Pediatric emergency medicine trisk 1019

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 1 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.