Prevalence of focal lamina cribrosa defects in eyes with pachychoroid disease spectrum

Abstract

Aim: To determine the prevalence of focal lamina cribrosa (LC) defect among patients with pachychoroid disease spectrum (PDS) in the absence of peripapillary retinoschisis.

Methods: This retrospective, cross-sectional study comprised of 180 patients with PDS, including polypoidal choroidal vasculopathy (PCV), central serous chorioretinopathy, and pachychoroidal neovasculopathy. Medical records and optic nerve head evaluations conducted using spectral-domain optical coherence tomography with enhanced depth imaging were reviewed. As a control group, 236 patients who underwent ophthalmologic evaluation for vitreous floaters, without obvious ocular disease, were also included.

Results: The mean age of the PDS group, which included 118 male patients (65.6%), was 57.4±11.1y. There was no significant difference between the two groups in age (P=0.710) or sex (P=0.248). Six patients (3.3%) in the PDS group and none in the control group showed focal LC defect (P=0.318). Among the six patients with focal LC defect in the PDS group, four eyes had PCV, one eye was the fellow eye of a PCV eye, and one eye had pachychoroidal neovasculopathy.

Conclusion: Focal LC defect can be defected in patients with PDS in the absence of peripapillary retinoschisis. However, the prevalence of focal LC defect was not different significantly between PDS patients and those who did not have PDS.

Keywords: central serous chorioretinopathy; lamina cribrosa; pachychoroid; polypoidal choroidal vasculopathy.

Figure 1. Representative image of the ONH and SFCT, obtained by SD-OCT with EDI (Spectralis)

A: For the ONH assessment, serial horizontal cross-sectional scans, approximately 30 µm apart and covering the ONH, were obtained; B: Choroidal thickness was defined as the perpendicular distance from the outer border of the hyperreflective line demonstrating the RPE (upper arrowhead) to the chorio-scleral interface (lower arrowhead). Using digital calipers, SFCT was measured at the subfoveal region in both horizontal and vertical images; the two measurements were averaged.

Figure 2. Representative image of a 62-year-old female patient (Patient 1) with PCV in the right eye

A: Subretinal fluid with exudates and hemorrhage involving the macula of the right eye, shown by ultrawide fundus photography; B: Spectral domain optical coherence tomography revealed fibrovascular pigment epithelial detachment with subretinal fluid and exudates; dilated choroidal vessels were noticeable (asterisk); C: Focal LC defect was detected during ONH evaluation (arrow head).

Figure 3. Representative image of a 78-year-old female patient (Patient 2) with PCV in both eyes

PCV, which first occurred in the left eye, was treated by intravitreal bevacizumab injections; 23mo later, PCV recurred in the left eye along with subretinal fluid. A: Peripapillary chorioretinal atrophy with adjacent prominent choroidal vasculature in the right eye. B: Subretinal hemorrhage with exudates in the left eye. C: SD-OCT of the left eye showed fibrovascular pigment epithelial detachment with subretinal hemorrhage, exudates and fluid. D: Twenty-three months after the first visit, subretinal fluid developed in the right eye, as indicated by SD-OCT. E: Focal LC defect (arrow head) in the right eye was detected during ONH evaluation.

Figure 4. Representative image of a 79-year-old male patient (Patient 3) with PCV in the left eye

The patient had disciform scarring in the right eye. A: Subretinal exudative fluid with foveal involvement was noted on fundus photography. Chorioretinal atrophy was evident from the inferior to inferotemporal vascular arcade. B: SD-OCT revealed a fibrovascular membrane with subretinal fluid. Dilated choroidal vessels were noticeable (asterisk). C: Focal LC defect was detected during ONH evaluation (arrow head).

Figure 5. Representative images of the left eye of a 56-year-old patient with pachychoroid pigment epitheliopathy (Patient 4)

A: Tigroid fundus with prominent choroidal vasculature in the left eye; B: The contralateral right eye showed massive subretinal hemorrhagic detachment, associated with PCV; C: Dilated choroidal vessels and mild photoreceptor disruption without subretinal fluid were observed in the left eye using SD-OCT; D: ONH evaluation showed focal LC defect in the left eye (arrow head).

Figure 6. Images of a 44-year-old male patient with pachychoroid neovasculopathy in the right eye (Patient 5)

A: Subretinal turbid fluid with depigmentation involving the fovea was noted on fundus photography. B: SD-OCT showed fibrovascular pigment epithelial detachment with subretinal fluid involving the foveal center. Dilated choroidal vessels were noticeable (asterisk). C: ONH evaluation showed focal LC defect (arrow head).