Kartagener’s Syndrome: Presenting in Fourth Decade

Abstract

Kartagener’s Syndrome (KS) is a rare ciliopathic autosomal recessive genetic disorder characterized by a triad of situs inversus, bronchiectasis, and chronic sinusitis. Although Siewart first described this condition in 1904, it was Kartagener -a Swiss pediatrician, who recognized the etiological correlation between elements of the triad and reported four cases in 1933 1. It is a type of Primary Ciliary Dyskinesia (PCD). The frequency of KS is 1:32,000 to 1:50,000 live births. A 40-year-old male patient presented to the emergency department with complaints of fever, productive cough, dyspnea, and headache. He had a history or a recurrent cough for ten years with multiple hospitalizations. The patient had been married for 15 years and still had no offsprings for which he had been investigated previously which was suggestive of azoospermia. He was febrile with normal other vitals accept his respiratory rate which was 28 per minute with 88% saturation on room air. Apex beat could be localized on 5th ICS right side on mid clavicular line. On percussion heart, borders were confirmed on the right side with liver dullness on left side. On auscultation, S1S2 were heard on the right side with coarse, leathery crepitations on inspiration and rhonchi on expiration.On investigating further his ECG showed features of dextrocardia and chest roentgenography was suggestive of dextrocardia with aortic arch on right side of the trachea with gastro-hepatic transposition suggestive of situs inversus with bronchietatic changes. Further High resolution computed tomography was done to confirm those findings along with ultrasound of abdomen which showed liver in left upper quadrant and spleen in right upper quadrant. A skull roentgenogram was also done which was suggestive of sinusitis.The ‘nasal saccharin transit time’ test gave mucociliary clearance time of 60 minutes (Normal < 30 minutes). Thus patient was diagnosed with Kartagener’s syndrome clinically and also on the basis of investigations. The patient was treated with oral steroids, bronchodilators, mucolytics, and antibiotics and over the course of 10 days patient gradually recovered and was discharged.