International Journal of Pediatric Otorhinolaryngology
Review articleManagement of otitis media with effusion in children with primary ciliary dyskinesia: A literature review
Introduction
Primary ciliary dyskinesia (PCD), formerly known as immotile cilia syndrome, is a rare cause of chronic airway disease in white children [1]. PCD is an autosomal recessive, heterogenous group of disorders of ciliary ultrastructure with variable penetrance [2], [3], [4]. PCD is characterized by cilia that beat more slowly than normal, or with abnormal beat patterns, that result in impaired mucociliary clearance (MCC). PCD affects both genders equally and has an incidence varying from 1 in 15,000 to 1 in 40,000 births [1], [3], [5]. Kartagener's syndrome (KS) is a subgroup of these children with a triad of sinusitis, bronchiectasis and situs inversus and affects approximately 50% of PCD patients [6].
Otolaryngologists are frequently involved in the management of many of the common symptoms of PCD and KS including chronic rhinitis, sinusitis and otitis media with effusion (OME). OME is defined as the presence of middle ear fluid without symptoms or signs of acute inflammation of the middle ear [7] and [8]. Otitis media with effusion (OME) is the most common cause of acquired conductive hearing loss in childhood [9] with a 10–30% prevalence in the 1–3 year age group [7]. The treatment of chronic OME in children aims to improve hearing and to prevent the possible sequelae of long-term hearing loss such as impairments to speech and language development. Treatment also aims to prevent other potential sequelae of OME such as tympanic membrane atelectasis and retraction pockets, ossicular chain erosion or necrosis and cholesteatoma.
The dilemma for Otolaryngologists is whether ventilation tubes (VT) are of benefit in these children or whether, by placement of VT, a dry ear with OME and hearing loss is converted to a chronically discharging ear with OME and hearing loss. This paper presents a review of the current literature on the natural history of OME in PCD and also of the treatment of OME with VT in children with PCD. We look at the factors of hearing loss, post-operative otorrhoea and tympanic membrane changes. We report our experience and follow-up with one such patient.
Section snippets
Method
A review of the literature was undertaken to examine the natural history and treatment of OME in PCD. The literature review encompassed a search of the following databases: Premedline and Medline (1950–2008), Embase, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects, ACP Journal Club and Google Scholar. Also, a hand search of references of the relevant literature was undertaken and some authors were contacted for
Case report
K.M. was referred to our clinic at 9 months of age for an opinion regarding the management of his bilateral chronic OME. K.M. has dextrocardia, complete situs inversus, bronchiectasis and chronic sinusitis. Diagnosis of KS at birth was delayed due to a mislabeled chest X-ray (CXR) (Fig. 1). The CXR was performed due to respiratory distress after an uneventful pregnancy and delivery. A repeat CXR at six weeks of age, due to continued respiratory distress and feeding difficulties, revealed
Results
Eight papers were identified. Two studies specifically assessed the natural history of 87 patients with OME and PCD [10], [11]. Six papers reviewed the treatment of 81 patients with PCD and OME [6], [12], [13], [14], [15], [16]. All but 2 of the 8 papers identified [10], [16] were published 10 years ago or more. The 8 papers reviewed comprise: 5 retrospective observational studies/case series [6], [10], [12], [13], [15], one cross-sectional study [11], one case report [16] and one letter in
Discussion
KS is named after a paediatrician who described the triad of bronchiectasis, situs inversus and sinusitis in 4 patients in 1933 [30]. The aetiology and pathogenesis of KS was determined in 1975 when Pedersen and Rebbe [31], and then Afzelius et al. [32] in 1976, described the association of abnormal ultrastructure of spermatozoa with sinusitis, bronchiectasis and situs inversus in infertile men. More recently, Nonaka et al. [33], [34] suggested the role of the motile monocilia of the embryonic
Conclusion
Children in the general population with OME have a conductive hearing loss, which is usually temporary, variable in severity and duration, and may be asymmetric [47]. Normal hearing is usually achieved with conservative management by the age of 8 years [68], [69]. Hearing loss in children with OME and PCD is usually persistent throughout childhood, continues well into adulthood [10], [11], [15], [17], is variable in severity [10], [11] and is bilateral. By providing VT for children with PCD,
Conflict of interest statement
This study was not funded and no author had any conflict of interest regarding this article.
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2021, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :Prior studies of ENT complications of PCD have noted significant morbidity from sinonasal disease in adults as symptoms of otitis media decrease with age [16]. However, in the pediatric population the morbidity is often a result of otitis media, which may require intervention to prevent permanent hearing damage [17]. Although patients with PCD frequently present with serious and recurrent otitis media, this was not found to be a sensitive characteristic for disease diagnosis in the North American study and thus was not included in the ATS criteria [11].
Hearing loss in children with primary ciliary dyskinesia
2018, International Journal of Pediatric Otorhinolaryngology
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