Temporal bone pneumatization and its relationship to paranasal sinus development in cystic fibrosis

Abstract

PSP is impaired in CF and DeltaF508 homozygosity is related to poor PSP. TBP is well preserved in the CF population and DeltaF508 homozygosity correlates with greater TBP, with the underlying mechanisms being unclear. Genotype might play a role in skull base pneumatization.

Current views on chronic rhinosinusitis in patients with cystic fibrosis

“…Особенности пневматизации височной кости и околоносовых пазух были в свое время изучены с помощью компьютерной томографии [15]. Были выявлены генотипические данные для пациентов с МВ.…”

Cystic Fibrosis Chronic Rhinosinusitis: A Comprehensive Review

“…46 The general lack of chronic ear disease in CF patients and normal temporal bone development argues against this hypothesis. 47 However, genotype does appear to influence paranasal sinus development as individuals homozygous for the F508del mutation have shown a significantly increased frequency of underdeveloped frontal (98%), maxillary (70%), and sphenoid (100%) sinuses when compared with other genetic mutations (69,8, and 50%, respectively), suggesting CFTR may be a primary contributor to sinus development. 45 Studies from the recently developed CF pig model support the premise that CFTR dysfunction as opposed to chronic infection is responsible for decreased sinus pneumatization because pigs lacking intact CFTR have sinus underdevelopment before the development of infection.…”

Are Chronic Rhinosinusitis and Paranasal Sinus Pneumatization Related?

“…Among cystic fibrosis (CF) patients, for whom sinus mucosal inflammation is nearly universal [1,2], sinus hypoplasia is well documented [2][3][4][5][6]. Because of these findings in CF, it has been suggested that mucosal disease and infection may lead to arrested sinus development [6][7][8].…”

“…Previous studies using radiographic instruments have been successful at differentiating sinus hypoplasia in CF [3][4][5], but have not been able to discern a difference in CRS without CF from unaffected controls [4,6].…”

“…The instruments used to discern sinus hypoplasia in previous studies of CF patients are incompletely validated for use in a general population of sinus computed tomography (CT) scans [3][4][5]. Furthermore, volumetric or dimensional analysis of sinus CT tends to be too cumbersome for the large populations that may be necessary to detect small differences [6].…”