Influence of the Phenylephrine Test in Outcomes of External Levator Advancement for Involutional Ptosis.

PURPOSE: This study aims to evaluate the influence of preoperative phenylephrine testing on the surgical outcome of patients undergoing surgery for involutional ptosis by external levator advancement. METHODS: This was an observational, monocentric, retrospective study. Fifty-one eyelids from 32 patients, who had surgery between January 2018 and May 2023, were included for analysis. Preoperative clinical examination data were collected. Evaluation was performed at 1 month postoperatively. Surgical success was defined by a postoperative margin reflex distance between 3 and 5 mm inclusive. Symmetry success was defined by a difference in margin reflex distance between the 2 upper eyelids of no more than 1 mm. RESULTS: The surgical success rate was 86%. A positive preoperative phenylephrine test was significantly associated with a better surgical success rate (p = 0.01), including on symmetry (p = 0.01). The secondary outcomes, namely preoperative margin reflex distance, function of the upper eyelid levator muscle, and unilaterality of surgery, were not statistically associated with surgical outcome. CONCLUSIONS: The phenylephrine test is a predictive factor of surgical success in patients undergoing external levator advancement. Our study suggests that patients with a negative phenylephrine test should be overcorrected intraoperatively.

Risk factors associated with progression from papilloedema to optic atrophy: results from a cohort of 113 patients.

BACKGROUND: The aim of this study was to assess the risk factors for atrophic progression of patients with papilloedema secondary to intracranial hypertension, using optical coherence tomography parameters. METHODS: A retrospective study was conducted at Marseille University Hospitals' Ophthalmology departments between December 2015 and December 2021. All patients with papilloedema resulting from elevated intracranial hypertension at the initial presentation were included. Ophthalmological evaluations included analysing retinal nerve fibre layer (RNFL), ganglion cell layer (GCL) and total peripapillary retinal thickness (RT). RESULTS: The study included 222 eyes from 113 patients. The main aetiologies of intracranial hypertension were idiopathic intracranial hypertension (49/113), intracranial tumours (33/113) and cerebral venous thrombosis (15/113). The initial RNFL and RT showed significant correlations with optic atrophy. The mean RNFL was 199.63 µm in the 'no atrophy' group and 365.28 µm in the 'atrophy' group (p<0.001). Similarly, the mean RT was 483.72 µm in the 'non-atrophy' group and 796.69 µm in the 'atrophy' group (p<0.001). The presence of peripapillary haemorrhages showed a strong correlated with optic atrophy with an OR=19.12 (p<0.001). Impaired initial visual acuity was also associated with final optic atrophy with an OR=7.76 (p=0.020). Furthermore, impaired initial GCL was a major predictor of optic atrophy (OR=18.25 (p=0.021)). CONCLUSION: Our study highlights the risk factors for optic atrophy in papilloedema, aiming to facilitate the early detection of patients at a high risk of vision loss and enable more aggressive medical or surgical management.

Keratoconus and the Impact of Treatment on Patients' Quality of Life: A Qualitative Study.

INTRODUCTION: Keratoconus has a significant impact on patients' quality of life (QoL), from diagnosis to the advanced stages of the disease. The aim of this research was to identify domains of QoL affected by this disease and its treatment. METHODS: Phone interviews were conducted using a semi-structured interview guide, with patients with keratoconus stratified according to their current treatment. A board of keratoconus experts helped identify the guide's main themes. RESULTS: Thirty-five patients (rigid contact lenses, n = 9; cross-linking, n = 9; corneal ring implants, n = 8; and corneal transplantation, n = 9) were interviewed by qualitative researchers. Phone interviews revealed several QoL domains affected by the disease and its treatments: "psychological", "social life", "professional life", "financial costs" and "student life". All domains were impacted, independently of the treatment history. Few differences were found between treatment regimens and keratoconus stages. Qualitative analysis enabled the development of a conceptual framework based on Wilson and Cleary's model for patient outcomes common to all patients. This conceptual model describes the relationship between patients' characteristics, their symptoms, their environment, their functional visual impairment and the impact on their QoL. CONCLUSIONS: These qualitative findings supported the generation of a questionnaire to evaluate the impact of keratoconus and its treatment on patients' QoL. Cognitive debriefings confirmed its content validity. The questionnaire is applicable for all stages of keratoconus and treatments and may help tracking change over time in regular clinical settings. Psychometric validation is yet to be performed before its use in research and clinical practices.

Paracentral Acute Middle Maculopathy as a Specific Sign of Arteritic Anterior Ischemic Optic Neuropathy.

PURPOSE: The purpose of this study is to assess the diagnostic accuracy of paracentral acute middle maculopathy (PAMM) in the setting of anterior ischemic optic neuropathy (AION) to distinguish arteritic (A-AION) from nonarteritic (NA-AION) type. DESIGN: Retrospective cross-sectional diagnostic evaluation. METHODS: PAMM was evaluated by 3 physicians blinded to diagnosis using macular spectral-domain optical coherence tomography. We studied 45 patients with AION. Of those, 28 had NA-AION and 17 had A-AION. The study was conducted in the Department of Ophthalmology at the Hospital of Marseille-Assistance Publique, France, from January 1, 2018, to March 31, 2022. RESULTS: PAMM were only found in the A-AION group (N = 4) (P = .0143). As a distinctive sign of A-AION, we found a specificity of 100% (95% IC, 88.06%-100%) and a positive predictive value of 100%. In contrast, sensitivity and negative predictive value were lower, 19.1% (95% IC, 5.5-42.0) and 63.0% (95% CI, 58.1-67.7), respectively. CONCLUSIONS: The PAMM finding is highly specific for A-AION in the setting of AION. According to our results, macular spectral-domain optical coherence tomography looking for PAMM should be performed with any patient presenting with AION.

Evolution of Quantitative Optical Coherence Tomography Angiography Markers with Glycemic Control: A Pilot Study.

Aim: We aimed to analyze changes in retinal microvascularization with intensive reduction of glycated hemoglobin A1c (HbA1c) in patients with poorly controlled diabetes using quantitative optical coherence tomography angiography (OCT-A) metrics. Method: This was a retrospective observational study in patients with uncontrolled diabetes admitted to the hospital for glycemic control. A second set of 15 healthy volunteers was included to serve as a control group. OCT-A was performed at inclusion and at 3 months to measure foveal avascular zone area (FAZA), vessel density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP), acircularity index (AI), and fractal dimension (FD). Results: This analysis included 35 patients (35 eyes): 28 type-2 diabetics and 7 type-1 diabetics. Mean HbA1c was 13.1 ± 2.0% at inclusion and 7.0 ± 1.5% at 3 months. In the short period from inclusion to 3 months post-inclusion, patients showed significant decrease in VD−DCP (28.8% vs. 27.8%; p = 0.014), a significant increase in FAZA (0.300 mm2 vs. 0.310 mm2; p < 0.001), and a significant increase in AI (1.31 vs. 1.34; p < 0.01). Multivariate analysis found an increase in FAZA was correlated with baseline HbA1c level and age (R2 = 0.330), and a decrease in VD-DCP was correlated with HbA1c decrease and diabetes duration (R2 = 0.286). Conclusions: Rapid glycemic control in patients with uncontrolled diabetes led to possible short-term microvascular damage that correlated to both initial and decreased HbA1c.

Fundus Changes in the Offspring of Mothers With Confirmed Zika Virus Infection During Pregnancy in French Guiana, Guadeloupe, and Martinique, French West Indies.

Importance: Most ocular lesions have been described for children with congenital Zika syndrome. The frequency of finding ocular abnormalities is unknown among children exposed to Zika virus (ZIKV) during pregnancy. This study was conducted on newborns whose mothers were positive for ZIKV, confirmed with reverse-transcription polymerase chain reaction (RT-PCR) testing. Objective: To report ocular fundus manifestations in newborns with congenital ZIKV exposure in French Guiana, Martinique, and Guadeloupe, French West Indies, to assess its prevalence. Risk factors, such as the presence of extraocular fetopathies and the gestational term at infection, were sought. Design, Setting, and Participants: This was a cross-sectional multicentric study, conducted from August 1, 2016, to April 30, 2019, for which data were collected prospectively. The study inception was at the beginning of 2016 from the onset of the ZIKV epidemic in the French West Indies. Newborns whose mothers tested positive (by RT-PCR) for ZIKV during pregnancy were included. Interventions: Fundus examination was performed using widefield retinal imaging after pupil dilation. Infection date, delivery mode, and newborn measurements were collected. Main Outcomes and Measures: Anomalies of the vitreous, choroid, retina, and optic disc. Results: A total of 330 children (mean [SD] age, 68 [IQR, 22-440] days; 170 girls [51.5%]) were included. Eleven children (3.3%) had perivascular retinal hemorrhages, and 3 (0.9%) had lesions compatible with congenital ZIKV infection: 1 child had torpedo maculopathy, 1 child had a chorioretinal scar with iris and lens coloboma, and 1 child had a chorioretinal scar. Retinal hemorrhages were found at childbirth during early screening. Lesions compatible with congenital ZIKV infection were not associated with the presence of extraocular fetopathy. Microcephaly was not associated with lesions compatible with congenital ZIKV infection (odds ratio [OR], 9.1; 95% CI, 0.8-105.3; P = .08), but severe microcephaly was associated with an OR of 81 (95% CI, 5.1-1297.8; P = .002). Conclusions and Relevance: Results of this cross-sectional study suggest that the ocular anomalies found may be associated with ZIKV in 0.9% of the exposed population. Ocular lesions were rare, affected mostly the choroid and retina, and seemed to be associated with choroiditis-related scarring that developed during fetal growth.

SUCCESSFUL CLOSURE OF A LARGE FULL-THICKNESS MACULAR HOLE SECONDARY TO AN IDIOPATHIC RETINAL VASOPROLIFERATIVE TUMOR USING THE INVERTED INTERNAL LIMITING MEMBRANE FLAP TECHNIQUE.

PURPOSE: To report a case of large full thickness macular hole (MH) as an uncommon complication of an idiopathic retinal vasoproliferative tumor (RPVT) and the successful closure of the full-thickness MH by the inverted internal limiting membrane Flap technique. METHODS: A case report. A 40-year-old woman was referred for a progressive and profound visual loss on the right eye. The fundus examination showed a full-thickness MH and the presence of an idiopathic RPVT surrounding by numerous exudates in the inferior temporal of the peripheral retina. In addition, the spectral domain optical coherence tomography imaging confirmed the large size of the full-thickness MH (740 µ m-diameter). First, we treated the RPVT using the transconjunctival cryotherapy. Then, we performed the macular surgery including 25 Gauge vitrectomy, inverted internal limiting membrane flap technique, and gas tamponade. RESULTS: In the postoperative period, the RPVT remained inactive with no recurrence of the MH or excessive gliosis proliferation on the internal limiting membrane flap area. Moreover, visual acuity rose up from 20/100 (0.2) at 1-month follow-up to 20/40 (0.5) at 6-month follow-up. CONCLUSION: In our patient, the full-thickness MH was precisely large and secondary to an RPVT. The treatment of the tumor by transconjunctival cryotherapy had been efficient and the postoperative follow-up after the macular procedure involving the inverted internal limiting membrane flap technique showed the anatomical and functional recovery of the macula.

CRB1-Related Retinal Dystrophies in a Cohort of 50 Patients: A Reappraisal in the Light of Specific Müller Cell and Photoreceptor CRB1 Isoforms.

Pathogenic variants in CRB1 lead to diverse recessive retinal disorders from severe Leber congenital amaurosis to isolated macular dystrophy. Until recently, no clear phenotype-genotype correlation and no appropriate mouse models existed. Herein, we reappraise the phenotype-genotype correlation of 50 patients with regards to the recently identified CRB1 isoforms: a canonical long isoform A localized in Müller cells (12 exons) and a short isoform B predominant in photoreceptors (7 exons). Twenty-eight patients with early onset retinal dystrophy (EORD) consistently had a severe Müller impairment, with variable impact on the photoreceptors, regardless of isoform B expression. Among them, two patients expressing wild type isoform B carried one variant in exon 12, which specifically damaged intracellular protein interactions in Müller cells. Thirteen retinitis pigmentosa patients had mainly missense variants in laminin G-like domains and expressed at least 50% of isoform A. Eight patients with the c.498_506del variant had macular dystrophy. In one family homozygous for the c.1562C>T variant, the brother had EORD and the sister macular dystrophy. In contrast with the mouse model, these data highlight the key role of Müller cells in the severity of CRB1-related dystrophies in humans, which should be taken into consideration for future clinical trials.

Impact of the Craniofacial Surgery Simulation in Anterior Plagiocephaly on Orbits and Oculomotor Muscles: Biomechanical Analysis With a Finite Element Model.

PURPOSE: The aim of this study was to show the displacements and strain induced by the supraorbital band advancement during a craniofacial surgery for an anterior plagiocephaly on the orbital bones and the orbital content thanks to a numerical surgical simulation using the finite element method. METHODS: A three-dimensional (3D) finite element model of a child with an anterior plagiocephaly was entirely created from a tomodensitometry of a patient followed by our Craniofacial Pediatric team. Data of the tomodensitometry were computed with Slicer 3D to re-create the orbit geometry. Mesh production, properties of the model, and simulations of the fronto-orbital advancement were conducted on Hyperworks software (Altair Engineering, Inc., Detroit, MI, USA). RESULTS: The resulting 3D Finite Element Model was used to perform the supraorbital advancement simulation. Displacement and strain patterns were studied for orbital bones, oculomotor muscles, and eyeballs. Relative high strain in the both trochlear area and excycloration of the right orbit are among the most interesting results as torsional strabismus as V-pattern strabismus are often described in children with an anterior plagiocephaly. CONCLUSIONS: This pediatric Finite-Element Model of both orbits of a child with an anterior plagiocephaly showed the impact of the fronto-orbital advancement on the oculomotor system. This model described the relationship between the craniofacial surgery and the strabismus in the unilateral coronal synostosis. The advantages of this model are the many opportunities for improvement, including postoperative period and additional surgical procedures.

Sickle Cell Maculopathy: Microstructural Analysis Using OCTA and Identification of Genetic, Systemic, and Biological Risk Factors.

PURPOSE: To identify genetic, systemic, and biological factors associated with the occurrence of sickle cell maculopathy (SCM). To evaluate microvascular macular alterations using optical coherence tomography angiography (OCTA) in sickle cell disease (SCD). DESIGN: Cross-sectional study. METHODS: One hundred fifty-one eyes of 78 adult SCD patients (43 HbSS, 30 HbSC, 4 S/ß+, and 1 HbS Lepore) and 40 eyes of 20 healthy controls underwent spectral-domain optical coherence tomography (SDOCT) and OCTA using Spectralis HRA+OCT (Heidelberg Engineering, Heidelberg, Germany). We analyzed the occurrence of SCM, the foveal avascular zone (FAZ) area, and the severity of macular ischemia and studied their relationships with genetic, systemic, and biological parameters using multivariate logistic regression analysis. RESULTS: Maculopathy occurred in 66 eyes (44%), and more frequently in HbSS patients (71%, P = .004). Multivariate analysis identified HbSS genotype and lower prothrombin ratio (PR) as independently associated with SCM (P = .01). Proliferative sickle cell retinopathy was also associated with SCM (P = .02). FAZ enlargement was associated with higher lactate dehydrogenase level (P = .02). Macular ischemia was more severe in patients with lower hemoglobin level (P = .004) and lower PR (P = .01). No flow areas were identified with OCTA even in eyes with no macular thinning (36 eyes, 42%) and appeared more frequently in the temporal superior subfield (36%). CONCLUSIONS: HbSS genotype, abnormal coagulation and hemolysis increase the risk of SCM. OCTA provides valuable criteria to identify potential risk factors of SCM. OCTA also improves detection of early microvascular changes before the onset of macular thinning.

Association of Anterior Uveitis With Acute Zika Virus Infection in Adults.

Importance: Acute anterior uveitis has been reported to be associated with Zika virus (ZIKV) infection in case reports and retrospective studies based on systemic clinical signs of ZIKV and positive serologic tests. Objective: To describe the ophthalmic findings associated with systemic ZIKV infection during the 2016 ZIKV outbreak in Guadeloupe in the French West Indies. Design, Setting, and Participants: This cohort study assessed adult patients with red eye and virologic or serologic confirmation of acute ZIKV infection who were admitted to the Pointe-à-Pitre University Hospital from January 1, 2016, to January 1, 2017, for a systematic ophthalmologic examination. All patients with anterior uveitis also underwent a complete uveitis workup to rule out other causes of uveitis. Patients were excluded from the study if they had active uveitis and a history of uveitis or positive serology for other infectious diseases including dengue fever virus and chikungunya virus. Data were analyzed from January 1, 2016, to January 1, 2017. Exposures: Patients with confirmed ZIKV infection underwent full ophthalmic examination at study inception and 3, 6, and 12 months. Main Outcomes and Measures: Description of the presenting ocular characteristics, management, and outcome. Results: Of 62 total patients with a red eye, 32 (51.6%) had no uveitis (mean [SD] age, 27.8 [3.7] years; 26 women [81.3%]) and 30 (48.4%) had anterior uveitis (mean [SD] age, 27.5 [3.8] years; 26 women [86.7%]), which was bilateral in all cases. Acute anterior uveitis was nongranulomatous and without synechiae, exhibiting mild anterior chamber reaction with small keratic precipitates. Ocular hypertension occurred in 25 patients (83.3%) with uveitis at presentation and persisted in 15 patients (50%) at 1 year despite antiglaucoma therapy and control of uveitis. Uveitis responded to topical steroids in all cases. Intraocular pressure was within normal limits for all patients without uveitis throughout the follow-up period. Conclusions and Relevance: Anterior uveitis was present in almost half of patients with a red eye and acute systemic ZIKV infection; the uveitis was usually bilateral and often associated with increased intraocular pressure. These results suggest that ophthalmic evaluation should be performed in patients with red eye and acute ZIKV infection to potentially detect and manage hypertensive anterior uveitis.

Multifocal electroretinogram findings in sickle cell maculopathy.

BACKGROUND: The aim of the present work was to describe and compare multifocal electroretinogram findings (mfERG) between patients with sickle cell disease (SCD) without clinical sign of maculopathy and controls (HbAA). METHODS: Both HbSS (homozygous SCD) and HbSC (compound heterozygous SCD) patients, the two most frequent SCD genotypes, were included. All individuals underwent a full ophthalmologic examination (with a fundoscopy), a spectral domain ocular coherence tomography (SD-OCT) and a mfERG. RESULTS: A total of 86 subjects were included: 54 SCD patients (107 eyes) with 32 HbSS (63 eyes) and 22 HbSC (44 eyes) and 32 controls (64 eyes). None of the eyes showed retinal clinical abnormalities. SD-OCT analysis showed that macular thickness was statistically lower in SCD eyes than in controls. mfERG analysis demonstrated a significant reduction of N1 (initial-negative deflection), and P1 (positive peak) response amplitude densities of HbSS eyes compared to HbAA eyes from the centre (<2°) and to the periphery (>15°). Implicit time response was also reduced in the centre (<2°). N1 and P1 response amplitude densities of HbSC eyes were significantly lower than those of HbAA eyes from the centre (<2°) to the periphery (>15°). N1 implicit time was statistically reduced in HbSS compared to HbSC eyes. CONCLUSION: Our study is the first one to describe macular electrophysiological dysfunction in SCD patients. Moreover, we confirm that SCD maculopathy is equally frequent in HbSS and HbSC.

Proliferative retinopathy and maculopathy are two independent conditions in sickle cell disease: Is there a role of blood rheology?1.

OBJECTIVE: Our study investigated the prevalence of retinopathy and maculopathy in sickle cell patients and tested the association between these two conditions. In addition, we tested whether hematological and hemorheological parameters, as well as genotype, were involved in the development of these two conditions. METHODS: Seventy sickle cell adult patients were recruited: 37 with sickle cell anemia (SCA) and 33 with sickle cell hemoglobin C disease (SCC). All patients underwent retinal examination and macular ocular coherence tomography. Blood was sampled for the measurements of hematological and hemorheological parameters. RESULTS: Twenty-six patients had maculopathy and 30 had retinopathy with no significant difference between SCA and SCC patients. No association between the presence of retinopathy and maculopathy was detected. RBC aggregation was higher and RBC deformability lower at 3 Pa in SCA patients. Blood viscosity and hematocrit were higher in SCC than in SCA patients. However, no association was found between biological parameters and the ocular complications studied. CONCLUSIONS: Our study showed that retinopathy and maculopathy are common in sickle cell disease. Nevertheless, we found no association with hematological parameters, blood rheology or genotype.