While rare in children, primary tracheobronchial tumors may create symptoms due to varying degrees of airway obstruction Such lesions can be benign or malignant, and may be present at various locations in the tracheobronchial tree, with the majority of lesions occurring in the larynx, trachea, and larger bronchi Clinical Recognition Clinical presentation depends on the site and type of tumor, and less severe symptoms can masquerade as bronchitis, pneumonia, or asthma episodes with delayed ultimate diagnosis Right Middle Lobe Syndrome Right middle lobe syndrome is the recurrence or persistence of atelectasis or pneumonitis of the right middle lobe, sometimes associated with bronchiectasis It has been described in all age groups, in both the right middle and lower lobes concomitantly, and has also been observed in the lingula segment This process can be caused by extraluminal or intraluminal obstruction, or by nonobstructive causes The right middle lobe is anatomically predisposed to compression of its bronchus by the lymph nodes in the vicinity that encircle it, which can lead to sequestered areas of collapsed or infected lung Since it is bordered by two fissures, it also receives less collateral ventilation than other lobes, making reinflation of collapsed lung more challenging Because the right middle and lower lobes are favored sites for aspirated material (Fig 124.1 ), recurrent inflammation caused by pneumonia can lead to chronic atelectasis and adenopathy A similar situation can be seen with intraluminal tumors and other space-occupying lesions In children, nonobstructive causes such as asthma, cystic fibrosis, and immobile cilia syndrome can also result in prolonged atelectasis, which promotes recurrent infection and eventual bronchiectasis Clinical Recognition Right middle lobe syndrome is a radiographic diagnosis, so physical manifestations vary widely but commonly include persistent cough, intermittent wheezing, or dyspnea Patients may have wheezing, rales, or decreased aeration on lung examination, particularly appreciated anteriorly Chest radiographs typically reveal atelectasis of the right middle lobe, which may be recurrent or persistent They may also show bronchiectasis or pneumonitis of the affected area Recurrent episodes of pneumonia and associated atelectasis in the lingula or right middle (and often lower) lobes occur in these patients and are not responsive to chest percussion, postural drainage, or antibiotic treatment Management Patients with right middle lobe syndrome may respond to conventional medical management along with chest physical therapy and postural drainage When this is not the case, bronchoscopy and bronchoalveolar lavage may be helpful to restore patency of the bronchus, allow better postural drainage, and enhance antibiotic effectiveness Although the need for resection is far less common than in the past, patients with systemic symptoms such as failure to thrive, obstructing lesions, bronchiectasis, bronchial stenosis, or failure to respond to medical management should be considered candidates for lobectomy ESOPHAGUS-RELATED CAUSES OF AIRWAY DIFFICULTIES Goal of Treatment Patients with congenital and acquired esophageal abnormalities may present with a variety of urgent complaints, the most concerning of which include significant dysphagia, impacted food or foreign bodies, and in some cases even respiratory symptoms or distress In patients with airway concerns, prompt evaluation and treatment are critical Familiarity with the common congenital esophageal anomalies as well as acquired esophageal emergencies and urgencies are critical to arriving at a prompt diagnosis and delivering the appropriate treatment CLINICAL PEARLS AND PITFALLS Patients with a history of repaired esophageal atresia frequently have tracheomalacia If severe, a child may present with episodes of respiratory distress or “death spells.” Patients with an H-type esophageal fistula (patent trachea and esophagus with connection between the two structures) may present after the neonatal period with symptoms of recurrent choking and congestion with feeds, or with recurrent pneumonias Patients with a history of repaired esophageal atresia may present at any age with an esophageal stricture Typical symptoms are progressive intolerance of solid food followed later by intolerance of liquids as well In severe cases, patients may not be able to manage oral secretions Esophageal injuries in older children typically present with significant symptoms (e.g., retrosternal chest pain, dysphagia, stridor, retching) Younger children may present with more subtle or vague symptoms Esophageal Atresia and Tracheoesophageal Fistula TEF is typically a congenital condition but has been rarely reported as an acquired problem after suppuration of mediastinal nodes or as a result of iatrogenic injury or a complication following foreign-body ingestion The more common congenital TEF is accompanied by atresia of the esophagus in more than 85% of patients and generally presents in the immediate perinatal period with feeding intolerance and inability to handle secretions These patients undergo repair shortly after diagnosis, typically via a right thoracotomy or thoracoscopic approach Approximately 3% of all patients with TEF have an “H-type” fistula, in which both the trachea and the esophagus are intact and patent but are connected by a fistula that is frequently in the cervical region or high in the thorax (Fig 124.2 ) Because there is no accompanying esophageal atresia, these patients are more likely to present later in infancy or childhood with symptoms of recurrent respiratory distress or pulmonary aspiration The acquired form is usually in the distal trachea or proximal bronchial tree, and is extremely uncommon H-type fistulae are notoriously difficult to diagnose, particularly if the fistula tract is small Children generally develop recurrent pulmonary infections with no obvious source The characteristic history of choking or gagging with swallowing that accompanies esophageal atresia with TEF may not be present, but parents may describe excessive secretions or noisy breathing after feeds Contrast esophagram, specifically looking for an H-type TEF is necessary Most of these fistulae are quite small in diameter (millimeters) and short (also less than cm), making radiographic identification difficult Even when contrast appears in the tracheobronchial tree, it may be difficult to know whether primary aspiration of orally administered contrast is responsible Placing a feeding tube in the esophagus and injecting contrast while pulling the tube back up from the lower esophagus under fluoroscopic observation may be helpful This study is most accurate when performed with the patient in the prone position Highresolution CT scans may identify the anatomy, but are less commonly performed to diagnose such fistulae Bronchoscopy and esophagoscopy may both be diagnostic and may be useful at the time of repair if a small catheter or wire can be passed across the fistula to aid its identification by enabling palpation at operation Most such fistulae are cervical and can be repaired via a neck incision FIGURE 124.2 H-type tracheoesophageal fistula