Review
Nasal and sinus disease in cystic fibrosis

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Summary

Paranasal and sinus disease is present in almost every patient with cystic fibrosis. However, symptoms are rarely reported. Some aspects including polyposis and microbial flora change with patient age. Endoscopy and computerised tomography have broadened our understanding of how this disease affects the sinuses, including an increased recognition of polyposis than previously thought and identification of several disease specific entities such as abnormalities of the lateral nasal wall and uncinate process. Few randomised, controlled trials evaluating medical or surgical treatments of CF sinus disease exist. Sinus surgery may provide some benefit, though there are no established selection criteria for appropriate candidates. A link between sinus disease and lower respiratory tract function may contribute to general health and survival following lung transplantation. Complications of sinonasal disease in CF are rare and include mucoceles and periorbital abscesses.

Section snippets

Clinical presentation of sinonasal disease in CF

Despite significant disease, less than 10% of CF patients report sinonasal symptoms.5 In a survey of consecutive paediatric patients at a CF centre, the average subject answered ‘no problem’ to ‘mild to slight problem’ for most sinonasal symptoms.6 Even when they did complain, the complaint did not correlate with disease severity seen on endoscopic examination or on computed tomography (CT) .5 The lack of complaints may be secondary to the chronicity of the symptoms or the overshadowing of

Nasal Polyposis in CF

The reported prevalence of nasal polyposis in CF patients varies from 7% to 48%.11 This variation may be accounted for by differences in subject populations or diagnostic methods. Initial reports of polyp prevalence in CF ranged from 6.7% to 26.7%, however diagnosis was made only by physical exam of the external nares.1, 10, 12 These early studies were performed primarily in a younger population. Most polyps in CF are small13, 14 and over 60% are not visible outside the middle meatus.13, 15

Computed Tomography of CF sinonasal disease

Sinus computed tomography (CT) provides great detail of bony and soft tissue anatomy and has replaced planar radiographs for the diagnosis of sinus disease. Several CT sinonasal anomalies are characteristic in the CF population, such as bulging or displacement of the lateral nasal wall and demineralisation of the uncinate process.7, 8, 19, 20

Hypoplasia or aplasia of the paranasal sinuses has been uniformly reported in patients with CF.20 Minimal development of frontal and sphenoid sinuses has

Sinus Flora in CF

Most sinus cultures taken from CF subjects with documented sinus disease yield an organism. Polymicrobial results are obtained around 25–44.4% of the time22, 23 and the percentage of sterile cultures reported varies from 2.9%24 to 23.5%.22

The most common bacteria isolated from the sinuses of CF patients vary with age. Pseudomonas aeruginosa appears to be more significant in older patients, while Staphylococcus aureus and Haemophilus influenzae are found predominately in younger patients (see

Treatment of CF Sinonasal Disease

Although sinonasal disease is almost universal in CF there are relatively few randomised controlled trials (RCTs) evaluating medical or surgical therapy in CF. Nasal saline irrigation, topical or systemic nasal decongestants or antihistamines, the mucolytic N-acetylcysteine, systemic corticosteroids, topical aminoglycoside and systemic macrolide antibiotics are sometimes used to treat CF sinus disease but none of these therapies have been evaluated in RCTs.

Complications of CF Sinonasal Disease

Complications of acute or chronic sinonasal disease are much less common in CF patients than in the general population. Only two cases (both paediatric) of sub-periosteal orbital abscesses as complications of ethmoid sinus disease have been reported.39 Direct extension of sinus disease into cerebral abscesses in CF patients has not been reported.

CF sinus disease and lung transplant patients

Sinonasal disease in CF patients persists after lung transplantation and this has been implicated in some of the infection-related morbidity and mortality following transplantation. The benefit of maxillary sinus antrostomy and repeated lavage in CF heart-lung transplant patients has been evaluated.43 Mucoid impaction of the maxillary sinuses was found by non-contrast sinus CT in 11 consecutive subjects listed for lung transplantation at one centre. All subjects had bilateral maxillary sinus

Genotype-Phenotype correlations

The first report of CF genotype influencing sinonasal disease was reported in 1999.16 Out of 113 CF patients evaluated by an otolaryngologist at a regularly scheduled control visit, only 38% of asymptomatic patients were delta F508 homozygous compared with 79% of the candidates for sinus surgery. Delta F508 homozygous patients had greater severity of sinus disease and greater frequency of nasal polyps. Their relative risk for sinus surgery (2.33) was significantly greater than the ones for the

Intranasal gentamicin

Gentamicin has been found to correct the translation of defective CFTR alleles containing a premature termination codon.45 Topical application of gentamicin decreased the nasal potential difference of CF patients that were homozygous or heterozygous for CFTR stop mutations.46 In these patients, topical gentamicin may serve as a combined therapy for sinonasal disease treating the typical bacterial infection and correcting, at least temporarily, the underlying genetic defect.

Low dose systemic macrolide antibiotics

Low dose 14- and

Conclusion

Sinonasal disease is nearly ubiquitous in CF patients but symptoms are rarely reported. The paucity of complaints has made it difficult to assess the outcome of sinus medical or surgical therapy. Endoscopic sinus exams are almost always abnormal and give a better indication of the presence of nasal polyposis than physical examination of the nose alone. Nasal polyps may increase with age and may represent a proliferative airway repair mechanism. Sinus CT has demonstrated several anomalies

Acknowledgement

The authors would like to thank Dr John Hicks and Dr Mas Takashima for supplying Figure 1, Figure 2.

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