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

Authors

  • Jayashree V. Kanavi Department of Obstetrics and Gynecology, St Johns Medical College and Hospital, Bangalore, Karnataka, India
  • Jasmine Thangaraj Department of Obstetrics and Gynecology, St Johns Medical College and Hospital, Bangalore, Karnataka, India
  • Annamma Thomas Department of Obstetrics and Gynecology, St Johns Medical College and Hospital, Bangalore, Karnataka, India

DOI:

https://doi.org/10.18203/2320-1770.ijrcog20172329

Keywords:

CCAM, CTM, Non-immune hydrops

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 Post-natal 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.

References

Stocker JT, Madewell JE and Drake RM. Congenital cystic adenomatoid malformation of the lung classification and morphologic spectrum. Hum Pathol. 1977;8:155-71.

Chin KY and Tang MG. Congenital cystic adenomatoid malformation of one lobe of the lung with general anasarca. Arch Pathol. 1949:48:222-9.

Calvert JK and Calchoo K. Antenatally suspected congenital cystic adenomatoid malformation of the lung. Post natal investigation and timing of surgery. J Pediatr Surg. 2007;42(2):411-4.

Ben- Ishay O, Nicksa GA, Wilson JM, Buchmiller TL; Management of giant congenital pulmonary airway malformations requiring pneumonectomy. Ann Thaorac Surg. 2012;94(4):1073-8.

Adzick NS, Michael R, Harrison M, Timothy M. Foetal lung lesions; Management and outcome. Am J Obstet Gynecol.1998;179(4):884-9.

Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C et al: Antenatal and postnatal management of congenital cystic adenomatoid malformation. Paediatr Respir Rev. 2012;13(3):162-71.

Vu L, Tsao K, Lee M. Characteristics of congenital cystic adenomatoid malformations associated with non immune hydrops and outcome. J Pediatr Surg. 2007;42:1351-6.

David M, Lamas Pinheiro R, Henriques Coello T. Prenatal and Postnatal management of congenital pulmonary airway malformation. Neonatalogy. 2016; 110(2):101-15.

Crombleholme TM, Coleman B, Hedrick H, Leichty K. Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Paediatr Surg. 2002;37:331-8.

Azizkhan RG and Crombleholme TM. Congenital cystic lung disease: Contemporary antenatal and post -natal management. Pediatr Surg Int. 2008;24:643-57.

Downloads

Published

2017-05-25

Issue

Section

Original Research Articles