Images in internal medicine
Kartagener syndrome
Síndrome de Kartagener
Kartagener syndrome
Acta Medica Colombiana, vol. 47, no. 1, pp. 62-63, 2022
Asociacion Colombiana de Medicina Interna
Received: 29 August 2021
Accepted: 20 October 2021
A 23-year-old man with a history of recurrent sinusitis was ad mitted for pleuritic pain and fever. His tests showed left empyema, situs inversus, bronchiectasis and sinusitis (Figures 1 and 2). He was diagnosed with Kartagener syndrome, and required antibiotic and surgical treatment for his empyema, with clinical improvement. A nasal ciliary biopsy was compatible with ciliary dyskinesia.
Figure 1
A: anterior-posterior chest x-ray showing alveolar opacity in the left lower lobe, ipsilateral pleural effusion and signs of volume loss with hemidiaphragmatic retraction, dextrocardia and a right-sided gastric chamber. B: chest tomography, coronal plane, showing complete situs inversus: heart and stomach to the right and liver to the left.
Figure 2
A: chest tomography, axial plane, with left pleural effusion, peribronchial thickening and bilateral bronchiectases, some with a mucous plug. B: paranasal sinus tomography showing frontal sinusitis. C: bilateral ethmoid and maxillary
Kartagener syndrome is an autosomal recessive hereditary disorder, a subset of primary ciliary dyskinesia, characterized by the triad of chronic sinusitis, bronchiectasis and situs inversus1. The recommended diagnostic criteria include a history of chronic bronchial infections and rhinitis since childhood, with one or more of the following: (a) situs inversus or dextrocardia in the patient/ sibling, (b) live but immotile spermatozoa, (c) absent or abnor mal tracheobronchial clearance, and (d) a characteristic ciliary ultrastructural defect on electron microscopy 2. Management is multidisciplinary, with physical therapy, antibiotics, vaccinations and lung transplant in advanced disease recommended 1,3.
Referencias
Tadesse A, Alemu H, Silamsaw M, Gebrewold Y. Kartagener's syndrome: a case report. Journal of Medical Case Reports. 2018;12(1):5.
Fraser RS, Muller NL, Colman N, Pare PD. Bronchiectasis and other bronchial abnormalities. In: Fraser RS, Muller NL, Colman N, Pare PD, editors. Diagnosis of diseases of the chest. 4th ed. Philadelphia: W.B. Saunders Company; 1999. p. 2281-3.
Yamamoto H, Sugimoto S, Miyoshi K, Otani S, Yamane M, Toyooka S. Lung transplantation for Kartagener syndrome: technical aspects and morphological adaptation of the transplanted lungs. General Thoracic and Cardiovascular Surgery. 2021;69(3):588-92.
Author notes
* Correspondencia: Dra. Marysabel Cogollo-González. Bogotá, D.C. (Colombia). E-Mail: marysabelcogo@unisabana.edu.co