Congenital interstitial lung diseases: What the anesthesiologist needs to know

Paediatr Anaesth. 2022 Feb;32(2):138-147. doi: 10.1111/pan.14325. Epub 2021 Nov 11.


Congenital interstitial lung diseases can affect both adults and children. Pediatric congenital interstitial lung diseases generally carry high risk for morbidly and mortality and include congenital alveolar capillary dysplasia with misalignment of pulmonary veins, congenital alveolar dysplasia, acinar dysplasia, congenital pulmonary lymphangiectasis, diffuse pulmonary lymphangiomatosis, neuroendocrine cell hyperplasia of infancy, pulmonary hemosiderosis, pulmonary alveolar proteinosis, and pulmonary interstitial glycogenosis. Given their usual non-specific clinical presentation, they are frequently misdiagnosed and recognized late, particularly in children who have been apparently healthy for several years (eg, diffuse pulmonary lymphangiomatosis). Some diseases have a very poor prognosis, whereas others have a benign course with appropriate treatment. The current manuscript reviews congenital interstitial lung diseases that typically affect neonates and young children and may be encountered by the pediatric anesthesiologist.

Keywords: children's interstitial lung diseases; hypoxemia; interstitial lung diseases; lung biopsy; pulmonary hypertension.

Publication types

  • Review

MeSH terms

  • Adult
  • Anesthesiologists
  • Child
  • Child, Preschool
  • Humans
  • Infant, Newborn
  • Lung
  • Lung Diseases*
  • Lung Diseases, Interstitial* / diagnosis
  • Lung Diseases, Interstitial* / therapy
  • Lymphangiectasis*
  • Persistent Fetal Circulation Syndrome*
  • Pulmonary Alveoli