Background: The clinical spectrum of congenital cystic adenomatoid malformation of the lung (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 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.