Interstitial Lung Disease in Children Made Easier…Well, Almost

Abstract

Interstitial lung disease (ILD) in pediatric patients is different from that in adults, with a vast array of pathologic conditions unique to childhood, varied modes of presentation, and a different range of radiologic appearances. Although rare, childhood ILD (chILD) is associated with significant morbidity and mortality, most notably in conditions of disordered surfactant function, with respiratory failure in 100% of neonates with surfactant protein B dysfunction and 100% mortality without lung transplantation. The authors present a summary of lung development and anatomy, followed by an organized approach, using the structure and nomenclature of the 2013 update to the chILD Research Network classification system, to aid radiologic diagnosis of chILD. Index radiologic cases with contemporaneous histopathologic findings illustrate a summary of recent imaging studies covering the full spectrum of chILD. chILD is best grouped by age at presentation from infancy (diffuse developmental disorders, lung growth abnormalities, specific conditions of unknown origin, surfactant dysfunction mutations) to later childhood (disorders of the normal host, disorders related to systemic disease processes, disorders related to immunocompromise). Appreciation of the temporal division of chILD into infant and later childhood onset, along with a sound understanding of pulmonary organogenesis and surfactant homeostasis, will aid in providing useful insight into this important group of pediatric conditions. Application of secondary lobular anatomy to interpretation of thin-section computed tomographic images is pivotal to understanding patterns of ILD and will aid in selecting and narrowing a differential diagnosis. RSNA, 2017.

Congenital emphysematous lung disease associated with a novel Filamin A mutation. Case report and literature review

“…The pulmonary growth abnormality associated with FLNA deficiency consists of multilobar overinflation predominantly affecting the upper and lower lobes, with coarse septal thickening and varying lower lobe atelectasis with pruning of the peripheral pulmonary vasculature [27]. The role of FLNA in the development of lung disease is still not well elucidated.…”

Neurofibromatosis type‐1‐associated diffuse lung disease in children

“…In a large series of lung biopsies, it has been demonstrated that in children, 2 to 18 years of age, the pathologic spectrum of DLD overlaps with that seen in adults with some exceptions, but differs from that detected in infants . The disorders of the later childhood are subdivided into disorders of the normal host, disorders related to systemic disease processes, and disorders related to immunodeficiency …”

“…9 The disorders of the later childhood are subdivided into disorders of the normal host, disorders related to systemic disease processes, and disorders related to immunodeficiency. 10 Among disorders related to systemic diseases, NF-1 lung involvement has never been investigated in children.…”

The Common among the Rare: A Pictorial Essay of the Most Common Diffuse Lung Diseases in Children

“…Chest radiograph, albeit being the starting point, is highly non-specific with a limited sensitivity. CT is the gold standard in the work-up of interstitial lung disease in pediatric patients, since it provides information about the extent and distribution of parenchymal disorders (pattern recognition), contributing in estimating the severity of the disease, the response to treatment and the prognosis, as well as indicating a site for biopsy [2,3,10]. Occasionally, abnormalities affecting other systems (eg, cardiovascular) may be diagnosed.…”

“…Spontaneous pneumothorax is a potential complication. An enlarged thymus may also be seen with intrathymic calcification-cavitation being characteristic of the disease, rendering lung biopsy unnecessary (Figure 9) [7,10,13].…”