A clinical guide in the structured assessment of CT-imaging in congenital lung abnormalities

Introduction: While a distinct pathological description exists, standardized guidelines on the description of CT-features of congenital lung abnormalities(CLA) is lacking even though it is the golden standard in pre-operative imaging and diagnosis. Aims and Objectives: To develop a clinical guide for the structured assessment and uniform reporting of anomalies on CT-scan in patients with CLA. Methods: A systematic literature search was conducted for relevant articles on CLA including congenital pulmonary airway malformation(CPAM), bronchopulmonary sequestration(BPS), congenital lobar emphysema(CLE) and bronchogenic cyst(BC). Paediatric patient cohorts and reports describing CLA features exceeding 5 cases were included. Results: Of 1581 articles identified, 28 retrospective cohort-studies remained after assessing full-texts by two independent reviewers. Features exclusively seen in one diagnosis are anomalous arteries in BPS and contralateral herniation and vessel attenuation in CLE. Air-containing cysts are found in CPAM as well as BC. Soft tissue masses are seen in both CPAM and BPS; anomalous arterial blood supply in BPS can help distinguish between both. Perilesional low-attenuation, atelectasis and mediastinal shift can be found in both aforementioned abnormalities as well as CLE. Conclusions: Nomenclature used to describe CT-imaging findings in CLA are highly variable, and due to overlap between and within abnormalities, diagnostic features are difficult to standardize. Based on the literature and on consensus between experts we created a clinical guide to systematically assess CLA using objective radiologic features and uniform nomenclature.