Long-term outcome in patients with prenatally diagnosed cystic lung disease: special reference to ventilation and perfusion scan in the affected lung.
OBJECTIVE
The natural history of cystic lung disease (CLD) such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration has been altered by the advent of prenatal diagnosis. Although recent advances including fetal therapy have gradually improved outcome, the long-term course and the function of the residual lung have not been well clarified.


METHODS
Twenty-two patients with CLD who had been prenatally diagnosed and treated between 1990 and 2004 were reviewed. The clinical outcome and growth measurements were established, and, where possible, all infants underwent ventilation and perfusion lung scan.


RESULTS
Mediastinal shift was present in 14 fetuses. Fetal hydrops was present in 5 fetuses. Antenatal intervention was performed for hydrops in 2 fetuses (cyst-amniotic shunt and aspiration). Twenty-one infants underwent appropriate excisional surgery. Final diagnosis included CCAM (n = 12) and pulmonary sequestration (n = 7). No late death was observed. Common complications were failure to thrive (n = 5), frequent respiratory tract infection (n = 4), and asthmatic attack (n = 4). A significant decrease in lung ventilation and perfusion on the affected side was observed in patients with hydrops, lobectomy, and CCAM.


CONCLUSIONS
Long-term follow-up including respiratory care and growth assessment should be performed in prenatally diagnosed patients with CLD, especially those who present with hydrops.
Kamata
Shinkichi
S
Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan. kamata@pedsurg.med.osaka-u.ac.jp
Usui
Noriaki
N
Kamiyama
Masahumi
M
Nose
Keisuke
K
Sawai
Toshio
T
Fukuzawa
Masahiro
M
D001998:Bronchopulmonary Sequestration / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D006801:Humans; D011247:Pregnancy; D011296:Prenatal Diagnosis; D011877:Radionuclide Imaging; D016896:Treatment Outcome