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Chronic Rhinosinusitis in Children with Cystic Fibrosis

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Pediatric Rhinosinusitis
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Abstract

Chronic rhinosinusitis (CRS) is characterized by infection and inflammation of the nose and paranasal sinuses. CRS in children with cystic fibrosis (CF) is a challenging clinical condition and requires unique approaches to evaluation and both medical and surgical treatment. As the pathogenesis becomes clearer through ongoing clinical and basic science research, however, our ability to treat CRS in children with CF both effectively and safely should continue to improve. A number of studies over the past few years support the concept of the unified airway theory, suggesting an important relationship between disease and bacterial pathogens of the sinuses and the lower respiratory tract. While surgery, including adenoidectomy and functional endoscopic sinus surgery (FESS), can be beneficial in pediatric CF patients with CRS, the effect of surgery on lung pathogens and pulmonary function remains an area for further investigation. Knowing when a child with CF and CRS is likely to benefit from surgery can be difficult. In this chapter, we review the pathogenesis, evaluation, and treatment of CRS in children with CF and propose a framework for surgical decision-making based on outcomes for nasal polyposis, CRS symptoms, and pulmonary function.

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References

  1. Anand VK. Epidemiology and economic impact of rhinosinusitis. Ann Otol Rhinol Laryngol Suppl. 2004;193:3–5.

    Article  PubMed  Google Scholar 

  2. Benninger MS, Ferguson BJ, Hadley JA, et al. Adult chronic rhinosinusitis: definitions, diagnosis, epidemiology, and pathophysiology. Otolaryngol Head Neck Surg. 2003;129(3. Suppl):S1–32.

    Article  PubMed  Google Scholar 

  3. Lusk R. Pediatric chronic rhinosinusitis. Curr Opin Otolaryngol Head Neck Surg. 2006;14:393–6.

    Article  PubMed  Google Scholar 

  4. Tipirneni KB, Woodworth BA. Medical and surgical advancements in the management of cystic fibrosis chronic rhinosinusitis. Curr Otorhinolaryngol Rep. 2017;5(1):24–34.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Li W, Soave D, Miller MR, Keenan K, Lin F, Gong J, Chiang T, et al. Unraveling the complex genetic model for cystic fibrosis: Pleiotropic effects of modifier genes on early cystic fibrosis-related morbidities. Hum Genet. 2014;133(2):151–61.

    Article  CAS  PubMed  Google Scholar 

  6. Corvol H, Blackman SM, Boëlle PY, Gallins PJ, Pace RG, Stonebraker JR, et al. Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis. Nat Commun. 2015;6(1):8382.

    Article  CAS  PubMed  Google Scholar 

  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(3):347–55.

    CAS  PubMed  Google Scholar 

  8. Nelson J, Karempelis P, Dunitz J, Hunter R, Boyer H. Pulmonary aspiration of sinus secretions in patients with cystic fibrosis. Int Forum Allergy Rhinol. 2018;8(3):385–8. https://doi.org/10.1002/alr.22043.

    Article  PubMed  Google Scholar 

  9. 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(1):122–7.

    Article  PubMed  Google Scholar 

  10. Abreu NA, Nagalingam NA, Song Y, Roediger FC, Pletcher SD, Goldberg AN, Lynch SV. Sinus microbiome diversity depletion and Cornybactrium tuberculostearicum enrichment mediates rhinosinusitis. Sci Transl Med. 2012;12:4(151):151ra124.

    Google Scholar 

  11. Phipps CD, Wood E, Gibson WS, Cochran WJ. Gastroesophageal reflux contributing to chronic sinus disease in children. Arch Otolaryngol Head Neck Surg. 2000;126:831–6.

    Article  CAS  PubMed  Google Scholar 

  12. Bothwell MR, Parsons DS, Talbot A, Barbero GJ, Wilder B. Outcome of reflux therapy on pediatric chronic sinusitis. Otolaryngol Head Neck Surg. 1999;121(3):255–62.

    Article  CAS  PubMed  Google Scholar 

  13. Møller ME, Alanin MC, Grønhøj C, Aanæs K, Høiby N, von Buchwald C. Sinus bacteriology in patients with cystic fibrosis of primary ciliary dyskinesia: a systematic review. Am J Rhinol Allergy. 2017;31(5):293–8.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Comstock RH, Lam K, Mikula S. Topical antibiotic therapy of chronic rhinosinusitis. Curr Infect Dis Rep. 2010;12:88–95.

    Article  PubMed  Google Scholar 

  15. Wang M, Shi P, Chen B, Zhang H, Jian J, Chen X, et al. The role of superantigens in chronic rhinosinusitis with nasal polyps. ORL J Otorhinolaryngol Relat Spec. 2008;70(2):97–103.

    Article  CAS  PubMed  Google Scholar 

  16. Sanclement JA, Webster P, Thomas J, Ramadan HH. Bacterial biofilms in surgical specimens of patients with chronic rhinosinusitis. Laryngoscope. 2005;115(4):578–82.

    Article  PubMed  Google Scholar 

  17. Calò L, Passàli GC, Galli J, Fadda G, Paludetti G. Role of biofilms in chronic inflammatory diseases of the upper airways. Adv Otorhinolaryngol. 2011;72:93–6.

    PubMed  Google Scholar 

  18. Klodzińska SN, Priemel PA, Rades R, Mørck Nielsen H. Inhalable antimicrobials for treatment of bacterial-associated sinusitis in cystic fibrosis patients: challenges and drug delivery approaches. Int J Mol Sci. 2016;17(10):1688–707.

    Article  PubMed Central  CAS  Google Scholar 

  19. Sosnay PR, Siklosi KR, Van Goor F, Kaniecki K, Yu H, Sharma N, et al. Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Gentet. 2013;45(10):1160–7.

    Article  CAS  Google Scholar 

  20. Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, et al. From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol Biol Cell. 2016;27(3):424–33.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. MacKenzie T, Gifford AH, Sabadosa KA, Quinton HB, Knapp EA, Goss CH, et al. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the cystic fibrosis foundation patient registry. Ann Intern Med. 2014;161(4):233–41.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Abuzeid WM, Song C, Fastenberg JH, Fang CH, Ayoub N, Jerschow E, et al. Correlations between cystic fibrosis genotype and sinus disease severity in chronic rhinosinusitis. Laryngoscope. 2017;128(8):1752–8.

    Article  PubMed  Google Scholar 

  23. Aanaes K, Johansen HK, Slov M, Buchvald FF, Hjuler T, Pressler T, et al. Clinical effects of sinus surgery and adjuvant therapy in cystic fibrosis patients: can chronic lung infections be postponed? Rhinology. 2013;51(3):222–30.

    Article  CAS  PubMed  Google Scholar 

  24. Leo G, Triulzi F, Incorvaia C. Sinus imaging for diagnosis of chronic rhinosinusitis in children. Curr Allergy Asthma Rep. 2012;12(2):136–43.

    Article  PubMed  Google Scholar 

  25. Brenner DJ, Hall EJ. Computed tomography – an increasing source of radiation exposure. N Engl J Med. 2007;357:2277–84.

    Article  CAS  PubMed  Google Scholar 

  26. Setzen G, Ferguson BJ, Han JK, Rhee JS, Cornelius RS, Froum SJ, et al. Clinical consensus statement: appropriate use of computed tomography for paranasal sinus disease. Otolaryngol Head Neck Surg. 2012;147(5):808–16.

    Article  PubMed  Google Scholar 

  27. Elwany S, Helmy SA, El-Reweny EM, Harfoush R, Sobhy A. Endoscopically directed middle meatal cultures vs computed tomography scans in the diagnosis of bacterial sinusitis in intensive care units. J Crit Care. 2012;27(3):315:1–5.

    Article  Google Scholar 

  28. Andersen D. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathological study. Am J Dis Child. 1938;56:344–99.

    Article  Google Scholar 

  29. Kerem B, Rommens J, Buchanan J, Markiewicz D, Cox TK, Chakravarti A, et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989;245:1073–80.

    Article  CAS  PubMed  Google Scholar 

  30. Thomas M, Yawn B, 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(2):79–89.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Makary CA, Ramadan HH. The role of sinus surgery in children. Laryngoscope. 2013;00:1–5.

    Google Scholar 

  32. Sobol SE, Samadi DS, Kazahaya K, Tom LW. Trends in the management of pediatric chronic sinusitis: survey of the American Society of Pediatric Otolaryngology. Laryngoscope. 2005;115:78–80.

    Article  PubMed  Google Scholar 

  33. Shaikh N, Wald ER, Pi M. Decongestants, antihistamines and nasal irrigation for acute sinusitis in children. Cochrane Database Syst Rev. 2012;9:CD007909.

    Google Scholar 

  34. Wei JL, Sykes KJ, Johnson P, He J, Mayo MS. Safety and efficacy of once-daily nasal irrigation for the treatment of pediatric chronic rhinosinusitis. Laryngoscope. 2011;121(9):1989–2000.

    PubMed  Google Scholar 

  35. Khianey R, Oppenheimer J. Is nasal saline irrigation all it is cracked up to be? Ann Allergy Asthma Immunol. 2012;109(1):20–8.

    Article  CAS  PubMed  Google Scholar 

  36. Corcoran TE, Godovchik JE, Donn KH, Busick DR, Goralski J, Locke LW, et al. Overnight delivery of hypertonic saline by nasal cannula aerosol for cystic fibrosis. Pediatr Pulmonol. 2017;52(9):1142–9.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Zalmanovici A, Yaphe J. Intranasal steroids for acute sinusitis. Cochrane Database Syst Rev. 2009;4:CD005149.

    Google Scholar 

  38. Venekamp RP, Thompson MJ, Hayward G, Heneghan CJ, Del Mar CB, Perera R, et al. Systemic corticosteroids for acute sinusitis. Cochrane Database Syst Rev. 2014;3:CD008115.

    Google Scholar 

  39. Skoner D. Update of growth effects of inhaled and intranasal corticosteroids. Curr Opin Allergy Clin Immunol. 2002;2(1):7–10.

    Article  PubMed  Google Scholar 

  40. Dijkstra MD, Ebbens FA, Poublon RM, Fokkens WJ. Fluticasone propionate aqueous nasal spray does not influence the recurrence rate of chronic rhinosinusitis and nasal polyps 1 year after functional endoscopic sinus surgery. Clin Exp Allergy. 2004;34(9):1395–400.

    Article  CAS  PubMed  Google Scholar 

  41. Aukema AA, Mulder PG, Fokkens WJ. Treatment of nasal polyposis and chronic rhinosinusitis with fluticasone propionate nasal drops reduces need for sinus surgery. J Allergy Clin Immunol. 2005;115(5):1017–23.

    Article  CAS  PubMed  Google Scholar 

  42. Weber SAT, Iyomasa RM, Corrêa CC, Florentino WNM, Ferrari GF. Nasal polyposis in cystic fibrosis: follow-up of children and adolescents for a 3-year period. Braz J Otorhinolaryngol. 2017;83(6):677–82.

    Article  PubMed  Google Scholar 

  43. Beer H, Southern KW, Swift AC. Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis. Cochrane Database Syst Rev. 2015;6:CD008253.

    Google Scholar 

  44. Fokkens WJ, Lund VJ, Mullol J, Bachert C, Alobid I, Baroody F, et al. EPOS 2012: European position paper on rhinosinusitis and nasal polyps 2012 A summary for otorhinolaryngologists. Rhinology. 2012;50:1–12.

    Article  PubMed  Google Scholar 

  45. 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.

    Article  PubMed  Google Scholar 

  46. Adelson RT, Adappa ND. What is the proper role of oral antibiotics in the treatment of patients with chronic sinusitis? Curr Opin Otolaryngol Head Neck Surg. 2013;21(1):61–8.

    Article  PubMed  Google Scholar 

  47. Scheinberg PA, Otsuji A. Nebulized antibiotics for the treatment of acute exacerbations of chronic rhinosinusitis. Ear Nose Throat J. 2002;81:648–52.

    Article  PubMed  Google Scholar 

  48. Vaughan WC, Carvalho G. Use of nebulized antibiotics for acute infections in chronic sinusitis. Otolaryngol Head Neck Surg. 2002;127:558–68.

    Article  PubMed  Google Scholar 

  49. Uren B, Psaltis A, Wormald PJ. Nasal lavage with mupirocin for the treatment of surgically recalcitrant chronic rhinosinusitis. Laryngoscope. 2008;118:1677–80.

    Article  CAS  PubMed  Google Scholar 

  50. Mainz JG, Schädlich K, Schien C, Michl R, Schelhorn-Neise P, Koitschev A, 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 Devel Ther. 2014;10(8):209–17.

    Article  CAS  Google Scholar 

  51. Tatar EC, Tatar I, Ocal B, Korkmaz H, Saylam G, Ozdek A, et al. Prevalence of biofilms and their response to medical treatment in chronic rhinosinusitis without polyps. Otolaryngol Head Neck Surg. 2012;146(4):669–75.

    Article  PubMed  Google Scholar 

  52. Shah GB, De Keyzer L, Russell JA, Halderman A. Treatment of chronic rhinosinusitis with dornase alfa in patients with cystic fibrosis: a systematic review. Int Forum Allergy Rhinol. 2018;8(6):729–36.

    Article  PubMed  Google Scholar 

  53. Ren CL, Morgan RL, Oermann C, Resnick HE, Brady C, Campbell A, et al. Cystic Fibrosis Foundation Pulmonary Guidelines. Use of cystic fibrosis transmembrane conductance regulator modulator therapy in patients with cystic fibrosis. Ann Am Thorac Soc. 2018;15(3):271–80.

    Article  PubMed  Google Scholar 

  54. Accurso F, Rowe S, Clancy J, Boyle MP, Dunitz JM, Durie PR, et al. Effect of Vx-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med. 2010;363:1991–2003.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Davies JC, Wainwright C, Canny G, Chilvers MA, Howenstine MS, Munck A, et al. Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation. Am J Respir Crit Care Med. 2013;187:1219–25.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  56. McKone EF, Borowitz D, Drevinek P, Griese M, Konstan MW, Wainwright C, et al. Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST). Lancet Respir Med. 2014;2:902–10.

    Article  CAS  PubMed  Google Scholar 

  57. Wainwright CE, Elborn J, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. Lumacaftor- ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373:220–31.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  58. Quittner AL, Modi AC, Wainwright C, Otto K, Kirihara J, Montgomery AB. Determination of the minimal clinically important difference scores for the cystic fibrosis questionnaire-revised respiratory symptom scale in two populations of patients with cystic fibrosis and chronic pseudomonas aeruginosa airway infection. Chest. 2009;135:1610–8.

    Article  PubMed  PubMed Central  Google Scholar 

  59. Ren HY, Grove DE, De La Rosa O, Houck SA, Sopha P, Van Goor F, et al. VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1. Mol Biol Cell. 2013;24(19):3016–24.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  60. Donaldson SH, Pilewski JM, Griese M, Cooke J, Viswanathan L, Tullis E, et al. Tezacaftor/ivacaftor in subjects with cystic fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR. Am J Respir Crit Care Med. 2018;197(2):214–24.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Rowe SM, Daines C, Ringshausen FC, Kerem E, Wilson J, Tullis E, et al. Tezacaftor-ivacaftor in residual-function heterozygotes with cystic fibrosis. N Engl J Med. 2017;377(21):2024–35.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Taylor-Cousar JL, Munck A, McKone EF, van der Ent CK, Moeller A, Simard C, et al. Tezacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del. N Engl J Med. 2017;377(21):2013–23.

    Article  CAS  PubMed  Google Scholar 

  63. Conger BT, Zhang S, Skinner D, Hicks SB, Sorscher EJ, Rowe SM, et al. Comparison of cystic fibrosis transmembrane conductance regulator (CFTR) and ciliary beat frequency activation by the CFTR modulators genistein, VRT-532, and UCCF-152 in primary sinonasal epithelial cultures. JAMA Otolaryngol Head Neck Surg. 2013;139(8):822–7.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Ramsey B, Richardson MA. Impact of sinusitis in cystic fibrosis. J Allergy Clin Immunol. 1992;90(3,Pt2):547–52.

    Article  CAS  PubMed  Google Scholar 

  65. Coticchia J, Zuliani G, Coleman C, Carron M, Gurrola J, Haupert M, et al. Biofilm surface area in the pediatric nasopharynx: chronic rhinosinusitis vs obstructive sleep apnea. Arch Otolaryngol Head Neck Surg. 2007;133(2):110–4.

    Article  PubMed  Google Scholar 

  66. Brietzke SE, Brigger MT. Adenoidectomy outcomes in pediatric rhinosinusitis: a meta-analysis. Int J Pediatr Otorhinolaryngol. 2008;72(10):1541–5.

    Article  PubMed  Google Scholar 

  67. Ramadan HH. Surgical management of chronic sinusitis in children. Laryngoscope. 2004;114(12):2103–9.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  PubMed Central  Google Scholar 

  69. Ramadan HH, McLaughlin K, Josephson G, Rimell F, Bent J, Parikh SR. Balloon catheter sinuplasty in young children. Am J Rhinol Allergy. 2010;24:e54–6.

    Article  PubMed  Google Scholar 

  70. Mainz JG, Koitschev A. Management of chronic rhinosinusitis in CF. J Cyst Fibros. 2009;8(Suppl 1):S10–4.

    Article  PubMed  CAS  Google Scholar 

  71. Siedek V, Stelter K, Betz CS, Berghaus A, Leunig A. Functional endoscopic sinus surgery – a retrospective analysis of 115 children and adolescents with chronic rhinosinusitis. Int J Pediatr Otorhinolaryngol. 2009;73:741–5.

    Article  PubMed  Google Scholar 

  72. Smith TL, Kern R, Palmer JN, Schlosser R, Chandra RK, Chiu AG, et al. Medical therapy vs surgery for chronic rhinosinusitis: a prospective, multi-institutional study with 1-year follow-up. Int Forum Allergy Rhinol. 2013;3(1):4–9.

    Article  PubMed  Google Scholar 

  73. Van Peteghem A, Clement PA. Influence of extensive functional endoscopic sinus surgery (FESS) on facial growth in children with cystic fibrosis. Comparison of 10 cephalometric parameters of the midface for three study groups. Int J Pediatr Otorhinolaryngol. 2006;70(8):1407–13.

    Article  PubMed  Google Scholar 

  74. Rowe-Jones JM, Mackay IS. Endoscopic sinus surgery in the treatment of cystic fibrosis with nasal polyposis. Laryngoscope. 1996;106(12, Pt. 1):1540–4.

    Article  CAS  PubMed  Google Scholar 

  75. Konstantinidis I, Fotoulaki M, Iakovou I. Vitamin D3 deficiency and its association with nasal polyposis in patients with cystic fibrosis and patients with chronic rhinosinusitis. Am J Rhinol Allergy. 2017;31(6):395–400.

    Article  PubMed  Google Scholar 

  76. Fetta M, Tsillis NS, Segas JV, Nikolopoulos TP, Vlastarakos PV. Functional endoscopic sinus surgery improves quality of life in children suffering from chronic rhinosinusitis with nasal polyps. Int J Pediatr Otorhinolaryngol. 2017;100:145–8.

    Article  PubMed  Google Scholar 

  77. Virgin FW. Clinical chronic rhinosinusitis outcomes in pediatric patients with cystic fibrosis. Laryngoscope Investig Otolaryngol. 2017;2(5):276–80.

    Article  PubMed  PubMed Central  Google Scholar 

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

    Article  PubMed  PubMed Central  Google Scholar 

  79. 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.

    Article  PubMed  Google Scholar 

  80. Choi KJ, Cheng TZ, Honeybrook AL. Correlation between sinus and lung cultures in lung transplant patients with cystic fibrosis. Int Forum Allergy Rhinol. 2018;8(3):389–93.

    Article  PubMed  Google Scholar 

  81. Aanaes K. Bacterial sinusitis can be a focus for initial lung colonization and chronic lung infection in patients with cystic fibrosis. J Cyst Fibros. 2013;12(Suppl 2):S1–20.

    Article  PubMed  Google Scholar 

  82. Khalfoun S, Tumin D, Ghossein M, Lind M, Hayes D Jr, Kikby S. Improved lung function after sinus surgery in cystic fibrosis patients with moderate obstruction. Otolaryngol Head Neck Surg. 2018;158(2):381–5.

    Article  PubMed  Google Scholar 

  83. 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.

    Article  CAS  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  85. 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(6):542–7.

    Article  PubMed  Google Scholar 

  86. Crockett DJ, Wilson KF, Meier JD. Perioperative strategies to improve sinus surgery outcomes in patients with cystic fibrosis: a systematic review. Otolaryngol Head Neck Surg. 2013;149(1):30–9.

    Article  PubMed  Google Scholar 

  87. De Boeck K, Amaral MD. Progress in therapies for cystic fibrosis. Lancet Respir Med. 2016;4(8):662–74.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

The authors would like to thank Dr. Terry L. Noah, MD, and Dr. Charles R. Esther, Jr., MD, PhD, of the UNC Division of Pediatric Pulmonology for their input and careful review of our manuscript. We greatly appreciate their time and assistance. We would also like to thank Dr. Hassan Ramadan, MD, and Dr. Fuad Baroody, MD, for their guidance and inspiration in the field of pediatric rhinology, as well as the opportunity to contribute to this outstanding book.

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Correspondence to Austin S. Rose .

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Akil, N., Rose, A.S. (2020). Chronic Rhinosinusitis in Children with Cystic Fibrosis. In: Ramadan, H., Baroody, F. (eds) Pediatric Rhinosinusitis. Springer, Cham. https://doi.org/10.1007/978-3-030-22891-0_9

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