Search

Objective Primary ciliary dyskinesia is an autosomal recessively inherited group of disorders of ciliary ultrastructure. Otolaryngologists are frequently involved in the management of some of the most common symptoms of primary ciliary dyskinesia including chronic rhinitis, sinusitis and otitis media with effusion. A dilemma for otorhinolaryngologists is whether ventilation tubes are of benefit in children with primary ciliary dyskinesia and otitis media with effusion and what effective alternatives exist. This paper aims to address this issue via a literature review and case presentation. Methods An extensive review of the literature was undertaken and a discussion of the advantages and disadvantages of ventilation tubes in the management of otitis media with effusion in these children is presented and compared with that of the general population. We present a case of a 9 months old boy with Kartagener's Syndrome and chronic bilateral otitis media with effusion to illustrate our findings. Results Eight papers were identified, all with small study numbers. The main outcome measures were hearing, otorrhoea and tympanic membrane structural changes. The natural history of otitis media with effusion and hearing loss in primary ciliary dyskinesia appears to be fluctuant into adulthood. Therefore, otitis media with effusion in primary ciliary dyskinesia does not resolve by the age of 9 years, regardless of treatment, as previously assumed. Ventilation tube insertion improves hearing in primary ciliary dyskinesia, but may lead to a higher rate of otorrhoea when compared to the general population. Tympanic membrane changes were clinically insignificant. Our patient eventually underwent successful insertion of bilateral ventilation tubes with a marked improvement in hearing and language with minimal otorrhoea. Conclusion/Discussion The highest level of evidence found for the management of otitis media with effusion in children with primary ciliary dyskinesia was level IV. Currently, the evidence is inconclusive and conflicting. Whilst our results are promising, clearly higher quality research on a larger number of patients is required to definitively evaluate the management options for otitis media with effusion in these children.

Primary ciliary dyskinesia (PCD), also known as immotile-cilia syndrome, is a rare genetic disease that is inherited in an autosomal recessive manner. Several studies have explored certain aspects of PCD in the Arab world, yet much is still lacking in terms of identifying the different characteristics of this disease. In this paper, we aim to briefly cover those studies published about PCD in Arab countries, as well as to provide recommendations and guidelines for future studies.

Primary ciliary dyskinesia [PCD] is an uncommon, autosomal recessively inherited condition that is often overlooked and undertreated in childhood. Amidst the myriad of children with coloured nasal secretions, otitis media and a wet cough, there exists a subset with PCD as the underlying unifying diagnosis. In this paper we have highlighted the varying clinical manifestations of PCD, emphasising different presentations between neonates, toddlers, school aged children and adults.

This paper describes a clinical case of both giant cell granuloma and Kartagener syndrome in a 15-year-old male patient, with emphasis on the radiographic aspects of this extremely unusual pathology. To our knowledge, the presence of these 2 rare clinical conditions in the same patient has not been previously reported.