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Kartagener’s
Syndrome
Dyskinetic 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
- Ciliary dyskinesis can also be acquired
- Imaging findings
- Bronchial wall thickening
- Hyperinflation
- Segmental atelectasis or bronchiectasis
- Situs inversus is present in half of
patients with Dyskinetic Cilia Syndrome
Upper CT shows dextrocardia and
bronchiectasis at both bases;
lower CT shows situs inversus of abdominal organs
- Bronchiectasis is indistinguishable from
bronchiectasis of many other causes
- 50% occurs in lower lobes
- Clinically
- 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
Fraser and Pare, 4th Edition
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