Primary ciliary dyskinesia (Kartegner syndrome)

Case contributed by Dr Abdallah Alqudah


Cough in patient with known history of chronic lung disease.

Patient Data

Age: 40 years
Gender: Female

Cylindrical and varicose bronchiectatic changes seen predominantly in the right middle and lower lung lobes and the lingula. The upper lobes are relatively spared.

Diffuse bilateral centrilobular nodular changes with tree-in-bud configuration seen predominantly in the middle and lower lung lobes bilaterally.

The cardiac apex is seen pointed to the right side indicating dextrocardia.

The visible abdomen confirms situs inversus totalis.

Case Discussion

The characteristic lung findings of cylindrical and varicose bronchiectasis with tree-in-bud configuration in the lingula, middle and lower lung lobes along with dextrocardia make primary ciliary dyskinesia the most likely diagnosis.

Note the relative sparing of the upper lobes.

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