Skip to main content

Cystic Fibrosis and Chronic Rhinosinusitis: Surgical Management and Outcomes

  • Chapter
  • First Online:
The Unified Airway

Abstract

Rates of chronic rhinosinusitis (CRS) in patients with cystic fibrosis (CF) approach 100%. A combination of surgical interventions and aggressive systemic and topical therapy is required for the adequate treatment of CF CRS. Appropriate CRS treatment reduces sinonasal colonization of pathogenic bacteria and delays gram-negative pulmonary infections.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 16.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Rey MM, Bonk MP, Hadjiliadis D. Cystic fibrosis: emerging understanding and therapies. Annu Rev Med. 2019;70:197–210.

    CAS  PubMed  Google Scholar 

  2. Tipirneni KE, Woodworth BA. Medical and surgical advancements in the management of cystic fibrosis chronic rhinosinusitis. Curr Otorhinolaryngol Rep. 2017;5:24–34.

    PubMed  PubMed Central  Google Scholar 

  3. Illing EA, Woodworth BA. Management of the upper airway in cystic fibrosis. Curr Opin Pulm Med. 2014;20:623–31.

    PubMed  PubMed Central  Google Scholar 

  4. Johansen HK, Aanaes K, Pressler T, Nielsen KG, Fisker J, Skov M, Høiby N, von Buchwald C. Colonisation and infection of the paranasal sinuses in cystic fibrosis patients is accompanied by a reduced PMN response. J Cyst Fibros. 2012;11:525–31.

    CAS  PubMed  Google Scholar 

  5. Alanin MC, Aanaes K, Høiby N, Pressler T, Skov M, Nielsen KG, Taylor-Robinson D, Waldmann E, Krogh HJ. Sinus surgery postpones chronic gram-negative lung infection: cohort study of 106 patients with cystic fibrosis. Rhinology. 2016;54:206–13.

    CAS  PubMed  Google Scholar 

  6. Orlandi RR, Kingdom TT, Hwang PH, Smith TL, Alt JA, Baroody FM, Batra PS, Bernal-Sprekelsen M, Bhattacharyya N, Chandra RK, et al. International consensus statement on allergy and rhinology: rhinosinusitis. Int Forum Allergy Rhinol. 2016;6:S22–S209.

    PubMed  Google Scholar 

  7. Berkhout MC, Van Rooden CJ, Rijntjes E, Fokkens WJ, El Bouazzaoui LH, Heijerman HG. Sinonasal manifestations of cystic fibrosis: a correlation between genotype and phenotype? J Cyst Fibros. 2014;13:442–8.

    CAS  PubMed  Google Scholar 

  8. Casserly P, Harrison M, O’Connell O, O’Donovan N, Plant BJ, O’Sullivan P. Nasal endoscopy and paranasal sinus computerised tomography (CT) findings in an Irish cystic fibrosis adult patient group. Eur Arch Otorhinolaryngol. 2015;272:3353–9.

    CAS  PubMed  Google Scholar 

  9. Kang SH, Piltcher OB, de Tarso Roth Dalcin P. Sinonasal alterations in computed tomography scans in cystic fibrosis: a literature review of observational studies. Int Forum Allergy Rhinol. 2014;4:223–31.

    PubMed  Google Scholar 

  10. Berkhout MC, Klerx-Melis F, Fokkens WJ, Nuijsink M, van Aalderen WM, Heijerman HG. CT-abnormalities, bacteriology and symptoms of sinonasal disease in children with cystic fibrosis. J Cyst Fibros. 2016;15:816–24.

    CAS  PubMed  Google Scholar 

  11. Woodworth BA, Ahn C, Flume PA, Schlosser RJ. The delta F508 mutation in cystic fibrosis and impact on sinus development. Am J Rhinol. 2007;21:122–7.

    PubMed  Google Scholar 

  12. Rasmussen J, Aanæs K, Norling R, Nielsen KG, Johansen HK, von Buchwald C. CT of the paranasal sinuses is not a valid indicator for sinus surgery in CF patients. J Cyst Fibros. 2012;11:93–9.

    PubMed  Google Scholar 

  13. Habib AR, Quon BS, Buxton JA, Alsaleh S, Singer J, Manji J, Wicox PG, Javer AR. The Sino-Nasal Outcome Test–22 as a tool to identify chronic rhinosinusitis in adults with cystic fibrosis. Int Forum Allergy Rhinol. 2015;5:1111–7.

    PubMed  Google Scholar 

  14. Habib AR, Buxton JA, Singer J, Wilcox PG, Javer AR, Quon BS. Association between chronic rhinosinusitis and health-related quality of life in adults with cystic fibrosis. Ann Am Thorac Soc. 2015;12:1163–9.

    PubMed  Google Scholar 

  15. Mainz JG, Koitschev A. Management of chronic rhinosinusitis in CF. J Cyst Fibros. 2009;8:S10–4.

    PubMed  Google Scholar 

  16. Cimmino M, Nardone M, Cavaliere M, Plantulli A, Sepe A, Esposito V, Mazzarella G, Raia V. Dornase alfa as postoperative therapy in cystic fibrosis sinonasal disease. Arch Otolaryngol Head Neck Surg. 2005;131:1097–101.

    PubMed  Google Scholar 

  17. Mainz JG, Schien C, Schiller I, Schädlich K, Koitschev A, Koitschev C, Riethmüller J, Graepler-Mainka U, Wiedemann B, Beck JF. Sinonasal inhalation of dornase alfa administered by vibrating aerosol to cystic fibrosis patients: a double-blind placebo-controlled cross-over trial. J Cyst Fibros. 2014;13:461–70.

    CAS  PubMed  Google Scholar 

  18. Mainz JG, Schumacher U, Schädlich K, Hentschel J, Koitschev C, Koitschev A, Riethmüller J, Prenzel F, Sommerburg O, Wiedemann B, et al. Sino nasal inhalation of isotonic versus hypertonic saline (6.0%) in CF patients with chronic rhinosinusitis—Results of a multicenter, prospective, randomized, double-blind, controlled trial. J Cyst Fibros. 2016;15:e57–66.

    CAS  PubMed  Google Scholar 

  19. Aanaes K, Alanin MC, Nielsen KG, Møller MJ, Høiby N, Johansen HK, Johannesen HH, Mortensen J. The accessibility of topical treatment in the paranasal sinuses on operated cystic fibrosis patients assessed by scintigraphy. Rhinology. 2018;56:268–73.

    CAS  PubMed  Google Scholar 

  20. Hadfield PJ, Rowe-Jones JM, Mackay IS. A prospective treatment trial of nasal polyps in adults with cystic fibrosis. Rhinology. 2000;38:63–5.

    CAS  PubMed  Google Scholar 

  21. Donaldson JD, Gillespie CT. Observations on the efficacy of intranasal beclomethasone dipropionate in cystic fibrosis patients. J Otolaryngol. 1988;17:43–5.

    CAS  PubMed  Google Scholar 

  22. Mainz JG, Schädlich K, Schien C, Michl R, Schelhorn-Neise P, Koitschev A, Koitschev C, Keller PM, Riethmüller J, Wiedemann B, et al. Sinonasal inhalation of tobramycin vibrating aerosol in cystic fibrosis patients with upper airway Pseudomonas aeruginosa colonization: results of a randomized, double-blind, placebo-controlled pilot study. Drug Des Dev Ther. 2014;8:209.

    CAS  Google Scholar 

  23. Moss RB, King VV. Management of sinusitis in cystic fibrosis by endoscopic surgery and serial antimicrobial lavage: reduction in recurrence requiring surgery. Arch Otolaryngol Head Neck Surg. 1995;121:566–72.

    CAS  PubMed  Google Scholar 

  24. McCormick J, Cho DY, Lampkin B, Richman J, Hathorne H, Rowe SM, Woodworth BA. Ivacaftor improves rhinologic, psychologic, and sleep-related quality of life in G551D cystic fibrosis patients. Int Forum Allergy Rhinol. 2019;9:292–7.

    PubMed  Google Scholar 

  25. Chang EH, Tang XX, Shah VS, Launspach JL, Ernst SE, Hilkin B, Karp PH, Abou Alaiwa MH, Graham SM, Hornick DB, et al. Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor. Int Forum Allergy Rhinol. 2015;5:178–81.

    PubMed  Google Scholar 

  26. Lindstrom DR, Conley SF, Splaingard ML, Gershan WM. Ibuprofen therapy and nasal polyposis in cystic fibrosis patients. J Otolaryngol. 2007;36:309–14.

    PubMed  Google Scholar 

  27. Ayoub N, Thamboo A, Habib A-R, Nayak JV, Hwang PH. Determinants and outcomes of upfront surgery versus medical therapy for chronic rhinosinusitis in cystic fibrosis. Int Forum Allergy Rhinol. 2017;7(5):450–8.

    PubMed  Google Scholar 

  28. Khalid AN, Mace J, Smith TL. Outcomes of sinus surgery in adults with cystic fibrosis. Otolaryngol Head Neck Surg. 2009;141(3):358–63.

    PubMed  PubMed Central  Google Scholar 

  29. MacDonald KI, Gipsman A, Magit A, et al. Endoscopic sinus surgery in patients with cystic fibrosis: a systematic review and meta-analysis of pulmonary function. Rhinology. 2012;50(4):360–9.

    CAS  PubMed  Google Scholar 

  30. Liang J, Higgins TS, Ishman SL, Boss EF, Benke JR, Lin SY. Surgical management of chronic rhinosinusitis in cystic fibrosis: a systematic review. Int Forum Allergy Rhinol. 2013;3(10):814–22.

    PubMed  Google Scholar 

  31. Eggesbø HB, Søvik S, Dølvik S, Eiklid K, Kolmannskog F. CT characterization of developmental variations of the paranasal sinuses in cystic fibrosis. Acta Radiol. 2001;42(5):482–93.

    PubMed  Google Scholar 

  32. Do BAJ, Lands LC, Saint-Martin C, et al. Effect of the F508del genotype on outcomes of endoscopic sinus surgery in children with cystic fibrosis. Int J Pediatr Otorhinolaryngol. 2014;78(7):1133–7.

    PubMed  Google Scholar 

  33. Rickert S, Banuchi VE, Germana JD, Stewart MG, April MM. Cystic fibrosis and endoscopic sinus surgery: relationship between nasal polyposis and likelihood of revision endoscopic sinus surgery in patients with cystic fibrosis. Arch Otolaryngol Head Neck Surg. 2010;136(10):988–92.

    PubMed  Google Scholar 

  34. Jaberoo MC, Pulido MA, Saleh HA. Modified Lothrop procedure in cystic fibrosis patients: does it have a role? J Laryngol Otol. 2013;127(7):666–9.

    PubMed  Google Scholar 

  35. Virgin FW, Rowe SM, Wade MB, et al. Extensive surgical and comprehensive postoperative medical management for cystic fibrosis chronic rhinosinusitis. Am J Rhinol Allergy. 2012;26(1):70–5.

    PubMed  PubMed Central  Google Scholar 

  36. Alanin MC, Aanaes K, Hoiby N, et al. Sinus surgery postpones chronic gram-negative lung infection: cohort study of 106 patients with cystic fibrosis. Rhinology J. 2017;54(3):206–13.

    Google Scholar 

  37. Aanaes K, von Buchwald C, Hjuler T, Skov M, Alanin M, Johansen HK. The effect of sinus surgery with intensive follow-up on pathogenic sinus bacteria in patients with cystic fibrosis. Am J Rhinol Allergy. 2013;27(1):e1–4.

    PubMed  Google Scholar 

  38. Shatz A. Management of recurrent sinus disease in children with cystic fibrosis: a combined approach. Otolaryngol Head Neck Surg. 2006;135(2):248–52.

    PubMed  Google Scholar 

  39. Rosbe KW, Jones DT, Rahbar R, Lahiri T, Auerbach AD. Endoscopic sinus surgery in cystic fibrosis: do patients benefit from surgery? Int J Pediatr Otorhinolaryngol. 2001;61(2):113–9.

    CAS  PubMed  Google Scholar 

  40. Henriquez OA, Wolfenden LL, Stecenko A, DelGaudio JM, Wise SK. Endoscopic sinus surgery in adults with cystic fibrosis: effect on lung function, intravenous antibiotic use, and hospitalization. Arch Otolaryngol Head Neck Surg. 2012;138(12):1167–70.

    PubMed  Google Scholar 

  41. Triglia JM, Nicollas R. Nasal and sinus polyposis in children. Laryngoscope. 1997;107(7):963–6.

    CAS  PubMed  Google Scholar 

  42. Kovell LC, Wang J, Ishman SL, Zeitlin PL, Boss EF. Cystic fibrosis and sinusitis in children: outcomes and socioeconomic status. Otolaryngol Head Neck Surg. 2011;145(1):146–53.

    PubMed  Google Scholar 

  43. Holzmann D, Speich R, Kaufmann T, et al. Effects of sinus surgery in patients with cystic fibrosis after lung transplantation: a 10-year experience. Transplantation. 2004;77(1):134–6.

    PubMed  Google Scholar 

  44. Vital D, Hofer M, Benden C, Holzmann D, Boehler A. Impact of sinus surgery on pseudomonal airway colonization, bronchiolitis obliterans syndrome and survival in cystic fibrosis lung transplant recipients. Respiration. 2013;86(1):25–31.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to David A. Gudis .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Spielman, D., Safi, C., Overdevest, J., Gudis, D.A. (2020). Cystic Fibrosis and Chronic Rhinosinusitis: Surgical Management and Outcomes. In: Gudis, D., Schlosser, R. (eds) The Unified Airway. Springer, Cham. https://doi.org/10.1007/978-3-030-50330-7_9

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-50330-7_9

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-50329-1

  • Online ISBN: 978-3-030-50330-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics