Inferior Vascular Arcade Research Articles

Characterization and topographical analysis of choroidal caverns in young and healthy individuals.

To describe and clarify a possible pathogenetic origin for choroidal caverns in young healthy individuals through a topographical analysis using swept-source optical coherence tomography angiography (SS-OCTA). A cross-sectional evaluation of 44 healthy volunteers (44 eyes), aged 20-32 years with no systemic or ocular comorbidities. The topographical analysis of choroidal caverns was performed through a 15 × 15 mm volumetric scan cube using SS-OCTA (PLEX Elite 9000). The number, topographic localization within the perifovea and midperipheral retina, as well as the density of choroidal caverns in the area with the highest number, were analyzed. Factors influencing the presence of choroidal caverns were identified using a logistic regression model. The overall prevalence of choroidal caverns was 18.2% in our cohort. The subjects exhibiting choroidal caverns presented a greater AXL (+1.19 mm, 95%CI: 0.09, 2.29). Areas with the highest density of caverns presented an average of 1.98 ± 1.01 caverns/mm2. On a qualitative evaluation, the area with the highest density was along the inferior vascular arcade in 6/8 (75%) eyes. The main predictor of choroidal cavern development was represented by AXL (adjusted risk ratio of 2.13, P = 0.02). Choroidal caverns in young, healthy individuals may form due to mechanical stress in predisposed eyes, where an increased lateral and anteroposterior choroidal stretching due to globe elongation can result in the formation of these cavities. Future longitudinal studies are encouraged to understand their prognostic implications.

Hypodense regions in the peripapillary region increased the risk of macular retinoschisis detected by optical coherence tomography.

The purpose of the present study was to investigate the clinical features of peripapillary regions in patients with myopic macular retinoschisis (MRS) and its association with the development of retinoschisis (RS). In this cross-sectional study, high-myopic patients with or without MRS were recruited, and the hypodense regions were analyzed in the peripapillary regions. The vitreoretinal adhesions around both macular and paravascular arcades were compared between groups. The risk factors for the development of MRS were analyzed by logistic regression. Of 88 myopic eyes, MRS was detected in 45 eyes (51%). The eyes with MRS showed a higher rate of peripapillary and paravascular retinoschisis (P < 0.001 and P = 0.006). Hypodense regions were detected in 25 eyes (20.35%). Higher rates of horizontal and vertical macular MRS were detected in the hypodense group (P = 0.012 and P = 0.002). Lower refractive error, longer axial length, and higher rates of outer retinoschisis both in horizontal and vertical macular regions were observed in the hypodense group (P = 0.012, P = 0.006, P = 0.038, and P = 0.034). Higher rates of inner and outer retinoschisis, vitreoschisis, and microfolds along superior vascular arcade were detected in the hypodense group (P = 0.005, P = 0.001, P = 0.014, and P = 0.014). Higher rates of internal limiting membrane (ILM) detachment, inner and outer RS were detected along the inferior vascular arcade in the hypodense group (P = 0.008, P = 0.001, and P = 0.028). Hypodense regions, the axial length and PICC (peripapillary intrachoroidal cavitation) were significantly correlated with the severity of MRS (Odds ratio = 0.207, P = 0.010; Odds ratio = 1.399, P = 0.016; Odds ratio = 0.142, P = 0.010). The hypodense regions were likely to affect outer retinoschisis both in macular and paravascular regions. It was a risk factor for the development of MRS.

EYS-Associated Sector Retinitis Pigmentosa.

Sector retinitis pigmentosa (RP) is a rare form of rod-cone degeneration typically associated with mutations in the RHO gene. We describe six unrelated patients presenting with this atypical phenotype in association with biallelic mutations in EYS gene. Multinational, multicentre cross-sectional case series. Patients with biallelic disease-causing variants in EYS and a clinical diagnosis of sector RP were recruited from specialized centres in Portugal and Brazil. All patients underwent a comprehensive ophthalmologic examination complemented by deep phenotyping. Peripheral blood samples were collected from all probands and available relatives for genetic analysis. Genetic counselling was provided to all subjects. Seven disease-causing variants (4 pathogenic; 3 likely pathogenic) were identified in 6 unrelated female patients. Best-corrected visual acuity ranged from 75 to 85 ETDRS letters. All eyes showed bilateral and symmetrical areas of outer retinal atrophy distributed along the inferior vascular arcades and extending temporally and/or nasally in a crescent-shaped pattern. On fundus autofluorescence (AF), a foveal-sparing curvilinear band of hyperAF encroaching the optic nerve head and extending temporally was seen in 4 patients. The remaining 2 presented bilateral and symmetrical patches of hypoAF inside crescent-shaped areas of hyperAF along the inferior temporal vascular arcade. Visual field testing revealed superior visual field defects of varying extents, always in close association with the fundus AF findings. Even though EYS has only recently been listed as a cause of the sector RP phenotype, we believe that this presentation is not infrequent and should be considered an important differential for sector RP.

Observation of the characteristics of the natural course of Bietti crystalline dystrophy by fundus fluorescein angiography

BackgroundBietti crystalline dystrophy (BCD) is an autosomal recessive genetic disorder that causes progressive vision loss. Here, 12 patients were followed up for 1–5 years with fundus fluorescein angiography (FFA) to observe BCD disease progression.MethodsFFA images were collected for 12 patients with BCD who visited our clinic twice or more over a 5-year period. Peripheral venous blood was collected to identify the pathogenic gene related to the clinical phenotype.ResultsWe observed two types in FFA images of patients with BCD. Type 1 showed retinal pigment epithelium (RPE) atrophy in the macular area, followed by choriocapillaris atrophy and the subsequent appearance of RPE atrophy appeared at the peripheral retina. Type 2 showed RPE atrophy at the posterior pole and peripheral retina, followed by choriocapillaris atrophy around the macula and along the superior and inferior vascular arcades and the nasal side of the optic disc. The posterior and peripheral lesions of both type 1 and type 2 BCD subsequently extended to the mid-periphery; finally, all the RPEs and choriocapillaris atrophied, exposing the choroid great vessels, but type 2 macular RPE atrophy could last longer.ConclusionsThe characterization of two different types of BCD development provides a better understanding of the phenotype and the progression of the disease for a precise prognosis and prediction of pathogenesis.

A novel homozygous frameshift variant in the cellular retinaldehyde-binding protein 1 (RLBP1) gene causes retinitis punctata albescens.

Retinitis punctata albescens is a form of retinitis pigmentosa characterized by white fleck-like deposits in the fundus, in most cases caused by pathogenic variants in RLBP1 gene. The purpose of this work is to report the phenotypic and genotypic data of a patient with retinitis punctata albescens carrying a deletion in the RLBP1 gene. An 8-year-old Caucasian female has been complaining of nyctalopia for the last 2 years. No other ocular symptoms were present. No relevant past medical or familiar history was described. At clinical examination, the patient's best-corrected visual acuity was 20/20 in both eyes. Anterior segment evaluation and intraocular pressure were normal in both eyes. At fundoscopy, multiple punctate whitish-yellow fleck-like lesions were observed in the proximity of temporal superior and inferior vascular arcades. Scotopic electroretinogram demonstrated severely reduced rod response, without improvement or recovery of rod system function after prolonged dark adaptation. Blood DNA samples of this patient and from her parents were screened for causal variants in RLBP1, RDH5, and PRPH2. A probable pathogenic frameshift variant was identified in homozygosity in the RLBP1 gene with an autosomal recessive transmission as another cause of retinitis punctata albescens. This DNA variant will aid ongoing functional studies and add to our understanding of the molecular pathology about RLBP1-associated retinopathies.

Picture quality comparison between panoptic ophthalmoscope and non-mydriatic fundus camera

ABSTRACT&#x0D; Background: Non-mydriatic fundus photography has been shown to be a potential alternative to direct ophthalmoscopy in the emergency department. Panoptic ophthalmoscope is a newer handheld alternative to non-mydriatic fundus camera. Its advantages include greater portability and lower price. Panoptic Ophthalmoscope compatibly utilizes mobile phone’s camera to capture high definition fundus photographs, however lower in quality in comparison with a fundus camera. This study compares the picture quality between panoptic ophthalmoscope (POO) and non-mydriatic fundus camera (NMFC).&#x0D; Patients and Methods: This comparative cross-sectional study was conducted in Lahore General Hospital, Lahore. Three twenty three (323) fundus pictures were taken from two different cameras. One hundred sixty pictures (160) were taken with NMFC and one hundred sixty three (163) with POO. Quality of each picture was assessed by two consultant ophthalmologists independently. The data was computed and analyzed by SPSS version 21. Chi square test was applied to the data. Confidence interval of 0.95 with α of 0.05 was taken.&#x0D; Results: There was statistically significant difference in picture quality of disc, macula, superior and inferior vascular arcades (p&lt;0.001). Total 122 out of 160 pictures taken by NMFC and 48 out of 163 pictures taken by POO were considered ideal by first ophthalmologist whereas 96 of 160 pictures taken by NMFC and none taken by POO were considered ideal by second ophthalmologist.&#x0D; Conclusion: Quality of images taken with NMFC is significantly better as compared to POO.

Multimodal imaging ocular sarcoidosis

The study objective was to present multimodal imaging of definite ocular sarcoidosis (OS) in a young adult. A 52-year old woman consulted for 5 days of photophobia, ocular pain on movement, and redness in her left eye. Slit-lamp examination showed bilateral inferior granulomatous keratic precipitates, high cellullarity in the anterior chamber, posterior synechiae and hypopigmented subretinal lesions in the inferior vascular arcade, and nasal retina. In addition to the complete ophthalmic examination fundus photography (FP), fluorescein angiography (FA) and optical coherence tomography-angiography (OCT-A) were performed.Multimodal imaging of both eyes of the patient was performed. FP showed periphlebitis, hypopigmented subretinal lesions in the inferior temporal vascular arcade, and nasal retina. FA showed late diffuse capillary leakage from the subretinal lesions and optic nerve. OCT-A showed decreased choroidal capillary density, small hypo-intense gray areas of retinal capillary hypoperfusion, and presence of confluent and isolated areas void of flow in the deep plexus and choriochapillaris. With these findings on the multimodal imaging, chest computed tomography scan, and negative laboratory workup for other diseases, a presumptive diagnosis of OS was made which was subsequently confirmed with a positive skin nodule biopsy. The combination of OCT-A and other diagnostic methods allows a more detailed evaluation of structural and functional clinical findings in OS. There are few studies on OCTA findings in OS. Further studies with larger samples are needed in order to establish specific OCTA findings that could be used as diagnostic criteria.

'Papaya Seed' Appearance of Subependymal Nodules on Magnetic Resonance Imaging in a Patient with Tuberous Sclerosis.

A 14-month-old male was referred to the Department of Child Health at the Sultan Qaboos University Hospital (SQUH), Muscat, Oman, in 2014 with a two-week history of frequent abnormal movements of the body. A video, recorded by the patient’s father, revealed that the patient suffered from typical flexor body spasms and left side partial motor seizures. On physical examination, the patient had several cutaneous features of tuberous sclerosis, including depigmented skin lesions and Shagreen patches. A neurological examination was unremarkable. Sleep electroencephalography displayed interictal generalised and focal discharges from the left frontocentral region. There was no evidence of hypsarrhythmia. Magnetic resonance imaging (MRI) of the brain revealed multifocal cortical and subcortical tubers with subependymal nodules (SEN) which had a ‘papaya seed’ appearance [Figure 1A]. Four to five rhabdomyomas in both ventricles were visible on echocardiography. Ophthalmological examination revealed an astrocytoma along the inferior vascular arcade close to the optic nerve of the left eye. The patient’s father had a possible skin lesion on his back, while the rest of the family had normal examinations. The child was diagnosed with tuberous sclerosis and epilepsy. He was initially prescribed the antiepileptic medication vigabatrin; due to a lack of response, this was changed to topiramate and levetiracetam after two weeks.

Quantified Incision Placement for Postseptal Approach Transconjunctival Blepharoplasty.

This study quantifies the incision location in transconjunctival lower eyelid blepharoplasty to optimize postspetal (direct) access to the eyelid/orbital fat. A retrospective chart review of patients undergoing transconjunctival blepharoplasty by one surgeon (GGM) from January 2013 to January 2014 was performed. Simultaneous globe retropulsion and lower eyelid inferior displacement was used to balloon the conjunctiva forward to maximally visualize the transconjunctival surface anatomical landmarks of importance. A caliper was used to measure the distance in millimeters from the inferior tarsus to the most superior projection of visible fat. The conjunctival incision was made 0.5 mm posterior to this measured distance. For each procedure it was noted whether the preseptal or postseptal plane was entered. Sixty-six patients were assessed. Fifty patients were women, and the mean patient age was 54 years (range 36-71 years). The mean distance from the inferior tarsus to the visualized superior tip of fat was 6.03 mm (range 5-7 mm) and the mean incision placement was 6.53 mm (range 5.5-7.5 mm). The postseptal space (direct access to fat) was entered in 54 cases (82%). The inferior vascular arcade was identified in 23 cases (35%) cases. In this instance, the incision was placed below this landmark in 16 cases (70%). There were 5 cases (7.6%) of postoperative chemosis which all resolved within 2 months with conservative measures. There were no other complications related to the conjunctival incision. Placing the conjunctival incision for postseptal approach transconjunctival blepharoplasty 0.5 mm posterior to the most superior projection of clinically visible fat (with adjunctive globe retropulsion and lower eyelid infraplacement) accesses the postspetal space directly in 82% of cases. Previously suggested incision placements: between 2 and 5 mm below the tarsus, at the fornix, or at the inferior vascular arcade are subjective/anecdotal at best and without similar quantitative validation.

Quality of Nonmydriatic Digital Fundus Photography Obtained by Nurse Practitioners in the Emergency Department: The FOTO-ED Study

Nonmydriatic fundus photography by non-ophthalmic-trained personnel has recently been shown to be a potential alternative to direct ophthalmoscopy in the emergency department (ED). We evaluated the reliability of a novel quality rating scale and applied this scale to nonmydriatic fundus photographs taken during routine ED patient encounters to determine factors associated with diminished photograph quality. Prospective, cross-sectional study. We included 350 patients enrolled in the Fundus photography versus Ophthalmoscopy Trials Outcomes in the Emergency Department study who were photographed by nurse practitioners after <30 minutes of training followed by supervision. Photographs of both eyes were graded for quality on 2 occasions by 2 neuro-ophthalmologists. Four regions were independently evaluated for quality: Optic disc, macula, and superior and inferior vascular arcades. Quality as a function of the number of photographs taken was evaluated by Kaplan-Meier analysis. Mixed effects ordinal logistic regression was used to evaluate for predictors of image quality while accounting for the repeated measures design. Overall photographic quality (1-5 scale; 5 best). We evaluated 1734 photographs. Inter- and intraobserver agreements between neuro-ophthalmologists were very good (weighted kappa, 0.84-0.87). Quality of the optic disc area was better than those of other retinal areas (P<0.002). Kaplan-Meier analysis showed that if a high-quality photograph of an eye was not obtained by the third attempt, it was unlikely that one would be obtained at all. A 10-second increase in the interphotograph interval before a total of 40 seconds increased the odds of a 1-unit higher quality rating by 1.81 times (95% confidence interval [CI], 1.68-1.98), and a 10-year increase in age decreased the odds by 0.76 times (95% CI, 0.69-0.85). Black patients had 0.42 times (95% CI, 0.28-0.63) the odds of a 1-unit higher quality rating compared with whites. Our 5-point scale is a reliable measure of nonmydriatic photograph quality. The region of interest, interphotograph interval, and patient age and race are significant predictors of image quality for nonmydriatic photographs taken by nurse practitioners in the ED. Addressing these factors may have a direct impact on the successful implementation of nonmydriatic fundus photography into the ED.

Identification of breaks in choroidal coloboma eyes with retinal detachment using subretinal trypan blue injection

Abstract Purpose To describe the use of subretinal trypan blue to localize retinal breaks during vitrectomy for rhegmatogenous retinal detachment in choroidal coloboma eyes. Methods Trypan blue 0.03% was injected subretinally through a retinotomy superonasal to optic disc in 108 choroidal coloboma eyes in which no breaks could be identified pre‐ or intra‐operatively outside the coloboma margins. Eyes were rotated to evenly spread the dye subretinally and plume of trypan blue into the vitreous cavity was used to locate the breaks. Communication between breaks in the intercalary membrane (ICM) and coloboma margin was also studied. Results Eighty six eyes had breaks within the coloboma margins only whereas 7 eyes had breaks within as well as outside the coloboma. In 7 eyes breaks were seen in peripheral retina alone and no breaks could be found in 8 eyes even intra‐operatively. In all eyes with break inside the coloboma there was a single communication at its margin between the detached retina outside the coloboma and the break in ICM through which trypan blue entered vitreous cavity. Mostly it was in the infero‐nasal or temporal quadrant outside the inferior vascular arcade. Conclusion Majority of retinal detachments that involve the coloboma were caused by breaks within the ICM. Moreover there is a small defect at the coloboma margin through which the sub‐ICM fluid seeps under the normal retina from the break. Based on intraoperative findings in this study, prophylactic laser to margins of coloboma sparing the vascular arcades may seal off this potential communication and prevent retinal detachment in such eyes.

Rapid, bloody, and blinding diabetic retinopathy

Rapid, bloody, and blinding diabetic retinopathy

Photoreceptor attenuation in acute zonal occult outer retinopathy.

The purpose of this study is to report a case of acute zonal occult outer retinopathy and to describe a novel method of imaging photoreceptor damage using Cirrus spectral-domain optical coherence tomography (SD-OCT; Cirrus, Carl Zeiss Meditec, Dublin, CA) en face visualization. Our examination also included color fundus photographs, Goldmann visual field, fluorescein and indocyanine green angiography, fundus autofluo-rescence, and Ganzfeld electroretinography. This is a case report of a single patient. Despite 20/20 visual acuity in both of our patient's eyes, Goldmann visual field showed a unilateral C-shaped scotoma centered on the left optic nerve, sparing the fovea. Fundus and angiographic findings showed nonspecific pigmentary changes and hyperfluo-rescent foci along the superior and inferior vascular arcades. The electroretinography showed decreased scotopic amplitudes, with impaired 30-Hz flicker response, and Cirrus SD-OCT showed loss of the inner segment/outer segment photoreceptor junction. Cirrus SD-OCT Advanced Visualization three-dimensional reconstruction with en face visualization delineated an area of inner segment/outer segment attenuation corresponding to the Goldmann visual field scotoma. The patient's condition was unchanged at 5 months follow-up. In addition to classic diagnostic modalities, photoreceptor attenuation observed with Cirrus SD-OCT can contribute to the diagnosis of acute zonal occult outer retinopathy and may help elucidate the etiology of this puzzling condition.

Breakthrough vitreous hemorrhage after ICGA guided PDT for PCV

Dear Editor, Polypoidal choroidal vasculopathy (PCV) is a type of choroidal neovascularization, associated with recurrent serosanguineous detachments of the retinal pigment epithelium and the neurosensory retina, secondary to leakage and bleeding from the choroidal vascular lesions.[1] Indocyanine green angiogram (ICGA)-guided photodynamic therapy (PDT) with verteporfin in the treatment of associated choroidal neovascular membrane (CNVM) has shown encouraging results, with the postulation being that ICGA-guided targeting reduces collateral damage to the choriocapillaris, thus reducing the up-regulation of vascular endothelial growth factor.[2] We report a case of breakthrough vitreous hemorrhage after ICGA-guided PDT for PCV. A 69-year-old Indian lady presented with sudden diminution of vision in the left eye since one week. Visual acuity was 20/20, N6 in the right eye and 20/30, N6 in the left eye. Anterior segment examination was essentially normal except for early cataractous changes in both eyes. Right fundus was normal. Left fundus revealed sub-macular hemorrhage. Fundus fluorescein angiography (FFA) [Fig. 1A] and ICGA suggested PCV with multiple subretinal polypoidal lesions and a peripapillary CNVM in the left eye. The patient underwent ICGA-guided PDT in that eye with two non-confluent spots of 3 mm each [Fig. 1B]. Three weeks later, vision dropped to bare perception of light. Fundus examination revealed vitreous hemorrhage with no retinal view. Ultrasound showed multiple vitreous echoes with attached retina.Figure 1A: Fundus fluorescein angiography (FFA) reveals multiple, hyperfluorescent, pinpoint subretinal lesions around the optic disc suggestive of polypoidal choroidal vasculopathy in the left eye. Blocked fluorescence is secondary to subretinal hemorrhageFigure 1B: Indocyanine Green Angiography (ICGA) reveals numerous other polypoidal lesions with a peripapillary choroidal neovascular membrane (CNVM) inferior to the optic disc typical of polypoidal choroidal vasculopathy. Circles indicate photodynamic therapy (PDT) treatment spotsThe patient underwent pars plana vitrectomy for ‘breakthrough vitreous hemorrhage’ and the suspected site of breakthrough vitreous bleed was lasered. Fourteen per cent perfluropropane served as endotamponade. Six weeks later, fundus evaluation revealed altered sub-retinal hemorrhage inferior to the macula [Fig. 2A]. FFA showed peripapillary staining and macular window defects with no leakage [Fig. 2B]. One month later, fundus remained stable. Best corrected visual acuity improved to 20/40, N6.Figure 2A: Color fundus montage of left eye at 13 weeks follow-up revealed altered subretinal hemorrhage, regressed peripapillary choroidal neovascular membrane (CNVM) and absorbing subretinal hemorrhage along the inferior vascular arcades. The yellow circle in the infero-nasal quadrant indicates the area of endolaser photocoagulationFigure 2B: Fundus fluorescein angiography (FFA) at 13 weeks followup reveals staining of peripapillary choroidal neovascular membrane (CNVM) with no leakage. Multiple peripapillary and macular window defects are also seenRecent case series analyzing the hemorrhagic complications of PDT for PCV have reported a 6.59% incidence of breakthrough vitreous hemorrhage subsequent to sub-retinal hemorrhage following treatment.[34] In our institute, 41 eyes of 26 patients underwent PDT for PCV between June 2001 and August 2007. However, breakthrough vitreous hemorrhage was noted only in a single eye. Hence, the incidence of vitreous hemorrhage after PDT for PCV in our practice amounts to 2%. This is much lower than that reported in the Japanese population (6.59%).[3] The earliest development of vitreous hemorrhage was noted at 29 days post-PDT. Significant visual decline was noted in 50% of cases, including that of massive suprachoroidal hemorrhage and profound visual loss.[34] Our case had the earliest reported presentation (21 days) following PDT. Although the patient had diabetes mellitus there was no evidence of proliferative retinopathy which could account for the vitreous hemorrhage. The PDT spot size in the reported case was 3.0 mm [Fig. 1B]. The mean PDT spot size in the other 40 eyes was 3.55 mm. Hence, we did not find a positive correlation between the PDT spot size and the tendency for breakthrough vitreous hemorrhage following PDT. To the best of our knowledge, this is the first case report of breakthrough vitreous hemorrhage, after PDT for PCV, from the Indian subcontinent. The patient achieved satisfactory clinical and visual stability after surgery, at four months.

Mapping posterior vitreous detachment by optical coherence tomography in eyes with idiopathic macular hole

PurposeTo determine the relationship between posterior vitreous detachment and idiopathic macular hole. DesignObservational case series. MethodsIn a prospective study, the posterior hyaloid face was scanned from the posterior pole to the far periphery by optical coherence tomography in 25 eyes (22 patients) with an idiopathic macular hole (stage 1 = 1, stage 2 = 7, stage 3 = 10, and stage 4 = 7), and a map of the posterior vitreous detachment was constructed. ResultsOne eye with a stage 1 macular hole had a posterior vitreous detachment confined to the vascular arcade, but attached to the fovea. In all seven eyes at stage 2, the detached posterior hyaloid enlarged upward beyond the superior vascular arcade, but stopped at the margin of inferior vascular arcade. In two cases, the posterior vitreous detachment also extended temporally and superonasally. In all cases, the vitreous face remained attached to the fovea. Six of the 10 eyes at stage 3 had larger partial posterior vitreous detachment that extended not only upward, but also beyond the inferior vascular arcade, while in the other four eyes, the size and position of the posterior vitreous detachment was similar to stage 2 macular holes. However, unlike stage 2, the posterior vitreous detachment included the fovea in all eyes. All seven eyes with a stage 4 macular hole had complete posterior vitreous detachment that extended to the far periphery in all directions. ConclusionsThere is a close correlation between the stage of the macular hole and the degree of posterior vitreous detachment. This close correlation suggests that progression of idiopathic macular hole is related to enlargement of the posterior vitreous detachment.

Incomplete congenital stationary night blindness associated with symmetrical retinal atrophy

PURPOSE: To describe a Japanese patient with incomplete congenital stationary night blindness (iCSNB) with atypical retinal atrophy and kinetic visual field defects. METHODS: An ophthalmologic examination was performed, and the CACNA1F gene was analyzed by direct genomic sequencing. RESULTS: The patient had a hemizygous Arg913stop mutation in CACNA1F and had electroretinographic changes that were typical of iCSNB. The fundus had atrophic retinal lesions around the inferior vascular arcades OU, and Goldmann kinetic perimetry showed relative scotomas in the corresponding areas. CONCLUSIONS: Although most patients with iCSNB show essentially normal fundi without visual field defects, this case demonstrated retinal atrophy associated with visual field defects indicating a phenotypic heterogeneity induced by the CACNA1F mutation.

Ocular Perforation During Retrobulbar and Peribulbar Injections

To determine the ocular perforations during retrobulbar and peribulbar injections. Twenty-five ocular perforations between 1976 and 1993 occurred after 13 retrobulbar and 12 peribulbar injections. Eighteen patients (72%) were women. Eighteen eyes were myopic (72%). Risk factors included high myopia in 11 cases (44%), use of Atkinson gaze in 21 cases (84%) and a sharp injection needle. Deep position of the posterior pole was common. Perforation signs comprised vitreous hemorrhage in 25 eyes (100%), subretinal hemorrhage in 19 eyes (76%), retinal breaks along the inferior vascular arcade in 19 eyes (76%), and retinal detachment in 14 eyes (56%). Proliferative vitreoretinopathy developed in 11 eyes (44%). Retinal detachment strongly correlated to poor visual outcome.