Congenital cystic adenomatoid malformation of the lung – The experience of five medical centres

Abstract

ResumoIntrodução: O espectro clínico da malformação adenomatóide cística pulmonar congénita (MACPC) varia desde lesões assintomáticas e dificuldade respiratória no recém-nascido a hidrópsia fetal. Objectivos: Avaliação da casuística de MACPC, realçando a história natural, tratamento e evolução. Material e métodos: Análise retrospectiva dos processos clínicos de todos os recém-nascidos com o diagnóstico de MACPC, admitidos nas unidades de cui- Abstract 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

Antenatal and postnatal management of congenital cystic adenomatoid lung malformation diagnosed by ultrasound and Magnetic Resonance Imaging (MRI)

“…CCAM is the most common (77%) of congenital cystic lung diseases, followed by pulmonary sequestration, bronchogenic cysts, and bronchial atresia [7][8][9]20]. The disease spectrum of CCAM is quite variable.…”

“…The disease spectrum of CCAM is quite variable. Lesions with spontaneous regression and, ultimately, complete disappearance during the third trimester have been reported; so have rapidly growing lung masses with nonimmune hydrops and in utero demise [7,16,20]. Close perinatal monitoring is essential to optimize outcome [7][8][9]16,[20][21][22].…”

“…Lesions with spontaneous regression and, ultimately, complete disappearance during the third trimester have been reported; so have rapidly growing lung masses with nonimmune hydrops and in utero demise [7,16,20]. Close perinatal monitoring is essential to optimize outcome [7][8][9]16,[20][21][22]. Serial antenatal US studies have been the imaging gold standard to characterize the features of CCAM, including the size of lesions, their appearance (macro-vs micro-cystic), and their location (lobe involvement) [13][14][15][16]23].…”

“…Initially asymptomatic neonates with isolated CCAM, in the absence of hydrops, may develop, shortly after birth, recurrent respiratory tract infections (10%) [15,23]. In addition, pleuropulmonary blastoma is a risk in childhood, and bronchoalveolar carcinoma is a risk in adolescence [20,21]. In contrast, nonhydropic fetuses with isolated CCAM have little or no hypoplasia of the lungs and, therefore, a much better prognosis [26][27][28][29].…”

“…The youngest described patient with malignant malformation due to CCAM was 13 months old [30]. Elective surgery is usually well tolerated, the risk of infection is lower, and compensatory lung growth with normal long-term respiratory function has been observed [13,20]. Despite current limitations, prognostic information is necessary to provide parental counseling throughout the pregnancy and appropriate perinatal management [7,8,27,31].…”