J Med Ultrasound

. 2021 May 4;29(4):284-287. doi: 10.4103/JMU.JMU_139_20. eCollection Oct-Dec 2021. https://pubmed.ncbi.nlm.nih.gov/35127410/

Atypical Antenatal Presentation of an Unusual Nonmucinous Papillary Variant of Giant Congenital Pulmonary Airway Malformation Masquerading as Congenital Diaphragmatic Hernia with Volvulus

Basil Mathews ¹, Balaganesh Karmegaraj ², C Vidya ³, Vivek Krishnan ¹Affiliations expand

• PMID: 35127410
• PMCID: PMC8772466
• DOI: 10.4103/JMU.JMU_139_20

Free PMC article

Abstract

We report a case of a huge congenital pulmonary airway malformation (CPAM) that was referred as congenital diaphragmatic hernia (CDH). Initial ultrasound evaluation revealed a huge cystic lesion with septations, in the thorax, causing mediastinal shift and compression effects, suggesting the possibility of a thoracic lymphangioma, or bowel herniation with obstruction. A fetal magnetic resonance imaging reported possible bowel herniation through a posterior defect in the diaphragm, with volvulus, reinforcing the diagnosis of CDH. It was only on autopsy and subsequent histopathology examination that the diagnosis of a rare variant of CPAM-nonmucinous papillary type, could be made. To the best of our knowledge, a CPAM this huge has not been reported prenatally at this gestation. We recommend considering the potential diagnosis of CPAM in any thoracic cystic irrespective of its size or appearance.

Keywords: Atypical presentation of congenital pulmonary airway malformation; congenital pulmonary airway malformation; nonmucinous papillary variant; type I congenital pulmonary airway malformation.

Copyright: © 2021 Journal of Medical Ultrasound.