Kartagener Syndrome in Two Siblings: A Familial Case Report With Phenotypic Variability and Destroyed Lung Syndrome...

Kartagener syndrome is a rare genetic disorder characterized by the classic triad of situs inversus, bronchiectasis, and chronic sinusitis. The progression to destroyed lung syndrome represents an exceptionally rare complication, with only a few cases reported in the literature. We present the cases of two Moroccan sisters, aged 20 and 26, born from a first-degree consanguineous marriage, presenting with chronic respiratory symptoms but with different phenotypes. The first patient had a severe phenotype including destroyed lung syndrome, chronic bronchitis, recurrent infections, and imaging findings of dextrocardia with extensive lung tissue destruction. The second patient presented with chronic cough, anosmia, and bronchiectasis with less severe pulmonary involvement than her sibling. Imaging studies confirmed complete situs inversus in both cases. Diagnosis relies on clinical and radiological criteria. These cases highlight the phenotypic variability of Kartagener syndrome in the same family and the possibility of severe pulmonary involvement leading to a completely destroyed lung, a very rare but possible complication. Hence, it is important to regularly screen patients for lung parenchymal destruction and regularly monitor those with severe radiographic changes.