Kartagener's syndrome: A case series

Abstract

Kartagener's syndrome is a rare, autosomal recessive genetic ciliary disorder comprising the triad of situs inversus, chronic sinusitis, and bronchiectasis. The basic problem lies in the defective movement of cilia, leading to recurrent chest infections, ear/nose/throat symptoms, and infertility. We hereby report three unusual cases of this rare entity - an infertile male with azoospermia in whom Bochdalek's diaphragmatic hernia coexisted, another case of an infertile female, and a third of an infertile male with oligospermia. The need for a high index of suspicion to make an early diagnosis cannot be overemphasized in such patients so that wherever possible, options for timely treatment of infertility may be offered and unnecessary evaluation of symptoms is avoided.

Keywords: Bronchiectasis; Kartagener's syndrome; sinusitis; situs inversus.

Figure 1

(a) Chest X-ray PA view showing dextrocardia and left-sided Bochdalek's hernia; (b) CECT-chest showing right-sided bronchiectasis, dextrocardia, and left-sided Bochdalek's herina

Figure 2

(a) X-ray PNS showing opacified maxillary sinuses; (b) chest X-ray PA view showing dextrocardia and left-sided bronchiectasis

Figure 3

(a) Cystic bronchiectactic changes in the lower and mid zones with dextrocardia; (b) axial CT image abdomen showing situs inversus with the liver and IVC on the left and the spleen and aorta on the right; (c) axial CT image paranasal sinuses showing mucosal thickening and opacified sinus cavities