Outcome of Cataract Surgery in Patients With Retinitis Pigmentosa

Nguyen, Xuan-Thanh-An; Thiadens, Alberta A H J; Fiocco, Marta; Tan, Weijen; McKibbin, Martin; Klaver, Caroline C.C.W.; Meester-Smoor, Magda A.; Cauwenbergh, Caroline Van; Strubbe, Ine; Vergaro, Andrea; Pott, Jan-Willem R.; Hoyng, Carel B.; Leroy, Bart P.; Žemaitienė, Reda; Khan, Kamron; Boon, Camiel J F

doi:10.1016/j.ajo.2022.10.001

Cited by 8 publications

(8 citation statements)

References 41 publications

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“…When present, AAb testing has been obtained and, if positive, surgeries have been strategically delayed until inflammation has been subdued. To date, contrary to what has been reported in the literature ( Hong et al, 2020 ; Antonio-Aguirre et al, 2022 ; Nguyen et al, 2023 ), among hundreds of IRD patients who have undergone cataract surgery at our facility since 2016, we have observed less than a handful of cases of minimal and rapidly resolved post-operative CME, having had each of these “inflammation-prone” IRD cases pretreated shortly before cataract surgery with—usually posterior subtenon—steroids, typically within a week of surgery. None of these patients, even after years of follow up, has to date suffered any sudden or major disease progression or vision loss after surgery not even in the patients who had experienced as much no-light-perception outcomes in the fellow eye operated on previously elsewhere before the autoimmune-mediated inflammatory substrate had been recognized and managed.…”

Section: Discussioncontrasting

confidence: 82%

“…A common account reported by IRD patients exhibiting autorectivities was also the exacerbation of vision loss within months of undergoing cataract surgery, performed elsewhere, whereby some patients had subsequently refused to have the fellow eye operated on out of fear of experiencing the same outcome. It has been recently reported that CME, the most frequent post-operative inflammatory complication of cataract surgery (also known as the Irvine-Gass syndrome) is much more common in RP patients than in the general population ( Hong et al, 2020 ; Antonio-Aguirre et al, 2022 ; Nguyen et al, 2023 ). There is no accounting in the literature for the optic nerve inflammatory complications in IRDs.…”

Section: Discussionmentioning

confidence: 99%

“…We aim to conduct a detailed retrospective review of cataract surgery-specific outcomes in our IRD patient population pretreated with our protocol in a separate setting, since this is not the focus of this manuscript. However, we wanted to emphasize herein the substantial practical relevance of this observation to outcomes and prognosis for IRD patients who so often need to undergo cataract surgery and, otherwise, tend to benefit greatly from cataract removal ( Hong et al, 2020 ; Antonio-Aguirre et al, 2022 ; Nguyen et al, 2023 ). We have found this to be yet another very important and immediately relevant ramification of our findings that has impactful implications when it comes to ensuring that we do everything possible to avoid accidentally contributing closing the door on the light that is at the end of the tunnel for IRDs.…”

Section: Discussionmentioning

confidence: 99%

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The double-edged sword of inflammation in inherited retinal degenerations: Clinical and preclinical evidence for mechanistically and prognostically impactful but treatable complications

Sarıcı

Vyas

Iannaccone

2023

Front. Cell Dev. Biol.

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We present retrospective data from our clinical research efforts of the past several years alongside a review of past and current clinical and preclinical data independently by several investigators supporting our clinical evidence for the importance of inflammation in inherited retinal degenerations (IRDs). We show how inflammation is a complicating factor in IRDs but, if recognized and managed, also a great opportunity to mitigate disease severity immediately, improve patient prognosis and quality of life, extend the treatment windows for gene-specific and agnostic therapeutic approaches, mitigate the impact of inflammatory complications on the accurate estimate of vision changes in IRD natural history studies, improve the chances of safer outcomes following cataract surgery, and potentially reduce the likelihood of inflammatory adverse events and augment the efficacy of viral vector-based treatment approaches to IRDs. Manuscript contribution to the field. Inflammation has been suspected to be at play in IRDs since the beginning of the 1900s and became a research focus through the early 1990s but was then largely abandoned in favor of genetic-focused research. Thanks to regained cognizance, better research tools, and a more holistic approach to IRDs, the recent reappraisal of the role of inflammation in IRDs has brought back to the surface its importance. A potential confounder in natural history studies and a limiting factor in clinical trials if not accounted for, inflammation can be managed and often offers an opportunity for immediately improved prognosis and outcomes for IRD patients. We present our retrospective clinical evidence for connections with a measurable secondary autoimmune component that can develop in IRDs and contribute to vision loss but is at least in part treatable. We also present ample lines of evidence from the literature corroborating our clinical observations at the preclinical level.

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Section: Discussioncontrasting

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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The double-edged sword of inflammation in inherited retinal degenerations: Clinical and preclinical evidence for mechanistically and prognostically impactful but treatable complications

Sarıcı

Vyas

Iannaccone

2023

Front. Cell Dev. Biol.

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“…Patients with RP are also at increased risk for intra- and postoperative complications, including intraoperative phototoxic damage to the retina, (increase in existing) CM and zonular dialysis, among others [ 34 , 173 , 174 , 175 , 176 , 177 ]. Furthermore, higher rates of posterior capsular opacification and anterior capsule phimosis have been described following cataract surgery in patients with RP, which may also negatively influence visual outcomes if left untreated [ 178 ]. Despite the challenges regarding cataract surgery in RP, several studies have demonstrated its benefit, showing average BCVA improvements between studies ( Table 1 ) [ 105 , 162 , 178 , 179 , 180 , 181 , 182 , 183 , 184 , 185 , 186 ].…”

Section: Management Of Rp-associated Complicationsmentioning

confidence: 99%

“…Furthermore, higher rates of posterior capsular opacification and anterior capsule phimosis have been described following cataract surgery in patients with RP, which may also negatively influence visual outcomes if left untreated [ 178 ]. Despite the challenges regarding cataract surgery in RP, several studies have demonstrated its benefit, showing average BCVA improvements between studies ( Table 1 ) [ 105 , 162 , 178 , 179 , 180 , 181 , 182 , 183 , 184 , 185 , 186 ]. Subjectively, visual improvement was reported in 44.8–96.7% of patients included in these studies [ 177 ].…”

Section: Management Of Rp-associated Complicationsmentioning

confidence: 99%

Retinitis Pigmentosa: Current Clinical Management and Emerging Therapies

Nguyen

Moekotte

Plomp

et al. 2023

IJMS

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Retinitis pigmentosa (RP) comprises a group of inherited retinal dystrophies characterized by the degeneration of rod photoreceptors, followed by the degeneration of cone photoreceptors. As a result of photoreceptor degeneration, affected individuals experience gradual loss of visual function, with primary symptoms of progressive nyctalopia, constricted visual fields and, ultimately, central vision loss. The onset, severity and clinical course of RP shows great variability and unpredictability, with most patients already experiencing some degree of visual disability in childhood. While RP is currently untreatable for the majority of patients, significant efforts have been made in the development of genetic therapies, which offer new hope for treatment for patients affected by inherited retinal dystrophies. In this exciting era of emerging gene therapies, it remains imperative to continue supporting patients with RP using all available options to manage their condition. Patients with RP experience a wide variety of physical, mental and social-emotional difficulties during their lifetime, of which some require timely intervention. This review aims to familiarize readers with clinical management options that are currently available for patients with RP.

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A cohort study of 19 patients with gyrate atrophy of the choroid and retina (GACR)

Balfoort,

Van Den Broeck,

Brands

et al. 2024

Graefes Arch Clin Exp Ophthalmol

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Purpose Gyrate atrophy of the choroid and retina (GACR) is an autosomal recessive inherited metabolic disorder (IMD) characterised by progressive retinal degeneration, leading to severe visual impairment. The rapid developments in ophthalmic genetic therapies warrant knowledge on clinical phenotype of eligible diseases such as GACR to define future therapeutic parameters in clinical trials. Methods Retrospective chart analysis was performed in nineteen patients. Data were analysed using IBM SPSS Statistics version 28.0.1.1. Results Nineteen patients were included with a mean age of 32.6 years (range 8–58). Mean age at onset of ophthalmic symptoms was 7.9 years (range 3–16). Median logMAR of visual acuity at inclusion was 0.26 (range -0.18–3.00). Mean age at cataract surgery was 28.8 years (n = 11 patients). Mean spherical equivalent of the refractive error was -8.96 (range -20.87 to -2.25). Cystoid maculopathy was present in 68% of patients, with a loss of integrity of the foveal ellipsoid zone (EZ) in 24/38 eyes. Of the 14 patients treated with dietary protein restriction, the four patients who started the diet before age 10 showed most benefit. Conclusion This study demonstrates the severe ophthalmic disease course associated with GACR, as well as possible benefit of early dietary treatment. In addition to visual loss, patients experience severe myopia, early-onset cataract, and CME. There is a loss of foveal EZ integrity at a young age, emphasising the need for early diagnosis enabling current and future therapeutic interventions.

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Outcome of Cataract Surgery in Patients With Retinitis Pigmentosa

Cited by 8 publications

References 41 publications

The double-edged sword of inflammation in inherited retinal degenerations: Clinical and preclinical evidence for mechanistically and prognostically impactful but treatable complications

The double-edged sword of inflammation in inherited retinal degenerations: Clinical and preclinical evidence for mechanistically and prognostically impactful but treatable complications

Retinitis Pigmentosa: Current Clinical Management and Emerging Therapies

A cohort study of 19 patients with gyrate atrophy of the choroid and retina (GACR)

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