CT and histopathological correlation of congenital cystic pulmonary lesions: a common pathogenesis?
Introduction
Congenital cystic lesions of the lung comprise a heterogeneous group of bronchopulmonary malformations including congenital cystic adenomatoid malformations (CCAMs), sequestrations, bronchial atresia and bronchogenic cysts. Many lesions are now detected antenatally by prenatal ultrasound, but they can also present in the postnatal period, and more rarely, in later life.
A pathology-based classification system has evolved1 with controversy as to the degree of overlap between entities as, for example, some sequestrations demonstrate histological features of type 2 CCAMs.2, 3 It has been suggested that a common pathogenetic mechanism is responsible for the development of many of these lesions.1 This has led to others proposing a more encompassing nomenclature.4
There have been few published series on computed tomography (CT) features of congenital cystic lesions, primarily limited to CCAMs.5, 6 Therefore, the aims of this study were to use CT–histopathology correlation to assess the degree of overlap between proposed entities, and to search for CT features that might distinguish such entities preoperatively and, therefore, potentially improve patient management.
Section snippets
Patient selection
Patients who had undergone surgical resection for the final histological diagnosis of congenital cystic lung lesions between 1999 and 2006 were identified from the histopathology database (n = 42). The subset of patients (n = 24) who had also undergone CT of the thorax at our institution prior to surgical resection then formed the basis of the study. CT studies were performed between March 1997 and March 2006. Ethical approval was obtained from the local ethics committee.
Image acquisition
CT studies were obtained on
Results
There were 24 patients (16 male) presenting with congenital cystic lung lesions with a median age of 3 years (interquartile range 2.1–9.5 years) at the time of surgical resection. The median interval between CT study and surgical resection was 79.5 days (interquartile range 20–319 days). Clinical and surgical data for a proportion of these lesions have been published previously.9, 10, 11 Overall, there were five type 1 CCAM, six type 2 CCAM, one type 4 CCAM, one bronchial atresia, four type 1
Discussion
This study documents CT and histological features of patients presenting with congenital cystic lung lesions over a 9-year period at a tertiary referral centre, and correlates these data with the aims of assessing their inter-relationships. The data show that there is an overlap in the CT and histopathological features in certain non-neoplastic congenital cystic lesions of the lung (type 1 and type 2 CCAMs, sequestrations and bronchial atresia). Type 1 and type 2 CCAMs were primarily
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2020, Journal of the American College of RadiologyCitation Excerpt :Similar to CT chest without IV contrast, CT chest with IV contrast can be used to diagnose anatomical abnormalities in the immunocompetent patient that could predispose children to recurrent localized infections like congenital lobar overinflation [54], foreign bodies that could cause recurrent postobstructive pneumonia [13], and underlying pulmonary disease like bronchopulmonary dysplasia [52]. CT chest with IV contrast provides benefit over CT chest without IV contrast for diagnosing conditions such as bronchial tumors, congenital pulmonary airway malformation [54-58], pulmonary sequestration [21,54,55,59-63], and bronchopulmonary foregut malformations [54,55]. CTA chest with IV contrast is especially helpful for presurgical planning, identifying feeding and draining vessels in patients with suspected pulmonary sequestration, and assessing for a vascular ring that may lead to tracheal narrowing [59,63].
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