Factors Influencing the Need for Endoscopic Sinus Surgery in Adult Patients with Cystic Fibrosis

Brook, Christopher; Maxfield, Alice Z.; Ahmed, Hena; Sedaghat, Ahmad R.; Holbrook, Eric H.; Gray, Stacey T.

doi:10.2500/ajra.2017.31.4385

Cited by 9 publications

(7 citation statements)

References 26 publications

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“…Therefore, recurrences seem to be inevitable in these patients, especially in those with severe mutations in the CFTR gene [ 7 ]. Individuals with severe CFTR mutations are definitely more likely to undergo ESS than those with mild CFTR mutations [ 66 ].…”

Section: Therapeutic Options For Cf-related Crsmentioning

confidence: 99%

Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options

Krajewska

Zub

Slowikowski³

et al. 2021

Eur Arch Otorhinolaryngol

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Purpose Chronic rhinosinusitis (CRS) is observed in almost 100% of patients with cystic fibrosis (CF). CF-related CRS treatment is extremely challenging because of the underlying genetic defect leading to its development. CRS in CF is often refractory to standard therapy, while recurrences after surgical treatment are inevitable in the majority of patients. This study provides a precise review of the current knowledge regarding possible therapeutic options for CF-related CRS. Methods The Medline and Web of Science databases were searched without a time limit using the terms “cystic fibrosis” in conjunction with “otorhinolaryngological manifestation”, “rhinology” and “sinusitis”. Results Precise guidelines for CF-induced CRS therapy are lacking due to the lack of large cohort randomized controlled trials. None of the existing therapeutic agents has already been recommended for CRS in CF. Therapy targeting the underlying genetic defect, intranasal dornase alfa administration, and topical delivery of colistin and tobramycin showed promising results in CF-related CRS therapy. Besides the potential effectiveness of nasal steroids, strong recommendations for their usage in CF have not been provided yet. Systemic corticosteroid usage is controversial due to its potential negative influence on pulmonary disease. Ibuprofen revealed some positive effects on CF-related CRS in molecular and small cohort studies. Intranasal irrigation with saline solutions could relieve sinonasal symptoms. Nasal decongestants are not recommended. Endoscopic sinus surgery is the first-line surgical option for refractory CRS. Extensive surgical approaches should be considered as they could improve long-term outcomes in CRS. Conclusion Further studies are warranted to establish consensus for CF-related CRS therapy.

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Section: Therapeutic Options For Cf-related Crsmentioning

confidence: 99%

Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options

Krajewska

Zub

Slowikowski³

et al. 2021

Eur Arch Otorhinolaryngol

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“…Despite appropriate medical therapy, 20 to 60% of CF patients go on to require ESS [2]. In a retrospective review, Brook et al showed that prior history of ESS and severe CFTR mutations are predictive of ESS while Sinonasal Outcome Test (SNOT)-22 score was not [14]. In a different study, CF patients with nasal polyposis, prior history of ESS, lower forced expiratory volume in one second (FEV1), higher Lund-Mackay score, and higher SNOT-22 score was more likely to elect up-front ESS versus medical therapy.…”

Section: Surgical Managementmentioning

confidence: 99%

Surgical Management of Chronic Rhinosinusitis in Cystic Fibrosis

2019

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Cystic fibrosis patients frequently develop chronic rhinosinusitis as a result of their propensity to form inspissated mucus and impairment of mucociliary clearance. They exhibit variable symptom burden even in the setting of positive radiographic and endoscopic findings. Current evidence suggests a positive effect of managing sinonasal disease on pulmonary health. Topical antimicrobial and mucolytic therapies are frequently required to manage the disease with surgery reserved for refractory cases. Endoscopic sinus surgery has been demonstrated to be safe and efficacious in controlling symptoms of chronic rhinosinusitis in patients with comorbid cystic fibrosis. However, the impact of surgery on pulmonary health remains an active area of investigation. In addition, a growing body of research has suggested a more extended surgical approach creating large sinonasal cavities with gravity-dependent drainage pathways, followed by adjuvant medical therapies, as an ideal strategy to optimally control disease and prevent pulmonary exacerbations. In this manuscript, we provide an up-to-date review of current evidence in the surgical management of chronic rhinosinusitis in cystic fibrosis patients.

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“…In an analysis of 91 patients over the age of 16 years who had sinus CT scans, the SNOT‐22 revealed few symptoms, suggesting under‐reporting of symptoms by patients with CF . A retrospective review of adults who were referred to an otolaryngology clinic showed that predictors of future endoscopic sinus surgery (ESS) included previous ESS and severe CFTR mutations; however, the SNOT‐22 was not found to be predictive …”

Section: Extrapulmonary Complicationsmentioning

confidence: 99%