of the PA sling following pneumonectomy with potential fatal consequences. The patient’s family provided consent for publication of this case report, and it is compliant with Health Insurance Portability and Accountability Act (HIPAA) guidelines and our institution’s institutional review board policies.

A male infant born at 38 weeks’ gestational age presented with coughing episodes with oral feeding. An upper GI study showed anomalous connection of the right lung to the distal esophagus without esophageal atresia (Fig 1A). Bronchoscopy, MRI, CT scan, and echocardiography confirmed the absence of the right mainstem bronchus and also diagnosed a left PA sling (Figs 1B, 2). There was no evidence of pneumonia in the esophageal lung or symptoms from the PA sling, and he was being fed by a nasogastric tube distal to the bronchoesophageal fistula. After appropriate consultation, it was decided that the PA sling did not require intervention at this time because it was asymptomatic, but it was also decided that the esophageal lung posed a significant infectious risk secondary to its connection to the GI tract.

Figure Jump LinkFigure 1. A, Upper GI contrast study showing opacification of the right bronchial tree. Arrow notes contrast filling the aberrant right mainstream bronchus when contrast was infused into the stomach via nasogastric tube. B, CT chest scan showing the trachea (T) (arrow) with direct connection to the left lung as well as absence of right mainstem bronchus and atelectasis of right lung.Grahic Jump Location
Figure Jump LinkFigure 2. A, MRI image of the chest showing left PA sling looping around trachea. B, MRI image of the chest showing bronchoesophageal fistula. PA = pulmonary arterial.Grahic Jump Location

The patient was taken to the operating room for rigid bronchoscopy, bronchoesophageal fistula ligation, right pneumonectomy, and tissue-expander placement into the right hemithorax, with extracorporeal membrane oxygenation on standby. A right posterolateral thoracotomy was performed with dissection of the bronchoesophageal fistula and the hilar vasculature. The right lung was atelectatic but showed no external evidence of infection (Fig 3A). The large bronchoesophageal fistula was repaired with interrupted polydioxanone suture (Fig 3B). The pneumonectomy was then completed with suture ligation of the pulmonary vasculature (Fig 3C). The left PA sling was clearly visible as it looped around the distal trachea. A Versafil 100-mL tissue expander (Allergan, Inc) was then placed into the right-side chest and instilled with 45 mL of saline (Allergan, Inc) until it was abutting both the lateral chest wall and the mediastinum. The patient was then placed supine without hemodynamic compromise. Final pathology demonstrated a unilobar, hypoplastic lung with aberrant bronchial anatomy, acute bronchitis, and early pneumonia (Fig 3D).

Figure Jump LinkFigure 3. A, The atelectatic, but normal appearing RL. B, The isolated BEF in relation to the esophagus (E). C, Ligated pulmonary hilum (H) and esophagus (E) following pneumonectomy. D, The right lung following pneumonectomy, with BEF labeled. BEF = bronchoesophageal fistula; RL = right lung.Grahic Jump Location

The patient underwent a Ladd procedure and gastrostomy tube placement prior to discharge. He was discharged home, tolerating full tube feeds as well as some oral feedings without respiratory distress or coughing spells. In ongoing follow-up with the cardiology department, his left PA sling remains asymptomatic and will not be repaired unless it becomes symptomatic. Similarly, his tissue expander remains in place without signs of foreign body reaction or infection and will be left in situ while he continues to grow normally. However, if the PA sling becomes symptomatic, requiring surgical intervention, or if foreign-body reaction or infection were to occur, the tissue expander would be removed at that time. Removal of the tissue expander at that point would carry with it a minimal risk of mediastinal shift as there has been ample time to allow scarring and fixation of the mediastinum.

To our knowledge, this is the first case reported of esophageal lung in conjunction with a PA sling. Because the esophageal lung was a true space-occupying lesion, removal would precipitate at least some degree of rightward mediastinal and tracheal shift. Given that the pulmonary blood flow to the patient’s only functioning lung looped around the trachea (Fig 4), tracheal shift could have lethal consequences. Another factor influencing our decision-making was the right lung’s continuity with the GI tract and the progressive risk of pneumonia that could be life-threatening or, at the least, would make subsequent resection more difficult. The initial plan was to perform pneumonectomy at the time of PA sling repair, thus removing the risk of tracheal impingement on pulmonary blood flow. However, because the patient was asymptomatic, our consultants felt that repair was not warranted. We then proceeded with pneumonectomy and tissue-expander placement to buttress the mediastinum and trachea with extracorporeal membrane oxygenation standby.

Figure Jump LinkFigure 4. Artist rendition of the patient’s preoperative anatomy. The right mainstem bronchus originates from the distal esophagus moving superolateral to the right hilum. The left PA sling originates from the right PA and courses posterolateral to the trachea connecting with the left hilum (as opposed to its normal course anterior to the trachea). See Figure 2 legend for expansion of abbreviation.Grahic Jump Location

Although others have reported successful reimplantation of esophageal lung,6 we believe the absence of the right mainstem bronchus without a true carina would have made reimplantation without tension difficult if not impossible in this patient. Another option included pneumonectomy without tissue-expander placement, but we felt this presented to great a risk to pulmonary blood flow.

Postpneumonectomy syndrome is a complication which results from kinking of the trachea and remaining mainstem bronchus due to mediastinal shift and hyperinflation of the remaining lung.7-9 It is more common after right pneumonectomy due to the presence of the heart in the left hemithorax and is more common in children due to greater tissue elasticity in the mediastinum. Previous studies have described the use of prostheses to treat patients with postpneumonectomy syndrome,7,10-13 as well as prophylactically, without increased risk of infection.7 However, specific to this case, what is normally a delayed complication had potential to cause acute cardiopulmonary collapse as well. Hopefully, this case will increase the information available to practitioners who may encounter this unique condition.

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Ozimek CD, Grimson RC, Aylsworth AS. An epidemiologic study of tracheoesophageal fistula and esophageal atresia in North Carolina. Teratology. 1982;25(1):53-59.
 
Torfs CP, Curry CJ, Bateson TF. Population-based study of tracheoesophageal fistula and esophageal atresia. Teratology. 1995;52(4):220-232.
 
Saydam TC, Mychaliska GB, Harrison MR. Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management. J Pediatr Surg. 1999;34(4):615-618.
 
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(8):1634-1637.
 
Backer CL, Russell HM, Kaushal S, Rastatter JC, Rigsby CK, Holinger LD. Pulmonary artery sling: current results with cardiopulmonary bypass. J Thorac Cardiovasc Surg. 2012;143(1):144-151.
 
Michel JL, Revillon Y, Salakos C, et al. Successful bronchotracheal reconstruction in esophageal bronchus: two case reports. J Pediatr Surg. 1997;32(5):739-742.
 
Choi L, LaQuaglia MP, Cordeiro PG. Prevention of postpneumonectomy syndrome in children with prophylactic tissue expander insertion. J Pediatr Surg. 2012;47(7):1354-1357.
 
Rodefeld MD, Wile FD, Whyte RI, Pitlick PT, Black MD. Pulmonary vascular compromise in a child with postpneumonectomy syndrome. J Thorac Cardiovasc Surg. 2000;119(4 pt 1):851-853.
 
Stolar C, Berdon W, Reyes C, et al. Right pneumonectomy syndrome: a lethal complication of lung resection in a newborn with cystic adenomatoid malformation. J Pediatr Surg. 1988;23(12):1180-1183.
 
Audry G, Balquet P, Vazquez MP, et al. Expandable prosthesis in right postpneumonectomy syndrome in childhood and adolescence. Ann Thorac Surg. 1993;56(2):323-327.
 
Morrow SE, Glynn L, Ashcraft KW. Ping-pong ball plombage for right postpneumonectomy syndrome in children. J Pediatr Surg. 1998;33(7):1048-1051.
 
Ozcelik C, Onat S, Askar I, Topal E. Surgical correction of postpneumonectomy syndrome by intrapleural expandable prosthesis in a child. Interact Cardiovasc Thorac Surg. 2004;3(2):390-392.
 
Podevin G, Larroquet M, Camby C, Audry G, Plattner V, Heloury Y. Postpneumonectomy syndrome in children: advantages and long-term follow-up of expandable prosthesis. J Pediatr Surg. 2001;36(9):1425-1427.
 

Figures

Figure Jump LinkFigure 1. A, Upper GI contrast study showing opacification of the right bronchial tree. Arrow notes contrast filling the aberrant right mainstream bronchus when contrast was infused into the stomach via nasogastric tube. B, CT chest scan showing the trachea (T) (arrow) with direct connection to the left lung as well as absence of right mainstem bronchus and atelectasis of right lung.Grahic Jump Location
Figure Jump LinkFigure 2. A, MRI image of the chest showing left PA sling looping around trachea. B, MRI image of the chest showing bronchoesophageal fistula. PA = pulmonary arterial.Grahic Jump Location
Figure Jump LinkFigure 3. A, The atelectatic, but normal appearing RL. B, The isolated BEF in relation to the esophagus (E). C, Ligated pulmonary hilum (H) and esophagus (E) following pneumonectomy. D, The right lung following pneumonectomy, with BEF labeled. BEF = bronchoesophageal fistula; RL = right lung.Grahic Jump Location
Figure Jump LinkFigure 4. Artist rendition of the patient’s preoperative anatomy. The right mainstem bronchus originates from the distal esophagus moving superolateral to the right hilum. The left PA sling originates from the right PA and courses posterolateral to the trachea connecting with the left hilum (as opposed to its normal course anterior to the trachea). See Figure 2 legend for expansion of abbreviation.Grahic Jump Location

Tables

References

Ozimek CD, Grimson RC, Aylsworth AS. An epidemiologic study of tracheoesophageal fistula and esophageal atresia in North Carolina. Teratology. 1982;25(1):53-59.
 
Torfs CP, Curry CJ, Bateson TF. Population-based study of tracheoesophageal fistula and esophageal atresia. Teratology. 1995;52(4):220-232.
 
Saydam TC, Mychaliska GB, Harrison MR. Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management. J Pediatr Surg. 1999;34(4):615-618.
 
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(8):1634-1637.
 
Backer CL, Russell HM, Kaushal S, Rastatter JC, Rigsby CK, Holinger LD. Pulmonary artery sling: current results with cardiopulmonary bypass. J Thorac Cardiovasc Surg. 2012;143(1):144-151.
 
Michel JL, Revillon Y, Salakos C, et al. Successful bronchotracheal reconstruction in esophageal bronchus: two case reports. J Pediatr Surg. 1997;32(5):739-742.
 
Choi L, LaQuaglia MP, Cordeiro PG. Prevention of postpneumonectomy syndrome in children with prophylactic tissue expander insertion. J Pediatr Surg. 2012;47(7):1354-1357.
 
Rodefeld MD, Wile FD, Whyte RI, Pitlick PT, Black MD. Pulmonary vascular compromise in a child with postpneumonectomy syndrome. J Thorac Cardiovasc Surg. 2000;119(4 pt 1):851-853.
 
Stolar C, Berdon W, Reyes C, et al. Right pneumonectomy syndrome: a lethal complication of lung resection in a newborn with cystic adenomatoid malformation. J Pediatr Surg. 1988;23(12):1180-1183.
 
Audry G, Balquet P, Vazquez MP, et al. Expandable prosthesis in right postpneumonectomy syndrome in childhood and adolescence. Ann Thorac Surg. 1993;56(2):323-327.
 
Morrow SE, Glynn L, Ashcraft KW. Ping-pong ball plombage for right postpneumonectomy syndrome in children. J Pediatr Surg. 1998;33(7):1048-1051.
 
Ozcelik C, Onat S, Askar I, Topal E. Surgical correction of postpneumonectomy syndrome by intrapleural expandable prosthesis in a child. Interact Cardiovasc Thorac Surg. 2004;3(2):390-392.
 
Podevin G, Larroquet M, Camby C, Audry G, Plattner V, Heloury Y. Postpneumonectomy syndrome in children: advantages and long-term follow-up of expandable prosthesis. J Pediatr Surg. 2001;36(9):1425-1427.
 
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