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Imaging of Lady Windermere Syndrome (MAC infection) | Radiology Board Review Case

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CT imaging appearance of Pulmonary Mycobacterium Avian Complex (MAC) infection (Lady Windermere Syndrome) and approach to Imaging of bronchiectasis.

Etymology

What is Lady Windermere syndrome?

Lady Windermere syndrome is Mycobacterium avium complex (MAC) pulmonary infection limited to the right middle lobe or lingula, hypothesized secondary to voluntary suppression of cough, responsible for the inability to clear the secretions from the right middle lobe and lingula.

Who was Lady Windermere?

Lady Windermere was a character in Oscar Wilde’s play by the name Lady Windermere’s fan. In the play, Lady Windermere is a vivacious young woman, married only two years, who never coughs or displays any other signs of illness. Also, Victorian women presumably believed “ladies don’t spit,” and consequently might have been predisposed to develop lung infection.

Pathophysiology and imaging

  • Occurs in patients with a pre-existing pulmonary disease (COPD) or those with depressed immunity. 
  • Predilection for older women who deliberately suppress the cough reflex (Lady Windermere syndrome). 
  • Can cause right middle lobe syndrome – chronic right middle lobe collapse without obstructing lesion. 
  • Patients with MAC infection, unlike those with pulmonary tuberculosis, are not contagious.
  • Subtypes: 
    • Upper lobe fibrocavitary pattern/cavitary form (classic infection)
    • Nodular bronchiectatic form/bronchiectatic form (non-classic infection) – RML / Lingula
    • Mixed form
  • Hot tub lung – Imaging appearances can mimic a subacute hypersensitivity pneumonitis. 
Lady windermere syndrome radiology findings

Watch the video for the entire PACS based image set and case description.

Imaging of Bronciectasis

  • Pulmonary artery: Bronchus diameter ratio is reduced. Usually, the bronchi are smaller or equal in diameter to the pulmonary artery.
  • Bronchus visualized within 1 cm of the pleural surface.
  • Types of bronchiectasis (gradual progression in the following order):
    • Cylindrical
    • Varicose
    • Cystic
  • Signs in bronchiectasis:
    • Signet ring sign is a primary finding of bronchiectasis at CT and refers to the cross-section of a dilated air-filled bronchus adjacent to a smaller nodular opacity representing the accompanying pulmonary artery. 
    • Tram-track sign – cylindrical bronchiectasis.
    • Bunch of grapes sign – cystic bronchiectasis. 

Causes of bronchiectasis

Cause of bronchiectasisCommon location
Recurrent aspirationLower lung zone
Allergic bronchopulmonary aspergillosisUpper lung zone
Tuberculosis Upper lung zone
Chronic atypical mycobacterial infection / Lady Windermere syndromeRight middle lobe and lingula.
Cystic fibrosisUpper lung zone
Primary ciliary dyskinesia (Kartagener syndrome)Middle lobe and lingula
Mounier-Kuhn syndromeTrachea and central bronchi
Williams-Campbell syndromeMiddle lobe bronchi

Reference and further reading

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