Congenital cystic adenomatoid malformation (CCAM): antenatal and postnatal management

Abstract

Background: Congenital cystic adenomatoid malformation (CCAM) is a rare abnormality of lung development. The estimated incidence ranges from 1: 10,000 to 1.35,000 live births. It is diagnosed by prenatal screening at 18-20 weeks of gestation. Surgical excision of symptomatic lesions is relatively straight forward, but management of asymptomatic lesions is controversial. Methods: Among women who delivered at St. Johns medical college and hospital, Bangalore between Jan 2011 to Dec 2016, those with the diagnosis of CCAM during anomaly scan were included in the study. Antenatal and Postnatal period and their outcomes were evaluated. Follow up was extend up to the childhood in the affected foetus. Results: There were 5 cases of CCAM in 13057 deliveries during 5-year study. Incidence was 1:2611. Mean gestational age at diagnosis was 21.6±2.5weeks. All foetus had CVR (CCAM volume) ratio more than 1.6 and there was no compromise on lung volume. Mean lung volume was 62.8±8.6 cc. and mean Apgar score at 1minute was 6.8±2.7 and at 5 minutes was 8.0±2.2. Among 5 foetuses, 2 foetuses had regression of cyst by birth and 3 underwent surgery for resection after birth. Conclusions: CCAM remains a challenge for obstetricians, neonatologists and paediatric surgeons. The combination of prenatal MRI and serial ultrasound studies optimize foetal surveillance and postnatal care. In asymptomatic CCAM, babies should be followed up to adolescence and adulthood, as they can manifest with malignant changes.

Hybrid Pulmonary Sequestration, Cystic Pulmonary Adenomatoid Malformation, and Dextrocardia: A Triple Whammy

“…It can be detected by prenatal screening at 18–20 weeks of gestation. [ 5 ] CCAM is also categorized under many subtypes: Type 0, in which tumor develops from the trachea and this is the rarest type, Type 1 is the most common comprising 50%–70% of the total cases and develops from distal bronchus or proximal bronchioles and Type 2 arises from terminal bronchioles also represent a higher risk of associated anomalies. Type 3 generally represents acinar-like tissue and Type 4 presents as large cysts and have alveolar origin.…”

Tension Pneumothorax in 5-Months Old Infant - An Atypical Presentation of CPAM Type I

“…Tip I and IV are characterized with large cysts so it can be very difficult to distinguish them on antenatal ultrasound and even on postnatal CT scans, especially if a child develops pneumothorax or respiratory infection. Children with CPAM are born mostly as full-term neonates of regular birth weight [9]. Most babies are born asymptomatic, but about 30% of neonates with CPAM are at risk of acute respiratory failure at birth or show signs of respiratory distress in the neonatal period [10,11].…”

Malformação congênita das vias aéreas pulmonares: uma revisão bibliográfica

“…O prognóstico vai variar de acordo com o tamanho da massa pulmonar que pode causar complicações como o desvio no mediastino, hiperplasia pulmonar, polidrâmnio e o comprometimento cardiovascular que leva à hidropsia e morte (Domingues et al, 2020) (Martins, 2019). Além disso, pode causar complicações obstétricas como pré-eclâmpsia, polidrâmnio, maior predisposição à prematuridade e baixo peso ao nascer (Kanavi et al, 2017).…”

“…Por outro lado, as lesões podem diminuir e até mesmo desaparecer antes do parto (Kanavi et al, 2017). Sendo assim, é necessário que as lesões sejam avaliadas para que o médico determine se será necessário realizar alguma intervenção intraútero, ou se somente o acompanhamento ultrassonográfico com parto a termo e a ressecção da lesão no pós natal será necessário para o tratamento do neonato (Domingues et al, 2020).…”