Ultrastructural defects of the ciliary epithelium in a child with Kartagener's syndrome.

ABSTRACT

Kartagener's syndrome is a well known classical triad of presentations consisting of bronchiectasis, sinusitis and situs inversus. It is now recognized that the syndrome is an extreme presentation of primary ciliary dyskinesia, a large group of conditions with ultrastructural ciliary defects, leading to poor ciliary motility in various organ systems. A case of Kartagener's syndrome is presented in an eight year old Thai boy in whom the ultrastructural ciliary defects have been examined and described in detail for the first time in Thailand. Incomplete lack of dynein arms was recognized. In addition, disorientation of ciliary axis was noticed. Due to severe bronchiectatic changes of the right lower lobe and right lingular lobe which did not improve despite adequate antibiotics, these lobes were surgically removed. The child has done well since, but still suffers occasional and recurrent bouts of sinusitis.