title: Sinus surgery can improve quality of life, lung infections, and lung function in patients with primary ciliary dyskinesia.
abstract: Chronic rhinosinusitis (CRS) and bacterial sinusitis are ubiquitous in patients with primary ciliary dyskinesia (PCD). From the sinuses, Pseudomonas aeruginosa can infect the lungs.
  
  
  
  We studied the effect of endoscopic sinus surgery (ESS) on symptoms of CRS and lower airway infections in PCD patients in a prospective single-arm intervention study of ESS with adjuvant therapy using nasal irrigation with saline, topical nasal steroids, and 2 weeks of systemic antibiotics. Additional treatment with local colistin for 6 months was instigated when P. aeruginosa was cultured at ESS.
  
  
  
  Twenty-four PCD patients underwent ESS to search for an infectious focus (n = 10), due to severe symptoms of CRS (n = 8), or both (n = 6). Bacteria were cultured from sinus samples in 21 patients (88%), and simultaneous sinus and lung colonization with identical pathogens were observed in 13 patients (62%). Four patients with preoperative P. aeruginosa lung colonization (25%) had no regrowth during follow-up; 2 of these had P. aeruginosa sinusitis. Sinonasal symptoms were improved 12 months after ESS and we observed a trend toward better lung function after ESS.
  
  
  
  We demonstrated an improvement in CRS-related symptoms after ESS and adjuvant therapy. In selected PCD patients, the suggested regimen may postpone chronic lung infection with P. aeruginosa and stabilize lung function.
authors.affiliation: Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Mikkel Christian
authors.initials: MC
authors.lastname: Alanin
authors.affiliation: Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Kasper
authors.initials: K
authors.lastname: Aanaes
authors.affiliation: Department of Clinical Microbiology, Rigshospitalet, Copenhagen, Denmark.|Institute of Immunology and Microbiology, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Niels
authors.initials: N
authors.lastname: Høiby
authors.affiliation: Danish Primary Ciliary Dyskinesia (PCD) Centre, Pediatric Pulmonary Service, Department of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Tania
authors.initials: T
authors.lastname: Pressler
authors.affiliation: Danish Primary Ciliary Dyskinesia (PCD) Centre, Pediatric Pulmonary Service, Department of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Marianne
authors.initials: M
authors.lastname: Skov
authors.affiliation: Danish Primary Ciliary Dyskinesia (PCD) Centre, Pediatric Pulmonary Service, Department of Pediatrics and Adolescent Medicine, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Kim Gjerum
authors.initials: KG
authors.lastname: Nielsen
authors.affiliation: Department of Clinical Microbiology, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Helle Krogh
authors.initials: HK
authors.lastname: Johansen
authors.affiliation: Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Christian
authors.initials: C
authors.lastname: von Buchwald
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001992:Bronchoalveolar Lavage Fluid / Q000382:microbiology; D002908:Chronic Disease; D004724:Endoscopy; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000188:drug therapy / Q000382:microbiology / Q000503:physiopathology / Q000601:surgery*; D008168:Lung / Q000382:microbiology / Q000502:physiology; D008297:Male; D008875:Middle Aged; D009298:Nasal Polyps / Q000188:drug therapy / Q000382:microbiology / Q000503:physiopathology / Q000601:surgery*; D010256:Paranasal Sinuses / Q000601:surgery*; D011552:Pseudomonas Infections / Q000382:microbiology / Q000503:physiopathology / Q000517:prevention & control* / Q000628:therapy; D011550:Pseudomonas aeruginosa; D011788:Quality of Life; D012220:Rhinitis / Q000188:drug therapy / Q000382:microbiology / Q000503:physiopathology / Q000601:surgery*; D012852:Sinusitis / Q000188:drug therapy / Q000382:microbiology / Q000503:physiopathology / Q000601:surgery*; D013256:Steroids / Q000627:therapeutic use; D007507:Therapeutic Irrigation; D055815:Young Adult
chemical_list: D000900:Anti-Bacterial Agents; D013256:Steroids
keywords: FESS; bacteriology; chronic rhinosinusitis; computer assisted surgery; sinusitis