Recurrent Pneumonia Caused by Diverticulum at the Site of TEF Repair
An 18-year-old girl was referred for evaluation of “uncontrolled asthma.” She had a long-standing history of recurrent coughing, wheezing, and documented pneumonias that were treated with frequent bronchodilators, inhaled and oral corticosteroids, antibiotics, and anti-reflux medications. At 4 months of age, she had undergone correction of type A esophageal atresia (EA) and tracheoesophageal fistula (TEF).
Diagnostic evaluation included an esophagram with a catheter, which demonstrated no stricture, anastomotic leak, or recurrent TEF. Flexible bronchoscopy revealed tracheomalacia and secretions bubbling out of a diverticulum in the posterior tracheal wall at the repair site (A).This finding was confirmed by a 3-dimensional CT scan of the chest (B).
TEF, the most common congenital tracheal abnormality, is frequently associated with EA. Coexistence of EA and TEF is classified according to their anatomic configuration.1 Type A, which consists of a proximal esophageal pouch and a distal TEF, accounts for 85% of cases.1
Respiratory and GI complications are common in patients with all types of TEF, even after fistula repair.2 These complications include recurrent pneumonia,
impaired esophageal peristalsis, gastroesophageal reflux with aspiration of gastric contents, recurrent TEF, tracheomalacia, abnormal respiratory epithelium, esophageal stenosis, anastomosis leak, and tracheal diverticulum.3,4
Tracheal diverticulum as a complication of TEF repair has been reported.5 The diverticulum contributes to poor airway clearance and increased risk for recurrent pneumonia and atelectasis by several possible mechanisms, such as obstructing lower airway clearance and facilitating the pooling of secretions from the proximal airway.
Excision of the diverticulum has been shown to reduce symptoms.5 This patient’s diverticulum was obliterated with cauterization, and her respiratory symptoms subsequently resolved with no documented pneumonias.
It is important to exclude tracheal diverticula in addition to the well-recognized respiratory and GI complications in patients with a history of EA and/or TEF before symptoms are attributed to asthma.4,6
1. Clements BS. Congenital malformations of the lungs and airways. In: Taussig LM, Landau LI, eds. Pediatric Respiratory Medicine. St Louis: Mosby; 1999:1995.
2. Shah AR, Lazar EL, Atlas AB. Tracheal diverticula after tracheoesophageal fistula repair: case series and review of the literature. J Pediatr Surg. 2009;44(11):
3. Levy J. Pulmonary manifestations of gastrointestinal diseases. In: Turcios NL, Fink RJ, eds. Pulmonary Manifestations of Pediatric Diseases. Philadelphia: Elsevier; 2009:98.
4. Kovesi T, Rubin S. Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest. 2004;126(3):915-925.
5. Gaissert HA, Grillo HC. Complications of the tracheal diverticulum after division of congenital tracheoesophageal fistula. J Pediatr Surg. 2006;41(4):
6. Holland AJ, Fitzgerald DA. Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications. Paediatr Respir Rev. 2010;11(2):100-107.