Kartagener syndrome in a young Ethiopian boy: a case report

Kartagener syndrome is a subset of a larger group of ciliary motility disorders called primary ciliary dyskinesias. The syndrome includes the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. Patients usually present with recurrent respiratory tract infections due to ineffective mucociliary clearance. Females and males are equally affected. Kartagener syndrome occurs in about 1 in 32,000 to 40,000 births worldwide.

This case involved a 17-year-old Black African Semitic male patient who presented to our hospital with a complaint of intermittent productive cough, which started when he was 5 years old. He had a history of repeated treatment for lower respiratory tract infection and chronic sinusitis with frequent exacerbation. On examination, he had coarse crackles over the left posterior lower lung field. Heart sounds were appreciated on the right side. During imaging investigations, his chest X-ray posterior-anterior view showed dextrocardia and right side gastric shadow with left paracardiac bronchiectatic changes. A high-resolution chest computed tomography scan was suggestive of complete situs inversus. There were left lower lobe bronchiectatic changes. An electrocardiogram showed features of dextrocardia. Routine laboratory tests were within normal range. He was treated with thoracic physiotherapy, azithromycin 500 mg three times per week, and mucolytics, with no apparent exacerbations in the last 6 months.

The diagnosis of Kartagener syndrome is typically delayed because the clinical symptoms are easily mistaken for common infections. Since there is no specific treatment for Kartagener syndrome, early diagnosis and management of Kartagener syndrome are critical to prevent irreversible lung damage and chronic lifelong sequelae. A high index of suspicion is needed to make an early diagnosis so that timely treatment options may be offered to prevent problems associated with Kartagener syndrome.