Characterizing macular edema in retinitis pigmentosa through a combined structural and microvascular optical coherence tomography investigation

Abstract

The aim of the study was to characterize macular edema (ME) in retinitis pigmentosa (RP) by means of quantitative optical coherence tomography (OCT)-based imaging. The study was designed as observational, prospective case series, with 1-year follow-up. All RP patients underwent complete ophthalmologic assessment, including structural OCT, OCT angiography, and microperimetry (MP). The primary outcome was the characterization through quantitative OCT-based imaging of RP eyes complicated by ME. A total of 68 RP patients’ eyes (68 patients) and 68 eyes of 68 healthy controls were recruited. Mean BCVA was 0.14 ± 0.17 LogMAR at baseline and 0.18 ± 0.23 LogMAR at 1-year follow-up (p > 0.05). Thirty-four eyes (17 patients; 25%) showed ME, with a mean ME duration of 8 ± 2 months. Most of the eyes were characterized by recurrent ME. The ME was mainly localized in the inner nuclear layer in all eyes. LogMAR BCVA was similar in all RP eyes, whether with or without ME, although those with ME were associated with higher vessel density values, as well as thicker choroidal layers, than those without ME. In conclusion, the inner retina is closely involved in the pathogenesis of ME. The impairment of retinal-choroidal exchanges and Müller cell disruption might be a major pathogenic factor leading to the onset of ME in RP.

Dexamethasone implant versus topical carbonic anhydrase inhibitors in patients with bilateral retinitis pigmentosa-related cystoid macular edema – a prospective, paired-eye pilot study

“…Further support for our results comes from the hypothesis that the pattern of BCVA in patients with RP-related CME is influenced more by outer layer integrity than by CMT. [21][22][23] In this regard, chronic CME may play a role in disrupting the photoreceptor layers, thus limiting changes in functional metrics. Interestingly, eyes treated with topical CAI showed significant and progressive improvement at all time points except month 1: less fluctuation in CMT and reduced spikes with respect to IVDI treatment suggest a better effect on BCVA.…”

The clinical research on the effect of hydrogen-rich water on primary retinitis pigmentosa

“…In addition, thinning of the choroidal vessels also contributes to histopathological changes in patients with RP. OCT imaging could reveal characteristic morphological changes in RP, such as thinning of the retinal layer and choroid [ 21 ]. Previous animal studies have indicated that intraperitoneal injection of HRW could improve retinal morphology in an RP animal model induced by N-methyl-N-nitrosourea (MNU), as evidenced by the examinations of the retinal sections and OCT scanning [ 22 , 23 ].…”

Clinical Applications of Optical Coherence Tomography Angiography in Inherited Retinal Diseases: An Up-to-Date Review of the Literature

“…The mean follow-up ranged between 2 months and 36 months [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31]. Overall, significant reductions in both the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were observed in all the affected patients of the evaluated cohorts over time [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31]. In addition, all the studies that explored the involvement of choriocapillaris (CC) demonstrated its significant impairment in RP patients [12,[15][16][17][21][22][23]25,…”

“…Overall, significant reductions in both the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were observed in all the affected patients of the evaluated cohorts over time [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31]. In addition, all the studies that explored the involvement of choriocapillaris (CC) demonstrated its significant impairment in RP patients [12,[15][16][17][21][22][23]25,30,31]. Several authors focused on the variation of the foveal avascular zone (FAZ) area in RP patients, two-thirds of which described an increased avascular area [12,15,19,20,26,31], while the remaining third demonstrated its significant reduction [16,17,29].…”

“…In addition, all the studies that explored the involvement of choriocapillaris (CC) demonstrated its significant impairment in RP patients [12,[15][16][17][21][22][23]25,30,31]. Several authors focused on the variation of the foveal avascular zone (FAZ) area in RP patients, two-thirds of which described an increased avascular area [12,15,19,20,26,31], while the remaining third demonstrated its significant reduction [16,17,29]. Nakajima et al and Alnawaiseh et al explored an interesting association between the reduction in optic nerve head (ONH) vessel density (VD) in RP patients and the deterioration of the visual field mean deviation (MD) [15,27].…”