Kartagener syndrome associated with bronchopulmonary dysplasia and complicated by obstructive granulomatous bronchiolitis in children

Abstract

The rarity of Kartagener syndrome, as well as the presence of structural malformative changes associated with the progressive development of granulomatous bronchiolitis, was considered appropriate for the presentation of a clinical case with unfavorable prognosis. Analyzing the clinical laboratory, diagnostic imaging and histopathological results, the authors have concluded that computed tomography data, pulmonary perfusion disturbances found at pulmonary scintigraphy along with progressive deterioration of the pulmonary ventilation function allow identifing and adequately assessing the severity of structural-functional bronchopulmonary changes in children with Kartagener syndrome. The evolution and severity of obstructive syndrome in Kartagener syndrome are determined by the development of structural changes in bronchial-alveolar peripheral airway segments, which together with interstitial inflammatory changes, progressive pneumofibrosis and development of pulmonary hypertension have unfavorable consequences on the evolution and prognosis of the disease. The coexistence of pulmonary dysplasia can be considered as an aggravating factor in the development of Kartagener syndrome in children.

Keywords

• kartagener syndrome
• obstructive syndrome
• pneumofibrosis
• pulmonary dysplasia