Kartegener's Syndrome
Diskinetic Cilia Syndrome

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

Fraser and Pare, 4th Edition