Pulmonary imaging abnormalities in an adult case of congenital lobar emphysema

J Radiol Case Rep. 2015 Feb 28;9(2):9-15. doi: 10.3941/jrcr.v9i2.2048. eCollection 2015 Feb.

Abstract

Congenital lobar emphysema is mainly diagnosed in infants, although rare cases are reported in adults. A 20-yr-old female with acute dyspnea, chest pain and left upper lobe (LUL) chest x-ray hyperlucency underwent 3He magnetic resonance imaging (MRI) for ventilation and apparent diffusion coefficient (ADC) measurements, as well as CT for emphysema and airway wall measurements. Forced expiratory volume in 1s, residual volume, and airways-resistance were abnormal, but there was normal carbon-monoxide-diffusing-capacity. The LUL relative area of the density histogram <-950 HU and airway morphology were highly abnormal compared with the other lobes and coincident with highly abnormal MRI-derived acinar duct dimensions. CT also identified bronchial atresia and congenital lobar emphysema as the source of symptoms in this case where there was also functional imaging evidence of collateral ventilation from the fissure (and not the abnormally terminated airway) into the emphysematous LUL.

Keywords: Airways Disease; Bronchial Atresia; Computed Tomography; Congenital Lobar Emphysema; Emphysema; Hyperpolarized 3He; Magnetic Resonance Imaging.

Publication types

  • Case Reports

MeSH terms

  • Bronchi / abnormalities*
  • Bronchography
  • Female
  • Humans
  • Lung / diagnostic imaging*
  • Lung / pathology
  • Magnetic Resonance Imaging / methods
  • Pulmonary Emphysema / congenital*
  • Pulmonary Emphysema / diagnostic imaging
  • Pulmonary Emphysema / pathology
  • Respiratory Function Tests
  • Young Adult

Supplementary concepts

  • Congenital lobar emphysema