Kartagener syndrome

Case contributed by Issac Yang
Diagnosis certain

Presentation

Chronic cough

Patient Data

Age: 55 years
Gender: Male
x-ray

Presence of dextrocardia with situs inversus (right-sided aortic arch, elevation of left hilum relative to the right, right sided gastric bubble)

Presence of tram-track like opacities suggestive of bronchiectasis, predominantly in the lower lobes. 

CT thorax

ct

Dextrocardia with situs inversus.

There is bronchiectasis involving predominantly the bilateral middle and lower lobes with multiple mucous plugs and volume loss in the lingula. There are also numerous tree-in-bud nodules suggestive of bronchiolitis.

CT head

ct

A prior CT head demonstrates pansinus mucousal inflammatory changes (except the maxillary sinuses, which are hypoplastic). There is also bilateral mastoid and middle-ear effusion.

Case Discussion

Kartagener syndrome, first described in 1933, is characterized by the triad of situs inversus, bronchiectasis and sinusitis and/or nasal polyposis. It is present in 50% of patients with primary ciliary dyskinesia, a rare genetic disorder causing ciliary defects and impaired mucociliary clearance, thereby predisposing patients to otosinopulmonary disease.

Air-trapping (characterized by mosaic attenuation) and recurrent pulmonary infections (characterized by tree-in-bud nodules, ground-glass opacities, and consolidations) are frequently seen due to poor mucosal clearance.

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