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angiography, echocardiography, magnetic resonance imaging (MRI), and digital
subtraction angiography are needed in some combination to define the anatomy.
Management
Although some patients with constricting anomalies improve as they grow, most
will require surgical correction. Surgical treatment is usually indicated to relieve
the obstruction, with predictable and immediate resolution of symptoms and
excellent outcomes. Surgical repair is accomplished by dividing the vascular ring
and preserving the blood supply to the aortic branches. This is usually
accomplished by a left thoracotomy but more recently, video-assisted
thoracoscopic surgery (VATS) and endoscopic robotic-assisted techniques have
been adopted in some centers for select patients.
BRONCHIAL LESIONS
Bronchial Atresia
Congenital bronchial atresia is a rare anomaly characterized by a bronchocele
caused by a mucus-filled, blindly terminating segmental or lobar bronchus, with
resulting hyperinflation of the distal obstructed segment of lung. Hyperaeration is
believed to result from communication with the normally aerated lung via the
pores of Kohn and the channels of Lambert.
Clinical Recognition
Neonates and infants with this lesion are usually seen for respiratory distress. In
older patients, a history of episodic upper respiratory infection and wheezing may
be elicited. Some older patients may complain of dyspnea on exertion or
unilateral chest pain. Physical examination findings seldom suggest the diagnosis,
but unilaterally decreased breath sounds may be evident.
Management
Often, the diagnosis is suggested by chest radiograph, but high-resolution chest
CT scan may be necessary to more closely define the anatomy. Bronchoscopy is
the most efficient way to identify the atretic opening to the involved bronchus.
Complete atresia of a main stem or lobar bronchus may lead to infectious
complications or compression symptoms from overdistention of the affected lobe.