Medical Articles & Literatures - Cystic Fibrosis

Management of chronic sinusitis in cystic fibrosis

Laryngoscope. 1995 Apr;105(4 Pt 1):354-8. Related Articles, Books, LinkOut
Davidson TM, Murphy C, Mitchell M, Smith C, Light M.

Department of Surgery, University of California San Diego Medical Center 92103-8895, USA.

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.

PMID: 7715376 [PubMed - indexed for MEDLINE]

Nasal and paranasal disease in adult cystic fibrosis patients

Eur Respir J. 1992 Nov;5(10):1239-42. Related Articles, Books, LinkOut
Kerrebijn JD, Poublon RM, Overbeek SE.

Dept of Otolaryngology, University Hospital Rotterdam Dijkzigt, The Netherlands.

Children with cystic fibrosis frequently have nasal polyps and sinusitis. This study addresses (para-) nasal disease in 39 adult cystic fibrosis patients. Fifteen patients (39%) had recently had serious nasal symptoms and 26% sinusitis. Seventeen (44%) had nasal polyposis. Almost all sinus radiographs taken showed opacification, which was unrelated to symptoms. Polypectomies and antral irrigations were usually ineffective, whilst more extensive surgery generally gave better results. It is concluded that a substantial number of adult cystic fibrosis patients frequently have upper airway symptoms. Sinus radiographs have little or no diagnostic value. Treatment of (para-) nasal disease in cystic fibrosis patients can be difficult; a guideline for treatment is suggested, calling for simple interventions coupled with intranasal steroids and nasal irrigation in early disease and for endoscopic to radical sinus surgery in recurrent advanced disease.

PMID: 1486971 [PubMed - indexed for MEDLINE]

Otorhinolaryngologic involvement in cystic fibrosis

Ann Otolaryngol Chir Cervicofac. 1990;107(3):200-3; discussion 203-4. Related Articles, Books, LinkOut
[Article in French]

Denoyelle F, Garabedian EN, Sardet A, Tournier G, Bellity A.

Service d'ORL et de Chirurgie Cervico-Faciale, Hopital Trousseau, Paris.

ENT involvement is very frequent in mucoviscidosis, particularly rhinitis and sinusitis. This prospective study, which spanned from September 1st, 1988 to August 31st, 1989 anc included 27 children with cystic fibrosis of pancreas was carried out with the intent of determining the incidence of the various associated diseases, their bacteriologic profile, and the optimal therapeutic procedure. Less than a child in 5 is symptomless when the nose and sinuses are affected. Polyposis is found to occur in 6/27 cases, and all children have sinus radio-opacities. Ear disease is rare (1/4 of cases) and is manifested by asymptomatic tubal dysfunction with minimal audiometric repercussions. Bacteriologic specimens of sputum and sinus purulent discharge show simultaneous germ positivity (10 times out of 11). Our therapeutic recommendations are based on our own experience as well as literature data, and are, in our opinion, only relevant for children with clinical signs of disease, as opposed to advocating systematic treatment of sinus foci found on X-ray.

PMID: 2344126 [PubMed - indexed for MEDLINE]
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