Prevalence of CF-related chronic rhinosinusitis – results from a multicentre interdisciplinary study

Mainz, J.G.; Schien, M.; Naehrlich, L.; Käding, M.; Thoss, K.; Frey, G; Wiedemann, Bärbel; Beck, James F.

doi:10.1016/s1569-1993(10)60453-5

Cited by 3 publications

(4 citation statements)

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“…Current literature reports that ESS is performed in 20–60% of patients with CF 16,119,120 and results in both symptomatic and endoscopic improvement. 121 A retrospective review of 24 studies recently reported universal improvement of sinonasal symptoms following sinus surgery, which was evaluated with use of validated questionnaires or subjective reports.…”

Section: Surgical Management Of Cf-related Crsmentioning

confidence: 99%

Medical and Surgical Advancements in the Management of Cystic Fibrosis Chronic Rhinosinusitis

Tipirneni

Woodworth

2017

Curr Otorhinolaryngol Rep

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Purpose of review The purpose of this review is to provide otolaryngologists with the most up-to-date advancements in both the medical and surgical management of CF-related sinus disease. Recent findings Recent studies have supported more aggressive CRS management, often with a combination of both medical and surgical therapies. Comprehensive treatment strategies have been shown to reduce hospital admissions secondary to pulmonary exacerbations in addition to improving CRS symptoms. Still, current management strategies are lacking in both high-level evidence and standardized guidelines. Summary The unified airway model describes the bi-directional relationship between the upper and lower airways as a single functional unit and suggests that CRS may play a pivotal role in both the development and progression of lower airway disease. Current strategies for CF CRS focus primarily on amelioration of symptoms with antibiotics, nasal saline and/or topical medicated irrigations, and surgery. However, there are no definitive management guidelines and there remains a persistent need for additional studies. Nevertheless, otolaryngologists have a significant role in the overall management of CF, which requires a multi-disciplinary approach and a combination of both surgical and medical interventions for optimal outcomes of airway disease. Here we present a review of currently available literature and summarize medical and surgical therapies best suited for the management of CF-related sinus disease.

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Section: Surgical Management Of Cf-related Crsmentioning

confidence: 99%

Medical and Surgical Advancements in the Management of Cystic Fibrosis Chronic Rhinosinusitis

Tipirneni

Woodworth

2017

Curr Otorhinolaryngol Rep

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“…Within the last years, sinonasal involvement in CF is coming into the clinical and scientific focus [ 14 , 16 – 24 ]. Almost one-third of CF patients have symptoms that fulfil the “European Position Paper on Rhinosinusitis and Nasal Polyps” (EPOS) [ 25 ] criteria of chronic rhinosinusitis, and another third suffers from remittent symptoms [ 26 ]. Thereby, almost all CF patients reveal pathologic sinonasal CT scans [ 27 ].…”

Section: Discussionmentioning

confidence: 99%

Pseudomonas aeruginosaAcquisition in Cystic Fibrosis Patients in Context of Otorhinolaryngological Surgery or Dentist Attendance: Case Series and Discussion of Preventive Concepts

Mainz

Gerber

Lorenz

et al. 2015

Case Reports in Infectious Diseases

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Introduction. P. aeruginosa is the primary cause for pulmonary destruction and premature death in cystic fibrosis (CF). Therefore, prevention of airway colonization with the pathogen, ubiquitously present in water, is essential. Infection of CF patients with P. aeruginosa after dentist treatment was proven and dental unit waterlines were identified as source, suggesting prophylactic measures. For their almost regular sinonasal involvement, CF patients often require otorhinolaryngological (ORL) attendance. Despite some fields around ORL-procedures with comparable risk for acquisition of P. aeruginosa, such CF cases have not yet been reported. We present four CF patients, who primarily acquired P. aeruginosa around ORL surgery, and one around dentist treatment. Additionally, we discuss risks and preventive strategies for CF patients undergoing ORL-treatment. Perils include contact to pathogen-carriers in waiting rooms, instrumentation, suction, drilling, and flushing fluid, when droplets containing pathogens can be nebulized. Postsurgery mucosal damage and debridement impair sinonasal mucociliary clearance, facilitating pathogen proliferation and infestation. Therefore, sinonasal surgery and dentist treatment of CF patients without chronic P. aeruginosa colonization must be linked to repeated microbiological assessment. Further studies must elaborate whether all CF patients undergoing ORL-surgery require antipseudomonal prophylaxis, including nasal lavages containing antibiotics. Altogether, this underestimated risk requires structured prevention protocols.

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“…Nevertheless, CF-related chronic sinusitis relapsed soon and led to orbital hypertelorism with a broadening of her nasal bridge (Figures 1 and 2). from symptoms of rhinosinusitis in up to 62% [3][4][5]. Thereby, sinonasal involvement can play a relevant and underestimated role in colonization of the airway system with pathogens.…”

Section: Case Reportmentioning

confidence: 99%

“…Due to improved prognosis, upper airway involvement is coming into focus, and it has been found to have a much higher impact on CF overall health than expected [1,2]. Patients carrying the disease causing defective chloride and sodium channels in sinonasal mucosa are reported to suffer from symptoms of rhinosinusitis in up to 62% [3][4][5]. Thereby, sinonasal involvement can play a relevant and underestimated role in colonization of the airway system with pathogens.…”

Section: Introductionmentioning

confidence: 99%

Chronic Rhinosinusitis as a Crucial Symptom of Cystic Fibrosis—Case Report and Discussion on the Sinonasal Compartment as Site of Pseudomonas aeruginosa Acquisition into CF Airways

et al. 2016

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Cystic fibrosis (CF) is the most frequent congenital lethal disease in Caucasians. Impaired mucociliary clearance causes chronic bacterial rhinosinusitis in up to 62% of patients, and almost all patients exhibit sinonasal pathology in CT scans. Pathogens like Pseudomonas aeruginosa (P.a.) chronically colonize about 70% of the CF adults' lungs and are the major reason for pulmonary destruction and premature death. In our 34-year-old female CF patient, rhinosinusitis caused massive orbital hypertelorism despite three sinonasal operations. Her sputum samples had always been negative for P.a. Then, P.a. was primarily detected in her sputum and additionally in nasal lavage, which since then persisted in both, her upper and lower airways. The P.a. strains turned out to be genetically identical in both airway levels, indicating early colonization of the entire airway system with P.a. This first report on simultaneous primary P.a. detection in the sinonasal and pulmonary compartments highlights the need to include an assessment of upper airway colonization in the standards of CF care, particularly in patients without chronic P.a. colonization. Both airway levels need to be considered as one united system, and a strong cooperation between ENT and CF specialists should be established. Prospective longitudinal studies should assess the upper airways´role in acquisition and persistence of pathogens and evaluate conservative and surgical therapeutic options.

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Prevalence of CF-related chronic rhinosinusitis – results from a multicentre interdisciplinary study

Cited by 3 publications

References 0 publications

Medical and Surgical Advancements in the Management of Cystic Fibrosis Chronic Rhinosinusitis

Medical and Surgical Advancements in the Management of Cystic Fibrosis Chronic Rhinosinusitis

Pseudomonas aeruginosaAcquisition in Cystic Fibrosis Patients in Context of Otorhinolaryngological Surgery or Dentist Attendance: Case Series and Discussion of Preventive Concepts

Chronic Rhinosinusitis as a Crucial Symptom of Cystic Fibrosis—Case Report and Discussion on the Sinonasal Compartment as Site of Pseudomonas aeruginosa Acquisition into CF Airways

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