Kartagener syndrome
Presentation:
Chronic sinusitis since childhood, routine check-up.
Patient Data:
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CXR demonstrates situs inversus (right sided heart, aorta and stomach bubble). Striking bilateral peribronchial cuffing and dilated bronchial tree, mainly in upper and lower lobe on the left side.
CT confirms situs inversus (mirror image of thoracic and upper abdominal content). The lung window setting the varicose to cystic bronchiectasis is well demonstrated. Also the tree-in-bud sign is well visible in both lower lobes suggests current endobronchial infection.
Case Discussion:
Kartagener syndrome is an autosomal recessive hereditary disease and a combination of findings that are also described with the immotile cilia syndrome (also called primary ciliary dyskinesia). The syndrome typically presents with:
- situs inversus
- bronchiectasis
- chronic sinusitis
- infertility in males
The tree-in-bud sign is often associated with Kartagener Syndrome, indicative of obstructive bronchiolitis. Impaction of mucoid material is a typical finding in these patients.
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