Pre- and postoperative OCT features and surgical outcomes of advanced retinitis pigmentosa with macular hole: case series and literature review.

BACKGROUND: Macular hole (MH) is a rare complication of retinitis pigmentosa (RP) and has an adverse impact on residual visual function. At present, the underlying mechanisms are not fully understood and surgical experience is limited. METHODS: We reviewed the medical records and optical coherence tomography (OCT) scans in a cohort of eight eyes of seven RP patients with MH in order to report their OCT features and vitreoretinal surgical prognosis. RESULTS: This study includes four lamellar macular holes (LMHs) and four full-thickness macular holes (FTMHs). Pre-operative OCT revealed other macular abnormalities in all eyes, such as epiretinal membrane (ERM), cystoid macular edema (CME), lamellar hole-associated epiretinal proliferation (LHEP) and vitreoretinal traction. MH progression and subjective vision worsening were noted in one LMH eye during a seven-month follow-up. All holes closed after vitrectomy with internal limiting membrane (ILM) peeling. At final follow-up, one eye had improved vision and seven eyes remained stable compared to baseline. CONCLUSIONS: The occurrence of MH in RP is accompanied by various imaging characteristics, such as ERM, CME and LHEP, suggesting a multifactorial pathogenesis. Considering poor vision in most RP patients with potentially progressive MH, surgery appears to be effective in maintaining or improving the central vision in a period of time. Thus, vitrectomy should be performed as soon as possible and flap-assisted techniques or episcleral surgeries are needed for some special cases.

Emotional State Evaluation of Retinitis Pigmentosa Patients with the Beck Depression Inventory.

Objectives: To evaluate the incidence and severity of depression in patients with retinitis pigmentosa (RP). Materials and Methods: The Beck Depression Inventory (BDI) was administered to 74 patients with RP and 60 healthy controls. Biomicroscopic anterior segment and fundus examination, visual field, optical coherence tomography, and full-field electroretinography tests were performed in all cases. Variables were evaluated with bivariate, multiple linear, and ordinal logistic regression analyses. Results: The RP group included 40 (54%) male and 34 (46%) female patients, while the control group included 23 (38%) male and 37 (62%) female subjects. The patient group had a mean age of 39.20±12.4 years, median best corrected visual acuity (BCVA) of 0.10 decimal (1.0 logarithm of the minimum angle of resolution [logMAR]; range, 1.3-0.7 logMAR), and median visual field mean deviation (MD) score of -28.00 decibels (dB) (range, -1.00 to -34.00 dB). The median BDI score was statistically significantly higher in the patient group (19 points) than in the control group (12 points) (p<0.001). Moderate to severe depression (BDI ≥20) was detected in 61% of patients, while this rate was 25% in healthy controls. BCVA and visual field MD values were identified as predictors of depression score and severity level. The patients' age and gender did not affect total depression score or severity. Conclusion: The prevalence and severity of depression were found to be higher in RP patients than in healthy controls. There was a significant relationship between the patient's functional vision tests and the frequency and severity of depression. Depression reduces the reliability of visual function tests and impairs patients' quality of life. Therefore, assessing mental health as well as functional tests is important in patients with RP.

Spontaneous Ectopia Lentis in Retinitis Pigmentosa: A Case Report and Review of the Literature.

Purpose: We report the successful surgical treatment of a case of spontaneous complete anterior crystalline lens luxation in a patient affected by retinitis pigmentosa (RP), associated with elevated intraocular pressure and pupillary block. Additionally, we review the current literature regarding the association between ectopia lentis and RP. Case description: A 44-year-old female RP patient presented to our emergency department reporting severe ocular pain in her left eye (LE) and sickness. She had no history of ocular trauma and did not report systemic disorders. The best corrected visual acuity at presentation was 1/20 in her LE, the intraocular pressure was 60 mmHg, and slit lamp examination showed in her LE a complete dislocation of the lens in the anterior chamber, with mydriasis, atalamia, and a pupillary block. The patient had been administered intravenous mannitol 18% solution and dorzolamide-timolol eye drops and was hospitalized for urgent lens extraction. Anterior segment optical coherence tomography and ultrasound biomicroscopy were performed before surgery. Decompressive 23-gauge pars plana vitrectomy and phacoemulsification were performed, and the capsular bag was removed due to marked zonular weakness, with deferred intraocular lens implant. Conclusions: Acute angle closure glaucoma in patients with RP may be rarely caused by spontaneous anterior lens dislocation. To our knowledge, this is the first report of spontaneous anterior lens dislocation in an RP patient, documented through photographs, anterior segment optical coherence tomography, and ultrasound biomicroscopy.

Mucopolysaccharidosis type II B complicated by optic disc swelling, pigmentary retinopathy and macular edema.

PURPOSE: To report clinical and imaging features of optic nerve and retinal involvement in a patient with mucopolysaccharidosis (MPS) type II B. METHODS: A 27-year-old man, diagnosed with MPS type II B and undergoing enzymatic substitution therapy for the past 19 years, was referred to the retina service. An ophthalmological evaluation, which included multimodal imaging, was conducted to investigate potential retinal and optic disc involvement. RESULTS: The eye examination revealed a pigmentary retinopathy with a predominant loss of the outer retinal loss, primarily in the parafoveal and perifoveal regions. Notably, multimodal imaging identified macular edema without any signs of leakage, implying an association between macular edema and retinal neurodegeneration. Additionally, both eyes exhibited an optic disc with blurred margins. CONCLUSION: We herein describe the multimodal imaging findings of retinal and optic disc involvement in a patient with MPS type II B. This report describes for the first-time the presence of macular edema without leakage alongside photoreceptor damage and optic disc swelling.

Quality of Life Analysis in Patients with Retinitis Pigmentosa.

INTRODUCTION: Retinitis pigmentosa (RP) is a chronic progressive disease causing loss of visual acuity and ultimately blindness. This visual impairment can contribute to psychiatric comorbidity and worse overall quality of life (QOL). Our goal was to assess the relationship between the severity of disease for people with RP and QOL as it pertains to mental health, social support, disability resources, and financial factors. METHODS: This was a survey study conducted from June 2021 to February 2022 including 38 people with RP. QOL was assessed through a survey questionnaire focusing specifically on demographics, visual function, family, employment, social support, and mental health/well-being. Statistical analysis was conducted using a χ2 test for significance. RESULTS: A best corrected visual acuity (BCVA) of less than 20/200 (p = 0.0285) and living alone (p = 0.0358) were both statistically significant independent risk factors for experiencing depressive symptoms. Highest education level attained and unemployment rate were not found to be related to the development of depressive symptoms. Subjects had a higher unemployment rate (64% vs. US rate of 3.6%) and a high likelihood of reporting depressive symptoms (47.4%). CONCLUSION: People with RP are more likely to be unemployed and to develop depressive symptoms when compared to the general population. Similar to previous studies' findings, those with a BCVA of less than 20/200 were statistically more likely to experience depressive symptoms; living alone is a novel risk factor that is also associated with the presence of depressive symptoms. Contrary to prior findings, highest education level and unemployment status were found not to be related to the development of depressive symptoms. These patients may benefit from regular depression screenings and optional establishment of care with a psychiatrist or psychologist if they live alone or their BCVA is 20/200 or worse.

Linear streaks associated with retinitis pigmentosa.

PURPOSE: To describe a phenotypical manifestation characterized by the identification of peripheral linear streaks associated with retinitis pigmentosa (RP). METHODS: Study design is a prospective observational case series. All consecutive patients affected by RP underwent a complete ophthalmological examination. The diagnosis of peripheral linear streaks was based on the identification of curvilinear atrophic streaks in the periphery of the retina. RESULTS: Overall, six out of 140 patients (4.2%) were affected by peripheral linear streaks associated with RP. A single patient showed also punched out chorioretinal lesions at the posterior pole, with macular neovascularization development over the follow-up, treated with ranibizumab injections. CONCLUSIONS: RP phenotypical manifestation characterized by peripheral linear streaks is infrequent and may provide additional evidence to support the contribution of inflammation in the pathogenesis of RP.

Cataract Surgery Outcomes in Retinitis Pigmentosa A Comparative Clinical Database Study.

PURPOSE: To report visual acuity (VA) outcomes, intraoperative and postoperative complications of isolated cataract surgery in eyes with retinitis pigmentosa (RP), compared with non-RP-affected eyes. DESIGN: Retrospective clinical cohort study. METHODS: A total of 113,389 eyes underwent cataract surgery between July 2003 and March 2015 at 8 clinical sites in the United Kingdom. Eyes with RP as the only comorbid pathology and eyes without any ocular comorbidities (controls) undergoing cataract surgery were compared. VA at 4 to 12 weeks postoperatively and rates of intraoperative and postoperative complications are reported. RESULTS: Seventy-two eyes had RP. The mean age in the RP group was 57 ± 15 compared to 75 ± 10 in controls (P < .001). Females represented 46% of RP cases and 60% of controls (P = .06). Preoperative VA (mean LogMAR = 1.03 vs 0.59, P < .001) and postoperative VA (0.71 vs 0.14, P < .001) were worse in RP group. The mean VA gain was 0.25 ± 0.60 LogMAR in RP vs 0.43 ± 0.48 LogMAR in controls (P < .001). There were no significant differences in the rate of intraoperative pupil expansion use, posterior capsular tears, or zonular dialysis. Postoperative cystoid macular edema developed in 6.9% of RP eyes and 1% of controls (P < .001). The need for IOL repositioning or exchange was not statistically different between the two groups. CONCLUSION: Cataract surgery can improve vision in eyes with RP and cataract. Intraoperative complications were similar to control eyes; however, RP eyes experienced more frequent postoperative cystoid macular edema.

DEXAMETHASONE IMPLANT VERSUS TOPICAL CARBONIC ANHYDRASE INHIBITORS IN PATIENTS WITH BILATERAL RETINITIS PIGMENTOSA-RELATED CYSTOID MACULAR EDEMA: A Prospective, Paired-Eye Pilot Study.

PURPOSE: To compare within-subject efficacy and safety of intravitreal dexamethasone implant and topical carbonic anhydrase inhibitors in the treatment of retinitis pigmentosa-related cystoid macular edema. METHODS: Patients with bilateral retinitis pigmentosa-related cystoid macular edema were treated with intravitreal dexamethasone implant in one eye and topical carbonic anhydrase inhibitors in the contralateral eye. The primary endpoint was a change in central macular thickness. Secondary endpoints were changes in best-corrected visual acuity and microperimetric central retinal sensitivity. Intraocular pressure and other ocular complications were evaluated for safety assessment. RESULTS: Nine patients were recruited for this 12-month follow-up study. Central macular thickness was significantly lower in intravitreal dexamethasone implant-treated eyes than in topical carbonic anhydrase inhibitors-treated eyes at Months 1 and 7, whereas mean best-corrected visual acuity was better in eyes treated with topical carbonic anhydrase inhibitors at Month 12 (borderline significant P = 0.0510). There was no difference in microperimetric sensitivity between the two treatments. Three patients developed ocular hypertension after intravitreal dexamethasone implant. Intravitreal dexamethasone implant showed an effect on the contralateral eye in five of nine patients. CONCLUSION: Intravitreal dexamethasone implant was more effective than topical carbonic anhydrase inhibitors in reducing retinitis pigmentosa-related cystoid macular edema 1 month after treatment. Corticosteroids can play a key role in the management of retinitis pigmentosa-related cystoid macular edema; however, their routes, timing, and modes of administration should be further explored.

Evaluation of photoreceptor features in retinitis pigmentosa with cystoid macular edema by using an adaptive optics fundus camera.

OBJECTIVE: Cystoid macular edema (CME) in retinitis pigmentosa (RP) is an important complication causing visual dysfunction. We investigated the effect of CME on photoreceptors in RP patients with previous or current CME, using an adaptive optics (AO) fundus camera. METHODS: We retrospectively observed the CME and ellipsoid zone (EZ) length (average of horizontal and vertical sections) by optical coherence tomography. The density and regularity of the arrangement of photoreceptor cells (Voronoi analysis) were examined at four points around 1.5° from superior to inferior and temporal to nasal. We also performed a multivariate analysis using CME duration, central macular thickness and transversal length of CME. RESULTS: We evaluated 18 patients with previous or current CME (18 eyes; age, 48.7 ± 15.6 years) and 24 patients without previous or current CME (24 eyes; age, 46.0 ± 14.5 years). There were no significant differences in age, logMAR visual acuity, or EZ length. In groups with and without CME, cell density was 11967 ± 3148 and 16239 ± 2935 cells/mm2, and sequence regularity was 85.5 ± 3.4% and 88.5 ± 2.8%, respectively; both parameters were significantly different. The correlation between photoreceptor density and age was more negative in group with CME. The CME group tended toward greater reductions in duration of CME. CONCLUSION: Complications of CME in RP patients may lead to a decrease in photoreceptor density and regularity. Additionally, a longer duration of CME may result in a greater reduction in photoreceptor density.

Outcomes of vitreoretinal surgery in eyes with retinitis pigmentosa without retinal detachment.

PURPOSE: Outcomes of retinal detachment (RD) have been discussed in detail in many reports of patients with retinitis pigmentosa (RP). This study tries to understand the outcomes of vitreoretinal (VR) surgery for indications other than RD in the eyes with RP. METHODS: This is a retrospective study that includes clinical data from January 2013 to December 2021. Patients with RP who were treated with a VR surgical intervention were included in the study. The primary outcome of the study was to assess the changes in best-corrected visual acuity. RESULTS: Forty-four eyes of 40 patients with RP were included in the study. Nearly half of the eyes (43%, 19/44) presented from 1 month to 1 year after the onset of diminished vision, with or without floaters. The mean ± standard deviation (SD) best-corrected visual acuity (BCVA) at presentation was 1.30 ± 0.79 logMAR (20/400 ± 20/125). The major surgical indications were vitreous opacities (43.2%, 19/44) and subluxated/dislocated cataractous lenses (25%, 11/44). The median follow-up duration was 8 months (interquartile range (IQR): 1.5-27). Approximately 77% (34/44) of the eyes had improvement in vision. The mean postoperative BCVA at the last follow-up was 0.95 ± 0.73 logMAR (p-value: 0.03). CONCLUSIONS: Most eyes with RP recovered well after VR surgical interventions, with short-term improvements in visual acuity. It may be crucial to address the vitreous opacities and membranes as they hinder the residual central island of vision in RP. However, appropriate counselling is required regarding the progressive nature of retinal neuronal degeneration.

Protocol for the treatment of cystoid macular edema secondary to retinitis pigmentosa and other inherited retinal dystrophies.

Inherited retinal dystrophies (IRD) are the leading cause of legal blindness in the working population. Cystic macular edema (CME) is one of the treatable causes of visual loss, affecting up to 50% of the patients. A bibliographic review has been carried out combining "inherited retinal dystrophy", "retinitis pigmentosa", "macular oedema" and a diagnostic-therapeutic protocol according to the levels of evidence and recommendations of the "US Agency for Healthcare Research and Quality". This protocol has been discussed in the monthly meetings of the XAREA DHR group with the participation of more than 25 ophthalmologists, creating a consensus document. The etiology of CME is multifactorial: dysfunction of the blood-retinal barrier, retinal pigment epithelium, and Müller cells, inflammation, and vitreous traction. OCT is the test of choice for the diagnosis and follow-up of CME associated with IRD. The drugs with the highest degree of scientific evidence are carbonic anhydrase inhibitors (IAC). Intravitreal corticosteroids, anti-VEGF, and vitrectomy with peeling of the internal limiting membrane do not have sufficient evidence. A treatment scheme is proposed for the CME in IRD in adults, another for pediatric patients and another for IRD and cataract surgery. Oral and topical IACs are effective in the treatment of CME secondary to IRD. Treatment with corticosteroids, anti-VEGF, and vitrectomy are second-line options. Randomized clinical trials are required to establish the therapeutic scale in these patients.

Genotypes and clinical features of RHO-associated retinitis pigmentosa in a Japanese population.

PURPOSE: To report the genotypes and clinical features of RHO-associated retinitis pigmentosa (RHO-RP) in the Kyushu region of Japan. STUDY DESIGN: Retrospective, single-center study. METHODS: Sixteen RP patients with pathogenic RHO variants seen at Kyushu University Hospital were investigated. Clinical data including age, best-corrected visual acuity (BCVA) in logarithm of the minimum angle of resolution (logMAR) units, visual field, fundus photography, and optical coherence tomography were retrospectively obtained. Visual outcomes were compared between classical and sector phenotypes and among genetic variants. RESULTS: The mean age at the first visit was 54.0 ± 15.7 years, with a mean follow-up of 7.6 ± 4.0 years. Fourteen patients (87.5%) showed the classical RP phenotype, of whom four were associated with p.[Pro23Leu] and two had p.[Pro347Leu] variants. In addition, two patients with the sector phenotype harbored p.[Ala164Val] variants. Among the classical RHO-RP patients, the mean BCVA decreased from 0.60 to 1.08 logMAR over the follow-up period (7.4 ± 4.1 years) whereas BCVA was preserved at 0.04 logMAR in sector RHO-RP patients (9.0 ± 3.0 years). Genotype-to-phenotype analysis demonstrated that p.[Pro347Leu] was associated with severe vision loss at an earlier age. Macular complications such as epiretinal membrane and cystoid macular edema were observed in 5 classical RHO-RP patients. CONCLUSION: p.[Pro23Leu], but not p.[Pro23His], was a frequent variant causing RHO-RP in the Kyushu region of Japan. As reported in previous studies, patients with the p.[Pro347Leu] variant showed a more severe phenotype, and variants causing sector RHO-RP were associated with a good prognosis.

Atypical retinopathy in ataxia with vitamin E deficiency: report of a sibship.

Typical retinitis pigmentosa (RP) may not be the only retinal phenotype encountered in ataxia with vitamin E deficiency (AVED). The following short case series describes a novel form of retinopathy in AVED. We describe two patients with AVED belonging to the same consanguineous sibship. Both presented an unusual retinopathy consisting of scattered, multifocal, nummular, hyperautofluorescent atrophic retinal patches. The retinopathy remained stable under vitamin E supplementation. We hypothesize these changes to be the result of arrested AVED-related RP following early supplementation with α-tocopherol acetate.

HIGH MYOPIA IS COMMON IN PATIENTS WITH X-LINKED RETINOPATHIES: Myopic Maculopathy Analysis.

PURPOSE: High myopia can occur as a single or syndromic condition. The aim of this study was to evaluate the refractive error and myopic maculopathy in patients with X-linked retinopathies. METHODS: Whole exome sequencing, Sanger sequencing, and comprehensive ocular examinations were performed in patients with X-linked retinopathies. RESULTS: A total of 17 patients were recruited, including six with CACNA1F, seven with RPGR, three with NYX, and one with OPN1MW mutations. The diagnoses were congenital stationary night blindness (6), cone-rod dystrophy (4), retinitis pigmentosa (4), achromatopsia (1), Leber congenital amaurosis (1), and myopia (1). Myopia was present in 88.2% patients, and 64.7% patients had high myopia. Gene analysis showed that high myopia was present in 80% patients with CACNA1F, 100% patients with NYX, and 57.1% patients with RPGR mutations. In the ATN classification, 64.7% of the patients were A1T0N0 and 35.3% were A0T0N0. The refractive errors progressed over time, even in patients with congenital stationary night blindness. Two females with heterozygous de novo RPGR mutations presented with retinitis pigmentosa or cone rod dystrophy combined with high myopia. CONCLUSION: High myopia is common in patients with X-linked retinopathies, and myopic maculopathy was only mild atrophy without traction and neovascularization.

Association of Circulating Antiretinal Antibodies With Clinical Outcomes in Retinitis Pigmentosa.

Purpose: To determine if circulating antiretinal antibodies (ARAs) differ between patients affected by retinitis pigmentosa (RP) and control participants and to assess whether ARAs are associated with clinical outcomes in patients with RP. Methods: Cross-sectional study involving a group of patients clinically diagnosed with RP and a control group of healthy participants. Serum autoantibodies against enolase, heat shock protein 70 (HSP70), and carbonic anhydrase II (CAII) were tested in all participants using Jess capillary Western blot. We compared ARA prevalence between the RP and control groups and investigated the association of serum ARA positivity with macular edema and vitreomacular disorders in patients affected by RP. Results: Thirty-six patients affected by RP and a control group of 39 healthy individuals were included. Overall, at least one ARA positivity was detected in 89% and 80% of participants in the RP and control groups, respectively. We observed a similar prevalence of anti-CAII and anti-enolase ARA between patients and controls (P = 0.87 and P = 0.35, respectively). Sera from patients with RP tested positive for anti-HSP70 ARAs more frequently than those from controls (53% vs. 36%), albeit without reaching statistical significance (P = 0.29). Among the 72 eyes with RP, 25% presented with macular edema (most often bilateral) and 33% with epiretinal membrane and/or lamellar macular hole. None of the three ARAs was associated with an increased risk of any macular complications in eyes affected by RP (all P > 0.05). Conclusions: The prevalence of circulating ARAs against enolase, HSP70, and CAII is similar between patients affected by RP and healthy individuals. Our results provide evidence against the association of ARAs with macular edema and vitreomacular interface disorders in RP.

Effect of Retinitis Pigmentosa Blindness on Functional Brain Network Measures.

Our brain undergoes significant alteration in response to blindness. Understanding these neuroplastic changes and finding reliable methods for quantification of such reorganization is consequential for devising optimum sight restoration therapies and clinical rehabilitation. In recent years, graph theory measures have been applied to brain connectivity, but few studies have examined blindness-induced cortical alterations using these methods. In this study, we investigated alterations in key global and local measures of functional plasticity in the brain network after visual deprivation. Four resting-state functional MRI runs from 10 sighted and 10 blind subjects, with severe retinitis pigmentosa, provided data for our analysis. The whole-brain ROI-ROI resting-state functional connectivity (rsFC) matrices were created for all subjects. Using the resulting rsFC matrices, the global density and efficiency were extracted for the whole brain and the visual system. Additionally, weighted degree, as a local metric, was investigated for all the nodes in the functional brain network. Our results revealed a statistically significant decrease in global density and efficiency of the visual system and weighted degree for lower visual areas, following visual deprivation in the blind group. The explored network measures can serve as tools to gauge functional neuroplasticity following blindness, as a complement to the behavioral indices. Future investigation with a larger number of test subjects for this rare condition can enable correlation analysis with patients' characteristics and further verify the application of these metrics as biomarkers of brain reorganization following vision loss.

Application of intravitreal aflibercept to treat bilateral exudative retinal detachment secondary to retinitis pigmentosa: Case report and review of literature.

RATIONALE: Exudative retinal detachment with macular edema is one of the complications of retinitis pigmentosa (RP). In this report, we present a case who treated with intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) in RP-related exudative retinal detachment and subsequently improved with favorable outcome. PATIENT CONCERN: A 49-year-old man, with a history of RP, had persistent blurred vision and was newly diagnosed with bilateral shallow exudative retinal detachment and macular edema. DIAGNOSIS: Fluorescein angiography showed bilateral diffuse dye leakage with macular pooling, and systemic survey excluded the possibility of infection or autoimmune disease. INTERVENTIONS: The patient was treated with intravitreal injection of aflibercept, one of the anti-VEGF agents, for bilateral eyes. Recurrent exudative retinal detachment and macular edema were noted, and repeated intravitreal injections of aflibercept in bilateral eyes were then arranged. Subsequently, bilateral macular edema and exudative retinal detachment subsided again, and the treatment course lasted for approximately 1 year. OUTCOMES: After 1 year, the exudative retinal detachment with macular edema was much improved. In the meanwhile, visual functional improvement was also achieved. LESSONS: This case illustrated the possibility of intravitreal injection of anti-VEGF therapy for the treatment of this rare complication of RP, and it may be a newly explored alternative treatment.

Bilateral angle closure glaucoma with retinitis pigmentosa in young patients: case series.

BACKGROUND: To report the ocular characteristics and management of three cases of retinitis pigmentosa (RP) concurrent primary angle closure glaucoma (PACG). CASE PRESENTATION: Three middle-aged patients presenting with diminished vision, high intraocular pressure (IOP), and typical fundus manifestations of RP were clinically evaluated. The individualized treatment was based on the ocular conditions of each case. A novel genetic alteration in ZNF408 was identified in one patient. Two patients with short-axial eyes received unilateral combined trabeculectomy, cataract surgery, and Irido-zonulo-hyaloid-vitrectomy. One of them had a subluxated lens, managed with a capsular tension ring implantation. Their contralateral eyes, respectively, underwent laser peripheral iridotomy (LPI) and transscleral cyclophotocoagulation. The third patient underwent bilaterally combined laser peripheral iridoplasty, LPI, and medication. Ultimately, all patients achieved the target IOP during a two-year follow-up. CONCLUSION: Young patients with RP may have a risk of developing angle closure glaucoma, and conversely, patients with angle closure glaucoma at younger age should be aware of the presence of RP. Therefore, routine gonioscopy and IOP monitoring are required for RP patients, and detailed fundus examinations are warranted for young PACG patients.