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

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

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 1Balaganesh Karmegaraj 2C Vidya 3Vivek Krishnan 1Affiliations expand

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.

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