Management
Some cystic lung lesions shrink or disappear during gestation. Perinatologists
and fetal surgeons recommend that the child undergo postnatal CT or MRI to
determined whether abnormal lung tissue still exists Elective resection is recommended
even for asymptomatic lesions. Abnormal lung tissue is a nidus for infection due to
abnormal air drainage. In addtion, cystic lung lesions and sequestrations have been
associated with the development of tumors later in life.
A newborn born with a cystic malformation of the lung should be birthed in a
facility that has immediate access to pediatric surgeons and possible ECMO. If a baby
is asymptomatic (breathing normally, NO tachypnea), a CXR should be performed to
document anatomy. The baby should be observed for 24-48 hours to make certain that
symptoms do not develop with feeding. Asymptomatic babies are sent home with a
follow-up with a surgeon. Typically, imaging with MRI or CT scan is performed prior to
the operation. Advanced imaging can identify systemic vascular supply and the precise
anatomic lesion. The operation can be deferred for a few months if the baby remains
without symptoms.
Symptomatic babies require immediate NICU transfer. If CLO is suspected, an
emergent surgical consultation is made in preparation for possible thoractomy.
Symptomatic babies with CCAM or sequestration would need an operation prior to
discharge. Imaging with CT or MRI is usually required.