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Kartagener Syndrome
Case Detail
| Anatomy: Chest |
 Joseph Junewick, MD FACR |
| Diagnostic Category: Genetic |
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| Created: over 9 years ago |
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| Updated: over 9 years ago |
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| Tags:
PEDS
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| Modality/Study Types:
CT
CR
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Activities:  PDF  ImageJA |
History
10 year old female with congestion and recurrent pneumonia.
Case Images
Diagnosis
Kartagener Syndrome
Findings
CR – Dextrocardia and situs inversus with bilateral basilar bronchiectasis.
CT Sinus – Bilateral polypoid maxillary mucosal thickening.
CT Chest – Left greater than right tubular bronchiectasis.
Discussion
Kartagener’s triad consists of situs inversus, bronchiectasis, and sinusitis. The underlying defect is related abnormal cilia structure and function; all patients with Kartagener syndrome have primary ciliary dyskinesia. Primary ciliary dyskinesia is a genetically heterogeneous, autosomal recessive trait with a prevalence of approximately 1/12,000-1/60,000. Males demonstrate infertility related to abnormal cilia of the spermatozoa; female fertility is less affected since cilia only contribute tubal transit of the fertilized ovum. Patients may exhibit chronic/recurrent, thick, mucoid rhinorrhea from early in childhood and are at risk for nasal polyps. Bronchiectasis usually occurs in the lower lobes in patients with Kartagener syndrome. Normal ciliary motion is necessary for normal visceral rotation, consequently dextrocardia and situs inversus occur. Fortunately, sequelae of primary ciliary dyskinesia and Kartagener syndrome usually become less problematic in adulthood.
Reference
Kennedy MP, Noone PG, Leigh MW, et al. High-Resolution CT of Patients with Primary Ciliary Dyskinesia. AJR (2007; ) 188: 1232-1238.