Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2023 Jul;39(4):417-420.
doi: 10.1007/s12055-023-01510-x. Epub 2023 May 3.

Congenital pulmonary airway malformation manifesting with severe respiratory distress and pneumothorax in a neonate

Nisha Rana  1 , Jagjit Singh Dalal  1 , Shamsher Singh Lohchab  2 , Sandeep Singh  2 , Basant Kumar  1 , Sunita Singh  3
Affiliations
Case Reports

Congenital pulmonary airway malformation manifesting with severe respiratory distress and pneumothorax in a neonate

Nisha Rana et al. Indian J Thorac Cardiovasc Surg. 2023 Jul.

Abstract

Congenital pulmonary airway malformation (CPAM) is congenital pulmonary anomaly characterized by multicystic areas, over-distension, and proliferation of terminal bronchioles with lack of normal alveoli. Clinical presentation may vary from mild respiratory symptoms to severe respiratory distress and frequent pneumothoraxes. We report a rare case of neonatal CPAM type I manifested with neonatal respiratory distress and pneumothorax, which was managed successfully with left lower lobectomy.

Supplementary information: The online version contains supplementary material available at 10.1007/s12055-023-01510-x.

Keywords: Congenital lung malformation; Congenital pulmonary airway malformation; Neonates and respiratory distress; Pneumothorax.

PubMed Disclaimer

Conflict of interest statement

Conflict of interestNone.

Figures

Fig. 1
Fig. 1
A Chest radiograph anteroposterior (AP view) showing the visceral pleural line and pneumothorax on the left side with contralateral mediastinal shift and passive atelectasis. B CECT thorax with axial and C reformatted minimum intensity projection (MinIP) images showing hyperinflation of the left lower lobe, mediastinal shift to the right, and passive atelectasis of the right lung parenchyma
Fig. 2
Fig. 2
A The large cystic lesion in the left lung seen through thoracotomy incision. B Hematoxylin and eosin (H&E)–stained section showing a cyst with the lung tissue (200 ×). The cyst is lined by pseudostratified ciliated columnar epithelium (red arrow). C Post-lobectomy chest X-ray showing resolution
See this image and copyright information in PMC

References

    1. Durell J, Lakhoo K. Congenital cystic lesions of the lung. Early Hum Dev. 2014;90:935–939. doi: 10.1016/j.earlhumdev.2014.09.014. - DOI - PubMed
    1. Stocker JT. Congenital pulmonary airway malformation: a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung. Symposium 24: non-neoplastic lung disease. Histopathology. 2002;41:424–30.
    1. Dilorenzo G, Salinaro E, Favia V, Pavone S, Drimaco P, Laforgia N, et al. Pleural loculated empyema masking a CPAM 3 in a newborn infant: a case report with brief literature review. Respir Med Case Rep. 2018;25:274–9. - PMC - PubMed
    1. Hellmund A, Berg C, Geipel A, Bludau M, Heydweiller A, Bachour H, et al. Prenatal diagnosis and evaluation of sonographic predictors for intervention and adverse outcome in congenital pulmonary airway malformation. PloS One. 2016;11:e0150474. - PMC - PubMed
    1. Costanzo S, Filisetti C, Vella C, Rustico M, Fontana P, Lista G, et al. Pulmonary malformations: predictors of neonatal respiratory distress and early surgery. J Neonatal Surg. 2016;5:27. doi: 10.21699/jns.v5i3.375. - DOI - PMC - PubMed

Publication types

LinkOut - more resources