谷川 篤宏

Inherited retinal diseases (IRDs) comprise a phenotypically and genetically heterogeneous group of ocular disorders that cause visual loss via progressive retinal degeneration. Here, we report the genetic characterization of 1210 IRD pedigrees enrolled through the Japan Eye Genetic Consortium and analyzed by whole exome sequencing. The most common phenotype was retinitis pigmentosa (RP, 43%), followed by macular dystrophy/cone- or cone-rod dystrophy (MD/CORD, 13%). In total, 67 causal genes were identified in 37% (448/1210) of the pedigrees. The first and second most frequently mutated genes were EYS and RP1, associated primarily with autosomal recessive (ar) RP, and RP and arMD/CORD, respectively. Examinations of variant frequency in total and by phenotype showed high accountability of a frequent EYS missense variant (c.2528G>A). In addition to the two known EYS founder mutations (c.4957dupA and c.8805C>G) of arRP, we observed a frequent RP1 variant (c.5797C>T) in patients with arMD/CORD.

PURPOSE: To investigate the efficacy of modified internal limiting membrane (ILM) flap technique combined with vitreous surgery for treating macular holes and examine the outcomes in visual function and anatomic macular hole closure. STUDY DESIGN: Retrospective, observational. METHODS: Between July 1, 2015, and October 1, 2019, the modified inverted ILM flap technique combined with vitreous surgery was used to treat idiopathic macular holes, postoperative progression was then followed for at least 6 months in 96 participants (98 eyes). We modified the method by removing the lower half of the ILM while peeling and inverting the upper half. The mean age of the participants was 65.9 ± 11.9 years (41 men (42.7%) and 55 women (57.3%)). Retrospective evaluations of macular hole diameter, corrected visual acuity, and macular hole closure rates were performed using data from medical records. RESULTS: The mean macular hole diameter was 623.6 ± 207.4 μm. The mean corrected visual acuity (logMAR) was 0.79 ± 0.27 before surgery and 0.46 ± 0.35 at 1, 0.35 ± 0.39 at 3, and 0.31 ± 0.36 at 6 months Post surgery, showing significant differences before and after surgery (p = 2.30 × 10- 2). The macular hole closure rate was 98%. CONCLUSION: The modified inverted ILM flap technique combined with vitreous surgery was an effective method for treating macular holes, resulting in improvement in closure and visual acuity.

OBJECTIVES: The purpose of this study was to examine the incidence of, and risk factors for, epiretinal membrane (ERM) surgery after an initial pars plana vitrectomy (PPV) for rhegmatogenous retinal detachment (RRD). METHODS: The records of consecutive patients (3,495 eyes of 3,387 patients) who underwent RRD repair at Fujita Health University Hospital between January 1, 2008, and February 28, 2019, were retrospectively reviewed. A total of 1,736 eyes without an ERM in preoperative optical coherence tomography were included in this study. RESULTS: The incidence of ERM surgery after RRD repair was 2.4%. The mean time from RRD repair to ERM surgery was 19.5±27.2 months. The odds ratios after adjusting for age and sex were as follows: the preoperative visual acuity (logarithm of the minimum angle of resolution, logMAR), 2.17 (p=0.02; 95% confidence interval [CI], 1.11-5.16); axial length, 1.38 (p=0.002; 95% CI, 1.12-1.72); 20-gauge vitreous surgery instruments, 3.82 (p<0.0001; 95% CI, 2.02-7.16); internal limiting membrane (ILM) peeling, 0.28 (p=0.033; 95% CI, 0.05-0.92). ERM surgery improved visual acuity from 0.36 to 0.01 logMAR, even at ≥1.5 years after RRD repair. CONCLUSIONS: Careful follow-up is required in the following cases: long axial length before RRD repair, low visual acuity, use of 20-gauge vitreous surgery instruments, and a lack of ILM peeling.

We report a case of Fabry disease diagnosed after recurrent cerebral infarction in a patient with central retinal artery occlusion (CRAO). A 23-year-old man presented with vision loss in his right eye (20/2000), showing CRAO. There was no identified cause for the loss of vision; however, corneal verticillata was detected in both eyes on the recurrence of the cerebral infarction. The α-galactosidase activity in leukocytes was significantly reduced to <0.3 nmol/mg of protein/hour, leading to a definitive diagnosis of Fabry disease. Enzyme replacement therapy was commenced concomitant to rehabilitation. It is necessary to identify Fabry disease as a cause of CRAO in young individuals, and the detection of cornea verticillata, used frequently as an ocular finding, is helpful.

PURPOSE: We report a case of neuroretinitis associated with cat scratch disease (CSD) in young children. METHOD: Case report. RESULTS: A 16-month-old boy was admitted for a detailed examination and treatment of a fever of unknown origin. Blood tests revealed no significant findings other than a white blood cell count of 16,100/mm3 and C-reactive protein level of 9.89 mg/dL. Computed tomography revealed no relevant findings to determine the causative disease. Antibiotic therapy with cefotaxime was initiated; however, the fever did not resolve. The patient was referred to our department for further examination to detect the cause of the fever. Fundoscopy revealed neuroretinitis in the right eye. His mother reported a history of breeding cats. Cat scratch disease (CSD) was suspected based on the clinical course and fundus findings. Cefotaxime was discontinued, and azithromycin, rifampicin, and prednisolone were administered, following which the fever disappeared and fundus findings improved. Immunoglobulin G (IgG) and IgM antibodies against Bartonella henselae was positive, leading to a definitive diagnosis of CSD. CONCLUSION: Infants cannot complain of decreased visual acuity; therefore, these findings may be overlooked unless a fundus examination is performed. As in this case, the early detection of neuroretinitis by an ophthalmologist may help in the diagnosis of CSD. It is extremely difficult to capture a photograph of the fundus of an infant, and recording with a smartphone is relatively simple and useful for monitoring continuous changes. Summary. We describe a case of neuroretinitis associated with cat scratch disease (CSD) that was diagnosed on the basis of fundus findings. The findings suggest the importance of an aggressive ophthalmologic examination when CSD is suspected in young children who are unable to describe their symptoms.

OBJECTIVES: We compared the effects of sub-Tenon's capsule anesthesia (STA) and trans-Tenon's capsule retrobulbar anesthesia (TTRBA) in 68 patients with epiretinal membrane. METHODS: Either STA or TTRBA was induced with 3 mL of lidocaine (2%) before vitrectomy combined with phacoemulsification and aspiration (phacovitrectomy). Akinesia was evaluated by range of eye movement (ROEM) in upward, downward, nasal, and temporal directions at 4, 10, and 30 minutes after injection. Analgesia was evaluated with a visual analogue pain score, which ranged from 0 to 10. RESULTS: The mean cumulative ROEMs were 1.44±1.02 corneal diameters (CDs) at 4 minutes, 0.55±0.76 CDs at 10 minutes, and 0.26±0.33 CDs at 30 minutes in patients who received STA; these values were 0.39±0.35 CDs at 4 minutes, 0.22±0.30 CDs at 10 minutes, and 0.13±0.29 CDs at 30 minutes in patients who received TTRBA. At both 4 and 10 minutes, the cumulative ROEMs in all directions, as well as the temporal ROEMs, were significantly larger in patients who received STA than in patients who received TTRBA. Pain scores did not significantly differ between groups at any time point. CONCLUSIONS: STA and TTRBA produced identical degrees of analgesia, but akinesia was slower in patients who received STA. TTRBA might be preferable for patients undergoing brief vitrectomy.

INTRODUCTION: The ability to reduce illumination levels is generally accepted as one of the main benefits of a three-dimensional heads-up system (3D system: Ngenuity®; Alcon, CA, USA). Some studies have focused on illumination reduction in vitreoretinal procedures; however, information regarding illumination reduction in cataract surgery has not been published. PURPOSE: This study aimed to compare the illumination of the operational field with a 3D system and a standard microscope eyepiece during cataract surgery. SUBJECT AND METHODS: We retrospectively evaluated 91 eyes of 84 consecutive patients who were undergoing cataract surgery at our hospital. We used the 3D system and the eyepiece on alternative days. We determined the minimum light intensity required for safe surgery using the foot switch of the microscope (OMS800; Topcon, Tokyo, Japan). Illuminance on the ocular surface and the minimum illuminance required for the operation were calculated from the minimum light intensity. RESULTS: The 3D system was used in 45 eyes (3D group), and the eyepiece was used in 46 eyes (eyepiece group). The values of minimum illuminance in the 3D group were significantly lower than those in the eyepiece group (3D: 5500 ± 2000 lux, eyepiece: 11,900 ± 1800 lux; p < 0.001*). In addition, the illuminance of the operational field was reduced by 60.4% on average using the 3D system. CONCLUSION: With real-time digital processing and automated brightness control, the 3D system reduced ocular surface illumination by 50% or more. Hence, the 3D system may contribute to reducing the risk of retinal phototoxicity and patient photophobia.

PURPOSE: To investigate the effect of mild cataract and its surgery on the ISCEV standard electroretinogram (ERG) by comparing pre- and postoperative ERGs elicited from fully dilated eyes. METHODS: Twenty-two patients participated. Each eye had cataract of grade 2 according to Emery-Little classification. None had complications during and after phacoemulsification and intraocular lens implantation. According to the ISCEV standard, pre- and 1-week postoperative full-field ERGs, dark adapted (DA) 0.01, DA 3, DA 3 oscillatory potentials (OPs), DA 10, and light adapted (LA) 3, and LA 3 flicker, were elicited from fully dilated eyes using skin electrodes. Photopic negative response (PhNR) 1 amplitude was measured from the baseline to the trough before the i-wave in the LA 3 ERG waveform. Statistical analysis was performed using SigmaPlot version 11.0 (Systat Software, Inc., San Jose, CA, USA). Pre- and postoperative data were compared using a paired t-test. Non-normally distributed data were evaluated using the Wilcoxon signed rank test. RESULTS: The pre- and 1-week postoperative amplitudes in each component were as follows: DA 0.01: 48.4 ± 19.5 μV and 57.1 ± 20.0 μV (p = 0.002), a-wave of DA3: 51.5 ± 14.8 μV and 58.3 ± 17.3 μV (p = 0.003), b-wave of DA3: 88.3 ± 27.5 μV and 101.5 ± 29.7 μV (p = 0.003), DA 3 ΣOPs (sum of DA 3 OP1, OP2, and OP3 amplitude): 30.7 ± 16.3 μV and 37.1 ± 21.9 μV (p < 0.001), a-wave of DA 10: 65.5 ± 18.8 μV and 74.2 ± 19.5 μV (p < 0.001), b-wave of DA 10: 95.5 ± 29.6 μV and 111.1 ± 29.9 μV (p < 0.001), a-wave of LA 3: 7.2 ± 2.6 μV and 8.2 ± 2.2 μV (p = 0.025), b-wave of LA 3: 30.6 ± 12.9 μV and 35.3 ± 12.7 μV (p = 0.003), PhNR1: 5.8 ± 2.5 μV and 5.5 ± 2.6 μV (p = 0.562), and LA 3 flicker: 25.4 ± 8.9 μV and 27.8 ± 8.6 μV (p = 0.039), respectively. The implicit time of all the components did not reveal significant differences before and after surgery. CONCLUSIONS: Cataractous lens removal and intraocular lens implantation may influence ISCEV standard ERGs, even if the cataract is relatively mild.

PURPOSE: This study reports a case of unilateral retinopathy with extinguished full-field ERGs (ffERGs), wherein the visual acuity was 16/16 and the visual field was spared. METHODS: Observational case report. RESULTS: A 39-year-old female had developed nyctalopia in her left eye. Two years later, she visited an ophthalmologist who noted a bilaterally reduced pigmentation of the fundus. Her best-corrected visual acuity was 16/16 in both the eyes. Goldmann perimetry demonstrated that her visual field was bilaterally fully spared. ffERGs measurement was performed in accordance with the ISCEV standard protocol and indicated that her right eye was normal. However, all ERG responses were severely attenuated in her left eye. Multifocal ERG responses were found to be normal in the right eye and extinguished in the left eye except for residual responses that were exclusively located at the center. During the 7 years of the follow-up period, the visual field in the left eye, which was once normal, became shaded, and the development of a ring scotoma was identified. The visual field in the right eye is still full. CONCLUSIONS: The pathogenesis of this patient's condition still remains unknown, while unilateral retinitis pigmentosa, unilateral pigmentary retinopathy, acute zonal occult outer retinopathy, and autoimmune retinopathy can all be considered as possible explanations. The uniqueness of this case study is that the extinguished ERG responses are predictive of the functional alteration in the affected eye, when the initial visual acuity and the visual field were normal.

OBJECTIVES: The aim of this study was to determine whether age correlates with amplitude and latency, when full-field electroretinography (ERG) is performed using skin electrodes. The ability of pulse reference power line noise reduction (PURE) to dampen the noise associated with the use of skin electrodes, was also investigated. METHODS: ERG was performed on 77 eyes in 77 healthy subjects (mean age: 55.6±19.0 years; age range: 9 to 86 years). Subjects with -5D or higher myopia, Emery-Little grade III or higher cataracts, retinal disease, uveitis, glaucoma, ≤5 mm mydriasis, or a history of intraocular surgery other than cataract surgery, were excluded. The active, reference, and ground electrodes were placed on the lower eyelid, outer canthus, and earlobe, respectively. Responses were averaged 10 times for dark-adapted (DA) ERGs, and 32 to 64 times for light-adapted (LA) ERGs. Noise was removed using the PURE method. RESULTS: The DA ERGs without PURE were so noisy that the amplitude or latency could not be determined, whereas those with PURE were comparatively quieter. ERG with PURE demonstrated a significant negative correlation between age and amplitude and a significant positive correlation between age and latency. CONCLUSIONS: We could record the measurable ERG waveforms with skin electrodes by using the PURE method, especially in fewer averaged conditions. It is suggested that skin electrode with PURE is suitable to examine the pathological ERGs, and other types of electrodes. It is recommended that the aging effect should be taken into consideration when pathological ERGs are evaluated.

PURPOSE: We report a novel technique for visualizing the posterior surface of the lens nucleus during phacoemulsification. SURGICAL TECHNIQUE: Hydro-dissection was performed using a solution of 20 mg triamcinolone acetonide powder without preservatives mixed with 3 ml BSS-plus, and triamcinolone acetonide was clearly identifiable underneath the posterior surface of the lens nucleus. Using a phaco-tip, the nucleus was shaved to the level of the triamcinolone acetonide and could be easily divided. The remnant triamcinolone acetonide was aspirated as much as possible from the lens cortex with an infusion/aspiration tip. SUBJECTS AND EVALUATION TECHNIQUE: Twenty-eight eyes in 28 patients with cataracts were enrolled in this study. Triamcinolone acetonide-assisted phacoemulsification was performed in 13 eyes in 13 patients (triamcinolone acetonide-phacoemulsification group), and normal phacoemulsification was performed in 15 eyes in 15 patients (phacoemulsification group). Intraocular pressure was measured in all patients pre-operatively, 1 day after, and 1 week after surgery. Corneal endothelial cell density was measured pre-operatively and 1 month after surgery. The time of surgical phacoemulsification (surgical phaco time) was measured from the video of the surgery. RESULTS: Surgery was successively performed in all eyes. Pre-operative and post-operative intraocular pressures and cell densities did not significantly differ between the two groups. Surgical phaco time was shorter in the triamcinolone acetonide-phacoemulsification group than in the phacoemulsification group (157.1 ± 51.7 s vs 225.3 ± 45.1 s; p = 0.006). CONCLUSION: The triamcinolone acetonide-assisted phacoemulsification procedure is safe and useful for visualizing the posterior surface of the lens nucleus and facilitates removal of the lens nucleus by phacoemulsification.

目的:新しく開発したアークスキャン式広画角超音波診断装置を用いて、健常眼の眼軸と赤道部径を計測して眼球の形状を検討した。対象および方法:対象は正常眼41眼。B-mode画像より、眼軸長axial length(AL)、赤道部水平径horizontal diameter(HD)、赤道部垂直径vertical diameter(VD)を定量した。結果:全体でALとHD、VDは正の相関を認めた。長眼軸群と非長眼軸群に分けて検討すると長眼軸群でAL、VD、HDが有意に大きかった。赤道部径/眼軸長は非長眼軸群が有意に大きかった。角膜屈折力平均値とHD、VDは負の相関を認めた。結論:広画角超音波診断装置は眼球赤道部径を測定することができ、眼軸延長に伴う眼球形態の変化を3次元的にとらえることができた。この装置は眼球形態を測定するデバイスとして臨床上有用と考えられた。(著者抄録)

＜文献概要＞目的:網膜剥離を合併した朝顔症候群の治療法は確立していない。筆者らは,片眼性または両眼性の本症3例4眼の病状と治療経過,転帰について今後の治療法選択の一助となるよう報告する。症例:症例1は14歳の女児(片眼性),症例2は43歳の女性(片眼性),症例3は20歳の男性(両眼性)で,症例3の左眼は前医で治療された後に眼球癆となった。所見:症例1の矯正視力は,右1.0,左0.01で,左眼に朝顔症候群と黄斑を含まない乳頭周囲に限局した網膜剥離を認めた。網膜裂孔は同定されなかったため,経過観察を行い,初診から5年後に網膜は自然復位した。最終受診時視力は眼前30cm手動弁だった。症例2の矯正視力は右光覚弁,左1.0で右眼に朝顔症候群と網膜が全剥離した増殖硝子体網膜症を認めた。硝子体手術中に見つかった耳側の網膜裂孔に対して液空気置換後に網膜光凝固を行いシリコーンオイルを留置した。網膜は復位したが,術後5ヵ月で視力0となった。症例3の矯正視力は右0.03,左0で右眼に朝顔症候群と耳側の網膜裂孔による網膜剥離を認めた。強膜内陥術が施行され,網膜は復位した。最終受診時視力は右0.03であった。結論:筆者らの経験した本症4眼中3眼は,経過観察や手術で網膜が復位した。原因裂孔が見つからない場合の手術選択は,自然復位の可能性もあるため,剥離範囲の拡大の有無や,片眼性か両眼性か,他眼の視力などを考慮しながら慎重に行うのがよいと思われる。

＜文献概要＞目的:網膜と水晶体前嚢の異常を示し,水晶体再建術により良好な視力を得たアルポート症候群の症例の報告。症例:27歳男性。両眼の視力低下で当院紹介となった。既往歴にアルポート症候群で腎移植歴があった。所見:矯正視力は右0.3,左0.5で,両眼に前部円錐水晶体があり,両眼黄斑耳側に白色斑の散在を認め,光干渉断層計では同部位の網膜内層菲薄化を認めた。黄斑微小視野計では同部位に感度低下を認めず,多局所網膜電図でも同部位に一致した振幅の減弱はなかった。当院にて両眼の水晶体再建術を施行した。採取した前嚢は菲薄化し,亀裂を無数に認めた。水晶体上皮細胞の空胞化と細胞内小器官の減少が観察された。術後矯正視力は両眼とも1.0と改善した。結語:網膜形態異常と前部円錐水晶体を伴うアルポート症候群を経験した。検査上,前部円錐水晶体による屈折異常を認めたが,網膜菲薄部位に一致する明らかな機能的異常は認めず,水晶体再建術により良好な視力を得られた。前部円錐水晶体は,水晶体嚢の脆弱化による構造異常のためと考えられた。

PURPOSE: We present a novel technique for measuring volume of space replaced by air during fluid-air exchange in vitrectomy (vitrectomized space) for intravitreal gas injection. To confirm the feasibility of this technique, we assessed postoperative intraocular pressure (IOP) and the duration of injected gas. Using this technique, we found remarkable differences in volume between high myopic eyes with retinal detachment caused by peripheral break (PB-RD group) and those with macular hole retinal detachment (MH-RD group). We studied the relationships between the volume and biometric values, axial length (AL) and corneal curvature radius (CCR) in both PB-RD and MH-RD group. STUDY DESIGN: Retrospective study. METHODS: During fluid-air exchange, the aspirated fluid accumulates in the measuring cup between the infusion needle and vitrectomy instrument. Vitrectomized space volume is obtained by subtracting the volume of the tube between the infusion needle and the 3-way stopcock from the aspirated fluid volume. We performed phaco-vitrectomies by measuring the vitrectomized space volume and then injected pure SF6 at 15% of the volume into the vitreous cavity in 156 myopic eyes (AL > 26 mm) with RD consisting of 144 eyes in PB-RD group and 12 in MH-group. RESULTS: The IOP (mean ± SD) was 13.5 ± 3.4 mmHg preoperatively, 23.4 ± 10.4 mmHg on day 1, 18.2 ± 7.4 mmHg on day 2, and 16.1 ± 4.5 on day 7. The gas disappeared in 16.1 ± 1.9 days. Axial length was longer in the MH-RD group but the volume of vitrectomized space was larger in the PB-RD group. AL and volume were significantly correlated in both groups (P < 0.01), but the fitting lines differed. The CCR and volume significantly correlated in the PB-RD group (P < 0.01) but not in the MH-RD group. CONCLUSION: Our novel technique for measuring the volume of vitrectomized space can achieve target gas concentration in the vitreous cavity. The difference in the shape of eyeballs may explain discrepancies in relationship between volume and the biometric factors.

Purpose: To determine adherence to face-down positioning (FDP) among patients who underwent vitrectomy and gas tamponade for macular hole (MH) repair. Method: A total of 69 patients (37 females and 32 males) who underwent primary vitrectomy for MH repair were studied. Nurses recorded whether the patient complied with FDP each time they examined the patient. FDP score was obtained from the nursing records patients were given a score of 1 if they complied with the FDP and 0 if they did not. The score was recorded four times per day for the first 3 postoperative days. A perfect FDP score was 12. Results: The mean ± standard deviation of the FDP scores was 10.6±1.8 (range: 4-12). Overall, 32 (46.4%) patients scored a perfect 12, and 7 (10.1%) patients scored ˂8. Failure of the MH closure was observed in only one patient (1.4%), who showed the poorest adherence to FDP (score =4). Consequently, the closure rate in patients with FDP score ˂7 (2/3, 66.7%) was significantly lower than in patients with an FDP score ≥7 (66/66, 100%) (P˂0.05, Fisher’s exact probability test). Conclusion: While adherence to FDP after MH surgery was better than that observed after vitrectomy for rhegmatogenous retinal detachments in our previous study, the percentage of patients with FDP scores, ˂8 did not differ. Statistically, the poor adherence to FDP can negatively impact the effectiveness of the surgery for MH repair.

Background: Iridocorneal endothelial (ICE) syndrome occurs mainly in young and middle-aged women and typically presents as a unilateral disease characterized by abnormalities of the iris and corneal endothelium. While the ICE syndrome is known to be associated with glaucoma and bullous keratopathy, to our knowledge, only two cases of ICE syndrome complicated with cystoid macular edema (CME) have been reported to date. In this paper, we report a case of ICE syndrome complicated with CME treated at our institution. Case presentation: The subject was a 51-year-old woman. In October 2013, she was examined by a primary care physician for blurred vision in her left eye. Dyscoria and abnormality of the corneal endothelium were observed, and the patient was diagnosed with ICE syndrome. In November of the same year, she was referred to our institution with a decrease in visual acuity and CME, both in her left eye. At initial examination, her best corrected decimal visual acuity was 1.0 (Snellen equivalent: 20/20) in the right eye and 0.5 (20/40) in the left eye. Intraocular pressure was 12 mmHg in both eyes. She was diagnosed with Cogan-Reese syndrome based on marked ectropion uveae, peripheral anterior synechia, and abnormalities of the corneal endothelium. Marked CME was observed on ophthalmoscopy and optical coherence tomography. A topical non-steroidal anti-inflammatory drug (nepafenac 0.1 %) was applied to the left eye four times daily from January 2014. Four weeks later, the CME had resolved and her visual acuity was 1.0 (20/20). Conclusion: While non-steroidal anti-inflammatory drugs and steroids did not appear to be effective in two previously reported cases of ICE syndrome complicated with CME, topical nepafenac was effective in this case. However, more such cases are needed before concluding that topical nepafenac is effective in this situation.

Purpose:To determine the compliance with the face-down positioning (FDP) of the patients who had undergone vitrectomy and gas tamponade.Methods:One hundred and twenty-seven patients, 45 females and 82 males, who had undergone primary vitrectomy and gas tamponade for rhegmatogenous retinal detachments were studied. The FDP score was determined as 4 per day for 3 days after the surgery. The patient was given a score of one if the nurse found that the patient was in the FDP and zero if not in the FDP. A perfect FDP score was 12.Results:The mean SD FDP score was 10.2 +/- 1.8 with a range of 3 to 12. Females (10.7 +/- 1.2) scored significantly higher than males (9.9 +/- 2.1) (Mann-Whitney U test: P &lt; 0.05). Patients who scored a perfect 12 were 38 (29.9%; female, 14:31.1%; male 24:29.3%). Low scorers &lt;8 were all males (12:14.6%) and no females (0:0.0%). Age did not seem to be related. Only 1 patient failed to achieve retinal attachment, although she had scored a perfect 12.Conclusion:The compliance was considerably varied among patients. Some patients failed nearly or more than half times, and they were mostly males. The significance of the FDP to treat rhegmatogenous retinal detachment needs to be examined in a further investigation.

The current case involved a 59-year-old woman who was referred to our hospital with bilateral optic nerve abnormality and gradually progressive bilateral inferior visual field defects. An ophthalmological examination revealed superficial optic nerve head drusen (ONHD) and bilateral inferior altitudinal hemianopsia, but no intracranial lesions. The results of an angiogram revealed no evidence of retinal vascular occlusion or anterior ischaemic optic neuropathy (AION). Prior reports suggested that drusen-induced hemianopsia was slowly progressive but that ONHD-induced AION caused acute altitudinal hemianopsia, which led us to the diagnosis of bilateral drusen-induced altitudinal hemianopsia.

Purpose: The b-wave of the human photopic electroretinogram (ERG) elicited by a short-flash increases in amplitude with increasing stimulus intensities at lower stimulus levels, but then decreases at higher stimulus intensities. The purpose of the present study was to explore this phenomenon in more detail, using short- and long-flash stimuli. Methods: The intensity-response functions of the b-wave elicited by short- and long-flashes were compared from threshold to higher stimulus intensities in 5 normal subjects. Short- and long-flash ERGs were elicited under rod-saturating background levels using white light-emitting diodes built into a contact lens electrode. Results: Whereas the amplitude of the short-flash b-wave decreased at higher intensities, the amplitude of the long-flash ERG b-wave did not decrease but plateaued. The long-flash ERG d-wave or OFF-response decreased at higher stimulus levels as did the short-flash elicited b-wave.Conclusions: Because it is widely accepted that the b-wave and the OFF-response d-wave interact to produce a single positive response, our results suggest that the decrease in the b-wave amplitude at high stimulus intensity is caused by the decrease of the d-wave at the higher stimulus intensities. Copyright (C) 2000 Japanese Ophthalmological Society.

Purpose. To determine using the multifocal ERG technique whether there are any regional differences in the increase in the amplitude of cone electroretinograms (ERGs) during light adaptation. Methods. Multifocal ERGs were recorded with the Visual Evoked Response Imaging System from five normal subjects. Thirty-seven hexagonal stimulus elements and a recording time of 60 seconds were used. After 20 minutes of dark adaptation, multifocal ERGs were repeatedly recorded every 2 minutes over a period of 16 minutes. The amplitudes of the multifocal ERGs at different eccentricities were compared during the 16 minutes of light adaptation. Results. During the 16 minutes of light adaptation, the summed responses of the multifocal ERGs increased in amplitude an average of 36% and 47% for the negative and positive components, respectively. The magnitude of increase was minimal in the central retina at 22% and was significantly larger in the peripheral retina at 58%. The implicit time was slightly increased (&lt 4%) with light adaptation, but there were no regional differences. Conclusions. The results demonstrated that there are topographic variations in the amplitude increase of cone ERGs during light adaptation. This topographic variation indicates that the mechanism for the increase must be based on known regional differences in the retina.

PURPOSE: To study the functional state of the retina beneath an epimacular membrane by means of focal macular electroretinography. METHODS: Focal macular electroretinograms of 30 consecutive patients (age 25 to 75 years) with unilateral idiopathic epimacular membrane were recorded prospectively. The amplitudes and implicit times of the a and b waves and the oscillatory potentials (O1, O2, O3) recorded from the eye with idiopathic epimacular membrane were compared with those of the normal fellow eye. RESULTS: There was a statistically significant reduction in the amplitude of the a wave (P &lt .001), the b wave (P &lt .001), and the oscillatory potentials (O1 + O2 + O3 P &lt .001) of the affected eyes. The reduction of the b-wave amplitude was significantly greater than that of the a wave, resulting in a lower b/a wave ratio (P = .003) in the affected eyes. The reduction in the amplitude of the oscillatory potentials was significantly greater than that of the other two components (P .001). The implicit times were significantly prolonged for the a wave (P &lt .001), the b wave (P = .004), and O3 (P &lt .001). There was a significant correlation between relative b-wave amplitude (affected/normal fellow eye) and the visual acuity (r = -0.50, P = .007). These findings were similar to those we reported for eyes with aphakic cystoid macular edema. CONCLUSIONS: The focal macular electroretinograms in eyes with epimacular membrane disclosed abnormalities that were similar to those of eyes with cystoid macular edema. This suggests that the epimacular membrane probably induced damage to the neurons in the inner retinal layers. Dysfunction of these neurons is one possible cause of visual impairment in this disease.

OBJECTIVE. To study the origin of tile different components of the electroretinogram (ERG) elicited by a random binary m-sequence stimulus. METHODS. Electroretinograms were recorded from pigmented rabbits before and after the injection of glutamate analogues (2-amino-4-phosphono-butyric acid [APB DL form] and cis-2,3-piperidine-dicarboxylic acid [PDA]) and inhibitory neurotransmitters (glycine and γ-aminobutyric acid [GABA]) to abolish the contribution of different cell types to the ERG. Two types of stimuli were used: conventional full-field stimulation with short- and long-duration flashes and a random binary m-sequence of flashes designed to mimic the pseudorandom binary m-sequence stimulation used in the multifocal ERG technique. RESULTS. The effects of APB and PDA on the first-order kernel of the random ERGs were similar to those on the photopic short-flash ERG. Glycine and GABA minimized the oscillatory potentials (OPs) of the photopic ERGs, and also reduced the amplitude of the positive wave of the first-order kernel slightly but caused a large reduction in the amplitude of the second- order kernel. CONCLUSIONS. The data suggest that the ON and OFF bipolar cells contribute significantly to the photopic short-flash ERG, as previously shown, and to the first-order kernel of the responses elicited by the pseudorandom binary sequence stimuli. The second-order kernel and the OPs receive a strong contribution from the cells of the inner retinal layers.

PURPOSE. To record the on and off responses of the multifocal photopic electroretinogram (ERG) from the human retina and to explore how each ERG component (a-, b-, and d-waves) changes at different retinal eccentricities. METHODS. Multifocal ERGs were recorded with the visual evoked response imaging system. Sixty-one densely packed stimulus elements were square wave- modulated between stimulus on and stimulus off, according to a binary m- sequence at a rate of 4.7 Hz under a constant background illumination. The ERGs were recorded with a bipolar Burian-Allen bipolar contact lens electrode from five normal subjects. Response densities (amplitude per retinal area) were calculated for five different eccentric rings. RESULTS. The response densities for all components (a-, b-, and d-waves) decreased with increasing retinal eccentricities. The ratio of the d-wave to b-wave amplitudes was minimal in the central retina and increased toward the periphery. The ratio of a-wave to b-wave amplitudes also increased toward the peripheral retina. CONCLUSIONS. These results demonstrate that the on and off responses of the human cone ERGs have different spatial distributions across the human retina, and they suggest a change in the photopic retinal circuitry with increasing retinal eccentricities.

PURPOSE: To report changes in focal macular electroretinograms before and after successful macular hole surgery and to discuss whether preoperative retinal function can predict postoperative visual acuity. METHODS: In this prospective study, 28 consecutive eyes of 28 patients underwent vitrectomy for stage 2 or 3 full-thickness idiopathic macular hole. Focal macular electroretinograms using a 4-degree stimulus spot were obtained in 24 eyes of 28 patients, and those using a 5-degree stimulus spot were obtained in 24 eyes of 28 patients, to evaluate preoperative and postoperative amplitude and implicit time of the b-wave. Visual acuity was measured preoperatively and postoperatively, and results were translated into logMAR (minimum angle of resolution) values. RESULTS: After successful macular hole closure, the b- wave amplitude increased in 23 of 24 eyes measured with the 4-degree stimulus spot and in 19 of 24 eyes measured with the 5-degree stimulus spot. Postoperative b-wave amplitude recorded with either stimulus spot correlated with postoperative corrected visual acuity. The b-wave implicit time decreased in 15 of 20 eyes measured with the 4-degree spot and in 13 of 23 eyes measured with the 5-degree spot. Preoperative b-wave implicit time measured with the 5-degree stimulus spot correlated significantly (P = .001) with postoperative corrected visual acuity. CONCLUSIONS: Qualitative change (implicit time) is more important than quantitative change (amplitude) in electroretinograms for predicting postoperative corrected visual acuity. The function of the retinal tissue surrounding the hole may affect visual outcome.

Purpose. To record multifocal electroretinograms (ERGs) with simultaneous fundus monitoring. Methods. An infrared television fundus camera was used to monitor the fundus. A tightly packed array of 19 yellow light-emitting diodes (LEDs) was used as the stimulus source and LEDs were altered between on and off according to a binary m-sequence at a rate of 75 Hz. The stimulus array subtended approximately 25°of the visual angle. Multifocal ERGs were recorded with a Burian-Allen bipolar contact lens electrode from two normal subjects and a patient with macular dystrophy. Results. When the center of the stimulus was positioned on the fovea, the amplitude of the response was largest at the fovea and decreased toward peripheral retina the response pattern corresponded to the spatial distribution of cone cells. When the center of the stimulus was positioned on the optic disc, the response at the optic disc was undetectable. A reliable multifocal ERG was obtained from a patient with macular dystrophy. Conclusions. This system made it possible to monitor the stimulus on the fundus and to adjust the stimulus to the correct retinal locations with exact focusing. The authors' findings indicate that this method especially can be useful in patients with poor fixation. In addition, the authors' results suggest that the stray light effect in the multifocal stimulus system is minimal if any.

Seven patients with Oguchi's disease were examined by conventional full-field rod and cone electroretinograms (ERGs), spectral characteristic studies with photopically matched short- and long-wavelength stimuli (3 of the 7 patients) and electrooculogram (EOG) (6 patients). ON and OFF photopic ERG responses, recorded with rectangular stimuli, in 3 patients were compared with those of 13 normal subjects and 13 patients with complete congenital stationary night blindness (CSNB). Conventional full-field ERGs recorded after 30 minutes of dark adaptation revealed absent rod, but essentially normal cone and flicker ERGs in all patients. With the dark-adapted standard flash ERG, the a-wave amplitude was significantly lower than normal in all patients. The spectral characteristic study indicated that the ERG recorded after 30 minutes of dark adaptation represented cone activity. The EOG was abnormal in all patients examined. The ON and OFF responses of the photopic ERG were normal in Oguchi's disease patients however the ON responses were reduced in patients with complete CSNB. The absence of a rod a-wave, with the abnormal EOG, strongly suggests that the rod itself is abnormal in Oguchi's disease. This finding differs from the report by Carr and Gouras and Carr and Ripps. The normal ON and OFF responses in the photopic ERGs of Oguchi's disease patients contrast with those of the complete CSNB patients in whom only the ON response is reduced.