| Thorax. 1999 August; 54(8): 701–706. | PMCID: PMC1745542 |
Management of antenatally diagnosed pulmonary sequestration
associated with congenital cystic adenomatoid malformation M. Samuel and D. Burge Wessex Regional Center for Pediatric Surgery, Southampton General Hospital, Southampton SO16 6YD, UK. Abstract 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 favourable
natural history than that of sequestration without associated cystic
adenomatoid malformation. METHODS—An outline of
the postnatal work up leading to the management of extralobar or
intralobar pulmonary sequestration with congenital cystic
adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented 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
adenomatoid malformation was made postnatally. Postnatal ultrasound
and computerised axial tomographic scans confirmed the diagnosis of
sequestration by delineating anomalous vascular supply. Cystic changes
were also observed in the basal area of the sequestration in all
patients. Four children remained 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). Histopathological examination confirmed
intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and
extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean
period of follow up was four years (range 1-8). The children remain
well and are developing normally. CONCLUSIONS—The
importance of seeking an anomalous blood supply in children with
congenital lung lesions is emphasised. Pulmonary sequestration and
congenital cystic adenomatoid malformation probably share a common
embryogenesis despite diverse morphology. The natural history of
antenatally diagnosed lung masses is variable. Early postnatal surgical
resection of pulmonary sequestration with cystic adenomatoid
malformation is recommended. Surgical excision should be conservative,
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