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Management and outcome of antenatally diagnosed
congenital cystic adenomatoid malformation of the
lung
PC Chow, SL Lee, MHY Tang, KL Chan, CP Lee, BCC Lam, NS Tsoi
Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, University of Hong Kong, Pokfulam Road, Hong Kong
OBJECTIVE. To review the management and outcome of babies with antenatally
diagnosed congenital cystic adenomatoid malformation.
DESIGN. Retrospective cohort review.
SETTING. Tertiary neonatal care unit at Queen Mary Hospital and antenatal
diagnostic centre at Tsan Yuk Hospital.
PATIENTS. Consecutive patients with antenatally suspected congenital cystic
adenomatoid malformation in their concepti among antenatal
patients attending Tsan Yuk Hospital from 1994 to 2002. Twentyfour
of 33 cases were referred to Queen Mary Hospital for postnatal
management and for whom comprehensive records were available
for analysis in 23.
INTERVENTIONS. Postnatal interventions in their babies included investigational
imaging for congenital cystic adenomatoid malformation and
surgery.
MAIN OUTCOME MEASURES. Antenatal and postnatal outcome, as well as pathology of the
excised lesions.
RESULTS. Antenatal outcome: termination of pregnancy in two cases and
spontaneous abortion in one; in-utero regression was documented
in nine cases and in one hydropic change was apparent. Postnatal
outcome: only eight of 20 babies born alive had symptoms in
neonatal period. Two developed serious infective complications
in infancy, one with documented in-utero regression. Pulmonary
parenchymal abnormalities were detected on computed tomography
of the thorax in six of seven cases with normal or non-specific chest
radiograph findings. Among nine cases with in-utero regression,
congenital cystic adenomatoid malformation was confirmed by
operative histology in five and abnormal computed tomography
findings in three. Fifteen babies underwent surgical excision,
one of whom died because of severe pre-existing pulmonary
hypoplasia and nine endured minor postoperative complications.
A favourable outcome was documented at a mean follow-up of 22
months (range, 2 months-7 years).
CONCLUSIONS. In-utero regression of congenital cystic adenomatoid malformation
on antenatal ultrasound may not represent genuine resolution.
Computed tomographic thorax should be considered in all
newborns with antenatally diagnosed congenital cystic adenomatoid
malformation, and if confirmed early operation before first hospital
discharge is recommended.
Hong Kong Med J 2007;13:31-9
Key words: Cystic adenomatoid malformation of lung, congenital; Prenatal diagnosis; Treatment outcome
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