Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery.

BACKGROUND: We have previously reported the outcome of a cohort of cases over a 10-year period with antenatally suspected congenital cystic adenomatoid malformation (CCAM) and have recommended elective surgery within the first year of life for persistent, asymptomatic cases because of the risk of long-term complications. The aims of this study were to document optimal postnatal investigation and timing of surgery.

METHODS: Cases were identified using the Oxford Congenital Anomaly Register, theatre records, and histopathology reports. Nineteen cases from 2003 onwards were reviewed with respect to postnatal imaging with chest x-ray and computed tomography scan and timing of surgery.

RESULTS: Chest x-ray was poor at detecting CCAM with only 61% sensitivity. Computed tomography scan was 100% sensitive with no false-positive diagnoses. Of the 19 cases since 2003, 13 cases were asymptomatic and had elective surgery. Eight had surgery at 6 months of age or older, and of these, 4 (50%) had evidence of chronic inflammation/infection on histology including 1 case with multiple abscesses found at surgery and 1 case with multiple adhesions. Five cases had surgery at less than 6 months of age and only 1 of these, aged 5.5 months, had evidence of infection present at histology.

CONCLUSIONS: All cases of antenatally suspected CCAM should be followed up postnatally and investigated with computed tomography scan. Elective surgery in asymptomatic cases, at around 3 to 6 months of age, is recommended for persistent lesions because of the risk of long-term complications.