Predictors of Glaucoma After Pediatric Cataract Surgery.

PRCIS: Glaucoma after pediatric cataract surgery is common and challenging. Age at surgery and the presence of microcornea or other anterior segment (AS) abnormalities can be used to identify those at greatest risk. OBJECTIVE: To establish risk factors for developing glaucoma after pediatric cataract surgery [glaucoma following cataract surgery (GFCS)]. METHODS: Single-center, retrospective, longitudinal study of patients who underwent lensectomy for pediatric cataracts from 2008 to 2020. Included eyes presented with congenital or acquired pediatric cataracts or an anterior form of persistent fetal vasculature, and a follow-up of at least 1 year. Exclusion criteria were the presence of preexisting intraocular pressure elevation, congenital glaucoma, syndromic cataracts, and a history of trauma or uveitis. Demographic and clinical data were collected. Our primary outcome was the development of GFCS. Multivariable logistic regression with generalized estimating equations was used to model the association between potential predictors and the risk of GFCS. RESULTS: A total of 110 eyes from 74 patients were included, 38 with unilateral and 36 with bilateral pediatric cataract surgery. The average surgery age was 24.71 ± 37.26 months, with 74 eyes (67.3%) undergoing surgery ≤12 weeks of age. Patients were followed for 9.96 ± 3.64 years after surgery. Twenty-eight eyes (25.45%) developed GFCS, all requiring glaucoma surgery. In multivariable analysis, surgery before 12 weeks of age [odds ratio (OR): 34.74; P < 0.001], presence of microcornea (OR: 12.90; P = 0.002), and presence of other AS abnormalities (OR: 52.71; P < 0.001) were significantly associated with the development of GFCS. CONCLUSIONS: The development of GFCS is a common and relevant adverse event after pediatric cataract surgery whose management is challenging. Age at surgery, the presence of microcornea, and the presence of other AS abnormalities can be used to identify those at greatest risk.

Cohen Syndrome: Novel VPS13B Genetic Variants in a Male Portuguese Patient with Pigmentary Retinopathy.

The purpose of this clinical report was to describe a case of Cohen syndrome with its classical ophthalmological manifestations and novel VPS13B genetic variants. A 39-year-old Caucasian male patient with severe rod-cone retinal dystrophy and no history of parental consanguinity was referred to our ophthalmology department. Ophthalmologic history included high bilateral myopia and a 3-year prior bilateral cataract surgery. Systemic history included facial dysmorphism, intellectual disability, transient neutropenia, microcephaly, truncal obesity, and joint hyperextensibility. The patient presented classic fundoscopic features of pigmentary retinopathy in both eyes (OU). Optical coherence tomography (OCT) revealed bilateral central and diffuse retinal pigment epithelium (RPE) and outer retinal atrophy without concomitant macular edema, while fluorescein angiography (FA) demonstrated diffuse RPE atrophy with prominent choroidal vessels. The full-field ERG (ffERG) showed no dark-adapted or light-adapted responses and the P50 wave was not identified in the pattern ERG (pERG). The genetic study revealed two novel heterozygous variants in the VPS13B gene: (1) c.5138T>C p.(Leu1713Pro) and (2) c.10179del p.(Asn3393Lysfs*37), thus confirming the diagnosis of Cohen syndrome. This case report introduces these two novel genetic variants to the literature, in a patient with classic phenotypic characteristics of Cohen syndrome, a rare genetic disease which has been increasingly reported since its first description in 1973.

Comparison of Trabeculectomy Outcomes Performed by Residents in Training and Ophthalmologists.

PRCIS: Resident-performed trabeculectomies present higher intraocular pressure and lower complete success rate at 1 year. PURPOSE: To compare the 1-year outcomes of ab externo trabeculectomy between residents in training and staff ophthalmologists. PATIENTS AND METHODS: This retrospective study included all consecutive eyes submitted to ab externo trabeculectomy between January 2015 to June 2020. A 1-year complete success rate was considered using all the following criteria: intraocular pressure (IOP)≤21 mm Hg and ≥6mm Hg without ocular hypotensive medications; IOP reduction≥30%; without loss of light perception, phthisis bulbi, and further glaucoma surgery (excluding suture lysis and bleb needling). RESULTS: One hundred and ten eyes from 99 patients were included. Thirty percent (n=33) of the trabeculectomies were performed by residents. There were no significant preoperative differences between groups, apart from age at surgery, which was higher in the residents' group (72.39±6.83 vs. 62.00±15.07 years, P<0.001), and visual field index (Humphrey Field Analyzer), which was lower in the ophthalmologists' group (51.81±34.74% vs. 32.04±33.83%, P=0.013). IOP at 1-, 3-, 6 months, and 1 year after surgery was significantly higher in the resident's group (P<0.05). Resident-performed trabeculectomies achieved a significantly lower complete success rate when compared with the ophthalmologists' group (39.39% vs. 64.94%, P=0.013). The overall rate of the postoperative complications and reintervention did not differ between groups, but the occurrence of a shallow anterior chamber was more frequent in the residents' group (15.15% vs. 4.05%, P=0.037). CONCLUSIONS: Resident-performed trabeculectomies present significantly higher postoperative IOP levels and a lower complete success rate when compared with staff ophthalmologists. It is, therefore, fundamental to adopt strategies to change this gap, improve patient safety, and strengthen resident confidence.

Effect of Prior Phacoemulsification Surgery in Trabeculectomy Surgery Outcomes.

PURPOSE: To evaluate whether previous clear-cornea phacoemulsification surgery affects the surgical outcomes of trabeculectomy in open-angle glaucoma (OAG). METHODS: We performed a retrospective cohort study, which included 82 patients with OAG that underwent trabeculectomy between January 1, 2010, and December 31, 2017. The primary outcome was the probability of surgical failure. Failure was defined as IOP >21 mmHg or reduced <20% from baseline, IOP ≤5 mmHg in three consecutive visits, need for further glaucoma surgery, phthisis or loss of light perception vision due to glaucoma. RESULTS: Eighty-two eyes (58 phakic and 24 pseudophakic) were included. Phakic group patients were younger than those in the pseudophakic group, 65.8 ± 11.7 vs 76.2 ± 7.9 years (p < 0.001). The most common type of glaucoma was primary OAG [59% (n = 34) phakic vs 63% (n = 15) pseudophakic], followed by exfoliative and pigmentary glaucomas. The mean preoperative IOP was not significantly different between groups nor was the number of preoperative hypotensive medications. The rate of surgical failure was not significantly different between groups at year 1 [17% (n = 10) phakic vs 29% (n = 7) pseudophakic; p = 0.361] nor at year 2 [28% (n = 16) phakic vs 46% (n = 11) pseudophakic; p = 0.110]. No significant differences were observed regarding the postoperative IOP or any secondary outcome measures at year 1 or 2. CONCLUSION: Previous clear-cornea phacoemulsification surgery does not lead to statistically significant differences in the rate of trabeculectomy failure. Despite not being significant, clinically relevant differences were observed between groups. Future studies with a larger sample and/or randomized are needed to clarify this association.

WAGNER syndrome: anatomic, functional and genetic characterization of a Portuguese family.

PURPOSE: To report the clinical (anatomic and functional) and genetic findings of Wagner Syndrome (WS) in a Portuguese family. METHODS: Nine members of the family agreed to be examined. All had complete clinical eye examinations. The proband and selected patients underwent color fundus photography, spectral domain optical coherence tomography (SD-OCT), automatic static white-on-white computerized perimetry, and electrophysiology assessment (flash ERG, multifocal(mf) ERG and dark adaptometry). A pedigree was constructed based on interviews with known affected subjects. Genomic DNA samples derived from venous blood were collected from all affected family members examined. RESULTS: Twenty-eight family members are affected. This family has the typical features of Wagner Syndrome, namely an empty vitreous cavity with veils, mild myopia and cataract. Four examined patients underwent vitreoretinal surgery due to abnormal peripheral vitreoretinal adhesions with peripheral retinal traction (n = 3). Retinal detachment was observed in 5 of the examined subjects. Four of them occurred between the ages of 5 and 15 years. Chorioretinal atrophy is also a frequent finding which results in moderate to severe visual field and advanced rod-cone dystrophy from younger ages, also confirmed by absence of scotopic function on dark adaptation. The macular dysfunction on mfERG was profound and of early onset. A heterozygous mutation in intron 7 of the VCAN gene (c.4004-1G > A) was found. CONCLUSIONS: We described a rare autosomal dominant vitreoretinopathy with near complete penetrance in a Portuguese family. Abnormal peripheral vitreoretinal adhesions, retinal detachment and chorioretinal atrophy are present in most of the examined individuals at young ages. Early onset of advanced visual field and electrophysiologic abnormalities were observed in this family. We also added relevant information to the literature by reporting our experience in surgical management of Wagner Syndrome patients with, and at risk of, retinal detachment.

Long-Term Effect of Anti-VEGF Agents on Intraocular Pressure in Age-Related Macular Degeneration.

PURPOSE: To analyze the effect of anti-vascular endothelial growth factor (VEGF) agents on intraocular pressure (IOP) in patients with neovascular age-related macular degeneration (AMD). Materials andMethods: This is a retrospective study that included 72 patients treated unilaterally with anti-VEGF agents according to a pro re nata regimen. Fellow noninjected eyes (n = 72) were used as controls. IOP variation and the development of sustained ocular hypertension (OHT) were assessed both in the injected and in the fellow eyes. RESULTS: While the final IOP was not significantly different between the 2 groups, sustained OHT developed in 4.2% of the injected eyes and 1.4% of the controls. In the study group, no significant IOP variation was noted during follow-up in patients receiving ≤20 injections, but there was a significant increase in IOP with time in more frequently treated patients (p = 0.041). Comparison of both subgroups demonstrated that patients receiving >20 injections suffered significantly greater IOP variation (p = 0.034) during follow-up, and that these patients tended to require IOP-lowering treatment more frequently (p = 0.090). CONCLUSION: Multiple anti-VEGF injections lead to an increase in IOP, although this variation is not sufficient to cause development of OHT in the majority of patients.

Comparison of topical dorzolamide and ketorolac treatment for cystoid macular edema in retinitis pigmentosa and Usher's syndrome.

PURPOSE: To investigate the topical effect of dorzolamide versus ketorolac on retinitis pigmentosa (RP) and Usher's syndrome (US) macular edema. METHODS: Prospective, randomized and interventional study. A total of 28 eyes of 18 patients were included. Five eyes had US, 23 had RP. Fifteen eyes were allocated to ketorolac tromethamine 0.5% (4 drops daily regimen) and 13 eyes to dorzolamide hydrochloride 2% (3 drops daily regimen) treatment groups. Snellen's best-corrected visual acuity (BCVA), foveal thickness (FT) and foveal zone thickness (FZT) measured by Stratus® optical coherence tomography (OCT) were evaluated at baseline, 1, 3, 6 and 12 months after treatment. RESULTS: Patients assigned to ketorolac had a baseline BCVA of 0.37 ± 0.17 logMAR which improved at the end of 1 year to 0.28 ± 0.16 (p = 0.02). Three eyes (20%) of 2 patients improved by 7 letters or more. Mean FT and FZT did not change significantly during the study follow-up. After 1 year of treatment, 4 eyes (27%) of 3 patients showed an improvement of at least 16% of FT and 11% of FZT. Patients assigned to dorzolamide had a baseline BCVA of 0.48 ± 0.34 logMAR which improved in the first 6 months (0.40 ± 0.30; p = 0.01), with a decrease at 1 year (0.42 ± 0.27; p = 0.20). Seven eyes (54%) of 5 patients had an improvement of 7 letters or more. Mean FT and FZT did not change significantly either. After 1 year of treatment, 3 eyes (23%) of 2 patients showed an improvement of at least 16% on FT and 11% on FZT. CONCLUSIONS: RESULTS suggest that dorzolamide and ketorolac might improve visual acuity and therefore be of interest in selected cases. No relationship between retinal thickness fluctuation and visual acuity was found. Sample size was a limitation to the study.

Complete spontaneous posterior luxation of capsular bag-intraocular lens-capsular tension ring complex.

We describe the case of a monocular 72-year-old man who presented with spontaneous acute visual decrease in the left eye 2 years after uneventful phacoemulsification with intraocular lens (IOL) and standard capsular tension ring (CTR) implantation. Dilated fundoscopy revealed aphakia and complete vitreous luxation of the entire capsular bag-IOL-CTR complex.