島田 佳明

OBJECTIVE: To report a case of bilateral reversible optic neuropathy as the first sign of Waldenström macroglobulinemia (WM). METHODS: Observational case report. RESULTS: A 52-year-old man had a sudden loss of vision in the left eye. Examinations revealed the presence of a serum monoclonal immunoglobulin (IgM kappa) in the serum. Even after a session of steroid pulse therapy, optic neuropathy became bilateral and then resolved almost completely after 4 months. The condition progressed to WM with multiorgan lesions years later. There was no evidence of optic neuropathy recurrence. The literature revealed two cases of monoclonal gammopathy (MG): a 64-year-old man with multiple myeloma (MM) with IgA lambda and a 51-year-old man with MM with IgG kappa. These cases have similar conditions: 1) visual reduction as an initial symptom of MG, 2) bilateral involvement, 3) no sign of central nervous system (CNS) infiltration shown by normal brain magnetic resonance images, and 4) recovery to a visual acuity of ≥1.0 bilaterally with no reoccurrence. The excessive Igs or B-cell hyperactivity may activate an autoimmune mechanism that reversibly interferes with the bilateral optic nerves. CONCLUSION: Bilateral optic neuropathy was the initial symptom of WM. There was no evidence of CNS infiltration; it recovered and then did not reoccur. The pathogenesis remained unknown, but two cases of MG were reported in the literature with remarkably similar conditions.

INTRODUCTION: We report a case of cerebral venous sinus thrombosis (CVST) that presented with bilateral optic disc swelling and diplopia in the absence of headaches. CASE PRESENTATION: A 54-year-old woman with no relevant medical history presented with a 2-week history of diplopia and no loss of visual acuity in each eye. Eye movements revealed bilateral abduction deficits, and fundoscopic examination revealed bilateral optic disc swelling. Non-contrast computed tomography of the head showed no abnormalities. Magnetic resonance venography revealed the absence of flow in the superior sagittal and left transverse sinuses as a consequence of thrombosis. The patient was diagnosed with intracranial hypertension associated with abducens nerve palsies secondary to CVST and was initiated on anticoagulant therapy. CVST can lead to stroke even in younger individuals. CONCLUSION: CVST should be considered in differential diagnosis when bilateral papilledema and abducens nerve palsies are present, even in the absence of headache or other neurological findings.

OBJECTIVES: To compare the eye axial length (AL), equatorial horizontal diameter (HD), and equatorial vertical diameter (VD) of normal eyes using a novel wide-angle, arc-scanning, ultrasound diagnostic device for wide-angle B-mode echography. METHODS: In this cross-sectional study, wide-angle B-mode echography and magnetic resonance imaging (MRI) were conducted on 22 normal eyes; the AL, HD, and VD were measured. RESULTS: The mean ALs were as follows: wide-angle B-mode echography, 25.22±1.47 mm and MRI, 25.24±1.46 mm; a significant correlation was observed between the two measurements (β=0.995 [0.976, 1.013]; p<0.001; 95% R2=1.00). The mean HDs were as follows: wide-angle B-mode echography, 22.33±0.84 mm and MRI, 22.55±0.90 mm; a significant correlation was observed between the two measurements (β=0.902 [0.750, 1.179]; p<0.001; 95% R2=0.81). The mean VDs were as follows: wide-angle B-mode echography, 22.77±0.91 mm; and MRI, 22.88±0.92 mm; a significant correlation was observed between the two measurements (β=0.966 [0.853, 1.097]; p<0.001; 95% R2=0.93). CONCLUSIONS: There were no significant differences in the measurements for each parameter by wide-angle B-mode echography and MRI. Therefore, wide-angle B-mode echography permits accurate visualization of ocular morphology.

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.

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.

＜文献概要＞肥厚性硬膜炎(hypertrophic pachymeningitis:HP)は脳脊髄硬膜が種々の原因や基礎疾患により肥厚し,頭蓋や脊椎を貫通している脳,脊髄神経を圧迫したり循環障害をもたらしたりすることで疼痛や機能障害をきたす病態である。全身疾患の部分症候であることがあるので,独立した疾患としては扱いにくい。視力・眼球運動障害により患者のQOLが著しく損われることがあり,下位脳神経障害により誤嚥性肺炎をきたすことや原疾患の増悪で死亡することもある。硬膜のみでなく,くも膜や軟膜を含んで脳脊髄の実質に炎症が及ぶこともある。本邦において全国調査が施行され,近年は症例数を集めた報告が増加した。従来のANCA関連血管炎(anti-neutrophil cytoplasmic antibody related vasculopathy:AAV)の典型である多発血管炎性肉芽腫症(granulomatosis with polyangiitis:GPA)やその関連疾患である顕微鏡的多発血管炎(microscopic polyangiitis:MPA),好酸球性多発血管炎性肉芽腫症(eosinophilic granulomatosis with polyangiitis:EGPA)に伴うもの以外に,多巣性線維硬化症(multifocal fibrosclerosis:MFS)やIgG4関連疾患(IgG4-related disease:IgG4-RD)の提唱により,全身臓器疾患に伴って生じる肥厚性硬膜炎として新たな研究の進展がみられている。原因が感染症の場合にはそれに対する診断と治療が必須であるが,免疫性ではグルココルチコイド(glucocorticoid:GC)を含む種々の免疫抑制薬が試みられるものの難治症例の存在とGC漸減中の再発など課題が多く,発症機序の解明と新しい治療法が模索されている。

＜文献概要＞多局所ERG(multifocal electroretiongram:multifocal ERG,mfERG,多局所網膜電図)は,Sutterが1989年に米国特許を取得した網膜部位反応マップ(retinal area response mapping)を,ERGの一種と捉えて用いるようになった名称である。後極部網膜の多数の網膜局所のERG(局所ERG)が同時に記録され,ERGのbreakthroughになった。臨床的に重要な黄斑部のERGが得られることに加え,病変のある網膜領域とない領域のERGの比較,ERGの反応分布の表示など,幅広い応用を可能にした(図1)。最も普及した国/地域といわれた本邦では,2010年に診療報酬点数表に収載され,成熟した臨床検査になっている。しかし成功から四半世紀を経て,同時期に現れた光干渉断層計(OCT)が,改良により有用性や対象疾患を増大しているのに対して,多局所ERGは老朽化した記録装置を更新しても,記録の手間や得られる情報がほぼ変わっておらず,適応の拡大も限定的である。また,散瞳,表面麻酔,コンタクトレンズ電極装用,屈折矯正,瞬目抑制等々が求められる多局所ERGは,検者・被検者の負担が大きい,煩瑣な検査とみる傾向が強まった。記録の効率化,負担軽減は多局所ERGの課題である。

＜文献概要＞目的:総胆管拡張と総胆管結石を有する透析患者にみられたビタミンA欠乏症の1例を経験したので報告する。症例:65歳,女性。主訴は数ヵ月前からの夜盲。2年前から消化器内科で総胆管拡張および総胆管結石を経過観察され,また近医にて血液透析中であった。視力は両眼とも(1.0),眼底には小白斑を認めた。ゴールドマン視野検査では異常なく,網膜電図(ERG)ではa波およびb波の著明な振幅低下を認めた。光干渉断層計(OCT)ではellipsoid zone(EZ)とinterdigitation zone(IZ)の不整を認めた。血清亜鉛は54μg/dlと低下し,血清ビタミンAは19IU/dlと著明に低下していた。総胆管拡張と総胆管結石患者に伴ったビタミンA欠乏症と診断し,ビタミンAの経口投与を開始した。血清ビタミンAは531IU/dlに上昇,夜盲の自覚も消失し,ERGも改善した。血液透析中であり,ビタミンAの蓄積を考慮し,漸減投与としたが最終視力は左右ともに(1.0)に保たれ,夜盲の再発はない。結論:血液透析に伴う低亜鉛血症に加え,総胆管拡張と総胆管結石による胆汁酸の異常をきたし,ビタミンAの吸収障害により夜盲をきたしたと考えられた。透析患者ではビタミンAの投与にも慎重を要する。

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.

Background: The acute physiological changes induced by focal retinal photocoagulation (PC) have been largely unexplored. Methods: This was a case-series study. We recorded multifocal electroretinograms (mfERGs) just before PC, and mfERGs were also recorded 5′, 15′, one hour, 24 h, and one week after the PCs. Transient changes of mfERGs were analyzed in eyes which underwent PCs to treat diabetic macular edema. The mfERGs recorded from the predominantly irradiated area and that from non-irradiated areas were analyzed separately. Results: Fifteen eyes of 15 patients were included in this study. The mfERGs elicited from non-irradiated areas did not change after PC, but the mfERGs elicited from the irradiated area changed with time; the amplitude was larger at 60′ than that before (p &lt; 0.05) and at 5′ after PC (p &lt; 0.01) and significantly smaller at 24 h and 1 week than that before and at 60′ after the PC (p &lt; 0.01). The implicit time was significantly prolonged after PC. mfERG on irradiated area with the severe diabetic change was less altered after PCs. Conclusions: The transient increase in the amplitude at 60′ likely resulted from a biological amplification of partially damaged cells adjacent to the PC spots. The mfERGs manifested the dynamic alterations of the retinal function following PCs.

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.

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.

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)

Objective: To report a case of unintentional retina artery amputation during macular peeling. Methods: Observational case report Results: A 73-year-old female underwent surgery to treat epiretinal membrane (ERM) associated with severe staphyloma in her left eye. While the ERM/posterior hyaloid membrane visually enhanced with triamcinolone (TA) was peeled, a movement of the forceps unintentionally involved the inferior temporal branch artery near the inner edge of the staphyloma. The artery was avulsed and amputated. Oozing from the retinal cleft that had once enfolded the artery and microscopic bleeding from the amputation stump were observed. The vitreous hemorrhage obscured a view of the fundus more than 4 weeks after the surgery. After 8 weeks, postoperative visual acuity was improved; however, the superior nasal visual field was lost, and the patient was aware of the broken vessel as a floater in her vision. Conclusions: Macular peeling is technically challenging, so meticulous attention must be paid to avoid any damage on vessels. The retina tissue was stretched in a staphyloma and vessels were bulged into the vitreous space especially at the inner edge of the staphyloma. High levels of TA dye here buried the texture of the retina. Excessive TA should be removed prior to macular peeling.

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.

肥厚性硬膜炎は脳脊髄の硬膜に炎症性肥厚を生じ、硬膜を貫通する脳神経や脊髄神経根を圧迫することで痛み、麻痺、失明や難聴、また誤嚥性肺炎などをもたらしうる疾患である。感染や腫瘍など明らかな原因があるものと、ANCA関連疾患やIgG4関連疾患といった免疫機序が関わる場合があり、後者では他臓器の肉芽腫や線維化が生じ、発病の発端が眼科や耳鼻科領域ないしは腎臓などの全身臓器のことがある。原因が特定できないものも多く存在する。診断に造影MRIは必須で、続発性の鑑別のためには治療前に各種自己抗体、抗ANCA抗体、IgG4、β-D-グルカンの測定や細菌学的な検索、副鼻腔や硬膜の可能な限りの生検が必要である。真菌感染では糖尿病を合併することが多く、治療は抗真菌薬を先行させステロイドも使用する。非感染性のものは上記膠原病近縁ないしは免疫介在性と考えられ、種々の免疫修飾療法が試みられ、ステロイド、IVIg、タクロリムス、リツキシマブ、サイクロフォスファマイドなどの使用が報告されている。ステロイド漸減中に再発がみられることが多く、病態別の治療指針の確立が待たれる。(著者抄録)

眼科臨床を理解するうえで視覚生理は非常に重要である。網膜がどのように光を捉えどのように視覚に変換するか、また視覚中枢がその情報をどのように分析するか、著者らは長年にわたって研究を行ってきた。また臨床では、これらの知識に基づき手術や診断に関して生理学的なアプローチも行ってきた。現在までの研究成果について紹介する。I.基礎電気生理 1.網膜細胞内電位記録 網膜の細胞内に微小電極を挿入し、1つの細胞から電位を記録する方法は網膜生理学を大きく進歩させた。著者らはアフリカツメガエルの網膜を使用して水平細胞電位を記録し、暗所で杆体が水平細胞を介して錐体を抑制することを証明した。この研究で網膜電気生理にsilent substitutionという方法を導入し、その後の電気生理の研究に大きく貢献した。2.網膜神経細胞に対するパッチクランプ 網膜の細胞の中ではなく細胞膜に微小電極を吸着し、膜にあるチャネルを記録する方法である。著者らは網膜剥離などの硝子体手術中に得られる網膜の破片を用いてパッチクランプを行いヒトの杆体細胞や双極細胞にNa+チャネルがあり、強い光刺激がoffとなるときに杆体がスパイクを発生することを発見した。II.臨床電気生理 これまでに多くの網膜電図の研究を行ってきた。最近開発されたinverted internal limiting membrane(ILM)flap techniqueの網膜への影響、硝子体手術の網膜電図への影響などについて検討した。III.臨床心理物理 1.黄斑円孔の視力測定 黄斑円孔では中心窩の網膜が裂けて円孔となるため固視点が安定しない。著者らはLandolt環を多数提示して視力測定を行う方法を考案したところ、円孔周辺における視力が測定できた。2.黄斑円孔の視細胞移動 Watzke-Allen testは円孔による視細胞の移動により起きる歪みをスリット光で観察する検査である。スリット光を段階的に拡大して閾値を求め視細胞の移動範囲を測定したところ、80%以上の円孔では移動は縦と横で異なり、光干渉断層計(OCT)の結果とあわせて多くの黄斑円孔が正円ではないことを示した。3.黄斑上膜の変視症 黄斑上膜の変視症、大視症に関してはAmslerチャートやM-CHARTS、New Aniseikonia testを用いた研究があるが、著者らは、多数の患者を観察したところ、一眼にしか黄斑上膜がないにもかかわらず両眼に変視症がある症例を複数発見した。変視症には中枢が関与している可能性が高いことが分かった。IV.眼科臨床(手術と診断) 1.組織染色による内眼手術 内眼手術では透明な組織を視認する必要があり、顕微鏡や照明を改善するのみでは困難な場合がある。著者は成熟白内障の前嚢切開にてインドシアニングリーンを用いて前嚢を染色し、視認性を向上させる方法を考案した。組織を染色する方法が眼科領域で報告されたのは初めてであり、その後多くの内眼手術に応用された。2.Optical fiber-free intravitreal surgery system(OFFISS)とwide-angle view system 顕微鏡の照明で硝子体手術を行う方法を考案し、そのシステムを用いたwide-angle view systemを開発した。現在用いられているwide-angle view systemの多くはこれをもとにしている。3.広角超音波検査 通常の眼科用B-modeではmagnetic resonance imaging(MRI)のように眼球全体の画像が得られない。眼球全体の画像が得られる装置を開発した。眼科患者の眼球断面解析にMRIを用いることは難しく、広角超音波検査を用いることにより眼球の形状と眼科疾患の関係を研究できる。(著者抄録)

BACKGROUND: This study aimed to investigate patient adherence to face-down positioning (FDP) and non-supine positioning (NSP) following vitrectomy with gas tamponade for treating macular holes (MHs). METHODS: Nursing records of 92 patients who underwent vitrectomy with gas tamponade for small-diameter (diameter < 400 μm) MHs during April 2016-June 2017 were examined. Forty-seven and 45 patients were instructed to maintain FDP and NSP (FDP and NSP groups), respectively. Patient adherence was evaluated seven times a day for 3 days, and the adherence rate was calculated. RESULTS: The mean adherence rate was significantly higher in the NSP group (99.3% ± 2.7%) than in the FDP group (93.7% ± 13.3%; P < 0.001, Mann-Whitney U test). Forty-one patients (91.1%) in the NSP group had an adherence rate of 100%, which was significantly higher than that in the 24 patients in the FDP group (51.1%; P < 0.001, chi-squared test). No statistically significant difference was observed between the patients in the two groups regarding sex, age, MH diameter, and pre- and postoperative visual acuities. MH closure was achieved in all patients. CONCLUSIONS: Almost half of the patients in the FDP group did not obtain 100% adherence rate, suggesting that patient adherence was largely compromised. Patient adherence was better in the NSP group as patient compliance to NSP was better, however, 8.9% of patients were found in face-up positioning at least once. Incompleteness of patient adherence was common, although to differing degrees.

PURPOSE: The clinical course of reduced visual acuity, metamorphopsia, and aniseikonia are dissimilar in patients with an epiretinal membrane (ERM). We measured and compared the best-corrected visual acuity (BCVA), metamorphopsia, and aniseikonia of patients with ERM. METHODS: We measured metamorphopsia and aniseikonia horizontally as well as vertically with the M-CHART (MH and MV) and New Aniseikonia Test (NATH and NATV) in 61 patients with unilateral idiopathic ERM. We compared the BCVA with the other values. Comparisons between the MH and the NATV and between the MV and the NATH were performed, because the MH assesses vertical metamorphopsia, and the MV, horizontal metamorphopsia. RESULTS: BCVA was not significantly correlated with the other values (MH vs BCVA: P = .69; MV vs BCVA: P = .114; NATH vs BCVA: P = .656; NATV vs BCVA: P = .935). The MH and NATV magnitudes were significantly correlated, but the correlation coefficient was small (P = .007, r = 0.343); no significant correlation was found between the MV and NATH magnitudes (P = .065). We found patients with aniseikonia, but no metamorphopsia (n = 6), and more patients with metamorphopsia, but no aniseikonia (n = 11). Only 23 patients had coincident directions of metamorphopsia and aniseikonia. CONCLUSIONS: The magnitudes of metamorphopsia and aniseikonia were not closely correlated and their directions did not coincide in most patients. Neither the M-CHARTS nor the NAT measurements correlated with the BCVA. Quantitative testing of metamorphopsia and aniseikonia in addition to that for BCVA is necessary to assess visual function in patients with ERM.

The use of a conjunctival retractor that expands a small incision in the conjunctiva/Tenon's capsule facilitated the performance of strabismus surgery through a fornix incision. A wide field of exposure revealed the entire area of the muscle insertion. While there is a risk of conjunctival tearing in elderly patients, the use of the retractor is valuable.

OBJECTIVE: To determine the adherence to the face-down positioning (FDP) in 296 patients who had undergone vitrectomy and gas tamponade. RESULTS: We studied 119 female and 177 male patients who had undergone primary vitrectomy and gas tamponade for a macular hole (MH) or for rhegmatogenous retinal detachments (RRDs). Adherence was assessed and recorded four times per day for 3 days postsurgery. The mean ± standard deviation adherence rate was 88.3 ± 11.7 (range 50.0-100.0). Female patients (90.8 ± 10.0) had significantly better adherence than male patients (86.7 ± 13.3; P < 0.02, U test). The adherence was significantly better after MH surgery (90.8 ± 11.7) than after RRD surgery (87.5 ± 12.5; P < 0.02). There were diurnal variations in adherence, being highest in the evening and significantly lower at midnight, and we evidenced a decline in adherence over time, with it being significantly low on the last follow-up day. Adherence to the FDP varied considerably among patients. Adherence was higher in female than in male patients, and higher in patients with MH than in those with RRD. We found patients age had no effect on adherence. Adherence also varied with time, being worst at midnight and declining over time.

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.

PURPOSE: To study the effect of an inverted internal limiting membrane (ILM) flap on the retina. DESIGN: Prospective case series. PARTICIPANTS: Twenty-nine patients with large (>400 μm) unilateral macular holes underwent surgery using a modified inverted ILM flap technique. METHODS: The macular ILM was peeled, and a large (2-3 disc diameter) ILM flap was made on the superior side of the hole, and then the flap was inverted on the inferior side. MAIN OUTCOME MEASURES: In all patients, multifocal electroretinograms (mfERGs) were recorded from operated eyes and normal fellow eyes. The peak time and amplitude of N1, P1, and N2 in the focal ERG from the upper retina without the ILM flap and those from the lower retina with the ILM flap were evaluated. In 14 patients, microperimetry was also performed in both eyes, and the averaged sensitivity was measured from the upper and lower areas. RESULTS: The peak times of P1 and N2 from the upper and lower retina were significantly longer in operated eyes than in the fellow eyes (P1 upper and lower: P < 0.04, N2 upper: P < 0.01, and N2 lower: P < 0.04), although we could not identify a significant difference in peak time and amplitude of N1, P1, and N2 between the upper retina and lower retina in both eyes (fellow eye-N1 amplitude: P > 0.2, N1 peak time: P > 0.5, P1 amplitude: P > 0.9, P1 peak time: P > 0.4, N2 amplitude: P > 0.9, N2 peak time: P > 0.9; operated eye-N1 amplitude: P > 0.8, N1 peak time: P > 0.4, P1 amplitude: P > 0.6, P1 peak time: P > 0.4, N2 amplitude: P > 0.9, N2 peak time: P > 0.9). We could not observe a significant difference in sensitivity between the upper and lower retinas in both eyes (fellow eye: P = 0.28, operated eye: P = 0.66). CONCLUSIONS: The results of this study revealed no significant difference between the upper retina without the ILM flap and the lower retina with the ILM flap, suggesting that an inverted ILM flap has little effect on retinal function.

To investigate the characteristics of the late foveal response component (lfrc) that presents on the first slice of the second-order kernel (K2.1) in multifocal electroretinograms (mfERGs).mfERGs with 37 hexagonal stimulus elements were obtained from 27 healthy subjects under a stimulus intensity of 2.67 cds/mThe foveal mfERG of the first-order kernel (K1) was shaped similarly to the K1 of the surrounding mfERGs. The foveal mfERG of K2.1 differed from the K2.1s of the surrounding mfERGs. This difference varied among subjects; however, the potential (0.34 ± 0.10 µV: mean ± SD) of the lfrc acutely changed at approximately 50 ms (range 48.56 ± 1.02-56.86 ± 1.99 ms). Whereas the amplitudes of the other major components of K1 and K2.1 significantly decreased with increasing refractive error, the amplitude of lfrc was not significantly correlated with refraction in this cohort.The lfrc was obtained only on the centermost hexagon within an appropriate recording time (2 min). This finding reflects the particular structure and peculiar adaptiveness of the fovea, a specialized area of the human retina, and enables the estimation of foveal function in clinical practice.

Leber hereditary optic neuropathy (LHON) causes visual loss, predominantly in healthy young men. We recently examined a patient who previously had bilateral macular holes and subsequently developed LHON at 74 years of age. Although his central scotomas were initially attributed to the macular holes, his visual acuity declined following an initial improvement after operative closure of the macular holes; thus, other diagnoses, including LHON, were considered. Furthermore, macular optical coherence tomography (OCT) images remained unchanged in this time. A mitochondrial genetic analysis identified a 11778G→A mutation. From this case, we propose that LHON remains in the differential diagnosis even in older patients, as has previously been reported.

目的:2種類の皮膚電極によるERG記録装置:PuREC(メイヨー)とRETeval complete(以下;レチバル)(LKC Technologies)を比較すること。対象:対象は正常者11名。散瞳下に暗順応後PuRECでISCEV standard ERGを記録した。その皮膚電極を装着したままレチバルに接続し直して再度ERGを記録した。結果:各成分の平均振幅(μV)はPuREC、レチバルそれぞれDA 0.01 ERG;63.6と59.0(p=0.256)、DA 3.0 ERG a波;63.8と57.9(p=0.239)、b波;99.6と95.5(p=0.966)、LA 3.0 ERG a波;9.1と9.6(p=0.457)、b波;40.4と40.7(p=0.905)、flicker;33.7と31.4(p=0.398)であった。平均潜時(ms)はDA 0.01 ERG;83.1と92.3(p=0.006)、DA 3.0 ERG a波;18.2と14.5(p=0.002)、b波;45.3と48.2(p=0.107)、LA 3.0 ERG a波;16.8と13.3(p<0.001)、b波;30.2と27.1(p<0.001)、flicker;27.6と24.4(検定せず)であった。結論:ERGの各成分の振幅はほぼ同じであった。しかしPuRECに比べレチバルの潜時はDA 0.01 ERGでは有意に遅く、DA 3.0 ERGのa波、LA 3.0 ERGのa波、b波では有意に早かった。これらの差は、両装置のノイズ除去システムの違いに起因する可能性がある。(著者抄録)

<p>Objectives: To compare the time of operation and the postoperative intraocular pressure (IOP) using a 27- versus 25-gauge three-port vitrectomy in eyes with epiretinal membrane (ERM).</p><p>Methods: The clinical records of eyes with ERM that underwent combined cataract surgery and vitrectomy from January to April 2016 were analyzed. Thirteen eyes were treated using a 27-gauge system (27-g group) and 12 eyes were treated using a 25-gauge system (25-g group). The operating times were determined from video recordings.</p><p>Results: The time of vitreous removal was significantly longer in the 27-g group (141.1 ± 34.1 s) than in the 25-g group (106.2 ± 24.1 s; P = 0.009). The IOP was significantly lower in the 25-g group than in the 27-g group on postoperative day 1 (27-g group, 18.3 ± 6.2 mmHg; 25-g group, 12.6 ± 3.6 mmHg; P = 0.008), but there was no significant difference on postoperative day 7. The times needed for removing the trocar and closing the port were not significantly different, but the number of the ports that required pressure to close was significantly greater in the 25-g group (35/36 ports) than in the 27-g group (31/39 ports; P = 0.0027), and the duration of pressure was significantly longer in the 25-g group (21.7 ± 13.8 s) than in the 27-g group (11.3 ± 5.2 s; P = 0.0183).</p><p>Conclusions: The 27-g system is better regarding closure of the scleral port, which may reduce postoperative complications, but the prolonged surgical time may be a disadvantage.</p>

We report a case of a 38-year-old man who presented with a recently self-detected lump under his left eyebrow. Previous ophthalmological history was unremarkable except for unilateral high myopia (left eye) since childhood. The appearance of the left eye was seemingly normal; however, with the top lid pulled up on downward gaze, a dark brown bulge emerged. The bulge was 10 × 7 mm and approximately 4 mm in height, and was covered by the extended superior rectus muscle. The diagnosis of equatorial staphyloma was made after coronal T1-weighted magnetic resonance imaging of the orbit revealed the dilatation of the vitreous cavity. Ocular movements were fully maintained and visual acuity was largely spared: 20/15 in the right eye without correction and 20/25 in the left eye with -10.00 spheres and -4.00 × 80 degrees cylinders. His past and family histories were unremarkable; however, small neurofibromas and café au lait spots all over his body led to the diagnosis of neurofibromatosis type 1 (NF1). From this case, similar to previous reports, we suggest that manifestations of NF1 are extremely variable and unpredictable.

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 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 , 0.05). Patients who scored a perfect 12 were 38 (29.9% female, 14:31.1% male 24:29.3%). Low scorers ,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.

BACKGROUND AND OBJECTIVE: The authors developed a new technique for retrobulbar anesthesia and compared it with the conventional transcuta-neous method in 223 eyes undergoing vitrectomy. PATIENTS AND METHODS: In the new technique, a 24-mm-long 19-gauge curved cannula with a 27-gauge straight needle with an oval tip was inserted into the sub-Tenon's space, and then the oval-tipped needle was replaced with a 50-mm-long 25-gauge curved needle. Anesthetic was then injected into the muscle cone from the syringe. The authors compared 118 eyes undergoing vitrectomy with the new technique and 105 eyes undergoing the procedure with conventional anesthesia. RESULTS: The pain scores were 0.1 ± 0.32 for the new technique and 1.57 ± 0.73 for the conventional technique (P &lt .001). Additional anesthetic required was 0.1 ± 0.33 mL using the new technique and 0.3 ± 0.68 mL using the conventional method (P = .03). CONCLUSION: This novel approach to retrobulbar anesthesia using a guarded cannula is safe and effective, causing less pain than the conventional technique.

患者は66歳の男性であり、起床時に一過性に開瞼困難と持続する複視を自覚した。頭部MRIでは下丘の高さの左尾側傍正中部中脳に急性期微小梗塞が認められた。Hess chartでは右上斜筋麻痺がもっとも示唆され、Parks 3stepテスト陽性であり右滑車神経麻痺と診断した。約3ヵ月で複視は改善した。一過性の開瞼困難は動眼神経最尾側の障害で説明されると考えた。滑車神経の髄内障害の報告では原因はほとんどが出血や血管奇形であり、梗塞によるものはきわめて稀であるが、脳虚血性の滑車神経麻痺は過小診断されている可能性がある。患者は左側にHorner徴候を有したが偶然の合併であり以前からあった。(著者抄録)

目的:ベースレート75Hzの多局所網膜電図(mfERG)の非線形成分K2.1において、中心の反応に現れる小波(中心窩小波、P50)の健常者における性質を調査する。対象および方法:健常者42名の刺激エレメント数37または509個のmfERGを分析した。結果:K2.1の50ms付近の陽性波P50が、37個刺激エレメントのmfERG全ての中心にのみ観察された。509個刺激エレメントでは、P50は中心視角半径約3度以内に分布し、その範囲内では潜時はほぼ一定に保たれていた。結論:中心窩の非線形成分群の波形は全視野刺激の場合と全く異なっており、中心窩網膜の特異性の表出と考えられた。中でもP50は健常者にほぼ例外なく観察されるので、疾患における中心窩視機能の評価に有望である。P50を活用するために、P50が記録されやすい、mfERGの刺激条件やグループ設定が工夫されてもよいだろう。(著者抄録)

多局所視覚誘発電位(multifocal visual evoked potential、mfVEP、多局所VEP)は、理論物理学者Sutterが発明した多局所入力技術を用い、視野上の多数の局所領域の視覚刺激により誘発される視覚誘発電位(VEP)を記録する技術、または記録されたVEP(局所VEP)のことである。数分間で数十個の局所VEPが一挙に記録されるものの、局所毎に反応の大きさが異なる性質と、著しい個人差のため、臨床には使えないと考えられていた。しかし多局所VEPがヒトの視覚の解剖と生理の表出であることが次第に認識され、多くの新知見をもたらした。多局所VEPを実用的な他覚的視野計に応用すると共に、従来のパターン反転VEPを置換しうる臨床検査に発達させた、研究と発見を紹介する。(著者抄録)

Purpose: The aim was to evaluate the value of multifocal electroretinogram (mfERG) and optical coherence tomography (OCT) in predicting post-operative visual acuity (post-VA) after removal of an idiopathic epiretinal membrane (ERM). Methods: Fifty-one eyes of 51 patients with an idiopathic macular ERM were examined by OCT and mfERG before three-port pars plana vitrectomy with removal of the ERM. The parameters of the OCT images and mfERGs, pre-operative visual acuity (pre-VA) and the duration of symptoms (DS) were compared with the post-VA. Results: The pre-VA (r = 0.570, p &lt; 0.001) and the duration of the symptoms (r = 0.389, p &lt; 0.035; n = 35) were significantly correlated with the post-VA. The central retinal thickness (CRT) was significantly correlated with the pre-VA (r = 0.379, p &lt; 0.01) and the post-VA (r = 0.318, p &lt; 0.03). The amplitudes of macular electroretinogram (amERGs) were not significantly correlated with the pre-VA or post-VA. The CRT to amERG ratio (CRT/amERG) was correlated with the post-VA (r = 0.429, p &lt; 0.002) but not with the pre-VA (p &gt; 0.1). Comparative analyses among groups of cases based on CRT/amERG showed significant differences in the post-VA but not in the pre-VA and the duration of symptoms. Conclusion: As with the large CRT/amERG, the pre-operative morphologically thickened and electroretinographically reduced retinas have a greater likelihood of being affected by the irreversible photoreceptor damages that could have been masked or modified by other factors before the removal of the ERM. Because the CRT/amERG values are obtained objectively, they should be valuable in predicting the post-operative visual function along with the pre-VA and the duration of symptoms.

目的:有水晶体眼での単一裂孔と多発裂孔による網膜剥離眼の特性の報告。対象と方法:裂孔原性網膜剥離98例98眼を対象とした。男性58例,女性40例であり,50歳以上で,白内障と硝子体同時手術を行い,硝子体切除腔容積が測定できた症例とした。結果:単一裂孔は52眼,複数の裂孔は46眼にあった。眼軸長が23mm以下の12眼では11眼が単一裂孔であり,27mm以上の8眼では5眼が多発裂孔,23～27mmの中間群では78眼中38眼が単一裂孔で40眼が多発裂孔であった。角膜曲率半径と硝子体切除腔容積は,単一裂孔群よりも多発裂孔群で有意に大きかった。中間群では単一裂孔群と多発裂孔群とでは,眼軸長に有意差がなかった。結論:有水晶体の網膜剥離眼では,眼軸長と眼球の大きさや形状が裂孔の数に関係する。(著者抄録)