March 15, 2010

Kartagener Syndrome

Figure 1: Frontal chest radiograph demonstrates dextrocardia (cardiac apex pointing to the right with right aortic arch - arrowheads), bibasilar coarse reticular opacities and loss of volumes in the lower lungs in a patient with Kartagener syndrome.
Figure 2: Coronal reformatted CT image confirms the presence of extensive bibasilar bronchiectasis (arrows) and situs inversus. Bronchiectasis in patients with this syndrome tends to involve the dependent parts of the lungs.

Facts: Kartagener Syndrome
  • Genetically transmitted (autosomal recessive) syndrome characterized by bronchiectasis, situs inversus and chronic sinusitis
  • Genetic disorder first described in 1904, but identified as a syndrome by Manes Kartagener, a Swiss internist, in 1933
  • It is a subtype of primary cilia dyskinesia
  • Incidence 1:32000 live births
  • Abnormal function of cilia is believed to be responsible for visceral asymmetry (abnormal movement of cilia in certain embryonic epithelial tissues), respiratory disease, etc.
  • Onset of upper and lower respiratory tract symptoms shortly after birth in the presence of situs inversus
  • Family history of primary ciliary dyskinesia or Kartagener syndrome
  • Confirmation with biopsy of respiratory mucosa or microscopic examination of sperms
  • Other clinical signs: chronic rhinitis with nasal polyposis, agenesis of frontal sinuses, repeated otitis media, bronchiectasis (usually dependent parts of the lungs in contrast to cystic fibrosis that tends to affect the upper lobes), situs inversus (complete or partial)
Bissonnette B, Luginbuehl I, Dalens BJ. Syndromes: rapid recognition and perioperative implications, 2006.

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