Kartagener Syndrome

The patient presented with complains of chronic cough with mucoid expectorations since 10 years. Patient has recurrent episodes of rhinitis since childhood. Patient is married and unable to concieve for more than 1 year.

Bronchiectatic changes in form of cystic and varicoid bronchiectasis with bronchial wall thickening are noted in left middle lobe and right lower lobe with internal air-fluid level in few of them. Multiple centrilobular nodules in tree in bud pattern of distribution with surrounding ground glass opacity are noted involving bilateral lower lobes. Dextrocardia, a left-sided liver, right-sided stomach and spleen, and the left lung having three lobes as identified by a left-sided horizontal fissure.

Sinusitis, situs inversus, and bronchiectasis make up the characteristic trio of Kartagener's syndrome, an extremely rare hereditary condition. [1]. This syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive disorder with defective ciliary structure or function that impairs mucociliary clearanceRecurrent bronchitis, pneumonia, and sinusitis are the three most typical clinical symptoms. Infertility in men and decreased fertility in women may accompany it. Recurrent infections and airway inflammation are the main causes of bronchiectasis. The primary method used to illustrate and evaluate the severity of bronchiectasis is often an HRCT of the lung [2]. Bronchiectasis are classified into three morphologic types depending on the severity of bronchial dilatation (cylindrical, varicose and cystic) and wall abnormalities [2]. A mucosal biopsy is used to confirm the diagnosis and check for ciliary activity. Screening tests include the saccharin test and the measurement of nasal and exhaled nitric oxide [3]. Treatment of this rare congenital disorder includes antibiotics, intravenous or oral, intermittent or continuous, and are used to treat upper and lower airway infections. Bronchiectasis should be treated with inhaled bronchodilators, mucolytics, and chest physiotherapy. Influenza and pneumococcal vaccination should be encouraged to prevent complications [1].