Teaching Case

Situs viscerum inversus. Chronic rhinosinusitis and recurrent bronchitis.

The patient affected by situs viscerum inversus (previously diagnosed) complained of recurrent pulmonary infections and chronic rhinosinusitis. She was referred to our Institute to undergo a high resolution computerized tomography (HRCT) of the chest which revealed bronchiectasis and a CT study of the sinuses which confirmed local signs of inflammation.

Kartagener’s syndrome is a rare autosomal recessive inherited disease characterised by the clinical triad of bronchiectasis, sinusitis and situs viscerum inversus. The pathogenesis of this condition is a defect in ciliary motility, associated with structural abnormalities of cilia; males with this condition tend to be infertile, owing to ineffective mobility of the sperm tail. About half the patients with defective cilia don't have situs inversus; it is assumed that during early embryonic life ciliary beats determine the type of laterality: when ciliary movements are absent laterality may develop fortuitously, thus effecting a situs inversus in about half the affected cases. The lack of ciliary activity results in impaired mucociliary clearance, predisposing sinuses and bronchi to infection. Bronchiectasis are caused by chronic necrotizing infection of the bronchi leading to abnormal permanent dilatation of these airways. The clinical manifestations consist of cough, fever and expectoration of copious purulent, sometimes bloody sputum. In severe cases obstructive ventilatory insufficiency may occur and leads to marked dyspnea and cyanosis. The HRCT of the chest can demonstrate the dilatation of the airways; these dilatations may produce long, tubelike enlargements (“cylindrical bronchiectasis”) or in other cases may cause saccular(“cystic bronchiectasis) or varicose-like aspect of bronchi (“varicose bronchiectasis”).