Presentation
Chest pain, chronic productive cough and low-grade fever.
Patient Data
On these images, there are multiple foci of bronchiolitis, central bronchiectasis, peribronchial soft tissue thickening, cuffing, and also foci of air trapping more prominent in the middle to lower zone of the lungs. Multiple foci of bronchoceles in the lower lobes of the lung are also noted, more prominent on the left side.
Note the right-sided heart and aorta, right-sided spleen, right-sided stomach, and left-sided liver.
Case Discussion
Kartagener syndrome is a rare autosomal recessive primary ciliary dyskinesia and is defined as a triad - situs inversus, bronchiectasis, and chronic sinusitis.
In most cases, newborns suffering from the syndrome have respiratory distress in the first 12-24 hours of life. Adults with the syndrome may present with chronic productive cough due to recurrent bronchial and bronchiolar infection, and bronchiectasis. This syndrome is also one of the causes of infertility in males and females.
The only definitive diagnosis is based on the evaluation of:
- ciliary ultrastructure on electron microscopy
- ciliary beats less than 10Hz per second on videomicroscopy (using a scrape or brush biopsy specimen of the nasal cavity)
A MDCT chest scan to evaluate pulmonary and bronchial complications of the syndrome and cardiac situs can also be performed.
There is no cure for the syndrome but standard treatment includes chest physiotherapy, antibiotics and mucolytics. Additionally, influenza and pneumococcal vaccines should be also be prescribed annually.
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