Kartagener Syndrome

KS, first described in 1933, is part of a larger family of diseases classified as PCD.

The triad of KS consists of bronchiectasis, chronic sinusitis, and situs inversus; it has an incidence estimated at 1:15,000–40,000 births.

The disease is likely underdiagnosed because a limited amount of centers have resources to provide an accurate diagnosis.

No known predilection for race or gender.

Symptoms more prevalent in children in the first decade of life.

Morbidity: Lung infection, pulm edema, atelectasis, sinusitis

Pulm function and anatomy.

Airway obstruction due to ineffective clearance of secretions.

Bronchiectasis, which can lead to cor pulmonale, amyloidosis, and pulm edema and is usually found in the middle or lower lobes in KS pts, as opposed to the upper lobes in cystic fibrosis pts.

Chronic disease with variable onset.

Chemical injury from aspiration in left lung, which is the larger lung in pts with KS.

Unintended bronchial intubation with single-lumen ETT, resulting in nonventilation of right lung (in those with pulm inversion).

Left-sided double-lumen tubes may occlude orifice of left upper lobe.

Nasal catheters relatively contraindicated because of risk of paranasal sinusitis and ear infections.

Increased susceptibility to overall infection due to impaired neutrophil chemotaxis.

Complete situs inversus (including dextrocardia).

PCD resulting in chronic respiratory tract infections, bronchiectasis, and sinusitis.

Approximately half of patients with PCD have situs inversus and thus are classified as having KS.