The unpredictable character of congenital cystic lung lesions.
BACKGROUND
The spectrum of congenital cystic disease of the lung ranges from hydrops and neonatal respiratory distress to asymptomatic lesions. Surgical management is dictated by the presence of symptoms, recurrent infection, and the potential risk of malignant transformation.


METHODS
Since 1995, all consecutive patients with congenital cystic lung lesions underwent follow-up for symptoms, treatment, and correlation of presumptive with pathological diagnosis.


RESULTS
Twelve cystic lung lesions were identified. Seven were diagnosed with mediastinal shift in utero; in 6 of 7, the shift subsequently resolved. Overall, 6 of 7 lesions that were followed up serially decreased in size. Two patients were symptomatic in utero; 1 underwent thoracoamniotic shunting, 1 pleurocentesis for impending hydrops. Postnatally, these 2, and 2 other newborns required urgent surgery. Five of 8 asymptomatic patients had elective resection by 16 months, and 4 await operation. In 6 of the 9 surgical cases (67%), there was a discrepancy between preoperative and pathological diagnosis. There were 4 hybrid congenital cystic adenomatoid malformation (CCAM)/sequestrations.


CONCLUSIONS
At least 6 of 7 congenital cystic lung lesions decreased in size regardless of gestational age or presence of mediastinal shift. Antenatal intervention is therefore rarely indicated. Hybrid morphology may necessitate resection of stable, asymptomatic lesions to prevent the theoretical concern for associated malignancies as well as other complications of CCAM.
Roggin
K K
KK
Division of Pediatric Surgery, Brown University School of Medicine and Hasbro Children's Hospital, Providence, RI 02905, USA.
Breuer
C K
CK
Carr
S R
SR
Hansen
K
K
Kurkchubasche
A G
AG
Wesselhoeft
C W
CW
Tracy
T F
TF
Luks
F I
FI
D000328:Adult; D001998:Bronchopulmonary Sequestration / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011013:Pneumonectomy; D011247:Pregnancy; D011256:Pregnancy Outcome; D011296:Prenatal Diagnosis / Q000379:methods*; D011446:Prospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal