Abstract

BackgroundCystic Fibrosis is an autosomal recessive disorder with a mutation in the cystic fibrosis transmembrane regulator gene, leading to dysregulation of epithelial fluid transport, dehydration of airway surfaces and impaired mucociliary function in the sinuses, lungs, pancreas and other organs. This leads to thickened secretion which blocks the sinus ostia and promotes chronic bacterial overgrowth and destruction of the mucosa. Chronic rhinosinusitis in cystic fibrosis patients leads to a decreased quality of life involving increased hospitalizations, infections, pneumonia, and acute exacerbations, warranting multiple sinus surgeries. This study investigates the effects of a more aggressive surgical approach on reducing the need for repeat surgeries. MethodsA retrospective review of CT scans of nine cystic fibrosis patients who have had aggressive endoscopic sinus surgery for the treatment of chronic rhinosinusitis (CRS) was performed. The decrease in maxillary sinus volume was measured using pre-operative and post-operative scans. ResultsA significant decrease in maxillary sinus volume was observed in all subjects. Volume reduction occurred through osteoneogenesis, causing auto-obliteration of the sinuses with cancellous bone. ConclusionsAggressive endoscopic sinus surgery for maxillary sinuses incites an osteoneogenetic reaction that leads to auto-obliteration of the sinus and reduces sinus-related morbidity in children with cystic fibrosis. Through this osteoneogenesis, the sinus is transformed into a shallow cup that does not retain secretions and is easy to clear. It is our impression that this may lead to reduced morbidity and a decreased need for repeat maxillary sinus surgery.

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