Kartagener's Syndrome: A Case Series

Abstract

Kartagener's syndrome is an uncommon autosomal recessive ciliary dyskinesia. It combines a triad comprised of bronchiectasis, chronic sinusitis, and situs inversus. This work aims to describe the clinical and paraclinical aspects of primary ciliary dyskinesia using Kartagener's syndrome as a model and to highlight the difficulties of confirming the diagnosis in our context. We report four observations (three boys and one girl with an average age of 10 years) of Kartagener's syndrome collected in the department of pediatric pneumo-allergology. Chronic bronchorrhea and otorhinolaryngological manifestations were found in all cases. Signs of neonatal respiratory distress syndrome were found in only one case. One child had dysmorphic facial features suggestive of Noonan's syndrome and conductive hearing loss. Digital hippocratism was found in half of the cases, along with pulmonary crackles and heart sounds perceived on the right. A chest CT scan showed bronchiectasis in all patients and necrotic adenopathy suggestive of tuberculosis in one case. Sinus imaging showed an appearance of pansinusitis. All children had abdominal situs inversus with dextrocardia. They had received antibiotic therapy with amoxicillin-clavulanic acid associated with respiratory physiotherapy. The girl had benefited from a right lobectomy with a follow-up of 18 months and a good evolution. In light of these four observations, Kartagener's syndrome is a rare disease but can be compatible with normal life if the treatment is done early. However, in our context, the difficulty of confirming the diagnosis explains its delay with the risk of progression of pulmonary lesions.

Keywords: bronchiectasis; dextrocardia; kartagener's syndrome; sinusitis; situs inversus.

Figure 1. Chest X-ray showing the situs inversus (white arrow), bronchial syndrome, and alveolar focus of the lingula

Figure 2. Chest CT scan (parenchymal window) showing the bilateral bronchial dilatation (white arrows) associated with situs inversus

Figure 3. Sinus CT scan showing chronic sinusitis with nasosinusal polyposis (white arrows)

Figure 4. Chest X-ray showing dextrocardia (right arrow) and left upper lobar pneumonia with minimal homolateral pleural effusion (left arrow)

Figure 5. Thoracic CT scan (parenchymal window) showing situs inversus, bronchial dilatation (right arrow), and left laterobasal and posterobasal thoracic empyema (left arrow)