Current Allergy and Asthma Reports

, Volume 12, Issue 2, pp 163–174 | Cite as

Pathogenesis and Management of Nasal Polyposis in Cystic Fibrosis

  • Jochen G. MainzEmail author
  • Assen Koitschev
Sinusitis (ML Kowalski, Section Editor)


Beginning in preschool age, during their lives, up to 50% of cystic fibrosis (CF) patients experience obstructing nasal polyposis (NP), which is rare in non-CF children. Pathogenetic factors of NP in general and especially in CF are still obscure. However, defective epithelial ion transport from mucosal glands plays a central role in CF, and viscous secretions impair mucociliary clearance, promoting chronic pathogen colonization and neutrophil-dominated chronic inflammation. Presently, CF-NP is not curable but can be clinically stabilized, though the large variety of proposed treatment modalities indicates a lack of standardization and of evidence of treatment efficacy. When conservative measures are exhausted, surgical intervention combining individually adapted endoscopic sinus surgery and supportive conservative treatment is performed. Topical steroids, approved as the gold standard for non-CF NP, may be beneficial, but they are discussed to be less effective in neutrophilic inflammation, and CF-specific antimicrobial and mucolytic therapy, as is true of all treatment modalities, urgently requires evaluation by controlled clinical trials within interdisciplinary networks.


Nasal polyposis Cystic fibrosis Upper airways Rhinosinusitis Nose Ostiomeatal complex Inflammation Mucociliary clearance Pathogen colonization Pseudomonas aeruginosa Staphylococcus aureus Topical therapy Steroids Mucolytics Antibiotics Macrolides Sinonasal inhalation Nasal lavage Rhinoscopy Sinonasal surgery FESS Neutrophil Interleukins IL-8 Quality of life 



The authors thank Franziska Doht and Dr. Ruth Michl for their support in preparation of the manuscript.


Dr. Mainz has served on advisory boards for Grunenthal, Gilead, and Pharmaxis; has had travel expenses covered by Novartis, Pari, Roche, Infectopharm, and MSD; and has conducted clinical trials on behalf of Luxemburgian and Germany CF Foundations and Pari Germany, Roche, Infectopharm, and Forest.

Dr. Koitschev reported no potential conflicts of interest relevant to this article.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989;245:1066–73.PubMedCrossRefGoogle Scholar
  2. 2.
    Kerem B, Rommens JM, Buchanan JA, Markiewicz D, Cox TK, Chakravarti A, et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989;245:1073–80.PubMedCrossRefGoogle Scholar
  3. 3.
    Eggesbo HB, Sovik S, Dolvik S, Eiklid K, Kolmannskog F. CT characterization of developmental variations of the paranasal sinuses in cystic fibrosis. Acta Radiol. 2001;42:482–93.PubMedGoogle Scholar
  4. 4.
    King VV. Upper respiratory disease, sinusitis, and polyposis. Clin Rev Allergy. 1991;9:143–57.PubMedGoogle Scholar
  5. 5.
    Koitschev A, Wolff A, Koitschev C, Preyer S, Ziebach R, Stern M. Routine otorhinolaryngological examination in patients with cystic fibrosis. HNO. 2006;54:361–4. 366, 368.PubMedCrossRefGoogle Scholar
  6. 6.
    Robertson JM, Friedman EM, Rubin BK. Nasal and sinus disease in cystic fibrosis. Paediatr Respir Rev. 2008;9:213–9.PubMedCrossRefGoogle Scholar
  7. 7.
    Feuillet-Fieux MN, Lenoir G, Sermet I, Elie C, Djadi-Prat J, Ferrec M, et al. Nasal polyposis and cystic fibrosis(CF): review of the literature. Rhinology. 2011;49:347–55.PubMedGoogle Scholar
  8. 8.
    Kingdom TT, Lee KC, FitzSimmons SC, Cropp GJ. Clinical characteristics and genotype analysis of patients with cystic fibrosis and nasal polyposis requiring surgery. Arch Otolaryngol Head Neck Surg. 1996;122:1209–13.PubMedCrossRefGoogle Scholar
  9. 9.
    Henriksson G, Westrin KM, Karpati F, Wikstrom AC, Stierna P, Hjelte L. Nasal polyps in cystic fibrosis: clinical endoscopic study with nasal lavage fluid analysis. Chest. 2002;121:40–7.PubMedCrossRefGoogle Scholar
  10. 10.
    De Gaudemar I, Contencin P, Van den Abbeele T, Munck A, Navarro J, Narcy P. Is nasal polyposis in cystic fibrosis a direct manifestation of genetic mutation or a complication of chronic infection? Rhinology. 1996;34:194–7.PubMedGoogle Scholar
  11. 11.
    Cimmino M, Cavaliere M, Nardone M, Plantulli A, Orefice A, Esposito V, et al. Clinical characteristics and genotype analysis of patients with cystic fibrosis and nasal polyposis. Clin Otolaryngol Allied Sci. 2003;28:125–32.PubMedCrossRefGoogle Scholar
  12. 12.
    Hadfield PJ, Rowe-Jones JM, Mackay IS. The prevalence of nasal polyps in adults with cystic fibrosis. Clin Otolaryngol Allied Sci. 2000;25:19–22.PubMedCrossRefGoogle Scholar
  13. 13.
    Fokkens W, Lund V, Bachert C, Clement P, Helllings P, Holmstrom M, et al. EAACI position paper on rhinosinusitis and nasal polyps executive summary. Allergy. 2005;60:583–601.PubMedCrossRefGoogle Scholar
  14. 14.
    Gysin C, Alothman GA, Papsin BC. Sinonasal disease in cystic fibrosis: clinical characteristics, diagnosis, and management. Pediatr Pulmonol. 2000;30:481–9.PubMedCrossRefGoogle Scholar
  15. 15.
    Drake-Lee AB, Morgan DW. Nasal polyps and sinusitis in children with cystic fibrosis. J Laryngol Otol. 1989;103:753–5.PubMedGoogle Scholar
  16. 16.
    Babinski D, Trawinska-Bartnicka M. Rhinosinusitis in cystic fibrosis: not a simple story. Int J Pediatr Otorhinolaryngol. 2008;72:619–24.PubMedCrossRefGoogle Scholar
  17. 17.
    Jorissen MB, De Boeck K, Cuppens H. Genotype-phenotype correlations for the paranasal sinuses in cystic fibrosis. Am J Respir Crit Care Med. 1999;159:1412–6.PubMedGoogle Scholar
  18. 18.
    Newton JR, Ah-See KW. A review of nasal polyposis. Ther Clin Risk Manag. 2008;4:507–12.PubMedGoogle Scholar
  19. 19.
    Taylor RF, Morgan DW, Nicholson PS, Mackay IS, Hodson ME, Pitt TL. Extrapulmonary sites of Pseudomonas aeruginosa in adults with cystic fibrosis. Thorax. 1992;47:426–8.PubMedCrossRefGoogle Scholar
  20. 20.
    •• Mainz JG, Naehrlich L, Schien M, Kading M, Schiller I, Mayr S, et al. Concordant genotype of upper and lower airways P aeruginosa and S aureus isolates in cystic fibrosis. Thorax. 2009;64:535–40. This study provided proof of the cross-relation of upper and lower airways (“one airway hypothesis”) by genotypically identical P. aeruginosa clones in CF upper and lower airways. It also identified NL as the most sensitive method to sample the upper airways. PubMedCrossRefGoogle Scholar
  21. 21.
    Ratjen F, Doring G. Cystic fibrosis. Lancet. 2003;361:681–9.PubMedCrossRefGoogle Scholar
  22. 22.
    •• Schraven SP, Wehrmann M, Wagner W, Blumenstock G, Koitschev A. Prevalence and histopathology of chronic polypoid sinusitis in pediatric patients with cystic fibrosis. J Cyst Fibros. 2011;10:181–6. This article provided a histologic comparison of CF to non-CF NP, identifying abundant mucus glands in CF-NP with mucus retention. PubMedCrossRefGoogle Scholar
  23. 23.
    Knipping S, Holzhausen HJ, Riederer A, Bloching M. Cystic fibrosis: ultrastructural changes of nasal mucosa. Eur Arch Otorhinolaryngol. 2007;264:1413–8.PubMedCrossRefGoogle Scholar
  24. 24.
    Bergoin C, Gosset P, Lamblin C, Bolard F, Turck D, Tonnel AB, et al. Cell and cytokine profile in nasal secretions in cystic fibrosis. J Cyst Fibros. 2002;1:110–5.PubMedCrossRefGoogle Scholar
  25. 25.
    Beju D, Meek WD, Kramer JC. The ultrastructure of the nasal polyps in patients with and without cystic fibrosis. J Submicrosc Cytol Pathol. 2004;36:155–65.PubMedGoogle Scholar
  26. 26.
    Henderson Jr WR, Chi EY. Degranulation of cystic fibrosis nasal polyp mast cells. J Pathol. 1992;166:395–404.PubMedCrossRefGoogle Scholar
  27. 27.
    Rowe-Jones JM, Shembekar M, Trendell-Smith N, Mackay IS. Polypoidal rhinosinusitis in cystic fibrosis: a clinical and histopathological study. Clin Otolaryngol Allied Sci. 1997;22:167–71.PubMedCrossRefGoogle Scholar
  28. 28.
    Claeys S, Van Hoecke H, Holtappels G, Gevaert P, De Belder T, Verhasselt B, et al. Nasal polyps in patients with and without cystic fibrosis: a differentiation by innate markers and inflammatory mediators. Clin Exp Allergy. 2005;35:467–72.PubMedCrossRefGoogle Scholar
  29. 29.
    Derycke L, Zhang N, Holtappels G, Dutre T, Bachert C. IL-17A as a regulator of neutrophil survival in nasal polyp disease of patients with and without cystic fibrosis. J Cyst Fibros. 2011. doi: 10.1016/j/jcf.2011.11.007
  30. 30.
    Rachelefsky GS, Osher A, Dooley RE, Ank B, Stiehm ER. Coexistent respiratory allergy and cystic fibrosis. Am J Dis Child. 1974;128:355–9.PubMedGoogle Scholar
  31. 31.
    Biewenga J, Stoop AE, Baker HE, Swart SJ, Nauta JJ, van Kamp GJ, et al. Nasal secretions from patients with polyps and healthy individuals, collected with a new aspiration system: evaluation of total protein and immunoglobulin concentrations. Ann Clin Biochem. 1991;28(Pt 3):260–6.PubMedGoogle Scholar
  32. 32.
    Noah TL, Black HR, Cheng PW, Wood RE, Leigh MW. Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis. J Infect Dis. 1997;175:638–47.PubMedCrossRefGoogle Scholar
  33. 33.
    Wang D, Brihaye P, Smitz J, De Waele M, Clement P. Inflammatory cells and mediator concentrations in nasal secretions of patients with cystic fibrosis. Acta Otolaryngol. 1996;116:472–6.PubMedCrossRefGoogle Scholar
  34. 34.
    Lesprit E, Escudier E, Roger G, Pruliere V, Lenoir G, Reinert P, et al. Characterization of inflammatory reaction in upper airways of cystic fibrosis patients. Histol Histopathol. 2000;15:395–402.PubMedGoogle Scholar
  35. 35.
    Khan TZ, Wagener JS, Bost T, Martinez J, Accurso FJ, Riches DW. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 1995;151:1075–82.PubMedGoogle Scholar
  36. 36.
    Wolter JM, Rodwell RL, Bowler SD, McCormack JG. Cytokines and inflammatory mediators do not indicate acute infection in cystic fibrosis. Clin Diagn Lab Immunol. 1999;6:260–5.PubMedGoogle Scholar
  37. 37.
    Lund VJ, Mackay IS. Staging in rhinosinusitus. Rhinology. 1993;31:183–4.PubMedGoogle Scholar
  38. 38.
    Lund VJ, Kennedy DW. Staging for rhinosinusitis. Otolaryngol Head Neck Surg. 1997;117:S35–40.PubMedCrossRefGoogle Scholar
  39. 39.
    Gliklich RE, Metson R. The health impact of chronic sinusitis in patients seeking otolaryngologic care. Otolaryngol Head Neck Surg. 1995;113:104–9.PubMedCrossRefGoogle Scholar
  40. 40.
    Eggesbo HB, Dolvik S, Stiris M, Sovik S, Storrosten OT, Kolmannskog F. Complementary role of MR imaging of ethmomaxillary sinus disease depicted at CT in cystic fibrosis. Acta Radiol. 2001;42:144–50.PubMedGoogle Scholar
  41. 41.
    Mainz JG, Koitschev A. Management of chronic rhinosinusitis in CF. J Cyst Fibros. 2009;8 Suppl 1:S10–4.Google Scholar
  42. 42.
    Martinez-Devesa P, Patiar S. Oral steroids for nasal polyps. Cochrane Database Syst Rev. 2011;CD005232.Google Scholar
  43. 43.
    Harvey R, Hannan SA, Badia L, Scadding G. Nasal saline irrigations for the symptoms of chronic rhinosinusitis. Cochrane Database Syst Rev. 2007;CD006394.Google Scholar
  44. 44.
    Talbot AR, Herr TM, Parsons DS. Mucociliary clearance and buffered hypertonic saline solution. Laryngoscope. 1997;107:500–3.PubMedCrossRefGoogle Scholar
  45. 45.
    Bachmann G, Hommel G, Michel O. Effect of irrigation of the nose with isotonic salt solution on adult patients with chronic paranasal sinus disease. Eur Arch Otorhinolaryngol. 2000;257:537–41.PubMedCrossRefGoogle Scholar
  46. 46.
    Wormald PJ, Cain T, Oates L, Hawke L, Wong I. A comparative study of three methods of nasal irrigation. Laryngoscope. 2004;114:2224–7.PubMedCrossRefGoogle Scholar
  47. 47.
    Moller W, Schuschnig U, Meyer G, Mentzel H, Keller M. Ventilation and drug delivery to the paranasal sinuses: studies in a nasal cast using pulsating airflow. Rhinology. 2008;46:213–20.PubMedGoogle Scholar
  48. 48.
    •• Moller W, Schuschnig U, Khadem Saba G, Meyer G, Junge-Hulsing B, Keller M, et al. Pulsating aerosols for drug delivery to the sinuses in healthy volunteers. Otolaryngol Head Neck Surg. 2010;142:382–8. This study provided proof of deposition of vibrating aerosols into paranasal sinuses in vivo by scintigraphy, which is not achieved in nasal inhalation without pulsation or by nasal sprays or drops. PubMedCrossRefGoogle Scholar
  49. 49.
    •• Mainz JG, Schiller I, Ritschel C, Mentzel HJ, Riethmuller J, Koitschev A, et al. Sinonasal inhalation of dornase alfa in CF: a double-blind placebo-controlled cross-over pilot trial. Auris Nasus Larynx. 2011;38:220–7. This was the first study to assess sinonasal inhalation with vibrating aerosols in CRS patients with significantly positive results in SNOT-20 already in the pilot trial. PubMedCrossRefGoogle Scholar
  50. 50.
    Koch C, Hoiby N. Pathogenesis of cystic fibrosis. Lancet. 1993;341:1065–9.PubMedCrossRefGoogle Scholar
  51. 51.
    Shah PL, Scott SF, Knight RA, Marriott C, Ranasinha C, Hodson ME. In vivo effects of recombinant human DNase I on sputum in patients with cystic fibrosis. Thorax. 1996;51:119–25.PubMedCrossRefGoogle Scholar
  52. 52.
    Jones AP, Wallis C. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2010;CD001127.Google Scholar
  53. 53.
    Raynor EM, Butler A, Guill M, Bent 3rd JP. Nasally inhaled dornase alfa in the postoperative management of chronic sinusitis due to cystic fibrosis. Arch Otolaryngol Head Neck Surg. 2000;126:581–3.PubMedGoogle Scholar
  54. 54.
    Cimmino M, Nardone M, Cavaliere M, Plantulli A, Sepe A, Esposito V, et al. Dornase alfa as postoperative therapy in cystic fibrosis sinonasal disease. Arch Otolaryngol Head Neck Surg. 2005;131(12):1097–101. Google Scholar
  55. 55.
    • Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev. 2009;CD001506. This article discusses the significant effects of bronchial inhalation of hypertonic saline in CF. Google Scholar
  56. 56.
    Burgel PR, Cardell LO, Ueki IF, Nadel JA. Intranasal steroids decrease eosinophils but not mucin expression in nasal polyps. Eur Respir J. 2004;24:594–600.PubMedCrossRefGoogle Scholar
  57. 57.
    Lund VJ, Flood J, Sykes AP, Richards DH. Effect of fluticasone in severe polyposis. Arch Otolaryngol Head Neck Surg. 1998;124:513–8.PubMedGoogle Scholar
  58. 58.
    Thomas M, Yawn BP, Price D, Lund V, Mullol J, Fokkens W. EPOS primary care guidelines: European position paper on the primary care diagnosis and management of rhinosinusitis and nasal polyps 2007 - a summary. Prim Care Respir J. 2008;17:79–89.PubMedCrossRefGoogle Scholar
  59. 59.
    Donaldson JD, Gillespie CT. Observations on the efficacy of intranasal beclomethasone dipropionate in cystic fibrosis patients. J Otolaryngol. 1988;17:43–5.PubMedGoogle Scholar
  60. 60.
    Costantini D, Di Cicco M, Giunta A, Amabile G. Nasal polyposis in cystic fibrosis treated by beclomethasone dipropionate. Acta Univ Carol Med. 1990;36:220–1.Google Scholar
  61. 61.
    • Beer H, Southern KW, Swift AC. Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis. Cochrane Database Syst Rev. 2011;CD008253. This is a review on TS therapy in CF-NP. Google Scholar
  62. 62.
    Stern RC, Boat TF, Wood RE, Matthews LW, Doershuk CF. Treatment and prognosis of nasal polyps in cystic fibrosis. Am J Dis Child. 1982;136:1067–70.PubMedGoogle Scholar
  63. 63.
    Hadfield PJ, Rowe-Jones JM, Mackay IS. A prospective treatment trial of nasal polyps in adults with cystic fibrosis. Rhinology. 2000;38:63–5.PubMedGoogle Scholar
  64. 64.
    Davidson TM, Murphy C, Mitchell M, Smith C, Light M. Management of chronic sinusitis in cystic fibrosis. Laryngoscope. 1995;105:354–8.PubMedCrossRefGoogle Scholar
  65. 65.
    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.PubMedCrossRefGoogle Scholar
  66. 66.
    Graham SM, Launspach JL, Welsh MJ, Zabner J. Sequential magnetic resonance imaging analysis of the maxillary sinuses: implications for a model of gene therapy in cystic fibrosis. J Laryngol Otol. 1999;113:329–35.PubMedGoogle Scholar
  67. 67.
    • Ryan G, Singh M, Dwan K. Inhaled antibiotics for long-term therapy in cystic fibrosis. Cochrane Database Syst Rev. 2011;CD001021. This article provides proof of the therapeutic effect and safety of bronchial antibiotic inhalation in CF lung disease. Google Scholar
  68. 68.
    Mainz JG, Schien C, Schädlich K, Pfister W, Schelhorn-Neise P, Koitschev A, et al. Sinonasal inhalation of Tobramycin in CF-patients with P. aeruginosa-colonization of the upper airways - results of a multicentric placebo controlled pilot study - Oral and poster: 34rd European Cystic Fibrosis Conference, 06.2011, Hamburg. J Cyst Fibros. 2011;10S:21.Google Scholar
  69. 69.
    Mainz JG, Michl R, Pfister W, Beck JF. Cystic Fibrosis Upper Airways Primary Colonization with P. aeruginosa: Eradicated by Sinonasal Antibiotic Inhalation. Am J Respir Crit Care Med. 2011;184(9):1089–90.Google Scholar
  70. 70.
    Mainz JG, Hentschel J, Schien C, Cramer N, Pfister W, Beck JF, Tümmler B. P. aeruginosa persists in the upper airways after lung transplantation. J Cyst Fibros. 2011. doi: 10.1016/j.jcf.2011.10.009.
  71. 71.
    Jaffe A, Francis J, Rosenthal M, Bush A. Long-term azithromycin may improve lung function in children with cystic fibrosis. Lancet. 1998;351:420.PubMedCrossRefGoogle Scholar
  72. 72.
    Tamaoki J, Kadota J, Takizawa H. Clinical implications of the immunomodulatory effects of macrolides. Am J Med. 2004;117(Suppl 9A):5S–11S.PubMedGoogle Scholar
  73. 73.
    Nonaka M, Pawankar R, Tomiyama S, Yagi T. A macrolide antibiotic, roxithromycin, inhibits the growth of nasal polyp fibroblasts. Am J Rhinol. 1999;13:267–72.PubMedCrossRefGoogle Scholar
  74. 74.
    Suzuki H, Shimomura A, Ikeda K, Furukawa M, Oshima T, Takasaka T. Inhibitory effect of macrolides on interleukin-8 secretion from cultured human nasal epithelial cells. Laryngoscope. 1997;107:1661–6.PubMedCrossRefGoogle Scholar
  75. 75.
    Yamada T, Fujieda S, Mori S, Yamamoto H, Saito H. Macrolide treatment decreased the size of nasal polyps and IL-8 levels in nasal lavage. Am J Rhinol. 2000;14:143–8.PubMedCrossRefGoogle Scholar
  76. 76.
    Videler WJ, Badia L, Harvey RJ, Gane S, Georgalas C, van der Meulen FW, et al. Lack of efficacy of long-term, low-dose azithromycin in chronic rhinosinusitis: a randomized controlled trial. Allergy. 2011;66:1457–68.PubMedCrossRefGoogle Scholar
  77. 77.
    Konstan MW, Byard PJ, Hoppel CL, Davis PB. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med. 1995;332:848–54.PubMedCrossRefGoogle Scholar
  78. 78.
    Lindstrom DR, Conley SF, Splaingard ML, Gershan WM. Ibuprofen therapy and nasal polyposis in cystic fibrosis patients. J Otolaryngol. 2007;36:309–14.PubMedCrossRefGoogle Scholar
  79. 79.
    Mainz JG, Schien M, Naehrlich L, Käding M, Thoss K, Frey G, et al. Prevalence of CF-related chronic rhinosinusitis - results from a multicentre interdisciplinary study. In 33rd European Cystic Fibrosis Conference; 2010. J Cyst Fibros. 2010:9 S1, 118.Google Scholar
  80. 80.
    Beata RN, Wojciech F, Stanislaw S, Ryszard M. The influence of anti-inflammatory drugs on the proliferation of fibroblast derived from nasal polyps. Auris Nasus Larynx. 2005;32:225–9.PubMedCrossRefGoogle Scholar
  81. 81.
    Saji F, Nonaka M, Pawankar R. Expression of RANTES by IL-1 beta and TNF-alpha stimulated nasal polyp fibroblasts. Auris Nasus Larynx. 2000;27:247–52.PubMedCrossRefGoogle Scholar
  82. 82.
    Hamilos DL, Leung DY, Huston DP, Kamil A, Wood R, Hamid Q. GM-CSF, IL-5 and RANTES immunoreactivity and mRNA expression in chronic hyperplastic sinusitis with nasal polyposis (NP). Clin Exp Allergy. 1998;28:1145–52.PubMedCrossRefGoogle Scholar
  83. 83.
    Gevaert P, Van Bruaene N, Cattaert T, Van Steen K, Van Zele T, Acke F, et al. Mepolizumab, a humanized anti-IL-5 mAb, as a treatment option for severe nasal polyposis. J Allergy Clin Immunol. 2011;128:989–95. e981–988.PubMedCrossRefGoogle Scholar
  84. 84.
    Schulte DL, Kasperbauer JL. Safety of paranasal sinus surgery in patients with cystic fibrosis. Laryngoscope. 1998;108:1813–5.PubMedCrossRefGoogle Scholar
  85. 85.
    Messerklinger W. Background and evolution of endoscopic sinus surgery. Ear Nose Throat J. 1994;73:449–50.PubMedGoogle Scholar
  86. 86.
    Keck T, Rozsasi A. Medium-term symptom outcomes after paranasal sinus surgery in children and young adults with cystic fibrosis. Laryngoscope. 2007;117:475–9.PubMedCrossRefGoogle Scholar
  87. 87.
    • 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;136:988–92. This is an analysis of recurrences after CF-NP surgery. PubMedCrossRefGoogle Scholar
  88. 88.
    Madonna D, Isaacson G, Rosenfeld RM, Panitch H. Effect of sinus surgery on pulmonary function in patients with cystic fibrosis. Laryngoscope. 1997;107:328–31.PubMedCrossRefGoogle Scholar
  89. 89.
    • Osborn AJ, Leung R, Ratjen F, James AL. Effect of endoscopic sinus surgery on pulmonary function and microbial pathogens in a pediatric population with cystic fibrosis. Arch Otolaryngol Head Neck Surg. 2011;137:542–7. This article provides retrospective identification of lacking effects of sinonasal surgery in CF on pulmonary function, which requires further prospective investigation. PubMedCrossRefGoogle Scholar
  90. 90.
    Kayarkar R, Clifton NJ, Woolford TJ. An evaluation of the best head position for instillation of steroid nose drops. Clin Otolaryngol Allied Sci. 2002;27:18–21.PubMedCrossRefGoogle Scholar
  91. 91.
    Raghavan U, Jones NS. A prospective randomized blinded cross-over trial using nasal drops in patients with nasal polyposis: an evaluation of effectiveness and comfort level of two head positions. Am J Rhinol. 2006;20:397–400.PubMedCrossRefGoogle Scholar
  92. 92.
    Johansson L, Oberg D, Melen I, Bende M. Do topical nasal decongestants affect polyps? Acta Otolaryngol. 2006;126:288–90.PubMedCrossRefGoogle Scholar
  93. 93.
    Graf PM. Rhinitis medicamentosa. Clin Allergy Immunol. 2007;19:295–304.PubMedGoogle Scholar
  94. 94.
    Helbling A, Baumann A, Hanni C, Caversaccio M. Amphotericin B nasal spray has no effect on nasal polyps. J Laryngol Otol. 2006;120:1023–5.PubMedCrossRefGoogle Scholar
  95. 95.
    Heintz B, Schlenter WW, Kirsten R, Nelson K. Clinical efficacy of Broncho-Vaxom in adult patients with chronic purulent sinusitis–a multi-centric, placebo-controlled, double-blind study. Int J Clin Pharmacol Ther Toxicol. 1989;27:530–4.PubMedGoogle Scholar
  96. 96.
    Cuyler JP. Follow-up of endoscopic sinus surgery on children with cystic fibrosis. Arch Otolaryngology Head Neck Surg. 1992;118:505–6.CrossRefGoogle Scholar
  97. 97.
    Nishioka GJ, Barbero GJ, Konig P, Parsons DS, Cook PR, Davis WE. Symptom outcome after functional endoscopic sinus surgery in patients with cystic fibrosis: a prospective study. Otolaryngol Head Neck Surg. 1995;113:440–5.PubMedCrossRefGoogle Scholar
  98. 98.
    Coste A, Idrissi F, Beautru R, Lenoir G, Reinert P, Manach Y, et al. Endoscopic endonasal ethmoidectomy in severe sinusitis of cystic fibrosis. Mid-term results in 12 patients. Ann Otolaryngol Chir Cervicofac. 1997;114(4):99–104.Google Scholar
  99. 99.
    Triglia JM, Nicollas R. Nasal and sinus polyposis in children. Laryngoscope. 1997;107:963–6.PubMedCrossRefGoogle Scholar
  100. 100.
    Yung MW, Gould J, Upton GJ. Nasal polyposis in children with cystic fibrosis: a long-term follow-up study. Ann Otol Rhinol Laryngol. 2002;111:1081–6.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Cystic Fibrosis Center, Department of Paediatrics, Paediatric PulmonologyJena University HospitalJenaGermany
  2. 2.Division of Paediatric Otorhinolaryngology and Otology, Department of OtorhinolaryngologyKlinikum StuttgartStuttgartGermany

Personalised recommendations