Interstitial Lung Disease in Infants

Interstitial Lung Disease (ILD) in Infancy is rare and complex with distinct causes and differential diagnoses that are different than in older children or adults. Imaging is an essential part of the workup and diagnosis of children with suspected ILD. Chest XR and especially high resolution chest CT are the main imaging modalities

 Classification:

1. Diffuse developmental disorders

2. Alveolar growth abnormalities

3. Surfactant dysfunction disorders and related abnormalities

4. Specific conditions of unknown etiology

Rule out other causes of diffuse lung disease in infancy

  • Cystic fibrosis
  • Early presentation of primary ciliary dyskinesia
  • Congenital or acquired immunodeficiency
  • Congenital heart disease
  • Pulmonary infection
  • Recurrent Aspiration
  • Mimics of ILD e.g. lymphatic disorders, cardiac congestion, pulmonary hypertension, veno-occlusive disease
Diffuse Developmental Disorders
 
Include:
 
  • Acinar dysplasia
  • Congenital alveolar dysplasia
  • Alveolar capillary dysplasia with misalignment of pulmonary veins
diff

 

Congenital Alveolar Dysplasia

Full term newborn infant with respiratory distress

  • Increased lung volumes
  • Diffuse hazy opacity

 

 

 
Alveolar Growth Abnormalities
 
Include:
 
  • Chronic lung disease of prematurity
  • Pulmonary hypoplasia from in utero lung compression ( e.g. oligohydramnios, congenital diaphragmatic hernia)
  • Chromosome anomalies ( e.g. Down S, Filamin A mutation)
  • Congenital cardiovascular anomalies
ab1

Chronic lung disease of prematurity
5 month old 28 week gestation infant
ab2

CT at 5 months

  • Defective alveolarization , lobular simplification & decreased vascularization with  focal airspace enlargement.
  • Interspersed areas of scarring & fibroproliferation.
ab3

CT at 10 months

  • Evolution to emphysema  with less reticular change
  • Bibasilar atelectasis

Alveolar Growth Abnormalities: Subpleural Cysts in Down Syndrome

Example: 18 Month Male Trisomy 21 

  • Mild Respiratory Distress
  • Peripheral lung cysts
  • Focal areas of lung overinflatio
  • DS
  • 1-2mm subpleural, fissural and peribronchovascular cysts
  • 1/3 of patients with Down syndrome
  • Pulmonary growth disorder with pulmonary hypoplasia/dysplasia, defective alveolarization

Specific Conditions of Undentifed Etiology

Include:

  • Neurendocrine cell hyperplasia (NEHI)
  • Pulmonary interstitial  glycogenosis (PIG)

sp1

6mo F persistent respiratory distress Chest X Ray

  • Hyperinflated lungs
  • Hazy perihilar opacity

2

3

CT 6mos

  • General overinflation
  • Perihilar,RML and lingula ground glass opacity
  • C/W NEHI