The radiology of diffuse interstitial pulmonary disease in children: pearls, pitfalls and new kids on the block in 2015

Abstract

Diffuse interstitial lung disease in children differs markedly from interstitial lung disease in adults and is a distinct entity. The childhood interstitial lung disease (ChILD) classification, devised in 2010 separates conditions into those occurring in infancy, and those not specific to infants, the later group containing many conditions related to systemic diseases (including connective tissue diseases and depositional/storage disorders), and conditions occurring in immunocompromised children. In this article, we briefly review normal lung growth and development. We discuss our preferred technique for imaging the lungs with computed tomography in children, and review the recent literature regarding the radiological appearance of various ChILD. We illustrate this with cases from our institution and emphasize the more recently recognised conditions including pleuroparenchymal fibroelastosis and filamin A deficiency-related lung disease.

Keywords: Childhood interstitial lung disease; Diffuse lung disease; Filamin A deficiency-related lung disease; Paediatric CT; Pleuroparenchymal fibroelastosis.