Volume 120, Issue 3 , Pages 541-543, September 2007
Diagnosis of a symptomatic tracheal pouch by means of bronchoscopy after neonatal repair of a tracheoesophageal fistula
Article Outline
Key words: Tracheal pouch, diverticulum, tracheoesophageal fistula, thoracoscopic surgery, recurrent pneumonias
Abbreviations used: EA, Esophageal atresia, TEF, Tracheoesophageal fistula
A tracheoesophageal fistula (TEF) is a congenital or acquired communication between the trachea and esophagus. Although a TEF can exist as an isolated congenital anomaly, the majority of them are seen in association with esophageal atresia (EA). Several anatomic variations of EA with or without a TEF can occur. The most common anomaly consists of a blind esophageal pouch and a distal TEF. Most individuals with TEF are given diagnoses shortly after birth or during early infancy because TEF is often associated with life-threatening feeding and respiratory complications. These infants undergo surgical repair in the neonatal period after diagnosis. A TEF in a newborn infant is demonstrated by means of rigid bronchoscopy (see the video clip in this article's Online Repository at www.jacionline.org).
Respiratory and gastrointestinal complications commonly occur after surgical repair of TEF and EA and are significant causes of lifelong morbidity and occasional mortality. The most common problems are gastroesophageal reflux, anastomotic stricture, esophageal dysmotility, tracheomalacia, chronic aspiration, and recurrent pneumonias.1, 2, 3 The long-term respiratory complications of TEF were thoroughly reviewed by Kovesi and Rubin.3 Tracheomalacia is a common postrepair finding, and manifestations include a harsh productive cough, wheezing during respiratory tract infections, and occasional “dying spells” (characterized by cyanosis and bradycardia and progressing to apnea) caused by severe airway obstruction. Wheezing illnesses and recurrent respiratory tract infections, including pneumonias, are frequently seen after TEF repair. Bronchiectasis has also been reported in adults with a history of TEF repair.4 Other long-term respiratory complications include recurrent TEF, airways hyperreactivity, and chest wall deformities (most commonly scoliosis and anterior chest wall asymmetry).
In those with a history of neonatal TEF repair, respiratory symptoms of chronic brassy (barking or honking) cough, wheezing, and bronchitis are frequent in early childhood and can persist into adulthood. These respiratory symptoms might be attributed to airway obstruction caused by tracheomalacia, retained secretions caused by impaired mucociliary clearance, aspiration, recurrence of the TEF, and/or gastroesophageal reflux disease. Although children with a history of TEF repair commonly cough and wheeze and many are given a diagnosis of asthma, only a minority demonstrate a significant bronchodilator response, suggesting that wheezing is likely due to other causes.5 In fact, pulmonary function testing frequently reveals a restrictive pattern, with a decrease in lung volumes rather than airflow obstruction.3 Therefore in persons with a history of TEF/EA, it is essential that other known respiratory complications are excluded before respiratory symptoms are assumed to be due to asthma. Direct visualization of the airways with a flexible bronchoscopy might be indicated to evaluate the possibility of a persistent anatomic abnormality and to culture lower respiratory secretions for microbes.
The following case report focuses on a child with trisomy 21 and a history of neonatal TEF repair and recurrent respiratory tract illnesses. She had a presumptive diagnosis of asthma based on her symptoms. However, her medical course was not improved by asthma therapy. A flexible bronchoscopy was performed to aid in diagnosis. For this child, the bronchoscopy proved to be an extremely valuable tool in identifying a rarely reported complication of TEF/EA repair, a large tracheal pouch or diverticulum, as the cause of her recurrent respiratory symptoms.
Case report
A 6-year-old girl with trisomy 21 and a congenital TEF/EA repaired in the neonatal period presented with a history of asthma and recurrent pneumonias. She was given a presumptive diagnosis of asthma as a toddler because of recurrent coughing and wheezing symptoms and managed intermittently with albuterol and corticosteroids. In the year before our evaluation, she was hospitalized 6 times with diagnoses of asthma and pneumonia and treated with oxygen, antibiotics, bronchodilators, and corticosteroids. Diagnostic evaluations during this time included multiple chest radiographs indicative of pneumonia and airways disease, an upper gastrointestinal study that demonstrated mild dilatation of the proximal thoracic esophagus at the anastomosis site without evidence of esophageal stricture or gastroesophageal reflux, a modified barium swallow that showed trace aspiration of thin liquids, a normal electrocardiogram, an echocardiogram that revealed a small patent ductus arteriosus without evidence of pulmonary hypertension, and a polysomnogram suggestive of upper airway resistance syndrome. We then performed a flexible bronchoscopy that revealed a large tracheal pouch 2 to 3 cm proximal to the carina on the posterior wall of the trachea (Fig 1). Copious mucopurulent secretions filled the pouch and were present throughout the distal airways. With spontaneous respiration during bronchoscopy, the mucus-filled pouch would bulge into the tracheal lumen, resulting in moderate-to-severe tracheomalacia. After the bronchoscopy, a high-resolution computed tomographic scan of the chest demonstrated this large focal outpouching from the posterior wall of the distal trachea, bilateral patchy opacities indicative of pneumonitis and atelectasis, and mild cylindrical bronchiectasis in both lower lobes (Fig 2). She subsequently underwent a thoracoscopic resection of the tracheal pouch. There was no postoperative morbidity, and the child was discharged home the next day. In the year after surgery, she was hospitalized once and treated with antibiotics and augmented mucus clearance 3 times for respiratory tract illnesses.
Fig 1.
View of the distal tracheal pouch during flexible bronchoscopy.
Fig 2.
A, Chest computed tomographic scan demonstrating a focal outpouching of air extending posteriorly to the left in the distal trachea without obvious connection to the dilated esophagus seen more posteriorly and to the right in the mediastinum. RUL, Right upper lobe. B, Also noted were focal patchy opacities and mild cylindrical bronchiectasis in the right middle lobe and both lower lobes.
Discussion
A tracheal pouch or diverticulum can be either congenital or acquired. Acquired forms are less common and are usually seen after TEF repair. Review of the literature reveals only a handful of published cases of this complication.6, 7, 8 Johnson et al8 recently reported the largest case series of acquired symptomatic tracheal pouches as sequelae to TEF repairs and nicely described the varied clinical presentations. Some children present with recurrent pulmonary infections caused by pooling and stasis of secretions that occur within the pouch. In others the pouch is associated with significant tracheomalacia and airway obstruction. Our child had symptoms of both these clinical presentations. Infrequently, acute airway obstruction will occur if the tip of an endotracheal or tracheostomy tube is displaced into the pouch. Acquired pouches are typically larger than the congenital form, have a wider opening, and are therefore more likely to be symptomatic. Repair of a tracheal pouch is usually reserved for symptomatic cases.
Although the child in the case report was given a clinical diagnosis of asthma and managed as such, her clinical course should have raised concerns for another cause of her recurrent respiratory tract illnesses. There is a tendency to attribute lower respiratory tract symptoms to asthma in this group of patients. Yet most persons with a history of TEF probably do not have asthma because previous studies have observed only minor improvements in lung function after bronchodilator administration in these patients.5
This case report highlights the possibility of a tracheal pouch as an infrequent and commonly misdiagnosed cause of persistent or recurrent respiratory symptoms that frequently occur after primary repairs of EA and TEFs. In persons with a history of repaired TEFs who present with persistent or recurrent respiratory problems and in whom the more common causes have been ruled out, direct visualization of the trachea by means of flexible bronchoscopy should be included in the evaluation process. A more timely detection and repair of this unusual complication of congenital TEF could provide symptomatic relief and might prevent bronchiectasis and other structural lung injuries.
Appendix. Supplementary data
Video clip. Rigid bronchoscopy demonstrating a TEF in a newborn infant. The TEF is best visualized near the end of the video clip after secretions have been suctioned from the airway. The TEF is identified with an arrow.
References
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- . Respiratory morbidity after repair of oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child. 1993;68:167–170
- . Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest. 2004;126:915–925
- . Adults who survived repair of congenital oesophageal atresia and tracheo-oesophageal fistula. BMJ. 1988;297:344–346
- . Respiratory function in childhood following repair of oesophageal atresia and tracheoesophageal fistula. Arch Dis Child. 1999;81:404–408
- . Tracheal diverticulum after surgical correction of esophageal atresia: 2 cases. Arch Fr Pediatr. 1978;35:641–645
- . Endoscopic treatment of tracheal diverticulum after primary repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1998;33:1323–1324
- . Management of symptomatic tracheal pouches. Int J Pediatr Otorhinolaryngol. 2007;71:527–531
Disclosure of potential conflict of interest: M. E. Krawiec has consulting arrangements with Novartis and is on the speakers' bureau for Merck and Glaxo Wellcome. The rest of the authors have declared that they have no conflict of interest.
PII: S0091-6749(07)01451-0
doi:10.1016/j.jaci.2007.07.050
© 2007 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.
Volume 120, Issue 3 , Pages 541-543, September 2007
