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Atlanto-occipital Dislocation
Joseph Junewick, MD FACR
over 8 years ago
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Kartagener Syndrome

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Genetic
Created: over 6 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: CT CR
Activities:
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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.



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