Cystic Fibrosis and New Trends by Ophthalmological Evaluation: A Pilot Study

Abstract

Background. Cystic fibrosis (CF) is characterized by hypoxia that affects several organic tissues. Retinal ganglion cells may suffer from the hypoxic status, and this may lead to alterations of retinal nerve fiber. Methods. Twenty-two eyes in CF patients were analyzed. A complete ocular evaluation and visual field exams of the 30 central degrees were performed using the frequency doubling technology (FDT). Forced expiratory volume in one second (FEV1%), forced vital capacity (FVC%), oxyhaemoglobin saturation (SpO2%), and hematocrit (Ht%) have been calculated. FDT analyzed parameters were mean deviation (MD) and pattern standard deviation (PSD). Pearson's correlation was chosen as statistical analysis. Results. Data showed statistically significant relationship between MD and Ht% (r value −0.18; P = 0.04), MD and FEV1% (r value −0.68; P = 0.001), and MD and FVC% (r value −0.45; P = 0.005). Moreover, there were correlations between PSD and Ht% (r value 0.29; P = 0.03), PSD and SpO2% (r value −0.31; P = 0.01), PSD and FEV1% (r value 0.71; P = 0.0005), and PSD and FVC% (r value 0.63; P = 0.003). Conclusions. The oxygen supply alterations might determine hypoxia of the ganglion cells causing a decrease of receptive optic nerve fiber activity. This method could be also useful to evaluate indirectly pulmonary activity of the CF disease.

Ophthalmologic manifestations of adult patients with cystic fibrosis

“…Our finding of no statistically significant difference in IOP measurements between CF patients and controls, is in accordance with a previous CF study. 18 This finding is pathophysiologically supported by other studies that demonstrated no contribution by the CFTR protein to aqueous humor production. 28,29 Our study comes with certain limitations, including lack of information on other parameters assessing nerve fiber loss, both anatomical (ganglion cell layer, cell-inner plexiform layer, and cell complex) and functional (visual fields).…”

“…15,16 Pathophysiological studies demonstrate temporary optic nerve hypoperfusion, potentially due to the afore-described mechanisms. 17 In relevance to CF, where lung function is also impaired, Nebbioso et al 18 performed visual field examination by Humphrey Frequency Doubling Technology (FDT) and observed lowered average responses in the nasal-inferior sector of patients versus controls, as well as abnormal glaucoma hemifield test (GHT) and mean deviation at FDT perimetry. These results were assumed to be a consequence of the pulmonary disease causing long term hypoxia and vascular dysregulation in this group of patients.…”

The pathological association between the anterior eye segment and the retina in a murine model of neovascular glaucoma

“…In retinal diseases, fibrosis leads to changes in retinal tissues and retinal ischemic conditions, including inflammation, ischemia, and neurodegeneration 18 . The expression of TGF‐β, MMP‐9, and Snail increased after 7 days post vascular occlusion (Figure 1A–D).…”

Correlation between optic nerve involvement and chronic obstructive pulmonary disease

“…It can be concluded that hypoxia caused by cystic fibrosis may decrease the optic nerve activity. 17 A similar mechanism of optic nerve involvement can be considered for COPD patients as both sufferers endure chronic hypoxia due to lung disorders.…”