Current Update on Interstitial Lung Disease of Infancy: New Classification System, Diagnostic Evaluation, Imaging Algorithms, Imaging Findings, and Prognosis

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Key points

  • Childhood interstitial lung disease (chILD) encompasses a spectrum of diffuse childhood lung diseases presenting with at least 3 of 4 criteria: (1) respiratory symptoms, (2) hypoxemia, (3) respiratory signs, (4) diffuse abnormalities on chest radiographs or computed tomography (CT).

  • Imaging in chILD primarily relies on CT with whole lung volumetric CT for the initial evaluation and with high-resolution CT for the follow-up evaluation.

  • Imaging findings of chILD are typically nonspecific for a

Clinical presentation

The most consistent clinical presentation in those affected by chILD is tachypnea, which occurs in 75% to 93% of cases.9, 10, 11, 12 Cough, failure to thrive, and/or crackles are also common clinical presentations.8, 10, 12 However, affected patients may present only with mild wheezing or have normal breath sounds. Profound respiratory failure may occur in the neonatal period, whereas a more insidious course can be seen in older children presenting with failure to thrive or intolerance to

Nonimaging Evaluation

Nonradiologic diagnostic testing in chILD includes echocardiography, pulmonary function tests (PFTs), genetic testing, bronchoscopy with bronchoalveolar lavage, and lung biopsy.

The utility of echocardiography is twofold. First, both congenital heart disease and pulmonary vascular disease can coexist with or mimic chILD.6 Second, in patients with isolated chILD, echocardiography can help in the evaluation of concurrent pulmonary hypertension, a finding that is associated with a worse prognosis.9

Spectrum of imaging findings

Communication of imaging findings in chILD should closely follow the standard lexicon as put forth by the Fleischner Society in their glossary of terms for thoracic imaging.30 Using this lexicon, the predominant findings in chILD consist of varying combinations of air-trapping, ground-glass opacities, consolidations, nodules, cysts, and linear/reticular opacities.2 As can be ascertained, these imaging findings are nonspecific and can be seen in a host of infectious, inflammatory, genetic,

Summary

Despite their rarity, childhood interstitial lung diseases are an important cause of morbidity and mortality in infants and young children. Many have nonspecific imaging findings, and, thus, a definitive diagnosis cannot be made on imaging alone. For the interpreting radiologist, obtaining appropriate and adequate imaging is paramount. Furthermore, familiarity with these diseases and their classification is important, as the treating physician may be unfamiliar, or at least not considering,

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