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Congenital cystic adenomatoid malformation of the lung - the experience of five medical centres
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Advisor(s)
Abstract(s)
Background: The clinical spectrum of congenital
cystic adenomatoid malformation of the lung to the neonatal intensive care units of five tertiary
medical centres in the north of Portugal between
1996 and 2005.
Results: Fifteen neonates with CCAML were identified,
9F/6M, birth weight 3100 g (645-3975), gestational
age 38 weeks (24-40). The incidence of
CCAML was 1: 9300 births. There were 11 (73%)
cases of cystic lung lesion diagnosed during pregnancy,
median age 22 weeks (19-30). The lesion was
right sided in six (40%) and left sided in nine (60%)
cases. In utero spontaneous regression of the lesion
was observed in two cases. Antenatal intervention
(pleurocentesis and thoracoamniotic shunting) was
performed in one foetus with impending hydrops.
Normal lung radiographic findings at birth were
present in five cases, with an abnormal CT scan.
Three (20%) neonates became symptomatic during
the neonatal period (respiratory distress) and one
(70%) after the neonatal period (spontaneous pneumothorax).
Two neonates (13%) died. Six (40%) patients
underwent thoracotomy and appropriate excisional
surgery. Histological examination showed
definitive features of CCAML (Stocker classification:
type I = 4; type II = 1; type III = 2). Eight
(53%) patients remain asymptomatic and did not
undergo surgery.
Conclusions: Antenatally diagnosed CCAML has a
good prognosis in the absence of severe foetal distress;
normal radiographic findings at birth do not rule
out CCAML; treatment of asymptomatic CCAML is
controversial; surgery may be advocated because of
its low morbidity and the prevention of late complications
such as malignancy.
(CCAML) ranges from asymptomatic lesions to neonatal
respiratory distress and hydrops fetalis.
Aim: To review our experience with CCAML, emphasising
natural history, management and outcome.
Material and methods: A retrospective chart review
of all CCAML-diagnosed neonates admitted
Description
Keywords
Congénito Congenital malformação adenomatóide cística cystic adenomatoid malformation
Citation
Rev Port Pneumol. 2007 Jul‐Aug;13(4):511‐23. English, Portuguese. PMID: 17898911
Publisher
Sociedade Portuguesa de Pneumologia