Kartagener syndrome: a case report

Introduction: Kartagener's Syndrome is the most severe form of primary ciliary dyskinesia, caused by a rare autosomal recessive disease and presents in the triad: chronic pansinusitis, bronchiectasis and situs inversus. The incidence is 1/25,000 people. Carriers of this syndrome are prone to recurrent respiratory tract infections Objective: To report a case of a rare syndrome, its clinical presentation and the patient's evolution during outpatient follow-up. Methodology: This is a descriptive observational study based on the analysis of the patient's medical record and literature review. Results: A 37-year-old woman was being followed up at the pulmonology outpatient clinic for Kartagener's syndrome, asthma and bronchiectasis. On physical examination, respiratory auscultation revealed bilateral vesicular murmurs, diffuse rales in the right hemithorax and oxygen saturation of 90%. During outpatient follow-up, the patient reported hoarseness and cough, as well as a recent flu-like illness with the use of levofloxacin. A chest CT scan showed situs inversus totalis, as well as cystic, cylindrical and varicose bronchiectasis located in the right lower lobe and in the left paracardiac lung field, associated with consolidation and atelectasis of the lung parenchyma, which could be related to mucociliary dyskinesia. Diffuse thickening of the bronchial walls with mucoid impaction and centrilobular nodules with a budding tree appearance were also found. All these findings suggest Kartagener's syndrome associated with an inflammatory/infectious process with endobronchial dissemination. At the last consultation, due to the patient's tomographic and clinical findings, the patient was prescribed budesonide + tiotropium + holodanterol + azithromycin for continuous use, to control and improve the current condition. Conclusion: Finally, this study presents a case of a rare syndrome and the control of its clinical repercussions. In addition, when diagnosed and treated early, it prevents the development of complications such as hearing loss due to repeated otitis media and bronchiectasis.