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Kartegener's Syndrome
Diskinetic Cilia Syndrome

General Considerations

  • Classic triad consists of
    • Situs inversus
    • Sinusitis
    • Bronchiectasis
  • Other associations
    • Transposition of great vessels
    • Trilocular or bilocular heart
    • Pyloric stenosis
    • Postcricoid web
    • Marfan’s syndrome
    • Polysplenia
    • Hepatic steatosis
    • Hydrocephalus
    • Usher’s syndrome       
      • Deafness
      • Vestibular dysfunction
      • Retinitis pigmentosa
  • All have ciliary dyskinesia in common
  • Incidence
    • 1:12,500-40,000
    • Autosomal recessive inheritance
  • Cilary dyskinesis can also be acquired
    • Smokers most commonly

Imaging findings

    • Bronchial wall thickening
    • Hyperinflation
    • Segmental atelectasis or bronchiectasis
    • Situs inversus is present in half of patients with Dyskinetic Cilia Syndrome
    • Bronchiectasis is indistinguishable from bronchiectasis of many other causes
      • 50% occurs in lower lobes

Clinical Findings

    • Sinusitis
    • Recurrent bronchitis
    • Bronchiectasis
    • Male sterility
    • Corneal abnormalities and poor sense of smell

Pulmonary function test abnormalities

    • Small airway dysfunction
    • Increased bronchial responsiveness to methacholine

 Kartegener Syndrome.

 Kartegener Syndrome. Upper CT shows dextrocardia and bronchiectasis at both bases;
lower CT shows situs inversus of abdominal organs

Fraser and Pare, 4th Edition