Ophthalmologic manifestations of adult patients with cystic fibrosis. Issue 2 (March 2022)

• Record Type: Journal Article
• Authors: Giannakouras, Panagiotis; Kanakis, Menelaos; Diamantea, Filia; Tzetis, Maria; Koutsandrea, Chrysanthi; Papaconstantinou, Dimitrios; Georgalas, Ilias
• Is Part Of: European journal of ophthalmology. Volume 32:Issue 2(2022)
• Page Start: 976
• Page End: 983
• Publication Date: 2022-03
• Subjects: Genetics -- diagnostic techniques -- glaucoma -- hereditary/genetics factors -- diseases of the ocular surface: lid inflammation affecting the ocular surface -- cornea/external disease -- preventive medicine/screening -- socioeconomics and education in medicine/ophthalmology; Ophthalmology -- Periodicals; Eye -- Diseases -- Periodicals; 617.7005
• DOI: 10.1177/11206721211008780
• Languages: English
• ISSNs: 1120-6721

Abstract

Introduction: Cystic fibrosis (CF) is the most common life-shortening recessive genetic disease in Caucasians, affecting primarily the lungs. The objective of our study was to investigate potential ophthalmologic involvement in adult patients with CF. Methods: Fifty adult patients with cystic fibrosis and 60 age- and sex-matched controls underwent complete ophthalmologic examination including tear-film Break-Up Time (BUT), Macular Thickness, and peripapillary Retinal Nerve Fiber Layer (pRNFL) thickness measurements using Spectral Domain-OCT. Results: CF patients had significantly lower nasal-inferior pRNFL thickness (median 82 IQR 67–102 vs 92.5 IQR 82–107, p = 0.005) and lower percentage of normal tear Break-Up Time (56.0% vs 96.7%, p = 0.001) than healthy controls. All CF patients with BUT <10 s were diagnosed with blepharitis at the time of our assessement. The subgroup of patients homozygous for the most common CF mutation, F508del, had lower nasal-inferior pRNFL thickness ( p = 0.014) and lower percentage of normal tear Break-Up Time ( p = 0.001) compared to the control group. Additional findings, present in the CF group only, were punctuate retinal hemorrhages (four patients), vessel tortuosity (four patients), snail-track degeneration, and retinal tufts (two patients without refractive error). There were no significant differences in visual acuity, refractive errors, gonioscopic findings, or intraocular pressure between the groups. Conclusions: Our study is, to the best of our knowledge, the largest ophthalmologic study of patients with cystic fibrosis. We found that CF patients had significantly decreased inferior-quadrant peripapillary retinal nerve fiber layer thickness and decreased tear-film break-up time compared to controls. We highlight the importance of careful regular ophthalmologic assessment and follow-up of these patients.