Search

Purpose: To report a case of lacrimal drainage pathway disease-associated keratopathy (LDAK) with endophthalmitis. Observations: An 80-year-old man with diabetic retinopathy and nephropathy was referred to our hospital with endophthalmitis of the left eye. Slit-lamp examination revealed slight eye discharge, peripheral corneal ulcers, diffuse hyperemia of the conjunctiva, iris synechia, and a large amount of fibrin in the anterior chamber of the left eye. No puncta of the left eye were observed. The patient had undergone trabeculectomy for primary open-angle glaucoma 5 years previously. B-mode echo examination confirmed vitreous opacity in the left eye. We suspected endophthalmitis and performed a par-plana vitrectomy, bacterial culture, and polymerase chain reaction examination for eye discharge, aqueous humor, and vitreous humor. However, no bacteria or viruses were detected. The eye discharge and corneal peripheral ulcers did not improve following surgery. A lacrimal syringe test was performed two weeks after surgery, and bacterial concretion and discharge were observed. We detected Actinomyces in the bacterial concretions and performed dacryocystorhinostomy. After surgery, the corneal ulcer improved, and eye discharge disappeared. Conclusions and importance: LDAK causes corneal perforation and endophthalmitis. In cases of intraocular inflammation with corneal ulcers, a lacrimal syringing test should be performed, even in the absence of lacrimal findings on slit-lamp examination.

Abstract Lacrimal drainage pathway disease-associated keratopathy (LDAK) has been associated with corneal perforation, which arises from both infectious and non-infectious corneal disorders. However, patients with corneal perforation are often not routinely tested for LDAK, and the potential risk posed by LDAK in the development of corneal ulcers has not been investigated in detail. This study aimed to assess the proportion and characteristics of LDAK in patients with non-infectious corneal perforation using lacrimal syringing test. This study enrolled 56 patients with corneal perforation treated at Saitama Medical University Hospital between January 2016 and September 2022. The causes of corneal perforation were trauma (n = 17, 30%), infection (n = 19, 34%), non-infection (n = 16, 29%), and unknown (n = 4, 7%). A lacrimal syringing test was performed on 12 patients with non-infectious corneal perforation and 4 with an unknown diagnosis. Among the 16 patients with non-infectious corneal perforation, 13 (81%) had lacrimal drainage disease, but only 3 (19%) patients had lacrimal puncta, as revealed by slit-lamp examinations. The primary bacterial species identified in lacrimal obstructive disease and lacrimal canaliculitis were Staphylococcus spp. and Actinomycetes spp. respectively. Lower temporal and peripheral corneal perforations were common. All patients underwent lacrimal surgery, and 6 (38%) were treated for corneal perforation without corneal surgery. Interestingly, several patients with LDAK who did not exhibit any lacrimal duct obstruction on slit-lamp examination. The study findings demonstrate the significance of the lacrimal syringing test for assessing LDAK in patients with corneal perforation, indicating LDAK as a potential cause of corneal perforation.

Introduction: Dellen is a corneal disease characterized by shallow, saucer-like excavations at the corneal margin. Herein, we presented a case of dellen formation that developed following a 27-gauge pars plana vitrectomy (PPV) and was rapidly resolved using scleral and conjunctival sutures. Case Presentation: A 73-year-old woman underwent a 27-gauge PPV for the epiretinal membrane of the right eye. Intraoperatively, owing to coexisting diabetic retinopathy, the peripheral vitreous was shaved, and retinal photocoagulation was performed on the peripheral retina. The intraocular pressure (IOP) was 15 mm Hg on 1 day after the PPV and on postoperative day 4; however, on day 14, the IOP decreased to 10 mm Hg, and conjunctival bleb formation was observed. By day 18, the bleb height remained unchanged, and dellen formation was noted at the corneal periphery. Because of marked corneal thinning, conjunctival and scleral sutures were placed 20 days following the PPV. Intraoperative findings revealed leakage from the scleral wound at the trocar puncture site. The IOP increased to 20 mm Hg at 19 h following the suture, resulting in the disappearance of the bleb and dellen. Since then, the bleb or dellen did not recur. Conclusion: We encountered a case of late-onset scleral wound cleavage 2 weeks following microincision vitreous surgery, resulting in bleb and dellen formation, which was immediately managed by the scleral and conjunctiva suturing.

Conclusions: Our findings showed the course of recovery of the visual acuity and visual field in an eye with TON. The quantification of GVF was helpful in assessing the course of recovery after the treatments. The new quantitative index of the GVFs may be helpful in evaluating the effectiveness of treatments for optic nerve disorders.

IntroductionIt is reported that eyes with a branch retinal artery occlusion (BRAO) had normal full-field electroretinography (ERG) but the response of the multifocal electroretinography (mfERG) was reduced in the area of the arterial occlusion. Optical coherence tomography angiography (OCTA) is a recently appeared modality that can evaluate microvascularizations in different retinal layers and in different regions of the retina. The purpose of this study was to determine the density of the microcirculation and the function of the macular area of eyes with BRAO, and to determine whether they are significantly correlated.MethodsThe OCTA and mfERG findings of 7 eyes of 6 patients (3 men, 3 women) were studied. The mean age of the patients was 71.7±10.6 years. The OCTA examinations were made with volume scans of 3 × 3 and 6 × 6 mm squares centered on the fovea. The macular vessel densities (mVD) in the superficial retinal layer (SRL) and deep retinal layer (DRL) were measured for the superior and inferior halves of 3 × 3 and 6 × 6 mm diameter concentric circles. The mfERGs were recorded with targets set to stimulate the focal areas of the retina corresponding to the areas examined by OCTA.ResultsThe OCTA examinations showed that the mVD of the 3 mm concentric circle in the SRL was significantly lower on the affected side than on the unaffected side (P = 0.022). No such difference was observed in the DRL. The N1 amplitude of the 20.2° concentric circle and the N1–P1 amplitude of the 10.1° concentric circle of the mfERGs were significantly smaller on the affected side than on the unaffected side (P = 0.047 and 0.031). A significant positive correlation was found between the mVD of the 6 mm concentric circle in the DRL and the P1–N2 amplitude of the 20.2° concentric circle (ρ = −0.929 and p = 0.003).DiscussionThese findings indicate that OCTA images may be able to show changes in the density of the retinal macular microcirculation, and the mfERGs may be able to show alterations in the function of the macular area of the eyes with BRAO. A layer-by-layer analysis of the local retinal microcirculation and function should help in determining the pathogenesis of BRAO.

Purpose We investigated the effects of a warm compress immediately before surgery on the ocular surface and intraoperative visibility during surgery.Methods A randomised controlled quasi-experiment at Saitama Medical University Hospital. From November 2020 to September 2021, 200 patients scheduled for endophthalmic surgery were randomly assigned to a group that received a hot compress with a spontaneously heating eye mask (HM group) or a group that received only an eye mask (control group). The eye masks were applied for 20 min from 2 hours before surgery, and before and after mask application in the non-invasive tear break-up time (NIBUT), tear meniscus height (TMH) and obstruction score of the meibomian gland (meiboscore) were evaluated. The time from wetting to dry blurring of the corneal surface (corneal blurring time, CBT) was also compared before and after the warm compress.Results We enrolled 100 patients in the HM group (mean age 69.0±13.3 years) and 99 patients in the control group (mean age 69.5±16.2 years). In the control group, there were no significant changes in the NIBUT, meiboscore or TMH before and after eye mask use, whereas in the HM group, the NIBUT increased from 6.7±5.1 to 9.5±5.6 s (p

Abstract The center of the fovea, termed the foveola, is the area of highest visual acuity, has the highest density of cone photoreceptors. We investigated the distance between the automatically-determined center of the foveal avascular zone (FAZ) and the manually-determined highest foveal bulge (FB) point using single swept-source optical coherence tomography angiography (OCTA) instrument. This cross-sectional study included 49 eyes of 49 individuals (34 women and 15 men; median age: 68 years) with no history of ocular disorders. The FAZ in the superficial capillary plexus was automatically determined using the Kanno–Saitama macro method, and the center of the FAZ was automatically determined using ellipse approximation. Another candidate foveal center, the highest FB point, was determined manually on the serial cross-sectional B-scan images. As a result, the foveal center was manually identified as the highest FB point on B-scan OCTA images. The center of the FAZ was more likely to be located inferior to the highest FB point (p = 0.031). In participants with a total (linear) distance of more than 50 μm between the center of the FAZ and the highest FB point, the displacement was significantly more in the horizontal direction than in the vertical direction (p = 0.017). These results can be applicable to further studies regarding the spatial relationships between the center of the FAZ and the highest FB point in various macular diseases or previously-treated eyes.

We describe a case of brolucizumab-related intraocular inflammation (IOI) detected using vitreous haze on optical coherence tomography (OCT) at an early stage before the patient was aware of any symptom. A 69-year-old female presented with decreased right vision. The patient was diagnosed with pachychoroidal neovasculopathy and started intravitreal aflibercept (IVA) with a 3+ treat-and-extend strategy (TAE). Although the serous retinal detachment (SRD) disappeared after IVA treatment, the patient was managed with treatment every 4 weeks without extending the treatment interval To shorten the treatment interval, intravitreal brolucizumab (IVBr) was started 44 weeks after starting IVA treatment. After initiating IVBr treatment, the SRD completely disappeared. However, 16 weeks after starting IVBr, OCT showed noise in the vitreous cavity, which had not been seen before, and infrared images showed a black smoke-like shadow over the macula. Despite these findings, the patient had no subjective symptoms, and so IVBr was re-administered with an 8-week TAE interval. Five days after IVBr treatment, vitreous inflammatory cells were observed, and the noise in the vitreous cavity and the smoke-like shadow in the infrared image were further enhanced. We diagnosed the patient with brolucizumab-related IOI, and anti-inflammatory treatment was initiated. After extensive treatment, the vitreous opacity gradually disappeared, and the vitreous noise on OCT and the black smoke-like shadow on infrared images disappeared. IOI may have already been present 16 weeks after starting IVBr treatment, when we judged that there was no inflammation and IVBr was re-administered. When following patients receiving IVBr, IOI may be detected by OCT at an earlier stage by evaluating vitreous haze.

We describe the electroretinographic findings of a case of primary intraocular lymphoma (PIOL) wherein the patient received intravitreal injections of methotrexate (ivMTX). A 62-year-old man developed blurred vision and complained of decreased visual acuity (VA) in his right eye. Fundus examination showed vitreous opacity and multiple subretinal yellowish lesions. Optical coherence tomography (OCT) revealed subretinal and intraretinal infiltrations. The full-field electroretinogram (ffERG) showed subnormal combined rod-cone response and multifocal electroretinogram (mfERG) recorded using skin electrodes showed severe attenuation of the response compared with the other eye. Pars prana vitrectomy, phacoemulsification, and lens implantation were performed to remove the opacity, and vitreous biopsy revealed a high ratio of interleukin 10–6 (76.0). There was no systemic malignant lesion, and the patient was diagnosed with PIOL. Treatment with ivMTX (400 μg/0.1 mL) was started. One month later, the intraretinal infiltration had disappeared, and mfERG revealed recovery of the response density from the central area. Two months later, OCT showed recovery of the foveal ellipsoid and interdigitation zones, and VA recovered to 20/17; mfERG showed maintenance of macular function. However, the amplitude of a- and b-waves in the ffERG gradually decreased. Macular function recovered, but there was also a decrease in total retinal function. mfERG and ffERG recorded using skin electrodes were useful in monitoring macular and entire retinal function with repeated examinations and showed recovery and maintenance of macular function in a case of PIOL treated with ivMTX.

Introduction: To evaluate the fundus autofluorescence (FAF) images 1 year after half-dose photodynamic therapy (hdPDT) for chronic central serous chorioretinopathy (CSC). Methods: Forty-six eyes of 46 consecutive patients with chronic CSC underwent hdPDT. Short wavelength-elicited FAF images and enhanced depth imaging optical coherence tomographic (EDI-OCT) images were recorded before and at 1, 3, 6, 9, and 12 months after the hdPDT. The FAF images at 1 month were compared to those at 12 months after the hdPDT. Results: The serous retinal detachment (SRD) was resolved in all eyes. The best-corrected visual acuity (BCVA) improved significantly from 0.13 ± 0.28 logarithm of minimum angle of resolution (logMAR) units before to 0.01 ± 0.17 logMAR units at 12 months after the hdPDT (p = 0.001; paired t test). The mean choroidal thickness decreased significantly from 365.4 ± 103.0 µm to 284.3 ± 92.5 µm at 12 months (p < 0.001). Abnormal FAF images were present within the irradiated area in all the eyes before the hdPDT. In 5 of 46 eyes, identifiable changes of the FAF images were observed 12 months after hdPDT. None of the eyes had the confluent hypo-FAF type during the follow-up period. Univariate analyses showed that the choroidal thickness before hdPDT correlated significantly with hypo-FAF enlargement (p = 0.005). However, multivariate analyses showed that the association was not significant (p = 0.06). Conclusions: The progression of the RPE damages occurred in approximately one-tenth of the eyes that underwent hdPDT for chronic CSC. The long-term effect of progression of hypo-FAF on visual functions remains to be determined.

Purpose: The aim of this study was to evaluate the foveal avascular zone (FAZ) of healthy subjects and examine the magnification effect. Methods: A total of 33 healthy volunteers were enrolled and all subjects were eligible for analysis. Optical coherence tomography angiography (OCTA) examination scanned 3 × 3 mm of the macular area. The FAZ area was measured on the superficial OCTA en face image with and without correction by axial length. The relationship between changes in the FAZ area after correction with the axial length was examined. Results: The mean age was 21.9 ± 0.6 years. The mean axial length was 24.87 ± 1.17 mm and mean spherical equivalent (SE) value was –3.64 ± 2.83 diopters (D). The FAZ area was 0.26 ± 0.10 mm2 before the axial length correction and 0.27 ± 0.10 mm2 after the correction. In the eyes that had an axial length longer than or equal to 26 mm or SE less than or equal to –6 D, the FAZ area after correction was significantly larger than that before correction (p < 0.01). The change of FAZ area after correction with axial length was significantly correlated with the axial length (R2 = 0.88, p < 0.01) or SE value (R2 = 0.55, p < 0.01). Conclusion: FAZ areas were comparable to previous reports. In high myopic cases, the magnification effect needs to be considered when evaluating the FAZ area.

Abstract Background Macular hole (MH) is a retinal break in the fovea involving partial or complete dehiscence of the neural retinal layers affecting the visual quality by decreasing visual acuity (VA) and visual deformation. We describe a case of secondary MH associated with submacular hemorrhage (SMH) due to polypoidal choroidal vasculopathy (PCV), which showed spontaneous closure. Case presentation A 67-year-old man developed decreased VA in his right eye due to an SMH. The VA was 20/50, and monthly intravitreal injection of aflibercept was administered three times. The SMH gradually decreased, and 10 months later the external limiting membrane was found to be perforated, resulting in MH. The old clot disappeared, and the MH remained for 10 months. Twenty-three months later, serous retinal detachment (SRD) involving the macula appeared and the MH had disappeared. SRD gradually disappeared, and macular configuration recovered. VA gradually improved and became 20/20 38 months later. Conclusion Dynamic change of the ultrastructure in an unusual case of secondary-developed and spontaneously closed MH was clearly observed. Although the mechanism was unknown, the small diameter size and exudative PCV are thought to have contributed to the closure.

The present report aimed to describe the macular structure’s recovery process in a case of optic disc pit maculopathy (ODP-M) with outer layer hole following pars plana vitrectomy (PPV) with juxtapapillary laser treatment (JPL). We performed repeated optical coherence tomography (OCT) examinations to evaluate the macular structural changes. An 80-year-old man presented with distorted vision and decreased visual acuity (VA) in his left eye, experienced for 1 year, prior to presentation. Fundus examination and OCT showed intraretinal fluid (IRF) in the inner and outer retinal layers. Serous retinal detachment (SRD) with an outer layer hole in the macula was also evident. The IRF was connected to the optic disc; however, the SRD was isolated. Best-corrected VA was 20/100. PPV combined with JPL was performed. Posterior vitreous detachment creation and tamponade with sulfur hexafluoride was performed. Postoperatively, the inner retinal IRF at the fovea disappeared. The outer layer hole gradually closed and had completely disappeared 1 month postoperatively. After resolution of the outer layer hole, SRD reduced gradually and disappeared 8 months postoperatively, although the macular outer retinal IRF remained. The outer retinal IRF had partially resolved by the 18th postoperative month. Macular structure was completely recovered 31 months postoperatively, with an improved VA of 20/20. In conclusion, SRD might be associated with outer retinal IRF and outer layer holes. In cases of ODP-M, outer layer holes might induce optic disc-isolated SRD.

Abstract Background To evaluate the retinal function before and soon after an intravitreal injection of an anti-vascular endothelial growth factor (anti-VEGF) agents. Methods Seventy-nine eyes of 79 patients that were treated by an intravitreal injection of an anti-VEGF agent for age-related macular degeneration (AMD), diabetic macular edema (DME), or retinal vein occlusion (RVO) with macular edema (ME) were studied. The RETeval® system was used to record 28 Hz flicker electroretinograms (ERGs) from the injected and non-injected eyes before (Phase 1, P1), within 2 h after the injection (P2), and 2 to 24 h after the injection (P3). Patients were grouped by disease or by the injected agent and compared. The significance of the changes in the implicit times and amplitudes was determined by t tests. Results The amplitudes were not significantly different at the three phases. The implicit time of the injected eye was 31.2 ± 3.2 msec at P1, and it was not significantly different at P2 (31.7 ± 3.1 msec) but it was significantly longer at P3 (32.2 ± 3.3 msec, P

[This corrects the article DOI: 10.1371/journal.pone.0216823.].

Abstract Background Patients with Leber hereditary optic neuropathy (LHON) have a progressive decrease of their visual acuity which can deteriorate to

Purpose: To describe a Japanese girl with unilateral optic neuritis who was seropositive for the anti-myelin-oligodendrocyte glycoprotein (MOG). Serial recordings of the pattern visual evoked potentials (pVEPs) were made to follow the dynamic changes of the disease activity.Observations: A 5-year-old girl developed a sudden reduction of vision and deep ocular pain in her right eye. On examination at our university hospital, the best-corrected visual acuity (BCVA) was light perception, and a swelling of the optic disc and tortuous vessels at the posterior pole of the right eye were observed. MRI demonstrated that her right optic nerve was hyperintense on short TI inversion recovery (STIR) sequence. A diagnosis of right papillitis was made, and she was treated with steroid pulse therapy followed by a gradual tapering of oral prednisolone. The visual acuity decreased to no light perception and plasmapheresis combined with high-dose intravenous immunoglobulin therapy was performed. The decimal visual acuity rapidly improved and recovered to 1.2, and no recurrence was observed for at least 1 year. On day 19, she was found to be anti-MOG antibody positive and anti-Aquaporin 4 antibody negative. pVEPs were recorded during the course of the disease process which showed the dynamic changes of the physiology of the visual pathways. The implicit times of the N75 and P100 components were prolonged in the right eye in the acute phase. The right visual acuity remained at 1.2 for at least 1 year, but the implicit times of the N75 and P100 components of the pVEPs of the right eye were still prolonged compared to left eye.Conclusion: Our findings indicate a positive relationship between the anti-MOG antibodies-positivity and the prolonged pVEPs. Further analyses of the pVEPs and other clinical findings of the optic neuritis are needed to establish the clinical significance of the anti-MOG antibodies positivity and optic neuritis for the diagnosis, treatment, and prognosis for this disease.

Purpose: To report a case with lacrimal fistula presumably associated with Sjögren's syndrome. Observations: A patient with Sjögren's syndrome was referred to our hospital for fistula from the inferior lacrimal canaliculus to the palpebral conjunctiva of her left lower eyelid. She also revealed severe dry eye in both eyes. She had no history of trauma or congenital lacrimal fistula. She was administering 0.3% purified sodium hyaluronate and 3% diquafosol sodium solution 6 times a day for dry eye. The Schirmer-1 test indicated that tear secretion was 0 mm for 5 min for both eyes. She was diagnosed with Sjögren's syndrome based on increased levels of blood Sjögren's-syndrome-related antigen A and Sjögren's-syndrome-related antigen B antibodies, decreased saliva volume, and lip biopsy. We performed silicone tube intubation and patched the fistula with conjunctiva. We observed the lacrimal sac and nasolacrimal duct under lacrimal micro-endoscopy; there was no bacterial concretion, obstruction, and inflammation of lacrimal mucosa. After the operation, her symptoms improved and lacrimal perforation healed after the removal of the silicone tube. Conclusions and importance: Sjögren's syndrome can cause not only corneal perforation but also mucosal perforation, which may lead to a lacrimal fistula. Sjögren's syndrome patients with severe dry eye should be managed carefully. Keywords: Lacrimal fistula, Sjögren's syndrome, dry eye

Purpose: We present our findings in a case with an intraocular foreign body in which the electroretinographic (ERG) findings were useful. Observations: A 37-year-old man was injured by an iron fragment that penetrated into his left eye through the cornea. His visual acuity was counting fingers, and a traumatic cataract prevented an examination of the fundus. B-mode ultrasonography showed a stick-like foreign body of approximately 14 mm in length in the eye. Preoperative ERGs with a contact lens electrode showed reduced responses with many blinking artifacts. Lensectomy and pars plana vitrectomy were performed and a fragment of a wire brush was seen embedded in the superior nasal retina which was removed. The decimal visual acuity improved to 1.2 two weeks later. The postoperative ERG performed with a skin electrode showed reduced responses in the injured eye. Conclusions and importance: We recommend that the physiology of the retina be assessed by recording ERGs with a skin-type electrode as soon as possible after a traumatic injury to the eye. Keywords: Electroretinography, Skin electrode, Intraocular foreign body, Pars plana vitrectomy

PurposeTo assess the physiology of the retina by electroretinography (ERG) with skin electrodes in eyes that had undergone vitrectomy with silicone oil (SO) tamponade.DesignRetrospective case series.MethodERGs were recorded from eleven eyes with complex vitreoretinal disorders and from the normal fellow eyes. The affected eyes underwent pars plana vitrectomy (PPV) with SO tamponade. ERGs were recorded before and after the SO was removed. The amplitudes and implicit times of the a- and b-waves of the affected eyes were compared to those of the normal fellow eyes. In addition, the ratios of the amplitudes of the b-waves of the affected eyes to those of the fellow eyes were compared before and after the SO was removed.ResultsERGs were recordable from 7 eyes (63.6%) before the SO was removed and 11 eyes (100%) after the SO was removed. The a- and b-wave amplitudes were significantly smaller in the affected eyes than those of the fellow eyes at the baseline. The b-wave amplitude before the removal of the SO was significantly and positively correlated with that after the SO removal. The ratios of the b-waves of the affected/normal fellow eye significantly increased after the SO was removed.ConclusionThe results indicate that ERGs picked up by skin electrode can be used to assess the physiology of the retina in eyes with a SO tamponade. The amplitude of the b-waves of the ERGs in silicone-filled eyes can be used to predict the amplitude after the silicone is removed.

Purpose. To determine the feasibility of performing intraocular surgeries in a heads-up position with low illuminance conditions by observing a display of the surgical field created by a three-dimensional imaging (3D) system. Methods. Seventy-four eyes of 56 patients underwent cataract surgery (72 eyes) with the heads-up 3D surgery system; 60 eyes with cataract surgery alone, 7 eyes with combined cataract and glaucoma microdevice implant surgery, 5 eyes with combined cataract and vitrectomy surgery, and two eyes with vitrectomy surgery alone were studied. The illuminance from the surgical microscope was set to be dimmer (Leica M822F40 main light 2%; otto-flex 6%) than the usual setting to minimize the discomfort and glare for the patient. The surgeries were performed under topical anesthesia. The luminance of the images observed through the eyepieces of the operating microscope and the image of a 3D system created by a high-sensitivity sensor Exmor R 3CMOS HD camera (Sony MCC-1000MD) were measured. Results. All surgeries were completed without any complications under the low illumination conditions. The surgical field on the display monitor was created by a 3D system using a high-sensitivity sensor camera and was observed in a heads-up position. The patients did not report any intolerable discomfort or glare during the surgery. Cataract surgeries were performed with a good view of the surgical field under the extremely low illumination from the surgical microscope. The high-sensitivity sensors and electronic amplifications of the image signals made the surgical field brighter and allowed the surgeon to perform the surgery confidently and safely. Conclusions. Heads-up, 3D-assisted intraocular surgeries can be performed safely and efficiently with low illuminance of the surgical field. This trial is registered with UMIN000037838.

PurposeTo evaluate retinal function by intraoperative electroretinograms (ERGs) before and after core vitrectomy.DesignRetrospective consecutive case series.MethodFull-field photopic ERGs were recorded prior to the beginning and just after core vitrectomy using a sterilized contact lens electrode in 20 eyes that underwent non-complicated vitreous surgery. A light-emitted diode was embedded into the contact lens, and a stimulus of 150 ms on and 350 ms off at 2 Hz was delivered. The amplitudes and latencies of the a-, b-, and d-waves, photopic negative response (PhNR), and oscillatory potentials (OPs) were analyzed. The intraocular temperature at the mid-vitreous was measured at the beginning and just after the surgery with a thermoprobe.ResultsThe intraocular temperature was 33.2 ± 1.3°C before and 29.4 ± 1.7°C after the vitrectomy. The amplitudes of the PhNR and OPs were significantly smaller after surgery, and the latencies of all components were prolonged after the surgery. These changes were not significantly correlated with the changes of the temperature.ConclusionRetinal function is reduced just after core vitrectomy in conjunction with significant temperature reduction. The differences in the degree of alterations of each ERG component suggests different sensitivity of each type of retinal neuron.

Purpose: To present our findings on the cause of an acute visual field defect (VFD) that developed in a patient on the day after vitrectomy for proliferative diabetic retinopathy. Case: A 50-year-old man complained of a blind area in the superior visual field that developed one day after vitrectomy. The patient had undergone uncomplicated vitrectomy for a long-duration vitreous hemorrhage associated with proliferative diabetic retinopathy. Residual vitreous hemorrhage hampered a clear view of the fundus. Goldmann perimetry showed a horizontal VFD in the superior field. The area corresponding to the VFD was examined by multifocal electroretinograms (mfERGs) and multifocal visual evoked potentials (mfVEPs). The amplitudes of the mfVEPs were reduced with prolonged implicit times especially when the superior hemifield was stimulated, while the amplitudes and implicit times were within the normal range when other parts of the visual field were stimulated. In addition, the full-field photopic ERGs and photopic negative responses were attenuated in the right eye. These findings suggested that the VFD did not originate from alterations in the retinal inner and middle layer but in the ganglion cells. The visual acuity improved to 1.2 but his optic disc became pale and the VFD remained unchanged more than 12 years after the surgery. Conclusion: We suggest that vitrectomy can cause ischemic optic neuropathy by interfering with the circulation associated with diabetes mellitus. Evaluations by mfERGs, mfVEPs, and full-field photopic ERGs were helpful in making the diagnosis.

Purpose: To report a case of a ruptured eye with a suprachoroidal hemorrhage (SCH) in which tissue plasminogen activator (tPA)-assisted vitrectomy was successful in reconstructing the globe and restoring good vision. Case: A 32-year-old man was struck on the right eye by a surfboard. His eye was ruptured and his visual acuity decreased to hand movements. Surgery was immediately performed to successfully close the ruptured globe. Nine days later, a second surgery was performed, and tPA (25 µg/0.1 ml monteplase) was used to liquefy and drain the SCH. This freed enough vitreous space for a more comprehensive vitrectomy. Eighteen months after the injury, the retina remained attached, and the decimal best-corrected visual acuity improved to 0.8. Conclusion: tPA was helpful in lysing a massive SCH, thereby contributing to the excellent visual outcome. tPA-assisted drainage should be considered in cases of massive SCH when drainage is difficult due to an incomplete lysis of the clot.

Purpose: Peripheral cone dystrophy is a subgroup of cone dystrophy, and only 4 cases have been reported. We present a patient with unilateral peripheral cone dysfunction and report the functional changes determined by electrophysiological tests and ultrastructural changes determined by spectral domain optical coherence tomography (SD-OCT). Case: A 34-year-old woman complained of blurred vision in both eyes. Our examination showed that her visual acuity was 0.05 OD and 0.2 OS. A relative afferent pupillary defect was present in her right eye. The results of slit-lamp examination, ophthalmoscopy, and fluorescein angiography were normal except for pallor of the right optic disc. SD-OCT showed a diffuse thinning of the retina in the posterior pole of the right eye. A severe constriction of the visual fields was found in both eyes but more in the right eye. The photopic full-field electroretinograms (ERGs) were reduced in the right eye but normal in the left eye. The multifocal ERGs were severely reduced throughout the visual field except in the central area of the right eye. The multifocal ERGs from the left eye were normal. The pattern visual evoked responses were within the normal range in both eyes. She had a 5-year history of sniffing paint thinner. Results: Although the visual dysfunction was initially suspected to be due to psychological problems from the results of subjective tests, objective tests indicated a peripheral cone dysfunction in the right eye. The pathophysiological mechanism and the relationship with thinner sniffing were not determined. Conclusions: Our findings indicate that peripheral cone dysfunction can occur unilaterally. Electrophysiology and SD-OCT are valuable tests to perform to determine the pathogenesis of unusual ocular findings objectively.

Purpose: Acute zonal occult outer retinopathy (AZOOR) is characterized by an acute zonal loss of outer retinal function with minimal ophthalmoscopic changes in one or both eyes. We present a patient with AZOOR whose ultrastructural and functional findings were followed for 8 months. Case: A 22-year-old woman developed an acute central scotoma in her right eye. Her best-corrected visual acuity (BCVA) was 0.5 OD and 1.2 OS. The ophthalmoscopic examinations, fluorescein angiography, and full-field electroretinograms (ERGs) were normal in both eyes. The amplitudes of the multifocal ERGs (mfERGs) were attenuated in the area corresponding to the scotoma. Spectral domain optical coherence tomography showed an absence of both the inner and outer segment (IS/OS) line of the photoreceptors and the cone outer segment tip (COST) line between the IS/OS line and the retinal pigment epithelium. These changes were seen in the area corresponding to the scotoma. One month later, the scotoma disappeared and the BCVA improved to 1.2 OD. The mfERGs increased to almost the same amplitude as the fellow eye. The IS/OS line became discernible but the COST line was still absent. The ophthalmological findings of the right macula remained normal during the 11-month follow-up period. Conclusions: Our findings indicate that the selective loss of the IS/OS and the COST lines is probably the morphological alterations corresponding with the reduced BCVA and the mfERGs in the areas of the visual field defects in the acute phase of AZOOR. But in the recovery phase, only the abnormality of the COST line is a subclinical sign for the disease. These findings should be important in understanding and evaluating the pathological mechanism in other outer retinal diseases.

Purpose: To present a patient with acute idiopathic blind spot enlargement syndrome who had abnormal changes in the outer retinal microstructure limited to areas with reduced responses on multifocal electroretinograms as well as to the area involving a scotoma. Methods and Results: We report the case of a 44-year-old man who developed an arcuate scotoma which was associated with a physiological blind spot in the left eye. The ophthalmoscopic, fluorescein angiographic, and full-field electroretinogram findings were normal. The amplitudes of the multifocal electroretinograms were reduced in the area of the scotoma. Optical coherence tomography showed that both the external limiting membrane and the inner and outer segment (IS/OS) line were intact, but that the middle cone outer segment tip line between the IS/OS line and the retinal pigment epithelium was absent in the nasal macular area of the left eye. Conclusions: These findings indicate that the integrity of not only the external limiting membrane and IS/OS line but also the cone outer segment tip line is important for the function of the retina.

Background: We present two patients who developed severe protrusion of the conjunctiva and chemosis secondary to Harlequin ichthyosis (HI). Case Reports: Case 1 was a male infant diagnosed with HI who had parchment-like appearance and conjunctival protrusion with severe chemosis. Case 2 was a female infant on whom HI had been suspected before birth through ultrasonography. She showed thickened skin over the entire body and conjunctival protrusion with severe chemosis. For both cases, a vitamin A derivative was applied and the hyperkeratotic layer was peeled off every day. Great care was taken to sterilize and moisten the ocular surface. The conjunctival protrusion gradually improved and other systemic conditions were successfully treated. HI is a rare condition, but affected infants are surviving longer than previously and hence guidelines for ocular management are now required. Conclusions: Gentle and patient debridement of the hyperkeratotic skin and moisturizing were important in treating the unusual conjunctival protrusion.

Celso S Matsumoto1,2, Kei Shinoda1, Kazuo Nakatsuka21Department of Ophthalmology, Teikyo University School of Medicine, Tokyo, Japan; 2Department of Ophthalmology, Faculty of Medicine, Oita University, Oita, JapanPurpose: The aim of this study was to determine whether a significant correlation exists between the scotopic and photopic components of electroretinograms (ERGs) and the degree of circulation disturbances caused by a central retinal artery occlusion (CRAO).Design: Observational clinical study.Participants: Sixteen patients with a CRAO (16 eyes).Methods: The circulatory disturbance in eyes with CRAO was graded as mild (group 1) when the arm-to-retina transmission time was

Background: Endogenous endophthalmitis is a rare disease and its visual prognosis is poor. Case Reports: We present two patients, a 60-year-old man and a 53-year-old man, who developed endogenous endophthalmitis caused byGram-positive organismsbut recovered good vision after antibiotics and vitrectomy. Results: The first patient complained of ocular pain and visual decrease in his right eye. Ophthalmoscopy showed inflammation in the anterior chamber and vitreous opacities. Antibiotic was administrated systemically, and blood culture detected Streptococcus anginosus. He underwent successful heart surgery for endocarditis and total dental extraction for severe gingivitis. Vitrectomy was performed 36 days after the onset and vision improved from 0.02 to 0.7. The second patient was referred for acute visual decrease in his left eye. Severe iritis and vitreous opacities were observed, and systemic examination showed acute pyelitis and prostatic abscesses. Blood cultures detected Staphylococcus sp., and systemic antibiotics were given. Vitrectomy was performed 12 days after the onset, and vision improved from 0.06 to 1.2. Conclusions: We conclude that the rapid treatment with systemic antibiotics for the organisms at the primary site, and the vitrectomy, even though delayed, can lead to a good recovery of vision.

Aim: Infliximab, an anti-tumor necrosis factor (TNF)-α monoclonal antibody, has been reported to be effective in refractory uveoretinitis in Behçet’s disease. Because it has been used clinically for a short time, information on its adverse effects is limited. We report a patient who developed cystoid macular edema (CME) following infliximab use for uveoretinitis associated with Behçet’s disease. Case Report: A 27-year-old man had refractory uveoretinitis and neuro-Behçet’s disease, and intravenous infliximab was administered. Results: One day after infliximab infusion, the patient complained of a decrease in the vision in his left eye. The visual acuity had decreased from 1.2 to 0.5. Daily optical coherence tomographic evaluations showed a progressive worsening of the CME, and fluorescein angiography showed a typical staining with a cystic pattern. Two weeks later, the height of CME appeared to reach a maximum level and thereafter gradually resolved in spite of the continuation of infliximab administration. The visual acuity improved while the patient was treated with repeated subtenon injections of steroids in addition to continuation of infliximab and finally increased from 0.15 to 1.2. Conclusions: Although the mechanism of CME is not known, clinicians should be aware that infliximab therapy might cause a development and worsening of CME. Thus, it is crucial to rule out preexisting abnormalities in the macula prior to commencing infliximab infusion.

To describe a new technique to record focal macular electroretinograms (FMERGs) during vitrectomy to assess macular function.Intraoperative FMERGs (iFMERGs) were recorded in ten patients (10 eyes) who undergo vitrectomy. iFMERGs were elicited by focal macular stimulation. The stimulus light was directed to the macular area through a 25 gauge (25G) glass fiber optic bundle. Background light was delivered through a dual chandelier-type light fiber probe. Focal macular responses elicited with combinations of stimulus and background luminances were analyzed.A stimulus luminance that was approximately 1.75 log units brighter than the background light was able to elicit focal macular responses that were not contaminated by stray light responses. Thus, a stimulus luminance of 160 cd/m2 delivered on a background of 3 cd/m2 elicited iFMEGs from only the stimulated area. This combination of stimulus and background luminances did not elicit a response when the stimulus was projected onto the optic nerve head. The iFMERGs elicited by a 10° stimulus with a duration of 100 ms and an interstimulus interval of 150 ms consisted of an a-, b-, and d-waves, the oscillatory potentials, and the photopic negative response (PhNR).Focal ERGs with all components can be recorded from the macula and other retinal areas during vitreous surgery. This new technique will allow surgeons to assess the function of focal areas of the retina intraoperatively.

Background. To report the outcome of pars plana vitrectomy (PPV) combined with intraoperative endolaser focal photocoagulation (PC) on eyes with idiopathic macular telangiectasis (MacTel) type 1. Methods. This was a retrospective study of two female patients with MacTel type 1 who were resistant to focal photocoagulation, sub-Tenon triamcinolone injection, and/or antiangiogenic drugs. The best-corrected visual acuity (BCVA) was determined, and fluorescein angiography (FA) and spectral domain optical coherence tomography (SD-OCT) were performed before and after surgery for up to 19 months. Results. After surgery, the BCVA gradually improved from 20/100 to 20/20 at 19 months in Case 1 and from 20/50 to 20/13 at 13 months in Case 2. Fluorescein angiography (FA) showed leakage at the late phase, and OCT showed that the cystoid macular edema was resolved and the fovea was considerably thinner postoperatively. Conclusion. Patients with MacTel type 1 who are refractory to the other types of treatments can benefit from PPV combined with intraoperative endolaser focal PC with functional and morphological improvements.

OBJECTIVE:The purpose of this study was to investigate frequent disease-causing gene mutations in autosomal recessive retinitis pigmentosa (arRP) in the Japanese population. METHODS:In total, 99 Japanese patients with non-syndromic and unrelated arRP or sporadic RP (spRP) were recruited in this study and ophthalmic examinations were conducted for the diagnosis of RP. Among these patients, whole exome sequencing analysis of 30 RP patients and direct sequencing screening of all CNGA1 exons of the other 69 RP patients were performed. RESULTS:Whole exome sequencing of 30 arRP/spRP patients identified disease-causing gene mutations of CNGA1 (four patients), EYS (three patients) and SAG (one patient) in eight patients and potential disease-causing gene variants of USH2A (two patients), EYS (one patient), TULP1 (one patient) and C2orf71 (one patient) in five patients. Screening of an additional 69 arRP/spRP patients for the CNGA1 gene mutation revealed one patient with a homozygous mutation. CONCLUSIONS:This is the first identification of CNGA1 mutations in arRP Japanese patients. The frequency of CNGA1 gene mutation was 5.1% (5/99 patients). CNGA1 mutations are one of the most frequent arRP-causing mutations in Japanese patients.

Makoto Inoue1,2, Kei Shinoda3, Tomoko Matsuda-Yamamitsu2, Ronaldo Yuiti Sano1, Susumu Ishida2,41Kyorin Eye Center, Kyorin University School of Medicine, Mitaka, Tokyo, Japan; 2Department of Ophthalmology, Keio University School of Medicine, Shinjuku, Tokyo, Japan; 3Department of Ophthalmology, Teikyo University School of Medicine, Itabashi, Tokyo, Japan; 4Department of Ophthalmology, Hokkaido University School of Medicine, Sapporo, Hokkaido, JapanAbstract: We report a case of a patient with a highly hyperopic eye who underwent cataract surgery combined with vitreous surgery to create a posterior vitreous detachment (PVD) to prevent choroidal neovascularization (CNV). A 78-year-old man noticed a decrease in his vision due to a cataract in his right eye. The patient had a severe visual loss in his left eye because of a CNV 2 years after a cataract surgery. His visual acuities were 20/30 OD and 20/600 OS, and funduscopic examination showed an orange-colored lesion OD and degenerative subretinal fibrosis OS. The posterior vitreous was attached to the retina in both eyes. The axial length was 18.9 mm OD and 19.0 mm OS. Cataract surgery combined with vitreous surgery to create PVD was performed on the right eye, and the vision improved to 20/20 with no signs of developing CNV after 5 years. We conclude that cataract surgery combined with vitreous surgery to create a PVD may prevent the development of CNV in highly hyperopic eyes.Keywords: hyperopia, macular degeneration, posterior vitreous detachment, cataract surgery

Background/aims To evaluate the electroretinographic (ERG) changes in the early postoperative period following glaucoma filtration surgery, and its relationship with choroidal detachment (CD). Methods This retrospective observational singlecentre study included 57 consecutive patients with primary open-angle glaucoma who underwent unilateral glaucoma filtration surgery. The patients were divided into two groups according to the presence or absence of CD. ERG components, including the photopic negative response (PhNR), a-wave and b-wave were compared before and after surgery using skin electrodes. Results There were 46 patients in the non-CD group and 11 in the CD group. ERG was recorded within 5.1 (2.1 to 8.1) (mean (95%CI)) days after surgery. In the non-CD group, the PhNR amplitude, PhNR/bwave amplitude ratio and PhNR implicit time improved significantly after surgery (p=0.008, 0.002 and 0.039, respectively). In the CD group, the amplitude of the PhNR, a-wave and b-wave were significantly deteriorated after surgery (p=0.002, 0.001 and 0.001, respectively). Postoperative intraocular pressure (IOP) (p=0.031) and postoperative CD (p<0.001) were significantly associated with change in the PhNR amplitude in the univariate models. In the multivariate analysis, severe CD (stage 3) cases tended to be deteriorated more. Conclusion Even in the early postoperative period within several days, the PhNR amplitude increased with IOP lowering following filtration surgery in the absence of CD. The presence of CD may arrest the improvement of the retinal ganglion cell function. The present results enhance understanding the structural and functional recovery after glaucoma surgery and the role of postoperative CD. [ABSTRACT FROM AUTHOR]