Nasal endoscopy and paranasal sinus computerised tomography (CT) findings in an Irish cystic fibrosis adult patient group

Abstract

Cystic fibrosis (CF) is a common inherited disorder in Caucasians in Ireland having the highest reported incidence. CF has well-recognised clinical sequelae in several physiological systems. Its' impact on the sinonasal system is less well established. We evaluated symptoms, endoscopic and computerised tomographic (CT) findings in an Irish adult CF group with the aim of characterising the relationship between these clinical features in an Irish CF group. Adult CF patients attending a specialist clinic underwent prospective evaluation of sinonasal symptoms using a specifically designed questionnaire. They subsequently underwent nasoendoscopy and CT scanning of their paranasal sinuses. Abnormalities identified were quantified using established radiological (Lund-Mackay) and endoscopic (Lund-Kennedy) scoring systems. The relationship between symptoms of chronic rhinosinusitis (CRS), endoscopic findings and CT abnormalities were then compared. Sixty-three CF patients (n = 63) were studied. 29 patients had a CT scan. Thirty-three CF patients (52%) had no symptoms of CRS. Fifty CF patients (80% of CF group) had evidence of CRS on nasoendoscopy including thirteen patients (20%) with nasal polyposis. 98% of patients scanned have positive findings on CT scan. There was no significant difference between symptomatic and asymptomatic CF groups with respect to their Lund-Kennedy endoscopic score or their Lund-Mackay CT score. 86% demonstrated one or more hypoplastic sinus. There was no increased incidence of hypoplastic sinuses amongst Δf508 homozygotes than other mutation groups.

Clinical predictors of cystic fibrosis chronic rhinosinusitis severity

“…Other radiographic findings of upper airway disease include sinus opacification, mucosal edema, mucoceles, medial bulging of the maxillary walls, polyposis, and chronic osteitis . As would be expected from the radiographic evidence of chronic inflammation, patients with CF‐CRS exhibit mucosal edema, crusting, and purulent discharge . Both radiographically and on physical exam, CF is marked by evidence of severe, chronic rhinosinusitis (CRS) in nearly all individuals.…”

“…Both radiographically and on physical exam, CF is marked by evidence of severe, chronic rhinosinusitis (CRS) in nearly all individuals. Although objective findings of CRS are nearly universal, only two thirds of patients report symptoms of CRS even when specifically questioned, and only one third report that the CRS symptoms are bothersome . Symptoms of CRS in CF include mucopurulent drainage, nasal obstruction, headache, orbital pain, and decreased sense of smell .…”

“…Several studies correlating CFTR genotype with patient‐reported symptoms could not identify an association between more severe symptoms and more severe CFTR mutations . There was no correlation found between severity of radiographic disease and patient‐reported symptoms in multiple studies involving both children and adults with CF . Endoscopic assessment also has shown limited correlation with patient symptomatology in CF .…”

Massive Nasal Polyposis in a Patient with Newly Diagnosed Cystic Fibrosis

“…CT scan is the imaging modality of choice to identify location of the polyps and its relationship with surrounding structures [1,8].…”

“…However, nasal polyposis in CF is often resistant to standard therapy and requires surgical intervention [4]. FESS is less invasive technique which permits to restore drainage of the paranasal sinuses and ventilation between the nose and sinus cavities and allows shorter hospital stay [1,8]. In the postoperative period the patient has been recommended to keep on doing frequent nasal washings with saline solution.…”