Congenital bronchopulmonary malformation: CT histopathological correlation

Abstract

Background. This study evaluated the accuracy of postnatal computed tomography (CT) imaging in the identification of congenital bronchopulmonary malformation (BPM) in comparison with histopathological analysis. Methods. CT scans of prenatally diagnosed BPMs from 24 patients with available histology were analysed retrospectively. The CT images were reviewed blinded to histological findings by two radiologists. Specific diagnosis was assigned based on predetermined criteria. The accuracy of CT was evaluated. Results. The agreement rate in CT diagnosis between two radiologists was 100%. In 75% the lesions were located in the lower lobes. An overlap of 71% in CT and histopathological diagnoses was reached. The least matching diagnosis was type 2 CPAM. Conclusion. Contrast enhanced chest CT is very accurate in characterizing the BPM spectrum and provides important information on lesion type and structure.

Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature

“…Etiology of CPAMs. The etiology of CPAMs involves disruption of the normal airway patterning during the course of lung development [ 4 , 9 ] most likely occurring in the pseudo-glandular stage [ 11 , 13 ]. During lung morphogenesis, which begins as early as 3 weeks’ gestation and continues postnatally, airway branching involves extensive signaling crosstalk between respiratory epithelium and the surrounding mesenchyme [ 13 ].…”

“…Radiographic findings of CPAMs. When present, CPAM is commonly diagnosed on prenatal ultrasound between 18 and 20 weeks of gestation [ 4 , 6 – 9 ]. Prenatal ultrasound findings typically include a heterogenous mass with a single or multiple hyperechoic cystic structure(s) [ 4 , 11 , 16 ].…”

“…Initial steps in postnatal management of CPAMs often involve further characterization of the mass, as this is often not reliable on prenatal imaging. Postnatal CT remains the gold standard for confirming diagnosis, with varied but overall good concordance of radiographic diagnosis and histopathologic diagnosis in multiple studies [ 4 , 9 , 10 ]. Additionally, CT with contrast allows for the definitive delineation of vascular supply to the lesion and whether that supply originates from the pulmonary or the systemic circulation [ 4 , 10 , 16 ].…”

“…In general, CPAMs, as well as other congenital lung malformations, are isolated anomalies that are diagnosed on routine prenatal ultrasound in up to 70% of cases [ 5 ], typically between 18 and 20 weeks gestational age, but are often difficult to characterize fully in the fetal period [ 4 , 6 – 9 ]. Fetal magnetic resonance imaging (MRI) has shown promise as a prenatal imaging modality frequently consistent with postnatal pathological diagnosis [ 2 , 10 ].…”

Uniportal Thoracoscopic Lobectomy for Congenital Lung Malformations in Children

“…Up to 26% of prenatally diagnosed lesions can have occult infection, cell dysplasia or even tumour [26]. In addition, few congenital lung anomalies other than CPAM may have similar appearance in prenatal or postnatal images, which will lead to different disease progression [27][28][29]. No single reliable indicator was found, that is, which asymptomatic patient would remain asymptomatic throughout his/her life span and which patient would develop symptoms in the watchful waiting period.…”