and extend below the diaphragm. The presence and location of these vessels should
be identified by imaging preoperatively as inadvertent division of the vessel can result in
its retraction of a vessel below the diaphragm and uncontrolled bleeding.
CCAM’s usually occur in the upper lobes of the lungs. Histologically, they are
classified according to the size of the cysts within the lesion. Type I CCAM’s have large
cysts, type III CCAM have dense small cysts , and type II cysts have a combination of
both.. CCAM’s may involve one or multiple lobes.
Congenital lobal overinflation (also known as congenital lobar emphysema) is a
lesion that typically occurs in the upper lobes, more commonly the left. There is an
anatomic defect in the lobar bronchus that does not allow complete emptying of the lobe
during exhalation. Overinflation of the lobe can cause mediastinal shift and a tension
pneumothorax physiology. These patients may require emergent thoracotomy and
lobectomy after birth.
Associated anomalies: Extralobar sequestrations may be seen with a diaphragmatic
hernia. 30% of extralobar sequestrations are associated with other anomalies such as
cardiac and gastrointestinal anomalies. 10% of intralobar sequestrations may have a
communication with the gastrointestinal tract. If a lung anomaly involves the right lower
lobe, a Scimitar syndrome should be suspected. This involves anomalous pulmonary
venous return into the heart. Depending on the anatomic variant, a baby may require a
combined lung lobectomy and cardiac surgery or embolization of anomalous vessels
prior to surgery.