Congenital Lung Abnormalities on MRI

Congenital lung abnormalities (CLA) are rare developmental anomalies which are
increasingly being detected by prenatal ultrasonography. CPAM*s are the most
prevalent abnormality comprising up to 30% of all CLA*s. They are cystic lung
tissue malformations with pulmonary vascularization and an intact but abnormal
connection to the tracheobronchial tree. In symptomatic patient a surgical
resection is warranted but there is currently no consensus on the best mode of
treatment in asymptomatic patients. They are either operated or undergo
structured long-term follow-up. Computed tomography (CT) is the postnatal
diagnostic method of choice and most frequently used imaging modality for
long-term monitoring and as pre-operative workup because of high accuracy and
its excellent spatial resolution. Due to the exposure of ionizing radiation its
use should be limited. With recent technological advances allowing shorter scan
times, MRI is increasingly being used in diagnosis of various childhood lung
diseases including CLA*s, avoiding radiation exposure. Furthermore, the use of
functional MRI techniques have added value due to real time imaging. However,
there is still lack of information on the optimal scan protocol in CLA and the
appearance of these parenchymal anomalies on MRI. As follow-up is warranted in
CPAM, a reduction in unnecessary radiation exposure may be achieved by using
MRI instead of CT-imaging. Because disease behavior is unknown, standardized
follow up is done adhering to general protocols. By imaging these patients the
course of the disease may be monitored and follow up may be adapted to these
findings.

Our main objective is to validate a MRI scan protocol for imaging parenchymal
abnormalities in CPAM and describe the appearance of these abnormalities on
MRI. Furthermore, we want to image postoperative changes in developing lungs of
patients operated for CPAM. Correlation between imaging and lung function
findings to each other and clinical parameters is a secondary objective.

Prospective, cross-sectional study of patients with (history of) CPAM conducted
at the Erasmus MC * Sophia Children*s Hospital. All subjects are enrolled in a
prospective follow-up program in children with congenital anomalies (CHIL,
surgical long-term follow-up). Each included patient will visit the hospital
once during which a lung function test will be obtained and a subsequent MRI
will be made after obtaining signed informed consent.

CPAM is a rare disease and the majority of asymptomatic cases have been
detected since the introduction of the prenatal structured ultrasound in the
last two decades. As this is the case, the majority of unresected CPAM cases
are still in the pediatric age range. As the general practice is to
avoid/minimize radiation exposure in the pediatric population, little is known
about structural changes of developing lungs after a lung resection.
Information obtained from these subjects might possibly aid in further research
on lung resection procedures and subsequent follow-up in the pediatric
population. MRI is considered a safe imaging technique with no exposure to
ionizing radiation. Other than anxiety due to claustrophobia and noise produced
by the MRI, no other side-effects are known. Our MRI scan protocol is focused
on obtaining the most clear pictures in the least amount of time not exceeding
45 minutes in total.