Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation

Background-Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favou rable natural history than that of sequestration without associated cystic adenomatoid malformation. Methods-An outline of the postnatal work up leading to the management of ex tralobar or intralobar pulmonary sequestration with congenital cystic adeno matoid malformation diagnosed antenatally as pulmonary malformation is pres ented and the indications for surgical intervention are discussed. Results-In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic ad enomatoid malformation was made postnatally. Postnatal ultrasound and compu terised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed i n the basal area of the sequestration in all patients. Four children remain ed asymptomatic and one infant presented at 10 months of age with pneumonia . The mean age at surgical resection was 6.8 months (range 2-10). Histopath ological examination confirmed intralobar pulmonary sequestration with asso ciated Stocker type 2 congenital cystic adenomatoid malformation in two pat ients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean per iod of follow up was four years (range 1-8). The children remain well and a re developing normally. Conclusions-The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and co ngenital cystic adenomatoid malformation probably share a common embryogene sis despite diverse morphology. The natural history of antenatally diagnose d lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.