A new strategy to calculate the intraocular lens power in congenital cataracts according to age and axial length at implantation.

PURPOSE: The purpose of the study was to suggest a new method to calculate the intraocular lens (IOL) power in paediatric cataracts targeting emmetropia at the age of 15 years. METHODS: Data of children younger than 15 years who underwent cataract surgery with IOL implantation between 2005 and 2020 in the ophthalmological department of Marseille (South of France) was collected retrospectively. A logarithmic regression model was used to predict the axial length growth of the included eyes between implantation and 15 years. The predicted myopic shift served as target refraction to calculate a theoretical IOL power aiming for emmetropia at 15 years. Refractive error with the theoretical lens power was estimated as the spherical equivalent at the last follow-up minus the difference of target refractions between the implanted IOL and the theoretical one. Refractive errors using Dahan, Enyedi and Trivedi guidelines were also estimated and compared to the logarithmic model. RESULTS: Thirty-five eyes of 26 children were analysed. At the last follow-up, the median age of children was 10 years old and the median spherical equivalent was -1.88 dioptres (D) (IQR -3.81, -0.75). The estimated median refractive errors were 0.18 D (IQR -1.11, 1.42) with the logarithmic formula, -1.47 D (IQR -3.84, -0.65) with Dahan formula, -0.63 D (IQR -2.15, 0.32) with Enyedi formula and 0.38 D (IQR -1.58, 1.07) with Trivedi formula. CONCLUSION: The estimated refractive error with the new logarithmic formula is the closest to emmetropia at the last follow-up.

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.

Bilateral acute retinal necrosis with encephalitis in a premature infant.

INTRODUCTION: Neonatal herpes infections can be extremely severe and their early recognition allows for appropriate management and increases the child's chances of survival. CASE DESCRIPTION: We report the case of a premature infant born by vaginal delivery and transferred to intensive care after neonatal misadaptation. Examination of the fundus revealed lesions of acute bilateral retinal necrosis, strongly suggesting a herpetic etiology. CONCLUSIONS: This case highlights the clinical benefit of an ophthalmologic exam in newborns with equivocal brain lesions. Herein, the fundus examination allowed for high suspicion of herpes virus infection.

Threatening Blindness in a Child With Typical Hemolytic Uremic Syndrome.

BACKGROUND: Hemolytic uremic syndrome (HUS) is the most common cause of acute kidney failure in children younger than five years. Central nervous system involvement occurs in 15% of patients, with clinical manifestations including confusion, coma, seizures, stroke, and cortical blindness. Ocular involvement in children with HUS is rare, but retinal and choroidal hemorrhages as well as ischemic retinopathy due to thrombotic microangiopathic lesions have been documented. PATIENT DESCRIPTION: We describe a 26-month-old girl with typical HUS who experienced severe visual loss likely resulting from cytotoxic injury of both lateral geniculate nuclei with bilateral damage to optic pathways coupled with macular thrombotic microangiopathic lesions. Her vision recovered completely within a month in conjunction with the normalization of her imaging studies. CONCLUSIONS: Although this child's vision was severely impaired, the prognosis for this mixed visual impairment of peripheral and central origin was excellent with a full recovery.

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.

Safety and efficacy of high-intensity focused ultrasound in severe or refractory glaucoma.

PURPOSE: The aim of this study was to evaluate the efficacy and safety of ultrasonic cyclocoagulation in severe or refractory glaucoma, and to analyze the procedure-related inflammation. PATIENTS AND METHODS: In this retrospective study, 15 eyes of 13 patients suffering from severe or refractory glaucoma with uncontrolled intraocular pressure of ⩾21 mmHg underwent 8-s ultrasonic cyclocoagulation. A complete ophthalmic evaluation, quality of life assessment using Glau-Qol-17, endothelial cell count, and flare measurement were performed. Primary outcome was qualified surgical success (defined as intraocular pressure reduction from baseline ⩾20% and intraocular pressure >5 mmHg without hypotensive medication adjunction). Secondary outcomes were flare, endothelial cell loss, and quality of life. RESULTS: Qualified success was achieved in 67% of eyes at 6 months (mean intraocular pressure reduction = 42% in these eyes). During the first month after the procedure, the mean flare reached its maximum value when the intraocular pressure was minimal; the flare slowly decreased until normalization at month 3 when the maximal intraocular pressure was noted. At month 3, there was moderate but significant endothelial cell loss (11%), and no significant alteration in quality of life was demonstrated. CONCLUSION: The efficacy of ultrasonic cyclocoagulation in the present study is comparable to that reported in the literature. The kinetics of intraocular pressure and flare suggest that postoperative inflammation could be partly responsible for the early intraocular pressure decrease. The moderate endothelial cell loss, which could be caused by localized heating, and the preservation of quality of life confirm the safety of ultrasonic cyclocoagulation.

Covid-19-Associated Retinopathy: A Case Report.

A 53-year-old man presented with acute loss of vision, negative scotoma and dyschromatopsia in his left eye. He reported contact with people with severe respiratory syndrome - coronavirus-2 (SARS-CoV-2) 8 days prior symptoms. Funduscopic examination revealed several retinal hemorrhages. Spectral-domain optical coherence tomography showed lesions consistent with acute macular neuroretinopathy and paracentral acute middle maculopathy. Quickly after his presentation, SARSCov-2 was confirmed by chest computed tomography-scan and RT-PCR in this patient. Thrombotic complications associated with Covid-19 infection have high incidence and may involve the retina. We described a case of retinal involvement associated with Covid-19 infection. PRÉCIS: Funduscopic examination revealed retinal hemorrhages in a man with loss of vision. Optical coherence tomography showed an acute macular neuroretinopathy and paracentral acute middle maculopathy. Coronavirus disease was confirmed by chest computed tomography-scan and RT-PCR.

Interocular suppressive interactions in amblyopia depend on spatial frequency.

In amblyopia, there is an interocular suppressive imbalance that results in the fixing eye dominating perception. In this study, we aimed to determine whether these suppressive interactions were narrowband and tuned for spatial frequency or broadband and independent of spatial frequency. We measured the contrast sensitivity and masking functions of fifteen amblyopic subjects and seventeen control subjects using the quick Contrast Sensitivity Function (qCSF) approach (Lesmes, Lu, Baek, & Albright, 2010). We first measured the monocular sensitivity functions of each participant and thereafter corrected for it. We then measured masking sensitivity functions for low, mid and high spatial frequency masks, normalized to their visibility. In the control group, we observed that the strength of dichoptic masking is equivalent between the two eyes. It is also tuned such that masking by low spatial frequencies in one eye mainly affects low spatial frequencies in the other eye and masking by high spatial frequencies mainly affects high spatial frequencies. In amblyopes, although the interocular masking is also tuned for spatial frequency, it is not equivalent between the two eyes: the masking effect from the amblyopic to fixing eye is weaker than the other way around. The asymmetry observed in the strength of masking between the two eyes in amblyopia is tuned for spatial frequency. It is not the consequence of the contrast sensitivity deficit of the amblyopic eye nor is it the consequence of abnormally strong masking from the fixing eye. Rather it is due to an abnormally weak masking strength by the amblyopic eye per se.

Morphologic Analysis of Peripapillary Retinal Arteriole Using Adaptive Optics in Primary Open-angle Glaucoma.

PURPOSE: The purpose of this study was to better understand the role of vascular risk factors in the pathogenesis of primary open-angle glaucoma (POAG), a detailed analysis of retinal arterial wall thickness is needed. The purpose of the present study was to make a morphologic analysis of peripapillary arteriole in POAG using adaptive optics (AO) technology. PATIENTS AND METHODS: We included otherwise healthy subjects with an isolated confirmed diagnosis of bilateral POAG. Patients' clinical characteristics were noted. AO imaging followed by a complete ophthalmic examination was performed. A single operator masked to clinical data performed 5 measurements at different locations of each analyzed vessel. For each location, lumen diameter and wall thickness were measured. Total diameter, wall-to-lumen ratio (WLR), and whole cross-sectional area were calculated. RESULTS: Lumen diameter and total diameter were significantly lower in the glaucoma group (n=31) than in the control group (n=29): [median (interquartile ranges)] 88.3 (82.6-99.2) versus 102.3 (87-113.1) (P=0.03) and 121.1 (109.3-130.5) versus 134.4 (112.7-144.4), respectively (P=0.015). Wall thickness, WLR, and whole cross-sectional area were not significantly different. Apart from a significantly higher WLR in subjects with reported high cholesterol levels, we did not observe any correlation between patients' clinical characteristics and any of the parameters. CONCLUSIONS: We observed in POAG a narrowing of the arteriolar lumen without modification of the vessel wall thickness. To date, it is the first time that these data are obtained using AO. This suggests that the vascular risk factor in POAG only reduces the vascular caliber without inducing any patent atherosclerosis of the retinal arterial wall.

Multimodal imaging of torpedo maculopathy including adaptive optics.

PURPOSE: The etiology of torpedo maculopathy remains unknown, but it has been recently suggested that it could represent a persistent defect in the development of the retinal pigment epithelium. As retinal pigment epithelium and photoreceptors form a functional unit, an alteration of photoreceptor distribution or function is predictable. The aim of this study is to describe multimodal imaging, including adaptive optics, in three cases of torpedo maculopathy, and discuss its pathogenesis. METHODS: Multimodal imaging is presented, including fundus photographs, optical coherence tomography, adaptive optics, autofluorescence, fluorescein angiography, and ultra-widefield retinal imaging in three cases of torpedo maculopathy. RESULTS: An oval-shaped well-delimited chorioretinal lesion both hypopigmented centrally and with a hyperpigmented border in the temporal macula, consistent with torpedo maculopathy, was observed in three patients. Optical coherence tomography showed a preservation of the inner retina, a mild atrophy of the outer retina, an alteration of the ellipsoid zone and of the retinal pigment epithelium layer, and a neurosensory detachment. These lesions were hypoautofluorescent with a hyperautofluorescent border. Fluorescein angiography showed a hyperfluorescence by window effect. Adaptive optics imaging showed an alteration of the cone mosaic within the lesions, with a lower cone density and a higher spacing between cones. CONCLUSION: The alteration of the cone mosaic suggested by adaptive optics in torpedo maculopathy has never been described and could be explained by the alteration of the retinal pigment epithelium. Our results support the existing hypothesis on the pathogenesis of torpedo maculopathy that a persistent defect in the development of the retinal pigment epithelium may be responsible for this clinical entity.

Persistent Placoid Maculopathy: Prognosis Factors and Functional Outcomes.

Purpose: To identify prognosis factors and functional outcomes of persistent placoid maculopathy (PPM). Methods: We collected personal PPM cases and combined them with the data from the literature. Results: 68 eyes of 37 patients with PPM were analyzed, including six new cases. Twenty-six patients were men (70%) with a mean age of 51.8 years old. The mean initial visual acuity (VA) was 0.52 LogMar ± 0.55 for a mean final VA of 0.49 LogMar ± 0.51. Risk factors for poor VA included: initial VA less than 0.2 LogMar (p < .0001), cardiovascular risk factor (p = .008), autoimmune-related and/or systemic pro-inflammatory conditions (p = .003), choroidal neovascularization (p = .001), macular atrophy (p = .03) and absence of systemic corticosteroid treatment (p = .03). Conclusion: PPM is a choroidal inflammation. Identifying prognosis factors may help to guide treatment and follow-up. We showed that anti-inflammatory drugs, and anti-VEGF injections in cases of choroidal neovascularization, may lead to better outcomes.

Secondary Congenital Glaucoma Associated With Retro-orbital Infantile Hemangioma: A Masquerade Syndrome.

PURPOSE: The main purpose of this study was to describe a unique case of retro-orbital infantile hemangioma (IH) simulating a congenital glaucoma requiring prompt management with systemic propranolol. STUDY DESIGN: This was a case report. RESULTS: A 2-month-old white female was referred to our department of ophthalmology for progressive, unilateral protrusion of the left eye associated with epiphora. Clinical examination was suggestive of congenital glaucoma, as evidenced by high intraocular pressure, optic disc cup, increased corneal diameter, and axial length. The left eye proptosis was measured at 4 mm with the Hertel exophthalmometer. Cycloplegic refraction discloses high degree of astigmatism in the left eye. A cerebral and orbital magnetic resonance imaging revealed an intraconal vascular mass evocative of orbital IH.Oral propranolol was initiated and resulted in a rapid radiologic reduction of the IH. Concomitantly, the notable finding was the reversal of congenital glaucoma parameters without the need of surgery. CONCLUSIONS: To the best of our knowledge, this is the first observation of a secondary congenital glaucoma reversal with oral propranolol used for orbital IH management.

Decompression retinopathy following nonpenetrating deep sclerectomy for primary congenital glaucoma.

BACKGROUND: To describe a unique case of decompression retinopathy manifesting as pre-macular subhyaloid hemorrhage that occurs in a nine-day old child after undergoing a non-penetrating deep sclerectomy for primary congenital glaucoma. CASE PRESENTATION: We report a single case of a 9-day-old boy who was referred to our department of ophthalmology for bilateral buphtalmia and corneal edema. He presented marked elevation of the intraocular pressure in both eyes (22 mmHg and 26 mmHg, in the right eye and left eye respectively) associated with significant optic nerve cupping. Non-penetrating deep sclerectomy was performed for each eye, with effective reduction of the intraocular pressure during the first week postoperatively (11 mmHg and 7 mmHg in the right eye and left eye respectively). The right eye presented an isolated subhyaloid hemorrhage located in the pre-macular area, persisting 3 weeks after the initial surgery and requiring pars-plana vitrectomy to clear the visual axis. This uncommon complication was identified as decompression retinopathy. The intraocular pressure remained controlled in the normal range three years after initial surgery in both eyes, with reversal of optic disc cupping. CONCLUSIONS: Decompression retinopathy is a potential complication after non-penetrating deep sclerectomy in primary congenital glaucoma, requiring prompt treatment strategy to prevent potential organic amblyopia.

Diffuse subretinal fibrosis syndrome: A rare entity.

BACKGROUND/ PURPOSE:: Diffuse subretinal fibrosis and uveitis syndrome is an inflammatory disease rarely reported. The purpose of this article is to describe a rare case of diffuse subretinal fibrosis syndrome in an 8-year-old child. METHODS AND RESULTS:: The patient initially presented with a loss of vision in his right eye, with a visual acuity of 20/200. The visual acuity of the left eye was normal. Clinical examination showed bilateral anterior and posterior inflammation while the fundus revealed on the right eye an inter maculopapillar fibrosed lesion with a discrete retinal hemorrhage and a similar but smaller lesion on the left eye. The optical coherence tomography showed hyper-reflective material compatible with pre-retinal and subretinal pigment epithelial fibrosis and associated subretinal fluid. The angiography facilitated the diagnosis of neovascularization that was associated with the fibrotic lesion. The rest of the clinical pediatric examination remained negative and a diagnosis of diffuse subretinal fibrosis syndrome complicated with neovascularization was made. Treatment with systemic corticosteroids at the dose of 1 mg/kg/day for 1 month with a progressive decrease of 5 mg per month allowed for both anatomical and functional improvement. The visual acuity of the right eye improved from 20/200 to 20/63 at 1 month, 20/32 at 2 months, and 20/20 at 4 months. CONCLUSION:: To our knowledge, this is the first reported case of bilateral diffuse subretinal fibrosis complicated by neovascularization. General corticosteroid therapy proved to be effective in this case and appears to be a viable option in first-line treatment.