Management of chronic sinusitis in cystic fibrosis

Abstract

Chronic rhinosinusitis is extremely common in patients with cystic fibrosis. It causes numerous problems in these patients and can put them at risk for life-threatening illness. Potential problems include nasal obstruction, congestion, sinus pain and pressure, infection (usually with Pseudomonas organisms), hyposmia or anosmia, and the seeding of bacteria into the lower respiratory tract. Cystic fibrosis patients with chronically infected sinuses are at increased risk for pneumonia following lung transplantation. A prophylactic protocol has been developed for the management of chronic sinusitis in patients with cystic fibrosis. These patients are fully evaluated at the Nasal Dysfunction Clinic of the University of California, San Diego (UCSD), Medical Center. Based on the results of the evaluation, they are treated with endoscopic sinus surgery, partial middle turbinectomy, septoplasty, and a large middle meatal maxillary antrostomy. Surgery is followed by a rigorous regimen of pulsatile hypotonic saline nasal irrigation to wash away tenacious cystic secretions. Tobramycin (Nebcin) is given once daily in the nasal irrigant to inhibit the growth of Pseudomonas organisms. At the USCD Nasal Dysfunction Clinic, this prepulmonary transplantation protocol is now used in all cystic fibrosis patients with chronic sinusitis.

International Consensus Statement on Allergy and Rhinology: Rhinosinusitis

“…1462,1463 Topical antibiotic use has shown reduced postoperative CF sinus exacerbations, endoscopic score improvement, and reduced symptoms. 782,785,1464 Dornase alfa reduces mucus viscosity and improves objective CRS outcome measures. 1465,1466 Oral macrolides possess antibacterial and anti-inflammatory effects with support for use in CF pulmonary manifestations and may be utilized to treat CF-related CRS.…”

Outcomes of sinus surgery in adults with cystic fibrosis

“…This subgroup of CRS patients represents one of several challenging subtypes of CRS for which optimal management strategies remain a topic of intense investigation. [5][6][7][8]11 Management of CF patients involves a multi-disciplinary approach to assess maturation of lung function at a young age and vigilant attention to preventing infectious complications. The marked heterogeneity observed, in terms of disease severity and progression, makes it difficult to accurately gauge progress and estimate prognostic factors.…”

“…7 Surgical intervention is often sought to aid in initiating drainage of the paranasal sinuses and therefore attenuate the inflammatory cascade in patients with cystic fibrosis. 8,9,10 As the mean survival has improved over the last 30 years, CF patients increasingly present with CRS, refractive to conventional medical therapy, well into adulthood. 7,11 Little is known however about the effectiveness of surgical therapy in adult CF patients with recrudescent sinus disease.…”

Pseudomucocele bilateral associada à fibrose cística: relato de caso

“…Davidson et al indicate a rigorous regimen of nasal washings with saline solution in order to fluidize secretions, as well as nasal flushing with Tobramycin daily in order to inhibit the growth of pseudomonas. 11 Our approach in the post-operative period has been to keep on doing frequent nasal flushings with isotonic saline solution. The patient improved significantly from the nasal obstruction, without signs and symptoms of pseudomucocele recurrence during the three month observation period.…”