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 report morning glory syndrome with retinal detachment in 4 eyes of 3 patients. Cases: The present series comprised 2 females and one male. They were aged 14, 43 and 20 years. Only the third case was bilaterally affected. Findings and Clinical Course: Corrected visual acuity was 1.0 right and 0.01 left in the first case. Morning glory syndrome and retinal detachment in the left eye were localized in the peripapillary area sparing the macula. Retinal detachment became spontaneously reattached with visual acuity of hand motion 5 years later. Visual acuity in the second case was light perception right and 1.0 in the left. The right eye showed morning glory syndrome and proliferative vitreoretinopathy with total retinal detachment. A retinal hole was found during surgery in the temporal periphery. Silicone oil tamponade with photocoagulation was followed by resolution of retinal detachment but visual acuity became no light perception 5 months later. The third case showed visual acuity of 0.03 right and 0 left. Retinal detachment involved the temporal fundus and the macula in the right eye. A tear was present in the temporal periphery. The retina became reattached after buckling with final visual acuity of 0.03. Conclusion: The present cases show that retinal detachment in morning glory syndrome may show retinal breaks and is to be surgically treated. Even when no retinal break is present, retinal detachment show spontaneous resolution.

Purpose: To report a case of Alport syndrome with abnormal retina and anterior capsule in the lens. Case: A 27-year-old male was referred to us for reduced visual acuity in both eyes. He had received renal transplantation for renal failure due to Alport syndrome. Findings and Clinical Course: Corrected visual acuity was 0.3 right and 0.5 left. Both eyes showed anterior lenticonus in both eyes. Funduscopy showed dot-and-fleck lesions in the perimacular and peripheral retina. Optical coherence tomography showed thinning of inner layer in the temporal retina in both eyes. There was no loss of sensitivity at these sites by microperimetry. Multifocal ERG showed no amplitude attenuation in the thinned retinal area. Both eyes received cataract surgery. The removed anterior capsule showed thinning and multiple vertical dehiscences by electron microscopy. Lens epithelial cells were vacuolated and contained fewer intracellular organelles. Visual acuity improved to 1.0 in either eye. Conclusion: This case of Alport syndrome showed refractive abnormalities due to lenticonus but no obvious retinal dysfunction. The lens capsule showed structural abnormalities. Visual acuity improved after cataract surgery.

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: To report the relation between hard exudates and serum lipid levels in cases of branch retinal vein occlusion (BRVO). Cases and Method: This retrospective study was made on 89 eyes of 89 cases who received vitrectomy for BRVO. The series comprised 40 males and 49 females. The age averaged 67.1 years. Hard exudates were present or became apparent in 12 eyes and were absent throughout in 77 cases. Results: There was no difference between the two groups regarding age, duration of BRVO before surgery, visual acuity, or macular thickness. Cases with hard exudates had significantly higher serum levels of total cholesterol (p=0.005) and showed poorer recovery of cystoid macular edema 6 months after vitrectomy (p = 0.002). Conclusion: Eyes with BRVO were characterized by higher serum levels of total cholesterol and slower postoperative absorption of cystoid macular edema.

Purpose : To report the characteristics of eyes with phakic rhegmatogenous retinal detachment with single or multiple breaks. Cases and Method : This retrospective study was made on 98 eyes of 98 cases of rhegmatogenous retinal detachment. The series comprised 58 males and 40 females. All the cases were aged 50 years or over, received simultaneous cataract and vitreous surgery, and were measured for the volume of resected vitreous cavity. Results : There were 52 eyes with single retinal break and 46 eyes with multiple breaks. Out of 12 eyes with axial length of 23 mm or less, 11 eyes had single break. Out of 8 eyes with axial length of 27 mm or over, 5 eyes had multiple breaks. Out of 78 eyes with axial length between 23 mm and 27 mm, 38 eyes had single break and 40 eyes had multiple breaks. Corneal curvature radius and volume of vitreous cavity was significantly greater in eyes with multiple breaks than single break. There was no significant difference in axial length in eyes with multiple or single break between 23 mm and 27 mm. Conclusion : Axial length, vitreous volume, and cornel curvature radius are related with the number of retinal break in eyes with phakic retinal detachment.

Purpose : To report a case with severe retinal dysfunction suspected of chlorpromazine-induced retinopathy. Case : A 50-year-old female presented with blurring of vision in both eyes. She had been receiving 10 medications including chlorpromazine 50 mg daily for severe depression since 21 years before. She had received simultaneous cataract and vitreous surgery for cataract and macular edema in both eyes 2 years before. Findings : Corrected visual acuity was 0.07 right and 0.6 left. Both eyes showed paracentral scotoma and decreased sensitivity in the fovea. Fluorescein angiography showed extensive atrophy of retinal pigment epithelium. Optical coherence tomography showed thinning of retinal outer layer and absence of borderline between outer and inner segment of visual cells in the right eye and cystoid macular edema in the left. Electroretinogram was almost extinguished in the right eye and was markedly reduced in the left. Conclusion : This case illustrates that prolonged intake of chlorpromazine at a low dosis may induce severe retinal dysfunction.

Back ground: Acute zonal occult outer retinopathy (AZOOR) is characterized by acute loss of one or more zones of outer retinal function, minimal funduscopic changes, and abnormal electroretinography (ERG). Recently optical coherence tomography (OCT) showed a loss or irregularity of inner segment-outer segment (IS/OS) junction of photoreceptors corresponded to the scotomas in AZOOR patients. Case report : 46-year-old woman presented temporal visual field defect in her right eye. But she had no funduscopic abnormalities in her right eye. Goldman perimetry showed blind spot enlargement and multifocal ERG findings showed markedly decreased amplitudes corresponded to the blind spots. Her spectral domain OCT showed a loss of the IS/OS junction in the area corresponded to the scotomas. After six months, OCT showed a markedly restoration of the IS/OS junction, but she had no change in the ERG findings and Goldman perimetry. Conclusion : Our patient showed early restoration of the IS /OS junction defect, which was not associated with functional recovery. Further observation is needed.