Congenital pulmonary lymphangiectasia

Case contributed by Dr Lee-Anne Slater

Presentation

Persistent pulmonary hypertension

Patient Data

Age: 4 weeks
Gender: Female
  • Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

Diffusely increased reticular opacities.

Thickening of the interlobular septa with demarcation of the secondary pulmonary lobules. There is also bibasal consolidation and pleural effusions.

CT chest on mediastinal windows demonstrates narrowing of the left pulmonary vein.

Case Discussion

This 4 week old girl presented with persistent pulmonary hypertension. She decompensated acutely due to influenza A infection.

Once the infection resolved there was persistent interlobular septal thickening consistent with an interstitial process. There were also persistent pleural effusions. These features were suggestive of lymphangiectasia.

Wedge biopsy confirmed lymphangiectasia. The effusions were not chylous and this was thought to be because in this child the lymphangiectasia was secondary to obstructed pulmonary venous return.

The narrowing of the left pulmonary vein may be part of this same process.

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