Introduction
Congenital pulmonary airway malformations (CPAM), characterized by an
abnormal airway pattern, are developmental malformations of the lower
respiratory tract that occur during lung branching morphogenesis. CPAM
has the potential to cause cystic and/or adenomatous pulmonary areas.
Despite being a rare condition with a prevalence of only 0.81/10,000
fetuses, CPAM is considered to be the most common congenital lung
abnormality, being responsible for 30–40% of all malformations (1).
The pathogenesis and exact cellular mechanisms behind CPAM are not fully
understood yet; however, many potential genes were suspected to play a
part in the formation of lung cysts (2). Nowadays, with the advance of
prenatal ultrasound (90% sensitivity and 77% specificity for CPAM),
CPAM is often diagnosed antenatally, by detecting an echogenic mass
within the fetal lungs usually in the second trimester of gestation
(21–24 weeks), then followed up routinely during the course of
pregnancy (1). Magnetic resonance imaging (MRI) also plays an important
diagnostic role complementary to ultrasound since it is able to define
the type and extent of these lung lesions, thus guiding prenatal
intervention and postnatal care (2). CPAM can be classified into five
different types, each one of them vary in appearance and originate from
a different area in the lung (3). Prognosis, and therefore, management
usually depends on the classification and presence of symptoms and any
other fetal anomalies (3). We present a symptomatic case of CPAM with
the associated clinical, radiological and pathological details. The work
has been reported in line with the SCARE criteria (4).