Until recently, cystic fibrosis was frequently fatal during childhood.
However, with current medical management, many patients are living in
to adulthood. This has created a new population of patients with chron
ic sinusitis and severe medical problems. In this report, experience w
ith 22 patients, eight of whom have undergone sinus surgery, is presen
ted, and recommendations for management are proposed. Presenting sympt
oms are typical of sinusitis, but in a few patients, severe debilitati
ng headaches predominate. Oral antibiotics are often of little use due
to the numerous courses of high dose intravenous antibiotics used for
resistant pulmonary infections. Topical nasal steroids and mucolytics
have been of some benefit Fourteen operative procedures were performe
d on eight patients. These procedures included 12 endoscopic sphenoeth
moidectomies, four Caldwell-Luc procedures, two frontal sinus oblitera
tions, and one transseptal sphenoidotomy (many of these were in combin
ation.) Results from this experience indicate 1) Failure of endoscopic
surgery to control frontal and maxillary sinus disease; 2) Delayed he
aling of the ethmoid cavity with persistent crusting; and 3) Significa
nt, albeit short term, symptomatic relief following surgical intervent
ion. Based on this limited series, we conclude that surgery should be
delayed until absolutely necessary and that an aggressive approach sho
uld be adopted when surgery is performed. In our hands this included i
nitial endoscopic sphenoethmoidectomy with open surgical techniques us
ed for removal of trapped inspissated secretions. We recommend long te
rm intravenous antibiotics postoperatively and frequent cleaning of th
e ethmoid cavity after surgery, continuing indefinitely, to optimize t
he benefit of surgery.