PEMP 0317 Pneumothorax In Pediatric Patients.pdf

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Secondary Pneumothorax
A secondary pneumothorax is the result of an
underlying disease process or trauma. In children,
a pneumothorax may be the presenting sign of
the pathology or the sequela of a known diagnosis, such as cystic fibrosis. Pneumothoraces can
also result from excessive coughing, as in severe
asthma exacerbation,6 pertussis,25 or pneumonia.
Additionally, numerous case reports and case
series have identified patients with a rare genetic
disorder with mutations in the folliculin gene,
known as Birt-Hogg-Dubé syndrome, who are at
higher risk of spontaneous pneumothorax.26-28
Secondary pneumothoraces typically occur later
in a patient’s life, due to disease progression, such
as in the case of patients with bronchopleural
fistula (aberrant connections between the bronchi
and pleural space that can be seen in the setting
of diseases such as cystic fibrosis or after thoracic
trauma). See Table 2 for a list of diseases and conditions that increase the risk of pneumothorax.

A rare type of secondary pneumothorax is
catamenial pneumothorax. Presenting typically
as monthly recurrent pneumothorax in a young
woman, it is believed to be an atypical presentation
of endometriosis.32 Endometrial tissue embeds on
the surface of the lung parenchyma. During menses,
bleeding causes local inflammation and necrosis,
leading to the development of an air leak and subsequent pneumothorax.

Neonatal Pneumothorax
In full-term infants, pneumothorax is likely related
to barotrauma during vaginal delivery. Most typically, these pneumothoraces become symptomatic
during the hospitalization period and are therefore
rarely encountered in the ED. Risk factors for neonatal pneumothorax include prematurity, surfactant
deficiency, low birth weight, out-of-hospital birth,
chronic lung disease, sepsis, and mechanical ventilation. Pneumothorax should remain on the differential diagnosis for the infant with an extramural
delivery who presents with respiratory distress.
Traumatic Pneumothorax
Pneumothorax is a common complication of both
blunt and penetrating trauma to the chest and/or
abdomen, and may also be seen in blast injuries. The
mechanism of traumatic pneumothorax is clearly
different than that of PSPs; therefore, evaluation for
concomitant injuries such as rib fracture, pulmonary
contusions, subcutaneous emphysema, and cardiac
injuries should be undertaken.33
Open Pneumothorax
An open pneumothorax is the result of penetrating
chest trauma that leaves a defect in the chest wall,
typically the result of a stab wound or missile penetration of the thorax. This will lead to an ongoing
air leak into the pleural space, and can quickly lead
to tension physiology.
Occult Pneumothorax
An occult pneumothorax is generally small and clinically silent on initial presentation. It is not seen on
initial chest radiography, but may be found incidentally on computed tomography (CT) scans intended
to evaluate other suspected injuries. There are limited
data in both the adult and pediatric literature regarding management of an occult pneumothorax diagnosed in the trauma patient. Obvious questions arise
regarding whether the pneumothorax will expand or
whether the presence of the pneumothorax indicates
that there may be a significant pulmonary injury. In
a large prospective observational study through the
Pediatric Emergency Care Applied Research Network
(PECARN) that enrolled 12,044 pediatric patients
who had suffered a blunt torso trauma, 8020 of these
children underwent chest radiography. A pneumothorax was identified in 372 patients (4.6%). Sixty percent
of these patients with a pneumothorax identified on
CT scans met the definition of occult pneumothorax
as described by the study authors.34 While 3-dimensional imaging is very sensitive for identifying occult
pneumothorax, the clinical relevance of the entity
remains questionable.

Table 2. Diseases And Conditions
That Increase The Risk Of Secondary
• Asthma
• Cystic fibrosis
• Alpha-1 antitrypsin deficiency29
• Congenital pulmonary airway malformation
• Congenital lobar emphysema
• Pneumonia
• Tuberculosis
• Pneumocystis
• Connective tissues disorders (ie, Ehlers-Danlos,30 Marfan
• Birt-Hogg-Dubé syndrome
• Catamenial pneumothorax
• Barotrauma (ie, mechanical ventilation, bag-valve mask)
• Mechanical ventilation
• Percutaneous lung biopsy
• Liver biopsy31
• Subclavian central line placement
• Internal jugular central line placement
• Thoracentesis
• Thoracic radiation

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Tension Pneumothorax
A tension pneumothorax is a complication of either
primary or secondary pneumothorax, and it results in