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Purpose: To try to recreate the images reported by patients during vitreous surgery in a model eye. Methods: A fluid-filled model eye with a posterior frosted translucent surface which corresponded to the retina was used. Three holes were made in the model eye through which an endoillumination pipe and intraocular forceps could be inserted. A thin plastic sheet simulating an epiretinal membrane and an intraocular lens (IOL) simulating a dislocated IOL were placed on the retina. The images falling on the posterior surface were photographed from the rear. The images seen through the surgical microscope were also recorded. Results: The images from the rear were mirror images of those seen through the surgical microscope. Intraocular instruments were seen as black shafts from the rear. When the plastic sheet was picked up, the tip of the forceps was seen more sharply on the posterior surface. The images of the dislocated IOL from the posterior were similar to that seen through the surgical microscope, including the yellow optics and blue haptics. Conclusion: Intravitreal objects can form images on the surface of a model eye. Objects located closer to the surface are seen more sharply, and the colour of the objects can be identified.

Scleral indentation is widely used to examine the peripheral fundus, however it can increase the intraocular pressure (IOP) to high levels which can then affect retinal function. We evaluated the effects of scleral indentation on the macular function electrophysiologically. Intraoperative focal macular electroretinograms (iFMERGs) were recorded with and without controlling the IOP in 7 eyes. Without IOP control, the IOP increased from 21.7 ± 4.9 to 92.7 ± 20.2 mmHg significantly (P = 0.020) and the amplitudes of the b-wave (from 6.29 ± 1.160 to 3.71 ± 1.98 uV, P = 0.007), on-photopic negative response (from 2.29 ± 0.99 to 0.72 ± 0.47 uV, on-PhNR, P = 0.005), and d-wave (from 2.57 ± 0.41 to 1.64 ± 0.69 uV, P = 0.007) decreased significantly soon after beginning the indentation. All values returned to the baseline levels after releasing the indentation. In the eyes with IOP controlled, the IOP and the amplitude of all components did not change significantly during and after the indentation except the on-PhNR amplitude which was significantly reduced during the indentation. The changes in the iFMERGs and macular function caused by scleral indentation were transient and reversible. The changes can be minimized by controlling the IOP.

Intravitreal injections (IVI) have become a part of daily practice for a growing number of procedures. We evaluated the retinal function by recording intraoperative photopic electroretinograms (ERGs) before an injection (T1), just after the injection (T2) and after the aspiration of the anterior chamber fluid (T3) of 19 eyes of 19 patients (mean age 70.6 years; men = 11) who received an IVI of an anti-vascular endothelial growth factor. The mean amplitudes of the b-wave, photopic negative responses (PhNR) and oscillatory potentials (OPs) 1 and 2 at T2 were significantly smaller than that at T1, but no significant difference was observed between T3 and T1. The mean implicit times of the a-wave and OP1, 2 and 3 at T2 and the a-wave and the OP2 at T3 were significantly longer than that at T1. The mean intraocular pressure (IOP) at T2 (49.32 mm Hg) was significantly higher and the IOP at T3 (8.74 mm Hg) was significantly lower than that at T1 (21.05 mm Hg). The retinal function was reduced and the IOP elevated just after the IVI. The response of each ERG component was different suggesting a different sensitivity of each type of retinal neuron to IVI.

Purpose To investigate the relationship between the integrity of the photoreceptor microstructures and retinal sensitivity after half-dose photodynamic therapy (PDT) in eyes with chronic central serous chorioretinopathy (CSC). Design Prospective, noncomparative, interventional case series. Methods Thirteen eyes of 13 patients with chronic CSC who had received half-dose verteporfin PDT were studied. The best-corrected visual acuity (BCVA), macular retinal sensitivity in the central 12 degrees, and optical coherence tomographic (OCT) findings were evaluated at baseline and at 1, 3, 6, and 12 months after the half-dose PDT. The integrities of the external limiting membrane (ELM), the inner segment/outer segment junction (IS/OS) line, and the cone outer segment tips (COST) line were determined. The retinal sensitivity was determined by MP-1 microperimetry. Results The serous retinal detachment (SRD) was completely resolved in 11 eyes at 1 month and in 1 eye at 3 months. The remaining eye had a persistent SRD throughout the follow-up period. The mean retinal sensitivity was significantly better at 1, 3, 6, and 12 months after the half-dose PDT than at baseline. Before the PDT, 12 eyes had a continuous ELM and 1 eye had a fragmented ELM. The number of eyes with an intact IS/OS line and COST line increased with increasing post-PDT time. At 12 months after PDT, the IS/OS line was detected in 11 eyes and the COST line in 6 eyes. At 6 and 12 months, the retinal sensitivity was significantly higher in eyes with an intact COST line and IS/OS lines than in eyes with an intact IS/OS line only. However, no difference was found in the visual acuity of these 2 groups. The 1 eye with a persistent SRD and another eye with fragmented ELM and absent IS/OS and COST lines through the follow-up periods were associated with poor retinal sensitivity of 8.5 dB and 10.9 dB respectively. Conclusions Our findings show that there is a significant improvement in the macular sensitivity after half-dose PDT in eyes with chronic CSC. The improvement was correlated with the recovery of the IS/OS and COST lines at 6 and 12 months.

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.

We report the changes of the retinal pigment epithelium (RPE) determined by optical coherence tomography in a patient with atypical Vogt–Koyanagi–Harada (VKH) disease during the recovery phase. Case presentation. A 74-year-old woman presented with a central scotoma associated with disc hyperemia, serous retinal detachment, and scleral thickening in her left eye, and was diagnosed with VKH. OCT showed a serous retinal detachment and a wavy RPE contour that corresponded to the choroidal folds. The OCT images clearly showed the dynamic changes of the retina, RPE, and choroid during the recovery phase. The OCT was found to be more sensitive in detecting the choroidal folds than angiography. OCT images are valuable not only in the diagnosis, but also for monitoring the morphology of the retina and choroid during the course of VKH.

PURPOSE Retinal vascular abnormalities are rare in patients with Churg-Strauss syndrome. We present the findings in a patient with Churg-Strauss syndrome who developed bilateral central retinal artery occlusion simultaneously. METHODS Case report. RESULTS A 68-year-old Japanese man developed acute bilateral vision decrease to counting finger in the right eye and hand movements in the left eye. Ophthalmoscopic and angiographic examinations revealed a central retinal artery occlusion with choroidal circulatory disturbances in both the eyes. The patient had bronchial asthma, hypereosinophilia, radiographically determined migratory pulmonary opacities, and paranasal sinus abnormalities, thus fulfilling the American College of Rheumatology criteria for Churg-Strauss syndrome. Antineutrophil cytoplasmic antibody was absent. High-dose steroid therapy was used, but after 6 weeks, his visual acuity in the right eye did not improve, and the vision in the left eye was no light perception. Later, vitreous hemorrhage was developed in the left eye followed by retinal detachment associated with proliferative retinopathy. CONCLUSION Bilateral central retinal artery occlusion can occur in patients with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome. The cause of the central retinal artery occlusion is not known, but consideration for prophylactic steroid therapy may be recommended in antineutrophil cytoplasmic antibody-negative cases to prevent potential visual loss.

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.

a Ophthalmology, b Pediatrics, and c Obstetrics and Gynecology, Oita University Faculty of Medicine, Hasama-machi, Yufu-shi, d Abstract 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.

Purpose To evaluate the reproducibility of vessel density calculations using different binarization methods obtained via two commercially available swept-source optical coherence tomography angiography (SS-OCTA) systems. Methods Healthy volunteers were imaged using two swept-source optical coherence tomography angiography (SS-OCTA) devices, PLEXElite and Triton. SS-OCTA examinations were performed using a 3 × 3-mm volume scan pattern centered on the fovea. A total of six methods were used for binarization in ImageJ, two global thresholding and four local adaptive thresholding methods. Resultant vessel density values were compared between the instruments and binarization methods. Images for 60 eyes from 30 healthy subjects were assessed by two reviewers who were blinded to the scanning system used. Results Twenty-two eyes were excluded due to poor image quality (17 eyes from Triton, 4 eyes from PLEXElite, and 1 eye from both instruments, P = 0.003). A final 38 eyes from 23 subjects were eligible for analysis. Each binarization method and instrument led to different median values. The coefficients of variation for vessel density measurements ranged from 0.3% to 2.3% and 0.6% to 4.7% for the PLEXElite and Triton, respectively. Local adaptive thresholding methods revealed higher reproducibility than did global thresholding methods for both devices. Conclusions Macular scans with both SS-OCTA instruments showed good reproducibility for vessel density measurements. PLEXElite recorded fewer poor images and had higher reproducibility than did Triton. These findings will inform the selection of proper binarization methods for the clinical detection of vascular diseases affecting the central retina. Translational relevance The reproducibility for macular vessel measurements with SS-OCTA instruments was good. PLEXElite recorded fewer poor images and had higher reproducibility than did Triton.

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 by Gram-positive organisms but 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.

For human trials with retinal prostheses it is mandatory to develop procedures to safely explant and possibly reimplant the devices. This prompted us to investigate in a small exploratory study the safety of repeated transchoroidal implantation and explantation procedures of complex subretinal devices in laboratory animals.Repeated transchoroidal surgery was performed in four rabbits. The rabbits were examined by clinical examination and funduscopy. Function was assessed by electroretinography and cortical recordings following light and subretinal electrical stimulation. Sections of the retina and of the implantation channel were examined by light microscopy.Using the same access route, repeated transchoroidal subretinal implantation surgery was successfully performed in all cases. Fixation of implants was stable for up to 13 months; retinas remained attached at all examination dates. Electroretinograms and visual evoked cortical potential proved retinal and visual pathway integrity. Subretinal electrical stimulation elicited retinal and cortical responses. While retinal morphology at earlier stages was found to be essentially unaltered, atrophic disorganization in the region of the subretinal channel was observed after 10 months and after subretinal electrical stimulation.Repeated transchoroidal surgery can be safely performed for implantation, explantation, and reimplantation of subretinal devices in rabbits. With modifications, we believe the technique can be applied in human surgery.

PURPOSE To determine the extent of retinal damage in Purtscher retinopathy (PR). METHODS A longitudinal analysis of the retinal morphology and function was made by optical coherence tomography and electroretinography, respectively, on a patient with unilateral PR. RESULTS Optical coherence tomography showed a marked thickening of the inner retinal layers in the acute phase and a thinning of the inner retinal layers and alteration of the outer layers in the chronic phase. The site of these changes corresponded with the cotton-wool patches. The results of electroretinography indicated reduced inner layer function in the acute phase and partial recovery in the chronic phase. CONCLUSION The inner and outer retinal layers are altered morphologically and functionally in eyes with PR. The dynamic evaluation on retinal morphology and function may be useful in understanding the pathophysiology of PR.

Purpose: To report the jamming of 25-gauge instruments in the cannula during vitreous surgery for non-clearing vitreous haemorrhage. Methods: Forty-five eyes underwent vitrectomy with 25-gauge instruments for non-clearing vitreous haemorrhage (VH group). The incidence of 25-gauge instruments jamming in the cannula was determined retrospectively and compared with that in 112 eyes that underwent vitrectomy for epiretinal membrane (ERM group), also using 25-gauge instruments. Results: The 25-gauge vitreous cutter or light pipe became jammed in the cannula in three eyes (7%) in the VH group and the instrument locked inside the cannula had to be removed with the cannula. None of the 25-gauge instruments in the ERM group jammed (p = 0.022, Fisher's exact probability test). Two of three eyes developed giant retinal breaks near the sclerotomy but no retinal break related to the sclerotomy was detected in the ERM group. Examination of the cutter revealed blood trapped between the cutter and the cannula. Conclusions: Twenty-five gauge instruments may become jammed in the cannula in eyes with non-clearing vitreous haemorrhage. Clinicians should be aware of this surgical complication when 25-gauge instruments are used in vitreous haemorrhage.

The superoxide dismutase (SOD) family is a major antioxidant system, and deficiency of Cu,Zn-superoxide dismutase (SOD1) in mice leads to many different phenotypes that resemble accelerated aging. The purpose of this study was to examine the morphology and physiology of the sensory retina in Sod1(-/-) mice. The amplitudes of the a- and b-waves of electroretinograms elicited by stimuli of different intensity were reduced in senescent Sod1(-/-) mice, and this reduction in amplitude was more pronounced with increasing age. Retinal morphometric analyses showed a reduced number of nuclei in both the inner nuclear cell layer and outer nuclear cell layer. Electron microscopy revealed swollen cells and degenerated mitochondria in the inner nuclear cell and outer nuclear cell layer of senescent Sod1(-/-) mice indicating necrotic cell death. Terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling revealed no significant differences in the number of apoptotic cells between Sod1(-/-) and wild-type mice, and activated caspase-3 could not be detected in the retina of Sod1(-/-) mice. In addition to the age-related macular degeneration-like phenotypes previously reported, Sod1(-/-) mice also present progressive retinal degeneration. Our results indicate that Sod1(-/-) mice may be a good model system in which to study the mechanism of reactive oxygen species-mediated retinal degeneration.

To determine the cause of the progressive glaucomatous visual field defects in three patients after an encircling scleral buckle for a rhegmatogenous retinal detachment (RRD).Scanning laser Doppler flowmetry and visual field tests were performed on three cases with unilateral progressive visual field defect after an encircling scleral buckling for a RRD. Similar measurements were made after the buckle was removed.After implanting the scleral buckle, the intraocular pressure was normal and chamber angle was open. The blood flow in the neuroretinal rim of the optic disk was lower than that in the healthy fellow eye. After removing the buckle, the blood flow improved to normal levels and a further worsening of the visual field was not detected.These results suggest that an encircling scleral buckle may impair choroidal circulation and lead to visual field defects similar to eyes with normal tension glaucoma.

Background: This study set out to document the early electrophysiological and immunohistochemical changes that occur in the retina of experimentally induced diabetic rats. Methods: Diabetes was induced in rats by intraperitoneal injection of 60 mg/kg of streptozotocin (STZ). Electroretinogram readings were taken monthly under either short-duration or long-duration stimuli for up to 3 months after STZ. Oscillatory potentials (OP) and the amplitudes and implicit times of a- and b-waves were analysed, and b-wave amplitudes were analysed using a Naka–Rushton fit. Scotopic a-waves were analysed with photoreceptor models, and Rmp3 (the maximum a-wave amplitude) and S (sensitivity) were calculated. Three months after STZ injection, immunohistochemistry for glial fibrillary acidic protein was performed on the retinas of the STZ-treated rats and age-matched controls. Results: The implicit OP times were significantly longer in the diabetic rats as compared with the controls, and this difference was noted as early as 1 month following STZ treatment. Other electrophysiological parameters, such as OP amplitudes, a- and b-wave amplitude as well as the implicit times, did not differ from controls at this stage. The sacrificed STZ-treated rats also demonstrated marked enhancement of glial fibrillary acidic protein immunoreactivity, suggesting that at least in experimentally induced diabetic retinopathy there is increased Muller cell reactivity. Conclusion: The results of this study indicated that functional alterations in the retina develop rapidly after the onset of diabetes. Analysis of each electroretinogram component may be useful in further investigating the development mechanisms of diabetic retinopathy.

Purpose: To compare the surgical outcomes and complications of vitrectomy with internal limiting membrane (ILM) peeling with or without indocyanine green (ICG) staining in eyes with an idiopathic macular hole. Methods: This study involved a non-randomized, single-centre, retrospective, interventional case series. Rates of anatomical closure, visual acuities (VAs) and postoperative complications in 35 eyes of 31 patients who underwent ICG-assisted ILM peeling during macular hole surgery (stained group) were compared with those in 18 eyes of 16 patients who underwent the same procedure without ICG staining (non-stained group). Results: Macular holes were closed following the initial surgery in 97% of the stained group and 94% of the non-stained group (p > 0.999). There was no significant difference in mean final VA between the stained and non-stained groups, but there was a lower percentage of eyes with postoperative vision > 20/25 in the stained group (15%) than in the non-stained group (44%) after 2 years (p = 0.036). Posterior retinal pigment epithelium atrophy, retinoschisis and visual field defects were observed only in the stained group. Conclusions: The difference in mean final VA between the two groups was not significant. However, a lower percentage of eyes obtained VA ≥ 20/25 and a higher incidence of postoperative complications occurred in the stained group. These results indicate that some consideration should be made before ICG is used in macular hole surgery.

To report the outcome of transcorneal electrical stimulation (TES) of the visual system on long-standing retinal artery occlusion (RAO).Open labeled, case series.Two patients with central RAO (15 and 33 months respectively) and one with branch RAO (26 months) underwent TES therapy. Subjective and objective ophthalmological evaluations were performed before and after the TES. The ages of the patients were 38, 49, and 63 years. The TES (20 Hz biphasic pulses, 30 minutes, up to 1100 uA) was delivered by a bipolar contact lens electrode once a month for 3 months. Perimetric and/or electrophysiological examinations were performed as outcome measures.The visual acuity improved by more than 0.2 logMAR units in two cases, and the visual fields were improved in all three cases. The multifocal ERGs which had been reduced in the loci corresponding to the ischemic retinal area were improved after the treatment in two cases. Neither ocular nor systemic adverse effects were observed except for transient superficial keratitis.TES of the retina can improve retinal function in eyes with long-standing RAOs.

To evaluate expression of proangiogenic matrix metalloproteinases (MMP) 2 and 9 at distinct intervals after verteporfin photodynamic therapy (PDT) in human choroidal neovascular membranes (CNV) secondary to age-related macular degeneration (AMD).Retrospective review of an interventional case series of 49 patients who underwent removal of CNV. Twenty-six patients were treated with PDT 3 to 383 days prior to surgery. Twenty-three CNV without previous treatment were used as controls. CNV were stained for CD34, cytokeratin 18, endostatin, MMP-2 and MMP-9 by immunohistochemistry.CNV without previous therapy disclosed MMP-2, MMP-9 in RPE-Bruch's membrane, vessels and stroma in different intensities. Three days after PDT, MMP-9 expression was significantly weaker in stroma (p = 0.0019). Endostatin was significantly reduced in vessels (p0.001). At longer post-PDT intervals, a significant increase of MMP-9 in stroma (p = 0.037) and of endostatin in RPE-Bruch's membrane (p = 0.02), vessels (p = 0.005) and stroma (p0.001) were disclosed. No significant changes in MMP-2 expression were detected.PDT induced an early, temporary decrease in MMP-9 and endostatin expression. At longer intervals, MMP-9 increase is possibly associated with the angiogenic process responsible for recurrence after PDT. MMP-9, however, acts as a double-edged sword by concomitant induction of endostatin, an endogenous inhibitor of angiogenesis.

Aim: To evaluate the effect of verteporfin photodynamic therapy (PDT) on endostatin with regard to expression of vascular endothelial growth factor (VEGF) in human choroidal neovascular membranes (CNVs) secondary to age-related macular degeneration. Methods: A retrospective review of an interventional case series of 68 patients who underwent removal of CNV. 29 patients were treated with PDT 3–655 days before surgery. 39 CNVs without previous treatment were used as controls. CNVs were stained for CD34, CD105, Ki-67, cytokeratin 18, endostatin, E-selectin and VEGF. “Predominance score of VEGF over endostatin” (mean) was defined as the difference between VEGF and endostatin staining scores. Results: In four CNVs treated by PDT 3 days previously, PS was significantly higher in the retinal pigment epithelium (mean = 2.5, p = 0.006) and stroma (mean = 2, p = 0.015) than in the control group (mean = 0). At longer post-PDT intervals, PS was significantly decreased in the retinal pigment epithelium (mean = 0, p = 0.019) and stroma (mean = 0, p = 0.015). Proliferative activity was high (p = 0.023), but mostly related to inflammatory cells. PDT did not influence E-selectin expression significantly. Conclusions: VEGF predominance over endostatin early after PDT might contribute to enhanced angiogenic activity associated with recurrences. Strategies upregulating or replacing endostatin early after PDT might increase the effectiveness of PDT.

To investigate the tissue blood flow in the neuroretinal rim of the optic disk and macula after rhegmatogenous retinal detachment (RRD) surgery.Tissue blood flow in the neuroretinal rim of the optic disk and macula was measured with the Heidelberg retina flowmeter in 53 eyes of 53 patients who had undergone successful surgery for unilateral RRD. Patients were divided into three groups; those who had the RRD treated by conventional encircling scleral buckling (group E), by local buckling (group L), and by primary vitrectomy (group V). Blood flow measurements were made more than 6 months after surgery in a 10 degrees x 2.5 degrees area of the superior and inferior margins of the neuroretinal disk rim and of the superior and inferior macula area. The mean blood flow (MBF) and the ratio of the MBF in the affected eye to the healthy fellow eye (a/f ratio) were compared among the three groups. The influence of several clinical factors on the MBF was also investigated.The MBF rate and mean a/f ratios of the MBF of the three groups were not significantly different. Multiple regression analysis revealed that the averaged MBF both at superior and inferior disk rims was significantly correlated with only the gas tamponade procedure.The ocular microcirculation is normal 6 months after scleral buckling or vitrectomy for RRD. However, the use of gas tamponade might have a subclinical adverse effect on the circulation in the neuroretinal disk rim.

Purpose To describe a new technique to record focal macular electroretinograms (FMERGs) during vitrectomy to assess macular function. Methods 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. Results 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). Conclusions 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.

Oxidative stress has long been linked to the pathogenesis of neurodegenerative diseases; however, whether it is a cause or merely a consequence of the degenerative process is still unknown. We show that mice deficient in Cu, Zn-superoxide dismutase (SOD1) have features typical of age-related macular degeneration in humans. Investigations of senescent Sod1 −/− mice of different ages showed that the older animals had drusen, thickened Bruch’s membrane, and choroidal neovascularization. The number of drusen increased with age, and exposure of young Sod1 −/− mice to excess light induced drusen. The retinal pigment epithelial cells of Sod1 −/− mice showed oxidative damage, and their β-catenin-mediated cellular integrity was disrupted, suggesting that oxidative stress may affect the junctional proteins necessary for the barrier integrity of the retinal pigment epithelium. These observations strongly suggest that oxidative stress may play a causative role in age-related retinal degeneration, and our findings provide evidence for the free radical theory of aging. In addition, these results demonstrate that the Sod1 −/− mouse is a valuable animal model to study human age-related macular degeneration.

Infrared (IR) irradiation is frequently used in ophthalmological diagnosis and treatment. It has been used to selectively stimulate photodiode-based retinal prostheses to prove their function. Data concerning the natural IR-sensitivity of the retina are contradictory. In our experiments in dark-adapted cats an IR-laser (826 nm) and IR emitting diodes (875 nm) elicited clear scotopic threshold responses. Comparison of the two lasers (IR and a visible laser at 670 nm) using Lambs template and our experimental data revealed very similar differences in retinal sensitivity (4.28 and 3.94 ± 0.29 log units, respectively). The fact that the cat retina is sensitive to IR-irradiation under certain conditions has important implications in interpreting the results from retinal prostheses and rewards further attention in its use in many ophthalmological applications.

To report the incidence of posterior vitreous detachments (PVDs) and the surgical results of vitrectomy with intravitreal triamcinolone acetonide (TA) to detect vitreomacular adhesions in eyes with asteroid hyalosis (AH). Ten eyes of nine patients with AH underwent vitrectomy, six eyes with TA and four without TA. The presence of a PVD was determined preoperatively by ultrasound echography (USE) and intraoperatively by microscopic observations. The postoperative best-corrected visual acuities (BCVA) were evaluated. The BCVA was improved by >2 Snellen lines in nine eyes and maintained at 20/20 with symptomatic improvements in the other eye. A vitreomacular adhesion was clearly seen during TA-assisted vitrectomy, and none was seen when TA was not used, even though preoperative USE showed an incomplete PVD in all eyes. The BCVA was not significantly better in eyes with TA-assisted vitrectomy than without TA-assisted vitrectomy. In one eye with vitrectomy without TA, a second surgery was required for a persistent cystoid macular edema and an epiretinal membrane. The BCVA and the edema in this eye improved after removing the epiretinal membrane. All (ten) of the eyes with AH were found to have a vitreomacular adhesion by preoperative USE and intraoperative microscopic observations. The residual vitreous over the macula is more easily detected and removed after intravitreally injected TA, but the visual acuities were not significantly different from eyes without TA.

To investigate the efficacy of intraocular lens (IOL) implantation in reducing the incidence of postoperative retinal detachment after cataract surgery in patients with atopic dermatitis.Retrospective review.One hundred sixty-nine eyes of 126 patients who underwent cataract surgery for atopic cataract were followed for more than 1 year. None of the eyes previously had a retinal detachment or retinal detachment surgery.The eyes were divided into 132 eyes of 95 patients with an IOL implantation (IOL group) and 37 eyes of 31 patients without an IOL implantation (aphakia group). The postoperative visual acuity and incidence of postoperative retinal detachment were compared between the 2 groups. The effects of the location of the causative retinal breaks, intraoperative posterior capsule rupture, and postoperative posterior capsulotomy on the incidence of retinal detachments were evaluated.The postoperative corrected visual acuity, incidence of postoperative retinal detachment, and influence of intraoperative posterior capsule rupture on the retinal detachment.The final visual acuity was better than or equal to 20/20 in 128 eyes (97.0%) of the IOL group and in 29 eyes (78.4%) of the aphakia group (P = 0.0007). Retinal detachment after an uncomplicated cataract surgery occurred in 3 eyes (2.3%) of the IOL group and in 8 eyes (25.8%) of the aphakia group (P0.0001, Mantel-Cox). Two of 3 eyes (66.7%) in the IOL group and 1 of 8 eyes (16.7%) in the aphakia group that later developed a retinal detachment had an intraoperative posterior capsule rupture. Posterior capsulotomy by yttrium-aluminum-garnet laser did not seem to alter the incidence of postoperative retinal detachment in either the IOL (2.0%) or the aphakia group (25.0%).The rate of postoperative retinal detachment in eyes with IOL and no intraoperative posterior capsule rupture seems to be low. Intraocular lens implantation with capsular bag fixation may reduce the incidence of postoperative retinal detachment triggered by lens surgery for atopic cataract.

To investigate retinal microcirculation changes in patients with rhegmatogenous retinal detachment (RRD).The tissue blood flow in the macular area was measured in 28 patients with RRD without macular involvement by scanning laser Doppler flowmetry before and after scleral buckling procedures. The mean blood flow (MBF) was calculated by the automatic full-field analysis program. The MBF ratios of the affected eye to the fellow eye (a/f ratio) in patients were compared with those of the right eye to the left eye (R/L ratio) in the control subjects.The mean preoperative a/f ratio in the patients (0.81 +/- 0.11) was lower than the mean R/L ratio in the control subjects (1.02 +/- 0.11, P0.0001) and correlated with the extent of RRD (P0.05). The mean a/f ratio tended to decrease 2 weeks after surgery (0.72 +/- 0.09) and recovered to an almost normal level after 1 month (0.96 +/- 0.09). The blood-flow change was not influenced by the type of buckling.The retinal microcirculation in the macular area was disturbed in RRD patients without macular involvement. It correlated with the extent of the RRD, and subsided 1 month after successful scleral buckling procedures.

Following multiple promising investigations into restoration of vision in degenerative retinal disease by implantation of a sub- or epiretinal prosthesis, the step to clinical use in humans is impending. In this study we intended to establish optical coherence tomography (OCT) and fluorescein angiography (FA) first in research animals for noninvasive assessment of the condition of the posterior pole of eyes after intraocular implant surgery. Three adult cats that had undergone subretinal implant surgery were evaluated by OCT and FA between 1 and 470 days postoperatively. Eight adult cats served as control. In addition histology was performed. In all three cats OCT demonstrated stable positioning of the implants in the subretinal space during the complete examination period. Transient retinal edema was found in the early postoperative period but decreased during follow-up. The retina over the implants was well attached at all times in cats 1 and 2; however, in cat 3 localized retinal detachment was demonstrated. FA showed intact retinal vasculature over the subretinal implant in high detail without interference from choroidal background fluorescence. OCT and FA have been fruitfully applied to cats to assess the morphological and circulatory conditions of the neuroretina and of its interface with the subretinal implant. The techniques may therefore provide a tool for objective, noninvasive in vivo evaluation of eyes that have undergone subretinal implant surgery, both in research animals and in humans.

PURPOSE Retinal vascular abnormalities are rare in patients with Duchenne muscular dystrophy. We present a patient with Duchenne muscular dystrophy who developed severe proliferative retinopathy for which vitrectomy was successfully performed in one eye. METHOD Case presentation. A 23-year-old Japanese man with Duchenne muscular dystrophy complicated by cardiac and respiratory insufficiency had reduced vision in both eyes. His best-corrected visual acuity was 0.01 in the right eye and hand movements in the left eye. Ophthalmoscopy showed vitreous hemorrhage and proliferative tissue attached to the optic disk bilaterally. Ultrasound echography showed tractional retinal detachment in the left eye. RESULTS Because general anesthesia was considered to be a high risk, vitrectomy, lensectomy, neovascular membrane removal, endolaser photocoagulation, and silicone oil injection were performed under local anesthesia on the right eye. After removal of the silicone oil and intraocular lens implantation, the best-corrected visual acuity was 0.8 in the right eye. Vitrectomy was performed on the left eye, but the retina could not be attached. CONCLUSION The etiology of the proliferative retinopathy in our case is not known. Because early treatment has the potential to improve and maintain vision, we recommend periodic fundus examinations in patients with Duchenne muscular dystrophy.

Purpose: To present two clinical cases diagnosed with predominant cone dystrophy and demonstrating early disturbance in the on-centre bipolar cells (ONpathway). Methods: Electrophysiological findings are presented in two siblings with predominant cone dystrophy. The subjects showed no remarkable ophthalmoscopic or fluorescein angiographic retinal changes, but demonstrated progressive visual disturbance during their 20s. Results: The electroretinograms (ERGs) showed reduced dark-adapted responses but the positive component of the photopic ERG was absent. Response to 30 Hz flicker was severely reduced. Electroretinograms elicited by long-duration stimuli showed a loss of the b-wave, and the off-response was slightly reduced. In both patients, multifocal ERGs (m-ERGs) were more reduced within the central 10ae, where the ON-pathway is normally a major contributor. Conclusion: We conclude that these patients may be affected by an abnormality of the synapses of the cone receptors and that their decrease in vision might, at least initially, be due to selective ON-pathway dysfunction.

PURPOSE Valsalva hemorrhagic retinopathy is characterized by retinal hemorrhages that develop after a Valsalva maneuver that consists of a forcible exhalation against a closed glottis, thereby creating a sudden increase in the intrathoracic or intraabdominal pressure. We present a patient who developed retinal and vitreous hemorrhages with multiple retinal nonperfused areas after extreme physical training with shouting on a mountainous area. This exercise was part of his training as a member of a Japanese traditional cheerleading team. METHOD Case presentation. RESULTS A 19-year-old man developed an acute decrease in the vision to 0.3 in his left eye after running on hilly roads at approximately 800 m while shouting fight songs for several hours. Ophthalmoscopy showed a fresh vitreous hemorrhage that covered the entire macula and was connected to the optic disk in the left eye. The vitreous hemorrhage spontaneously resolved and an intraretinal hemorrhage and nonperfused area emerged. His visual acuity returned to 1.2. CONCLUSION Prolonged, strenuous physical exertion with shouting during training exercises can lead to Valsalva hemorrhagic retinopathy. Several other factors, such as hot weather, altitude, and dehydration, may have played an additive role in increasing the venous pressure and hypoxia in the retinal vessels, which then caused the retinopathy.

To analyze the interaction of cortical potentials elicited by dichoptic stimulation of the dominant and fellow eyes at different frequencies, a pair of programmed power supply units were used to drive a light emitting diode (LED) mounted in the right and left eyes of light-proof goggles to elicit the visually evoked cortical responses (VECPs). The right eye was stimulated at 11.5 Hz and the left eye at 11.0 Hz. Then the stimulation was repeated with the frequency of stimulation switched to the other eyes. The stimulus duration was 5 ms. The sampling rate was 1.0 Hz, and the duration of collection was 200 ms. The VECP of each eye was extracted separately. Individual VECPs could be recorded separately after simultaneous dichoptic stimulation of each eye. The amplitudes of the VECPs were not significantly different after stimulating the dominant eye and the fellow eye separately. The implicit times of negative peak (N-2) and the second positive peak (P-2) were shorter after stimulation of the dominant eye than after stimulation of the fellow eye, but the difference was not significant. However, the implicit time of N-2 elicited by stimulating the dominant eye was significantly shorter when the stimulation rate was 11.5 Hz. The VECPs elicited by stimulating the two eyes can be recorded separately by simultaneous dichoptic stimulation. Dichoptic simultaneous stimulation required a shorter time and may be a more sensitive method of analyzing binocular interactions compared to the classic VECPs using monocular stimulation.

To evaluate the images created in a model eye during simulated cataract surgery.This study was conducted as a laboratory investigation and interventional case series. An artificial opaque lens, a clear intraocular lens (IOL), or an irrigation/aspiration (I/A) tip was inserted into the 'anterior chamber' of a model eye with the frosted posterior surface corresponding to the retina. Video images were recorded of the posterior surface of the model eye from the rear during simulated cataract surgery. The video clips were shown to 20 patients before cataract surgery, and the similarity of their visual perceptions to these images was evaluated postoperatively.The images of the moving lens fragments and I/A tip and the insertion of the IOL were seen from the rear. The image through the opaque lens and the IOL without moving objects was the light of the surgical microscope from the rear. However, when the microscope light was turned off after IOL insertion, the images of the microscope and operating room were observed by the room illumination from the rear. Seventy percent of the patients answered that the visual perceptions of moving lens fragments were similar to the video clips and 55% reported similarity with the IOL insertion. Eighty percent of the patients recommended that patients watch the video clip before their scheduled cataract surgery.The patients' visual perceptions during cataract surgery can be reproduced in the model eye. Watching the video images preoperatively may help relax the patients during surgery.

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.

Purpose: To determine the degree of metamorphopsia before and 1 year after half-dose verteporfin photodynamic therapy in eyes with chronic central serous chorioretinopathy. Methods: This was a retrospective, noncomparative, interventional case series. Forty-five eyes of 45 consecutive patients with chronic central serous chorioretinopathy were evaluated. The degree of metamorphopsia was measured with M-CHARTS before and at 1, 3, 6, 9, and 12 months after half-dose verteporfin photodynamic therapy. The best-corrected visual acuity was also measured. Results: Forty of the 45 eyes had a complete resolution of the serous retinal detachment at 1 month, 1 eye at 3 months, and 3 eyes at 6 months. The serous retinal detachment in one eye persisted throughout the follow-up period. The mean horizontal metamorphopsia score improved significantly from 0.61 ± 0.52° at baseline to 0.49 ± 0.56° at 12 months (P = 0.04). The vertical metamorphopsia score improved significantly from 0.52 ± 0.53° at baseline to 0.33 ± 0.46° at 12 months (P = 0.005). Conclusion: Half-dose verteporfin photodynamic therapy for chronic central serous chorioretinopathy results in significant improvements of metamorphopsia at 1 year, especially in eyes with good best-corrected visual acuity at the baseline. Half-dose verteporfin photodynamic therapy can be a therapeutic option for patients with good visual acuity who complain of metamorphopsia.

To examine the physiologic condition of the middle retinal layer of patients with X-linked juvenile retinoschisis (xlRS) by studying the on- and off-responses of the photopic electroretinograms (ERGs).Eleven unrelated Japanese men (mean age; 24.9 +/- 7.6 years) who were clinically diagnosed with xlRS and molecularly confirmed as having XLRS1 mutations were investigated. For the photopic ERGs, the a-, b- and d-wave amplitudes elicited by long duration stimuli were recorded, and the responses from the xlRS patients were compared to those recorded from normal subjects (n = 14, mean age, 27.5 +/- 4.5 years). We also examined the relationship between the photopic ERG responses and the genotype.No significant difference was found between the a- and d-wave amplitudes in the xlRS patients (34.2 +/- 8.7 microV, 52.5 +/- 10.4 microV, respectively), and those in normal subjects (40.4 +/- 10.3 microV, 44.7 +/- 6.3 microV, respectively). The mean b-wave amplitude in the xlRS patients was significantly smaller (10.5 +/- 7.7 microV) than the mean of normal subjects (46.4 +/- 10.2 microV) (P0.0001). No significant correlation was found between the ERG responses and the locus of the mutation.The photopic ERG demonstrated considerable impairment of the on-pathway arising from an abnormality of the on-bipolar cells or possibly secondary to Müller cell abnormality in xlRS.

Progressive outer retinal necrosis syndrome (PORN) is a variant of necrotizing herpetic retinopathy and the majority of the described cases were related to acquired immunodeficiency syndrome. We present a patient who is HIV negative with nephrotic syndrome and prednisolone use for 4 months who showed clinical features of PORN. Low CD4 counts and lymphocytopenia suggested immunosuppression. In the left eye, tractional retinal detachment at the posterior pole followed by incomplete posterior vitreous detachment developed. In addition to intravenous administration of acyclovir, vitreous surgeries including stripping of the posterior hyaloid and silicone-oil tamponade were successfully performed to repair the retinal detachment in the left eye and to prevent it in the right eye. [Ophthalmic Surg Lasers 2001;32:67-72]

Objective: To evaluate pathologic features of vitreomacular traction syndrome (VMT). Methods: Preretinal membranes removed from five patients during vitreous surgery for VMT syndrome were evaluated by electron microscopy (n = 4) and immunohistochemistry (n = 1). Results: Electron microscopic examination revealed large segments of internal limiting lamina (ILL) in three of the four cases. Other extracellular features included two types of abnormal collagen fibrils, determined to be type I and fibrous long-spacing collagen. The myofibrocyte was the predominant cell type in all cases. Collagen types I, II. and III, as well as glial fibrillary acidic protein and vimentin, were identified by immunohistochemistry. Conclusions: Epiretinal membranes in eyes with VMT syndrome adhered tightly to the ILL with abnormal collagen and contractile elements that included myofibrocytes.

To characterize the clinical features associated with XLRS1 gene mutations in Japanese patients with X-linked juvenile retinoschisis (xlRS), we evaluated the following data on 14 Japanese males from 13 unrelated families with XLRS1 mutations: age and symptoms at first visit to an ophthalmologist and ophthalmologic findings including visual acuity, refractive errors, fundoscopic appearance, and results of electroretinography (ERG) and electro-oculography (EOG). Each clinical finding was reviewed when the patients were between six and eight years of age. The best-corrected visual acuity in 12 patients (24 eyes) between the ages 6 and 8 years ranged from 1.0 to no light perception. Macular abnormalities were present in all cases. Peripheral retinoschisis was present in 14 of 26 eyes (53.8%). In the 21 eyes for which a single-flash ERG had been recorded, b-wave amplitude was reduced in 17 eyes. The EOG showed a low Arden ratio in three of the 13 eyes in the seven patients evaluated. No clear relationship was observed between the clinical features and the existing mutations. Three of four patients with a visual acuity less than 0.1 had retinal detachment or severe macular lesion that had occurred before the age of four years. Two patients harbored deletions of exon 1 or of the boundary region between exon 3 and intron 3, and one patient harbored R182C in exon 6. The present study shows a heterogeneity of mutations in the XLRS1 gene and phenotypic variations in 14 Japanese patients with xlRS.

To investigate clinical factors related to the aqueous humor levels of vascular endothelial growth factor (VEGF) and hepatocyte growth factor (HGF) in patients with proliferative diabetic retinopathy (PDR).Undiluted aqueous humor was obtained during ocular surgery from 46 eyes of 46 patients and the levels of growth factors were measured using enzyme-linked immunosorbent assays. VEGF and HGF levels were compared with the number of photocoagulation. VEGF and HGF levels in patient groups stratified according to the existence of vitreous hemorrhage, retinal detachment, and fibrovascular membrane were analyzed. And clinical parameters associated with the growth factors were determined by multiple regression analysis.The levels of VEGF decreased significantly as the extent of photocoagulation increased and showed significant positive correlation with the existence of vitreous hemorrhage. There was no significant correlation between VEGF levels and the existence of fibrovascular membrane and traction retinal detachment. The levels of HGF also decreased significantly as the extent of photocoagulation increased, but increased significantly when fibrovascular membrane existed. There were no significant correlations between HGF levels and the existence of vitreous hemorrhage and traction retinal detachment. Concerning systemic conditions, each growth factor has no significant correlation with duration and type of diabetes mellitus, the treatment regimen, the control of hemoglobin A1C, and the existence of hypertension or renal dysfunction.The therapeutic effect of panretinal photocoagulation on PDR might be partly exerted by reduction of the levels of VEGF and HGF in ocular fluid. Since the clinical parameters associated with VEGF were different from those associated with HGF, these growth factors might influence the progression of retinopathy in different ways.

7-year-old boy presented withdecreased vision and conjuncti-vitis in both eyes. He had a flu-likeepisode with fever about 2 weeks ear-lier, and the skin of both palms hadpeeled off. His visual acuity (VA) was0.15 OD and 0.1 OS. Slit-lamp exami-nation showed conjunctival and ciliaryinjection, cells in the anterior cham-ber, flare and fine keratic precipitatesbilaterally. Ophthalmoscopy revealeddiffuse, white perivascular sheathingof the arteries and veins, tortuous anddilatated veins, scattered retinal haem-orrhages and papilloedema in botheyes (Fig. 1A). Fluorescein angiogra-phy demonstrated extensive dye leak-age from almost all vessels (Fig. 1B)and both optic discs were hyperfluo-rescent in the late phase. Indocyaninegreen angiography (ICGA) showed amottled background pattern, stainingof the vessels especially in the poster-ior pole, and hypofluorescence of thediscs with blurred margins (Fig. 1C).Optical coherence tomography (OCTModel 2000; Carl Zeiss Meditec, Inc.,Dublin, CA, USA) showed a clear ser-ous detachment and oedema in themacula (Fig. 1D, E). Goldmann peri-metry showed an enlarged blind spot.The amplitudes of the a- and b-wavesand the oscillatory potentials of theelectroretinograms (ERGs) elicited bya bright flash were significantlyreduced (Fig. 1F).Systemic examinations disclosed noabnormal findings except for high titresof anti-streptolysin O (ASO, 1830·)and anti-streptokinase (ASK, 5120·).From these findings, the subject wasdiagnosed with frosted branch angiitis(FBA). He was treated with topical0.1% betamethasone and 1.0% atro-pine along with i.v. prednisolone(580 mg⁄day) for 3 days and subse-quently oral prednisolone for 15 days(total 165 mg). Three days later, theaqueous inflammation and the peri-vascular sheathing were markedlyreduced. Thereafter, OCT and oph-thalmoscopy of the macular areashowed a resolution of the oedemafollowed by the appearance of a star-shaped figure (Fig. 1G, H). The sub-ject’s ERGs were markedly improvedbut were still not normal (Fig. 1I).The serous detachment (Fig. 1J, K)and finally exudates disappeared.Despite the rapid improvement inthe appearance of the retina, thepatient’s VA improved much moreslowly and attained 1.0 only after3 months. Fundus angiography at thattime demonstrated the complete reso-lution of all abnormal findings.The increase in the number of casesdiagnosed as FBA raises questionsabout whether this disorder representsa distinct clinical syndrome or merelya clinical sign of eyes with inflamma-tory conditions. From their descrip-tions in the literature, cases diagnosedas FBA are characterized by bilat-eral, perivascular retinal blood vesselsheathing, retinal haemorrhages andoedema of different degrees, panuveitisand papilloedema (Walker et al. 2004).Our case had diffuse angiitis involvingthe vessels of the macular and periph-eral retina and the choroid. Anterioruveitis and papilloedema were alsopresent. The abnormal ICGA findingssuggested that the choroid was alsoaltered, which probably accounts forthe decreased a-waves on ERG. Thereduced b-waves indicated alterationsto the inner retina. Whether thesefindings are also found in other chori-oretinal inflammatory diseases is yet tobe determined. The prevalence of FBAis reported to be higher in Asians thanin White populations (Walker et al.2004).Despite the marked panophthalmicinflammation, the eye responded wellto steroids. Our subject’s VA recov-ered to normal levels, but some FBAsubjects suffer a permanent loss ofvision caused by macular scarring(Kleiner et al. 1988; Walker et al.2004).

To report the changes in the optical coherence tomography (OCT) images of the outer retinal layers after prolonged dark adaptation in a patient with Oguchi disease. A 75-year-old woman showed the typical golden-yellow fundus reflex of Oguchi disease, and the coloration returned to normal after prolonged dark adaptation. Fourier domain OCT (FD-OCT) was performed on the patient’s left eye before and after prolonged dark adaptation. Before dark adaptation, the FD-OCT images at the fovea had three identifiable reflection bands, namely, the external limiting membrane (ELM), the border between the photoreceptor inner and outer segments (IS/OS line), and the retinal pigment epithelium (RPE)/Bruch band. The paramacular area had only the ELM and RPE/Bruch bands. After 4 h of dark adaptation, the IS/OS line was also detected in the paramacular area. The absence of the IS/OS line in the extramacular regions in the partly dark adapted condition was most likely due to a defect in the rod photoreceptors of this area. The emergence of the IS/OS line after prolonged dark adaptation suggests that microarchitectural changes occur in the photoreceptors and that the changes may be correlated with the improvement of rod function.

AIMS—To determine the relation between the stages of diabetic retinopathy (DR) and the levels of hepatocyte growth factor (HGF) and vascular endothelial growth factor (VEGF) in aqueous fluid and serum. METHODS—Levels of HGF and VEGF in serum and aqueous humour obtained during ocular surgery were measured by enzyme linked immunosorbent assay in 58 diabetic patients with 32 non-diabetic patients (NDM) as controls. The patients with diabetes were classified into three groups according to the stage of DR: no DR (NDR; 15 cases), non-proliferative DR (NPDR; six cases), and proliferative DR (PDR; 37 cases). RESULTS—No significant differences were found between any of the groups in serum concentrations of HGF or VEGF. The aqueous HGF levels increased with the stage of DR: NDM, median 397 pg/ml, range 133-930 pg/ml; NDR, 371 pg/ml, 142-1536 pg/ml; NPDR, 455 pg/ml, 162-1007 pg/ml; and PDR, 638 pg/ml, 187-2222 pg/ml. The aqueous VEGF levels in PDR (median 212 pg/ml, range 14-1216 pg/ml) were significantly higher than in NDM (105 pg/ml, 9-203 pg/ml), but aqueous HGF concentrations were unrelated to those of VEGF. CONCLUSION—The results of the present study suggest that both HGF and VEGF present in the ocular tissues may play important roles in the progression of DR.

X-linked juvenile retinoschisis is a form of vitreoretinal dystrophy that is characterized by foveal and peripheral splitting of the retinal nerve fiber layer. Pathognomonic of this disorder is a microcystic radiate appearance in the fovea. We encountered a 10 year-old, mildly retarded, Japanese boy, who exhibited a widely extended macular retinoschisis bilaterally. A break in the inner layer of the left eye mimicked a lamellar macular hole, which is a rare manifestation of the disease. Peripheral retinoschisis was absent. Only a few reports have described marked bilateral macular retinoschisis that involved entire posterior pole, while various other macular findings have been reported. This patient with a severe form of retinoschisis was found to harbor the deletion of 33 base pairs, including the boundary region of exon 3 and intron 3 in the XLRS1 gene.