KARTAGENER'S SYNDROME

10 yrs old boy with recurrent respiratory infections

 

Axial CT sections of chest in lung window show dextrocardia with dense cystic bronchiectasis involving middle lobe and lingula. Multiple infiltrates in bilateral lower lobes (not shown here).

Coronal reformatted images showing the same findings.

Axial CT section through the upper abdomen showed inversion of abdominal organs also
suggestive of complete situs inversus.

KARTAGENER'S syndrome

Findings:

The chest radiograph demonstrates dextrocardia with the stomach bubble and aortic arch on the right side. The findings represent situs inversus totalis. The patient has marked peribronchial thickening and a focal area of atelectasis in the left lung. Close inspection of the lung markings demonstrated tubular lucencies indicating bronchiectasis in this patient with known KARTAGENER'S SYNDROME.

This syndrome consists of a classic triad of:

• Sinusitis
• Bronchiectasis
• Situs inversus totalis

Also known as:

• Ciliary dyskinesia
• Immotile cilia syndrome

Clinical manifestations:

• Chronic cough
• Recurrent respiratory infections
• Sinusitis
• Otitis media
• Reduced fertility in males

The clinical manifestations are directly related to abnormal ciliary structure and/or function. Furthermore, situs inversus totalis occurs in approximately half of patients with Kartagener's syndrome, with the remainder having normal situs. This implicates the role of ciliary function in the early positioning of internal organs in the developing fetus. Without normal ciliary functioning, there is random occurrence of normal and reversed situs. Therefore, although the classic triad includes situs inversus, the syndrome includes those patients with normal situs.

Differential diagnosis:

The presence of airway disease and recurrent pulmonary and sinus infections may resemble the clinical and radiographic appearances of cystic fibrosis. Also, immunodeficiency states may present in a similar manner. The correct diagnosis is usually first recognized by the presence of situs inversus in a patient with chronic respiratory symptoms, and can be confirmed by biopsy of nasal cilia.